Hepatosplenic T cell lymphoma: Difference between revisions

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|Leukopenia
|Leukopenia
|Constant fatigue
|Constant fatigue
|}
Differentiating different types of T-cell Non-Hodgkin lymphoma. The gold standard for differentiation different types of Non-Hodgkin lymphoma is biopsy.
! colspan="12" align="center" style="background:#7d7d7d; color: #FFFFFF;" + |Non-Hodgkin's Lymphoma (T Cell Lymphoma)
|-
! colspan="2" align="center" style="background:#DCDCDC;" + |Precursor T-cell lymphoblastic leukemia/lymphoma<br><ref name="pmid28892922">{{cite journal| author=Shelly D, Gujral S| title=Early T-Cell Precursor Acute Lymphoblastic Leukaemia/Lymphoma: Immunohistochemical Evaluation of Four Lymph Node Biopsies. | journal=J Clin Diagn Res | year= 2017 | volume= 11 | issue= 7 | pages= EL01-EL02 | pmid=28892922 | doi=10.7860/JCDR/2017/29352.10164 | pmc=5583929 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28892922  }}</ref><ref name="pmid26276771">{{cite journal| author=You MJ, Medeiros LJ, Hsi ED| title=T-lymphoblastic leukemia/lymphoma. | journal=Am J Clin Pathol | year= 2015 | volume= 144 | issue= 3 | pages= 411-22 | pmid=26276771 | doi=10.1309/AJCPMF03LVSBLHPJ | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26276771  }}</ref>
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* Precursor T-cell lymphomas
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* [[Fever]]
* [[Fatigue]]
* [[Weight loss]]
* [[Night sweats]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
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* In most patients the [[mediastinal mass]] is anterior; associated with [[Pleural effusion|pleural effusions]]
* Can produce such complications as [[superior vena cava syndrome]]
* Skin and bone abnormalities
* [[Hepatosplenomegaly|Enlarged liver and/or spleen]]
*[[Tracheal compression|Tracheal obstruction]]
*[[Pericardial effusion|Pericardial effusions]]
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* [[Skin rash]]
* [[Chest pain]]
| align="left" style="background:#F5F5F5;" + |
* [[CD3 (immunology)|CD3]]
* [[CD7]]
* TDT
| align="left" style="background:#F5F5F5;" + |
* Blast-like cells with scant [[cytoplasm]], convoluted [[Cell nucleus|nuclei]], fine [[chromatin]], indistinct [[Nucleolus|nucleoli]].
* Some [[Lymphoblast|lymphoblasts]] have cytoplasmic [[Pseudopod|pseudopods]]
| align="left" style="background:#F5F5F5;" + |
* Males in their teens to early twenties
* [[Lymphadenopathy CT scan|Lymphadenopathy]] in cervical, [[Supraclavicular lymph nodes|supraclavicular]],  [[Axillary lymph nodes|axillary]] ,and [[mediastinum]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" + |T-cell granular lymphocytic<br><ref name="Rose2004">{{cite journal|last1=Rose|first1=M. G.|title=T-Cell Large Granular Lymphocyte Leukemia and Related Disorders|journal=The Oncologist|volume=9|issue=3|year=2004|pages=247–258|issn=1083-7159|doi=10.1634/theoncologist.9-3-247}}</ref><ref name="pmid22800517">{{cite journal| author=Liu EB, Chen HS, Zhang PH, Li ZQ, Sun Q, Yang QY et al.| title=[Hematopathologic features of T-cell large granular lymphocytic leukemia]. | journal=Zhonghua Bing Li Xue Za Zhi | year= 2012 | volume= 41 | issue= 4 | pages= 229-33 | pmid=22800517 | doi=10.3760/cma.j.issn.0529-5807.2012.04.004 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22800517  }}</ref><ref name="pmid15958859">{{cite journal| author=Osuji N, Matutes E, Catovsky D, Lampert I, Wotherspoon A| title=Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review. | journal=Am J Surg Pathol | year= 2005 | volume= 29 | issue= 7 | pages= 935-41 | pmid=15958859 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15958859  }}</ref>
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* Clonal proliferation of [[CD8|CD8 T cells]]
* Constant immune stimulation
* Absence of homeostatic [[apoptosis]]
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* [[Fatigue]]
* [[Fever]]
* [[Sleep hyperhidrosis|Night sweats]]
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
* [[Lymphadenopathy]]
* [[Hepatosplenomegaly]]
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* Frequent [[Infection|infections]]
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* [[CD8]]
* [[CD57]]
* [[T cell receptor|TCRαβ]]
* [[CD3 (immunology)|CD3]]
* [[CD16]]
* [[Granzyme|Granzyme b]]
* [[TIA1]]
| align="left" style="background:#F5F5F5;" + |
* Medium / large [[Lymphocyte|lymphocytes]] with abundant cytoplasm having azurophilic [[Granule (cell biology)|granules]]
| align="left" style="background:#F5F5F5;" + |May be associated with the following conditions:
* [[Monoclonal gammopathy of undetermined significance|Monoclonal gammopathy of unknown significance]] (MGUS)
* [[Chronic lymphocytic leukemia|B-chronic lymphocytic leukemia]] (B-CLL)
* [[Hairy cell leukemia]]
* [[Myeloid|Myeloid hypoplasia]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" + |T-cell prolymphocytic leukemia<br><ref name="pmid23382603">{{cite journal| author=Graham RL, Cooper B, Krause JR| title=T-cell prolymphocytic leukemia. | journal=Proc (Bayl Univ Med Cent) | year= 2013 | volume= 26 | issue= 1 | pages= 19-21 | pmid=23382603 | doi= | pmc=3523759 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23382603  }}</ref>
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* Abnormal proliferation of mature [[T cell|T cells]] (post thymic)
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* [[Fever]]
* [[Fatigue]]
* [[Weight loss]]
* [[Night sweats]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
* [[Lymphadenopathy]]
* [[Splenomegaly]]
* [[Hepatomegaly]]
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* [[Skin rash]]
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* [[CD2]]
* [[CD3 (immunology)|CD3]]
* [[CD4]]
* [[CD5]]
* [[CD7]]
* CD56
* [[TCL1A|TCL1]]
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*[[Lymphocytes]] with abundant [[basophilic]], nongranular cytoplasm, atypical nucleus
*Cytoplasmic protrusions (blebs)
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*[[Pleural effusion]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" + |[[Adult T-cell leukemia|Adult T cell leukemia/lymphoma]]<br><ref name="pmid18042693">{{cite journal |vauthors=Matutes E |title=Adult T-cell leukaemia/lymphoma |journal=J. Clin. Pathol. |volume=60 |issue=12 |pages=1373–7 |year=2007 |pmid=18042693 |pmc=2095573 |doi=10.1136/jcp.2007.052456 |url=}}</ref><ref name="pmid180426932">Matutes E (2007) [http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&retmode=ref&cmd=prlinks&id=18042693 Adult T-cell leukaemia/lymphoma.] ''J Clin Pathol'' 60 (12):1373-7. [http://dx.doi.org/10.1136/jcp.2007.052456 DOI:10.1136/jcp.2007.052456] PMID: [http://pubmed.gov/18042693 18042693]</ref><ref name="pmid1751370">{{cite journal| author=Shimoyama M| title=Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87). | journal=Br J Haematol | year= 1991 | volume= 79 | issue= 3 | pages= 428-37 | pmid=1751370 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1751370  }}</ref>
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* Mature T-cell lymphoma
* [[Human T-lymphotropic virus|Human T-lymphotrophic virus 1 infection]]
| align="left" style="background:#F5F5F5;" + |
* [[Hepatosplenomegaly|Hepatosplenomagaly]]
* T-cell deficiency leading [[immunodeficiency]] causing [[opportunistic infection]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
* [[Hepatosplenomegaly]]
| align="left" style="background:#F5F5F5;" + |
* [[Hypercalcemia]]
* [[Skin lesion|Skin lesions]]
* Lytic bone lesions
* Elevated [[Lactate dehydrogenase|LDH]]
| align="left" style="background:#F5F5F5;" + |
* [[Adult T-cell leukemia]] cells are typically positive for:
* [[CD2]]
* [[CD4]]
* [[CD5]]
* [[CD8]]
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* Charecterstic leukemia cells with sharp [[nuclear]] indentations and a prominent [[nucleoli]]
* "Cloverleaf" or "flower" cells
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* [[Hypercalcemia]]
|-
! colspan="2" align="center" style="background:#DCDCDC;" + |[[Anaplastic large cell lymphoma]]<br><ref name="Al-AhmadMaertens2017">{{cite journal|last1=Al-Ahmad|first1=Selma|last2=Maertens|first2=Vincent|last3=Libeer|first3=Christophe|last4=Schelfhout|first4=Vera|last5=Vanhoenacker|first5=Filip|last6=Boeckx|first6=Nancy|last7=Vandevenne|first7=Marleen|title=The masquerading presentation of a systemic anaplastic large cell lymphoma, ALK positive: a case report and review of the literature|journal=Acta Clinica Belgica|volume=72|issue=6|year=2017|pages=454–460|issn=1784-3286|doi=10.1080/17843286.2017.1312057}}</ref><ref name="pmid25197351">{{cite journal| author=Yu L, Yan LL, Yang SJ| title=Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature. | journal=Int J Clin Exp Pathol | year= 2014 | volume= 7 | issue= 8 | pages= 4806-16 | pmid=25197351 | doi= | pmc=4152041 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25197351  }}</ref><ref name="pmid28652989">{{cite journal| author=de Campos FPF, Zerbini MCN, Felipe-Silva A, Simões AB, Lovisolo SM, da Fonseca LG et al.| title=Unusual clinical presentation of anaplastic large cell lymphoma. | journal=Autops Case Rep | year= 2014 | volume= 4 | issue= 1 | pages= 21-27 | pmid=28652989 | doi=10.4322/acr.2014.004 | pmc=5470561 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28652989  }}</ref><ref name="pmid17965727">{{cite journal |author=Watanabe M, Ogawa Y, Itoh K |title=Hypomethylation of CD30 CpG islands with aberrant JunB expression drives CD30 induction in Hodgkin lymphoma and anaplastic large cell lymphoma |journal=Lab. Invest. |volume=88 |issue=1 |pages=48–57 |date=January 2008 |pmid=17965727 |doi=10.1038/labinvest.3700696|display-authors=etal}}</ref>
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* Mature T-cell lymphoma
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*[[Fever]]
*[[Weight loss]]
*[[Night sweats]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
Painless swelling in :
* [[Neck masses|Neck]]
* [[Axilla]]
* [[Groin]]
* [[Chest|Thorax]]
* [[Abdominal mass|Abdomen]]
| align="center" style="background:#F5F5F5;" + |
* Skin rash
* [[Chest pain]]
* [[Abdominal pain]]
* [[Bone pain]]
| align="left" style="background:#F5F5F5;" + |Strongly immunoreactive for :
* [[CD30]]
* Anaplastic lymphoma kinase (ALK)
* EMA
* [[vimentin]]
| align="left" style="background:#F5F5F5;" + |
*
* Nucleoli tend to be more prominent
* The cytoplasm may be either [[basophilic]] or [[eosinophilic]] and the cell might have many nuclei with dispersed or clumped [[chromatin]]
| align="center" style="background:#F5F5F5;" + |
* May be caused by textured breast implants
|-
! align="center" style="background:#DCDCDC;" + |Cutaneous T-cell lymphoma
! align="center" style="background:#DCDCDC;" + |[[Mycosis fungoides]] / [[Sézary syndrome]]<br><ref name="FossGirardi2017">{{cite journal|last1=Foss|first1=Francine M.|last2=Girardi|first2=Michael|title=Mycosis Fungoides and Sezary Syndrome|journal=Hematology/Oncology Clinics of North America|volume=31|issue=2|year=2017|pages=297–315|issn=08898588|doi=10.1016/j.hoc.2016.11.008}}</ref><ref name="CampbellClark2010">{{cite journal|last1=Campbell|first1=J. J.|last2=Clark|first2=R. A.|last3=Watanabe|first3=R.|last4=Kupper|first4=T. S.|title=Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors|journal=Blood|volume=116|issue=5|year=2010|pages=767–771|issn=0006-4971|doi=10.1182/blood-2009-11-251926}}</ref><ref name="VonderheidBernengo2002">{{cite journal|last1=Vonderheid|first1=Eric C.|last2=Bernengo|first2=Maria Grazia|last3=Burg|first3=Günter|last4=Duvic|first4=Madeleine|last5=Heald|first5=Peter|last6=Laroche|first6=Liliane|last7=Olsen|first7=Elise|last8=Pittelkow|first8=Mark|last9=Russell-Jones|first9=Robin|last10=Takigawa|first10=Masahiro|last11=Willemze|first11=Rein|title=Update on erythrodermic cutaneous T-cell lymphoma: Report of the international society for cutaneous lymphomas|journal=Journal of the American Academy of Dermatology|volume=46|issue=1|year=2002|pages=95–106|issn=01909622|doi=10.1067/mjd.2002.118538}}</ref>
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* Mature T-cell Lymphoma
* The [[tumor]] [[Cell (biology)|cells]] originate from [[memory T cells]] or [[skin]] homing [[CD4+ T cells]] expressing [[cutaneous]] [[lymphocyte]] [[antigen]] (CLA) an<nowiki/>d [[chemokine]] [[receptors]] [[CCR4]]<nowiki/>and CCR7.
* It is understood that cutaneous t cell lymphoma (maycosis fungoides, Sezary sydrome ) is the result of malignant T cell that derived from a mature CD41 CD45RO1 memory T cells.
| align="left" style="background:#F5F5F5;" + |
* [[Weight loss]]
* [[Malaise]] and [[fatigue]]
* [[Anemia]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
* Cutaneous manifestations
| align="left" style="background:#F5F5F5;" + |
* [[Lymphadenopathy]]
* May progress to [[Sezary syndrome]]
| align="left" style="background:#F5F5F5;" + |
* Beta F1+
* [[CD2]]-/+
* [[CD3]]+
* [[CD3]]- ([[CD4]]-positive variant)
* [[CD4]]+ ([[CD4]]-positive variant)
* [[CD4]]-
* [[CD5]]-
* [[CD7]]+/-
* [[CD8]]+
* [[CD8]]- ([[CD4]]-positive variant),
* [[Granzyme B|Granzyme B+]]
* [[perforin]]+
| align="left" style="background:#F5F5F5;" + |
:* Polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical [[lymphoid]] cells
:* These cells may line up individually along the epidermal basal layer
:* The presence of spongiosis is highly suggestive of [[mycosis fungoides]]
| align="center" style="background:#F5F5F5;" + |
* [[Epidermis (skin)|Epidermal]] [[atrophy]] or poikiloderma
* Generalized [[itching]]([[pruritus]])
* [[Pain]] in the affected area of the skin.
* [[Insomnia]]
* Red ([[erythematous]]) patches scattered over the [[skin]] of the [[trunk]] and the [[extremities]]
|-
! rowspan="6" align="center" style="background:#DCDCDC;" + |Peripheral T-cell lymphoma
! align="center" style="background:#DCDCDC;" + |[[Subcutaneous panniculitis-like T-cell lymphoma]]<br><ref name="seer.cancer.gov">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016</ref><ref name="seer.cancer.gov2">Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016</ref><ref name="pmid191959752">{{cite journal| author=Parveen Z, Thompson K| title=Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. | journal=Arch Pathol Lab Med | year= 2009 | volume= 133 | issue= 2 | pages= 303-8 | pmid=19195975 | doi=10.1043/1543-2165-133.2.303 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19195975  }}</ref>
| align="left" style="background:#F5F5F5;" + |
* Mature T-cell lymphoma
| align="left" style="background:#F5F5F5;" + |
*[[Fever]]
*[[Chills]]
*[[Weight loss]]
*[[Night sweats]]
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |Painless swellings on:
* Extremities
* Trunks
| align="left" style="background:#F5F5F5;" + |
* [[Myalgias]]
| align="left" style="background:#F5F5F5;" + |Positive for:
* BetaF1
* [[CD8]]
* [[CD30]]
* [[CLA]]
* T-cell intracellular antigen (T1A1)
* [[Perforin]]
| align="left" style="background:#F5F5F5;" + |
* Atypical lymphoid cells
* [[Fat necrosis]]
* [[Karyorrhexis]]
| align="left" style="background:#F5F5F5;" + |
* [[Subcutaneous panniculitis-like T-cell lymphoma]] can mimic the following conditions making it difficult to diagnose
* [[Panniculitis|Begnign panniculitis]]
* [[Eczema]]
* [[Cellulitis]]
* [[Dermatitis]]
|-
! align="center" style="background:#DCDCDC;" + |[[Hepatosplenic T cell lymphoma|Hepatosplenic  T-cell lymphoma]]<br><ref name="pmid28058028">{{cite journal| author=van de Meeberg MM, Derikx LA, Sinnige HA, Nooijen P, Schipper DL, Nissen LH| title=Hepatosplenic T-cell lymphoma in a 47-year-old Crohn's disease patient on thiopurine monotherapy. | journal=World J Gastroenterol | year= 2016 | volume= 22 | issue= 47 | pages= 10465-10470 | pmid=28058028 | doi=10.3748/wjg.v22.i47.10465 | pmc=5175260 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28058028  }}</ref><ref name="ShiWang2015">{{cite journal|last1=Shi|first1=Yang|last2=Wang|first2=Endi|title=Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer–Cell Neoplasms|journal=Archives of Pathology & Laboratory Medicine|volume=139|issue=9|year=2015|pages=1173–1180|issn=0003-9985|doi=10.5858/arpa.2014-0079-RS}}</ref><ref name="pmid22379294">{{cite journal| author=Alsohaibani FI, Abdulla MA, Fagih MM| title=Hepatosplenic T-cell lymphoma. | journal=Indian J Hematol Blood Transfus | year= 2011 | volume= 27 | issue= 1 | pages= 39-42 | pmid=22379294 | doi=10.1007/s12288-010-0051-1 | pmc=3102508 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22379294  }}</ref>
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* Mature T-cell Lymphoma
* [[Inflammatory bowel disease]]
* Organ transplant patients (reciever)
* [[Immunosuppresive drug|Immunosuppresent medications]]
* [[Thiopurine|Thiopurines]]
* [[Infliximab]]
* [[Cyclophosphamide]]
* [[Vincristine sulfate|Vincristine]]
* [[Doxorubicin hydrochloride|Doxorubicin]]
| align="left" style="background:#F5F5F5;" + |
* [[Fever]]
* [[Fatigue]]
* [[Weight loss]]
* [[Night sweats]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |Painless swelling in :
* [[Neck masses|Neck]]
* [[Axilla]]
* [[Groin]]
* [[Chest|Thorax]]
* [[Abdominal mass|Abdomen]]
| align="center" style="background:#F5F5F5;" + |
* [[Skin rash]]
* [[Chest pain]]
* [[Abdominal pain]]
* [[Bone pain]]
| align="left" style="background:#F5F5F5;" + |
* [[CD2]]
* [[CD3 (immunology)|CD3]]
* [[CD7]]
* [[CD16]] 
* CD56  or [[CD57]] 
* [[TIA1]]
* [[Granzyme|Granzyme m]]
* [[Fas ligand]]
| align="left" style="background:#F5F5F5;" + |
* Intermediate sized  tumor cells with clear cytoplasm,  oval nuclei, slightly dispersed condensed chromatin, inconspicuous nucleoli
| align="left" style="background:#F5F5F5;" + |
* Patients with inflammatory bowel diseases taking immunosuppressant and anti-tumor necrosis factor-α agent are also have a higher risk for developing [[hepatosplenic  T-cell lymphoma]]
|-
! align="center" style="background:#DCDCDC;" + |[[Enteropathy-associated T-cell lymphoma|Enteropathy-type intestinal T-cell lymphoma]]<br><ref name="DelabieHolte2011">{{cite journal|last1=Delabie|first1=J.|last2=Holte|first2=H.|last3=Vose|first3=J. M.|last4=Ullrich|first4=F.|last5=Jaffe|first5=E. S.|last6=Savage|first6=K. J.|last7=Connors|first7=J. M.|last8=Rimsza|first8=L.|last9=Harris|first9=N. L.|last10=Muller-Hermelink|first10=K.|last11=Rudiger|first11=T.|last12=Coiffier|first12=B.|last13=Gascoyne|first13=R. D.|last14=Berger|first14=F.|last15=Tobinai|first15=K.|last16=Au|first16=W. Y.|last17=Liang|first17=R.|last18=Montserrat|first18=E.|last19=Hochberg|first19=E. P.|last20=Pileri|first20=S.|last21=Federico|first21=M.|last22=Nathwani|first22=B.|last23=Armitage|first23=J. O.|last24=Weisenburger|first24=D. D.|title=Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project|journal=Blood|volume=118|issue=1|year=2011|pages=148–155|issn=0006-4971|doi=10.1182/blood-2011-02-335216}}</ref><ref name="pmid22943012">{{cite journal |vauthors=Bautista-Quach MA, Ake CD, Chen M, Wang J |title=Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features |journal=J Gastrointest Oncol |volume=3 |issue=3 |pages=209–25 |date=September 2012 |pmid=22943012 |pmc=3418529 |doi=10.3978/j.issn.2078-6891.2012.024 |url=}}</ref><ref name="cancer.gov">Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016</ref>
| align="left" style="background:#F5F5F5;" + |
* Mature T-cell lymphoma
| align="left" style="background:#F5F5F5;" + |
*[[Fever]]
*[[Weight loss]]
*[[Night sweats]]
*[[Diarrhea]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | +
| align="left" style="background:#F5F5F5;" + |Painless swelling in the :
* [[Neck masses|Neck]]
* [[axilla]]
* [[groin]]
* [[thorax]],
* [[abdomen]]
| align="left" style="background:#F5F5F5;" + |
* [[Skin rash]]
* [[Chest pain]]
* [[Abdominal pain]]
* [[Bone pain]]
| align="left" style="background:#F5F5F5;" + |
* [[CD3]]+
* [[CD4]]-
* [[CD5]]-
* [[CD7]]+
* [[CD30]]+
* [[CD56]]+
* [[CD103]]+
* [[CD8]]+/-
* TCR beta+/-
| align="left" style="background:#F5F5F5;" + |
*Lymphomatous infiltrate with [[neoplastic]] large cells
*May have pleomorphic, multinucleated cells
*Adjacent mucosa shows villous atrophy, crypt [[hyperplasia]], increased inflammatory cells 
*[[Lymphocytosis]]
| align="left" style="background:#F5F5F5;" + |
* Mostly diagnosed in the small intestine
|-
! align="center" style="background:#DCDCDC;" + |Extranodal T-cell lymphoma, nasal type<br><ref name="Hindawi">Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016</ref><ref name="cancer.gov2">Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 02, 2016</ref><ref name="Hindawi2">Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016</ref>
| align="left" style="background:#F5F5F5;" + |
* Mature T-cell lymphoma
| align="left" style="background:#F5F5F5;" + |
* [[Fever]]
* [[Weight loss]]
* [[Night sweats]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |Painless swelling in :
* [[Neck masses|Neck]]
* [[Axilla]]
* [[Groin]]
* [[Chest|Thorax]]
* [[Abdominal mass|Abdomen]]
| align="left" style="background:#F5F5F5;" + |
* [[Rash|Skin rash]]
* [[Chest pain]]
* [[Abdominal pain]]
* [[Bone pain]]
| align="left" style="background:#F5F5F5;" + |
* Cytoplasmic [[CD3]] epsilon
* [[Granzyme|Granzyme B]]
* [[Perforin]]
* [[CD2]]
* [[CD56]]
| align="left" style="background:#F5F5F5;" + |
* Medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells
* [[Lymphoma|Lymphoma cells]] may be admixed with a polymorphic infiltrate of non-neoplastic inflammatory cells
| align="left" style="background:#F5F5F5;" + |
* [[Proptosis/exophthalmos|Protrusion of eye]]
* [[Swollen face|Swelling of the face]]
* Discharge from the nose
* [[Epistaxis|Nose bleeds]]
* [[Nasal congestion|Blockage of the nasal passages]]
|-
! align="center" style="background:#DCDCDC;" + |Angioimmunoblastic T-cell lymphoma<br><ref name="who1">{{cite book |last=Swerdlow |first=S.H. |last2=Campo |first2=E. |last3=Harris |first3=N.L. |last4=Jaffe |first4=E.S. |last5=Pileri |first5=S.A. |last6=Stein |first6=H. |last7=Thiele |first7=J. |last8=Vardiman |first8=J.W |chapter=11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma |title=WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues |edition=4th |series=IARC WHO Classification of Tumours |volume=2 |publisher=IARC |year=2008 |isbn=9283224310 |url=http://apps.who.int/bookorders/anglais/detart1.jsp?codlan=1&codcol=70&codcch=4002&content=1}}</ref><ref name="quin1">[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=10524524&query_hl=30&itool=pubmed_ExternalLink] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." '''Am J Surg Pathol'''. 1999 Oct;23(10):1233-40. PMID: 10524524</ref><ref name="seer">Angioimmunoblastic T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52dc/ Accessed on November 27, 2015</ref>
| align="left" style="background:#F5F5F5;" + |
* Mature T-cell lymphoma
| align="left" style="background:#F5F5F5;" + |
* [[Fever]] 
* [[Weight loss]]
* [[Night sweats]] 
* [[Edema]]
* [[Fatigue]]
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
* Painless swelling in :
* [[Neck masses|Neck]]
* [[Axilla]]
* [[Groin]]
* [[Chest|Thorax]]
* [[Abdominal mass|Abdomen]]
| align="left" style="background:#F5F5F5;" + |
* Skin rash
* [[abdominal pain]]
* [[bone pain]]
* [[dark urine]]
* [[shortness of breath]]
* [[chronic pain]] and swelling of joints
* Chest pain
| align="left" style="background:#F5F5F5;" + |
* [[CD2]]
* [[CD3]]
* [[CD4]]
* [[CD5]]
* [[CD10]]
* [[CD20]]
* Focal positivity for [[CXCL13]]
| align="left" style="background:#F5F5F5;" + |
* Leukocytic infiltrate, represented mostly by elongated cells with marked artifactual changes
* Hyperplastic [[germinal centers]] and [[Reed-Sternberg cells]]
| align="left" style="background:#F5F5F5;" + |
* [[Monoclonal gammopathy]]
* [[Autoimmune hemolytic anemia]]
|-
! align="center" style="background:#DCDCDC;" + |Peripheral T-cell lymphoma, unspecified<br><ref name="pmid22760778">{{cite journal |vauthors=Lemonnier F, Couronné L, Parrens M, Jaïs JP, Travert M, Lamant L, Tournillac O, Rousset T, Fabiani B, Cairns RA, Mak T, Bastard C, Bernard OA, de Leval L, Gaulard P |title=Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters |journal=Blood |volume=120 |issue=7 |pages=1466–9 |date=August 2012 |pmid=22760778 |doi=10.1182/blood-2012-02-408542 |url=}}</ref><ref name="JhaGupta2017">{{cite journal|last1=Jha|first1=KunalKishor|last2=Gupta|first2=SureshK|last3=Saluja|first3=Harpreet|last4=Subedi|first4=Nuwadatta|title=Peripheral T-cell lymphoma, not otherwise specified|journal=Journal of Family Medicine and Primary Care|volume=6|issue=2|year=2017|pages=427|issn=2249-4863|doi=10.4103/jfmpc.jfmpc_323_16}}</ref>
| align="left" style="background:#F5F5F5;" + |
* Mature T-cell lymphoma
| align="left" style="background:#F5F5F5;" + |
* [[Fatigue]]
* [[Fever]]
* Frequent [[Infection|infections]]
| align="center" style="background:#F5F5F5;" + | -
| align="center" style="background:#F5F5F5;" + | +
| align="center" style="background:#F5F5F5;" + | -
| align="left" style="background:#F5F5F5;" + |
* [[Lymphadenopathy]]
* [[Hepatosplenomegaly]]
| align="left" style="background:#F5F5F5;" + |
* [[Lymphadenopathy]] (swollen )
* [[Thirst]]
* [[Nausea and vomiting|Nausea]]
* [[Nausea and vomiting|Vomiting]]
* Skin and bone abnormalities
* Enlarged liver and/or spleen
| align="left" style="background:#F5F5F5;" + |
*[[CD2]]
*[[CD3]]
*[[TCR]]
*β F1
*Variable [[CD4]]
*[[CD5]]
*[[CD7]]
*Occasional [[CD56]]
*Cytotoxic granule expression
| align="left" style="background:#F5F5F5;" + |
*Clear cytoplasm, resemble [[Reed-Sternberg cell|Reed-Sternberg cells]], with irregular, pleomorphic, hyperchromatic or  nuclei
*A lot of mitotic figures; very broad cytologic spectrum
| align="left" style="background:#F5F5F5;" + |
* Endogenous [[immunosupression]]
* Patients may present with [[Pneumocystis jirovecii pneumonia|p.jiroveci]]  or [[Chickenpox|varicella zoster]] infection
* Can be confused with other [[infectious]] or rheumatologic diseases
|}
|}


Revision as of 13:41, 25 April 2019


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Hepatosplenic T cell lymphoma Microchapters

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Differentiating Hepatosplenic T cell lymphoma from other Diseases

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief: Jogeet Singh Sekhon, M.D. [3]

Synonyms and Keywords: Hepatosplenic γδ T-cell lymphoma, Hepatosplenic gamma delta T-cell lymphoma, HSTCL

Overview

Hepatosplenic T cell lymphoma is a rare type of non Hodgkins lymphoma that occurs in states of immunosupression such as post organ transplant and treatment of inflammatory bowel disease. Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990. It is also known as "gamma-delta" hepatosplenic T-cell lymphoma. The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow. The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year. If left untreated, patients can develop liver failure, pancytopenia or spleen rupture. Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic T-cell lymphoma. Chemotherapy including CHOP is the mainstay of the treatment along with bone marrow transplant and radiation therapy.

Historical Perspective

Hepatosplenic T cell lymphoma was discovered by Farcet et al in 1990.

Classification

There is no established system for the classification of hepatosplenic t cell lymphoma.

Pathophysiology

  • Hepatosplenic T-cell lymphoma is a peripheral T-cell lymphoma, a type of non Hodgkin's lymphoma.[1]
  • It is also known as "gamma-delta hepatosplenic T-cell lymphoma".[2]
  • It usually occurs in young men with history of immunosuppression including solid organ transplantation.[3]
  • Patients with inflammatory bowel disease receiving immunosuppressants and anti-tumor necrosis factor-α agent are also at risk for developing hepatosplenic T-cell lymphoma.
  • The T-cell receptor consists of either a gamma delta or alpha-beta entity on their cell surface which are a part of the innate immune system.[4][5]
  • Gamma delta T cells represent the first line of defense against bacterial peptides, such as heat-shock proteins.
  • Gamma delta T cells are CD4 and CD8 negative, but CD56 positive which is NK cell marker.
  • Gamma delta cells respond to a stimulus and are responsible for lymphokine production and proliferation.
  • Gamma delta cells are predominantly located in the spleen, liver sinusoids and intestinal epithelium.
  • 75 % of the cases are of gamma delta phenotype and the rest are apha beta phenotype.
  • Chronic antigen stimulation in states of immunosuppression is responsible for the development of the lymphoma.
  • Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome.
  • Mutations in SETD2, INO80, TET3 and STAT5B occur exclusively in hepatosplenic T cell lymphoma as compared to other T and B cell lymphoma types.
  • The lymphoma is characterized by malignant T-cell proliferation in the sinusoids of the liver, sinuses and red pulp of the spleen, and sinuses of the bone marrow.
  • Splenic involvement is characterized by diffuse involvement of the red pulp with small-to-medium-sized atypical lymphocytes.
  • The atypical lymphocytes are present within the cords and sinuses of the red pulp.
  • There occurs a complete loss of the white pulp.
  • The liver also shows sinusoidal infiltration by neoplastic lymphoid cells.
  • The bone marrow is characterized by neoplastic cells in the sinusoids.
  • Bone marrow infiltration results in pancytopenia.
  • The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression.
  • It manifests as hepatosplenomegaly without peripheral lymphadenopathy.
  • Pancytopenia and abnormal liver functions are the laboratory findings.
  • Histology and immunohistochemistry of the tumor biopsy shows portal and sinusoidal infiltration by atypical small-to-medium sized lymphocytes with hyperchromatic nuclei and low mitotic activity.

Causes

Common causes of hepatosplenic t cell lymphoma are: [6][7]

Differentiating hepatosplenic t cell lymphoma from Other Diseases

Differential diagnosis for the lymphoma is based on the below table:

Differentiating diagnosis of Lymphoma Symptoms Signs Diagnosis Additional Findings
Fever Rash Diarrhea Abdominal pain Weight loss Painful lymphadenopathy Hepatosplenomegaly Arthritis Lab Findings
Lymphoma + – – + + – + – Increase ESR, increased LDH Night sweats, constant fatigue
Brucellosis + + – + + + + + Relative lymphocytosis Night sweats, often with characteristic smell, likened to wet hay
Typhoid fever + + – + – – + + Decreased hemoglobin Incremental increase in temperature initially and than sustained fever as high as 40°C (104°F)
Malaria + – + + – – + + Microcytosis,

elevated LDH

"Tertian" fever: paroxysms occur every second day
Tuberculosis + + – + + + – + Mild normocytic anemia, hyponatremia, and

hypercalcemia

Night sweats, constant fatigue
Mumps + – – – – + – – Relative lymphocytosis, serum amylaseelevated Parotidswelling/tenderness
Rheumatoid arthritis – + – – – – – + ESR and CRP elevated, positive rheumatoid factor Morning stiffness
SLE – + – + + – – + ESR and CRP elevated, positive ANA Fatigue
HIV – – – + + + – + Leukopenia Constant fatigue

Differentiating different types of T-cell Non-Hodgkin lymphoma. The gold standard for differentiation different types of Non-Hodgkin lymphoma is biopsy.

! colspan="12" align="center" style="background:#7d7d7d; color: #FFFFFF;" + |Non-Hodgkin's Lymphoma (T Cell Lymphoma) |- ! colspan="2" align="center" style="background:#DCDCDC;" + |Precursor T-cell lymphoblastic leukemia/lymphoma
[8][9] | align="left" style="background:#F5F5F5;" + |

  • Precursor T-cell lymphomas

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

|- ! colspan="2" align="center" style="background:#DCDCDC;" + |T-cell granular lymphocytic
[10][11][12] | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |May be associated with the following conditions:

|- ! colspan="2" align="center" style="background:#DCDCDC;" + |T-cell prolymphocytic leukemia
[13] | align="left" style="background:#F5F5F5;" + |

  • Abnormal proliferation of mature T cells (post thymic)

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

  • Lymphocytes with abundant basophilic, nongranular cytoplasm, atypical nucleus
  • Cytoplasmic protrusions (blebs)

| align="left" style="background:#F5F5F5;" + |

|- ! colspan="2" align="center" style="background:#DCDCDC;" + |Adult T cell leukemia/lymphoma
[14][15][16] | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

  • Charecterstic leukemia cells with sharp nuclear indentations and a prominent nucleoli
  • "Cloverleaf" or "flower" cells

| align="left" style="background:#F5F5F5;" + |

|- ! colspan="2" align="center" style="background:#DCDCDC;" + |Anaplastic large cell lymphoma
[17][18][19][20] | align="left" style="background:#F5F5F5;" + |

  • Mature T-cell lymphoma

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + | Painless swelling in :

| align="center" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |Strongly immunoreactive for :

| align="left" style="background:#F5F5F5;" + |

  • Nucleoli tend to be more prominent
  • The cytoplasm may be either basophilic or eosinophilic and the cell might have many nuclei with dispersed or clumped chromatin

| align="center" style="background:#F5F5F5;" + |

  • May be caused by textured breast implants

|- ! align="center" style="background:#DCDCDC;" + |Cutaneous T-cell lymphoma ! align="center" style="background:#DCDCDC;" + |Mycosis fungoides / Sézary syndrome
[21][22][23] | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |

  • Cutaneous manifestations

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

  • Polymorphous inflammatory infiltrate in the dermis that contains small numbers of frankly atypical lymphoid cells
  • These cells may line up individually along the epidermal basal layer
  • The presence of spongiosis is highly suggestive of mycosis fungoides

| align="center" style="background:#F5F5F5;" + |

|- ! rowspan="6" align="center" style="background:#DCDCDC;" + |Peripheral T-cell lymphoma ! align="center" style="background:#DCDCDC;" + |Subcutaneous panniculitis-like T-cell lymphoma
[24][25][26] | align="left" style="background:#F5F5F5;" + |

  • Mature T-cell lymphoma

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |Painless swellings on:

  • Extremities
  • Trunks

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |Positive for:

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

|- ! align="center" style="background:#DCDCDC;" + |Hepatosplenic T-cell lymphoma
[27][28][29] | align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | - | align="left" style="background:#F5F5F5;" + |Painless swelling in :

| align="center" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

  • Intermediate sized tumor cells with clear cytoplasm, oval nuclei, slightly dispersed condensed chromatin, inconspicuous nucleoli

| align="left" style="background:#F5F5F5;" + |

  • Patients with inflammatory bowel diseases taking immunosuppressant and anti-tumor necrosis factor-α agent are also have a higher risk for developing hepatosplenic T-cell lymphoma

|- ! align="center" style="background:#DCDCDC;" + |Enteropathy-type intestinal T-cell lymphoma
[30][31][32] | align="left" style="background:#F5F5F5;" + |

  • Mature T-cell lymphoma

| align="left" style="background:#F5F5F5;" + |

| align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | + | align="center" style="background:#F5F5F5;" + | + | align="left" style="background:#F5F5F5;" + |Painless swelling in the :

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

| align="left" style="background:#F5F5F5;" + |

  • Lymphomatous infiltrate with neoplastic large cells
  • May have pleomorphic, multinucleated cells
  • Adjacent mucosa shows villous atrophy, crypt hyperplasia, increased inflammatory cells
  • Lymphocytosis

| align="left" style="background:#F5F5F5;" + |

  • Mostly diagnosed in the small intestine

|- ! align="center" style="background:#DCDCDC;" + |Extranodal T-cell lymphoma, nasal type
[33][34][35] | align="left" style="background:#F5F5F5;" + |

  • Mature T-cell lymphoma

| align="left" style="background:#F5F5F5;" + |

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  • Medium sized tumor cells and polymorphic infiltrate of non-neoplastic inflammatory cells
  • Lymphoma cells may be admixed with a polymorphic infiltrate of non-neoplastic inflammatory cells

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|- ! align="center" style="background:#DCDCDC;" + |Angioimmunoblastic T-cell lymphoma
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  • Mature T-cell lymphoma

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|- ! align="center" style="background:#DCDCDC;" + |Peripheral T-cell lymphoma, unspecified
[39][40] | align="left" style="background:#F5F5F5;" + |

  • Mature T-cell lymphoma

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  • Clear cytoplasm, resemble Reed-Sternberg cells, with irregular, pleomorphic, hyperchromatic or nuclei
  • A lot of mitotic figures; very broad cytologic spectrum

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Epidemiology and Demographics

  • The incidence of hepatosplenic t cell lymphoma is 0.3 per 100000 individuals per year.
  • It occurs in younger group of patients, most cases falling in 20-40 years of age group.
  • Men are more affected than the females.

Risk Factors

Common risk factors include:

Screening

There is insufficient evidence to recommend routine screening for hepatosplenic t cell lymphoma.

Natural History, Complications, and Prognosis

Natural history

  • Patients have a history of immunosupression such as inflammatory bowel disease under treatment or organ transplant.[41]
  • The mean age group is 35 years and most of the patients are males.
  • Initial symptoms are fever, weight loss, night sweats and then progress to more severe symptoms depending on the organ involvement but there is no lymphadenopathy.
  • Patients also present with symptoms of liver, spleen and bone marrow dysfunction.
  • If left untreated, patients can develop liver failure, pancytopenia, or spleen rupture.

Complications

Prognosis

  • The prognosis is very poor, with patients dying within 2-3 years of diagnosis even after recieving treatment[42].

Diagnosis

Diagnostic Study of Choice

  • Biopsy of the tumor is the gold standard diagnostic test for diagnosing hepatosplenic t cell lymphoma[43].
  • CT scan and PET scan are used to assess the spread of the lymphoma.

Symptoms

The most common symtoms are[44]:

  • Fever
  • Weight loss
  • Night sweats
  • Pain abdomen
  • Jaundice
  • Fatigue
  • Recurrent infections
  • Bleeding

Physical Examination

Temperature

Skin

Thorax

Abdomen

Extremities

Laboratory Findings

  • Biopsy of the tumor:
    • Histology - small-to intermediate sized T lymphocytes infiltrate the sinusoids of the liver and the splenic red pulp.
    • Flow cytometry and immunophenotyping - The lymphoma cells are typically CD2+, CD3+, CD4−, CD5−, CD7+, CD8− CD42+, CD52+, CD76+, CD82+ with either gamma-delta or alpha-beta T-cell phenotypic receptor expression[45].
    • Karyotyping - Isochromosome of the long arm of chromosome 7 is a genetic abnormality described in hepatosplenic t cell lymphoma. It could also be in association with trisomy 8 and a loss of a sex chromosome[46].
  • Bone marrow biopsy; The bone marrow is characterized by neoplastic cells in the sinusoids.
  • Complete blood count; Pancytopenia
  • Liver function tests: Deranged LFT

Electrocardiogram

There are no ECG findings associated with hepatosplenic t cell lymphoma.

X-ray

There are no x-ray findings associated with hepatosplenic t cell lymphoma but pleural effusion might be present.

Echocardiography or Ultrasound

  • There are no echocardiography findings associated with hepatosplenic t cell lymphoma.
  • On ultrasound of abdomen:

CT scan

Tumor mass can be seen in liver or spleen or both.

MRI

Tumor mass can be seen in liver or spleen or both.

Other Imaging Findings

PET scan: On PET scan, tumor mass can be seen in liver or spleen or if it has metastatsized to any other organ.

Treatment

Medical Therapy

Surgery

Surgical intervention is not recommended for the management of hepaosplenic t cell lymphoma.

Primary Prevention

There are no established measures for the primary prevention of hepatosplenic t cell lymphoma.

Secondary Prevention

There are no established measures for the secondary prevention of hepatosplenic t cell lymphoma.

References

  1. ↑ Armitage JO (2017). "The aggressive peripheral T-cell lymphomas: 2017". Am J Hematol. 92 (7): 706–715. doi:10.1002/ajh.24791. PMID 28516671.
  2. ↑ Brandt PH, Rahmat LT, Ali SS (2019). "A rare case of hepatosplenic gamma-delta T-cell lymphoma and secondary hemophagocytic lymphohistiocytosis". Clin Case Rep. 7 (1): 164–169. doi:10.1002/ccr3.1924. PMC 6333078. PMID 30656034.
  3. ↑ Choi Y, Jeon SY, Yoo WH (2018). "Hepatosplenic T-Cell Lymphoma Arising in a Patient Treated With Tumor Necrosis Factor-α Inhibitors for Ankylosing Spondylitis". J Clin Rheumatol. doi:10.1097/RHU.0000000000000805. PMID 29933321.
  4. ↑ Gowda L, Foss F (2019). "Hepatosplenic T-Cell Lymphomas". Cancer Treat Res. 176: 185–193. doi:10.1007/978-3-319-99716-2_9. PMID 30596219.
  5. ↑ Gaulard P, Bourquelot P, Kanavaros P, Haioun C, Le Couedic JP, Divine M; et al. (1990). "Expression of the alpha/beta and gamma/delta T-cell receptors in 57 cases of peripheral T-cell lymphomas. Identification of a subset of gamma/delta T-cell lymphomas". Am J Pathol. 137 (3): 617–28. PMC 1877506. PMID 1698028.
  6. ↑ Yabe M, Miranda RN, Medeiros LJ (2018). "Hepatosplenic T-cell Lymphoma: a review of clinicopathologic features, pathogenesis, and prognostic factors". Hum Pathol. 74: 5–16. doi:10.1016/j.humpath.2018.01.005. PMID 29337025.
  7. ↑ Gallamini A, Stelitano C, Calvi R, Bellei M, Mattei D, Vitolo U; et al. (2004). "Peripheral T-cell lymphoma unspecified (PTCL-U): a new prognostic model from a retrospective multicentric clinical study". Blood. 103 (7): 2474–9. doi:10.1182/blood-2003-09-3080. PMID 14645001.
  8. ↑ Shelly D, Gujral S (2017). "Early T-Cell Precursor Acute Lymphoblastic Leukaemia/Lymphoma: Immunohistochemical Evaluation of Four Lymph Node Biopsies". J Clin Diagn Res. 11 (7): EL01–EL02. doi:10.7860/JCDR/2017/29352.10164. PMC 5583929. PMID 28892922.
  9. ↑ You MJ, Medeiros LJ, Hsi ED (2015). "T-lymphoblastic leukemia/lymphoma". Am J Clin Pathol. 144 (3): 411–22. doi:10.1309/AJCPMF03LVSBLHPJ. PMID 26276771.
  10. ↑ Rose, M. G. (2004). "T-Cell Large Granular Lymphocyte Leukemia and Related Disorders". The Oncologist. 9 (3): 247–258. doi:10.1634/theoncologist.9-3-247. ISSN 1083-7159.
  11. ↑ Liu EB, Chen HS, Zhang PH, Li ZQ, Sun Q, Yang QY; et al. (2012). "[Hematopathologic features of T-cell large granular lymphocytic leukemia]". Zhonghua Bing Li Xue Za Zhi. 41 (4): 229–33. doi:10.3760/cma.j.issn.0529-5807.2012.04.004. PMID 22800517.
  12. ↑ Osuji N, Matutes E, Catovsky D, Lampert I, Wotherspoon A (2005). "Histopathology of the spleen in T-cell large granular lymphocyte leukemia and T-cell prolymphocytic leukemia: a comparative review". Am J Surg Pathol. 29 (7): 935–41. PMID 15958859.
  13. ↑ Graham RL, Cooper B, Krause JR (2013). "T-cell prolymphocytic leukemia". Proc (Bayl Univ Med Cent). 26 (1): 19–21. PMC 3523759. PMID 23382603.
  14. ↑ Matutes E (2007). "Adult T-cell leukaemia/lymphoma". J. Clin. Pathol. 60 (12): 1373–7. doi:10.1136/jcp.2007.052456. PMC 2095573. PMID 18042693.
  15. ↑ Matutes E (2007) Adult T-cell leukaemia/lymphoma. J Clin Pathol 60 (12):1373-7. DOI:10.1136/jcp.2007.052456 PMID: 18042693
  16. ↑ Shimoyama M (1991). "Diagnostic criteria and classification of clinical subtypes of adult T-cell leukaemia-lymphoma. A report from the Lymphoma Study Group (1984-87)". Br J Haematol. 79 (3): 428–37. PMID 1751370.
  17. ↑ Al-Ahmad, Selma; Maertens, Vincent; Libeer, Christophe; Schelfhout, Vera; Vanhoenacker, Filip; Boeckx, Nancy; Vandevenne, Marleen (2017). "The masquerading presentation of a systemic anaplastic large cell lymphoma, ALK positive: a case report and review of the literature". Acta Clinica Belgica. 72 (6): 454–460. doi:10.1080/17843286.2017.1312057. ISSN 1784-3286.
  18. ↑ Yu L, Yan LL, Yang SJ (2014). "Sarcomatoid variant of ALK- anaplastic large cell lymphoma involving multiple lymph nodes and both lungs with production of proinflammatory cytokines: report of a case and review of literature". Int J Clin Exp Pathol. 7 (8): 4806–16. PMC 4152041. PMID 25197351.
  19. ↑ de Campos FPF, Zerbini MCN, Felipe-Silva A, Simões AB, Lovisolo SM, da Fonseca LG; et al. (2014). "Unusual clinical presentation of anaplastic large cell lymphoma". Autops Case Rep. 4 (1): 21–27. doi:10.4322/acr.2014.004. PMC 5470561. PMID 28652989.
  20. ↑ Watanabe M, Ogawa Y, Itoh K; et al. (January 2008). "Hypomethylation of CD30 CpG islands with aberrant JunB expression drives CD30 induction in Hodgkin lymphoma and anaplastic large cell lymphoma". Lab. Invest. 88 (1): 48–57. doi:10.1038/labinvest.3700696. PMID 17965727.
  21. ↑ Foss, Francine M.; Girardi, Michael (2017). "Mycosis Fungoides and Sezary Syndrome". Hematology/Oncology Clinics of North America. 31 (2): 297–315. doi:10.1016/j.hoc.2016.11.008. ISSN 0889-8588.
  22. ↑ Campbell, J. J.; Clark, R. A.; Watanabe, R.; Kupper, T. S. (2010). "Sezary syndrome and mycosis fungoides arise from distinct T-cell subsets: a biologic rationale for their distinct clinical behaviors". Blood. 116 (5): 767–771. doi:10.1182/blood-2009-11-251926. ISSN 0006-4971.
  23. ↑ Vonderheid, Eric C.; Bernengo, Maria Grazia; Burg, Günter; Duvic, Madeleine; Heald, Peter; Laroche, Liliane; Olsen, Elise; Pittelkow, Mark; Russell-Jones, Robin; Takigawa, Masahiro; Willemze, Rein (2002). "Update on erythrodermic cutaneous T-cell lymphoma: Report of the international society for cutaneous lymphomas". Journal of the American Academy of Dermatology. 46 (1): 95–106. doi:10.1067/mjd.2002.118538. ISSN 0190-9622.
  24. ↑ Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016
  25. ↑ Subcutaneous panniculitis-like T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52df/. Accessed on March 08, 2016
  26. ↑ Parveen Z, Thompson K (2009). "Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas". Arch Pathol Lab Med. 133 (2): 303–8. doi:10.1043/1543-2165-133.2.303. PMID 19195975.
  27. ↑ van de Meeberg MM, Derikx LA, Sinnige HA, Nooijen P, Schipper DL, Nissen LH (2016). "Hepatosplenic T-cell lymphoma in a 47-year-old Crohn's disease patient on thiopurine monotherapy". World J Gastroenterol. 22 (47): 10465–10470. doi:10.3748/wjg.v22.i47.10465. PMC 5175260. PMID 28058028.
  28. ↑ Shi, Yang; Wang, Endi (2015). "Hepatosplenic T-Cell Lymphoma: A Clinicopathologic Review With an Emphasis on Diagnostic Differentiation From Other T-Cell/Natural Killer–Cell Neoplasms". Archives of Pathology & Laboratory Medicine. 139 (9): 1173–1180. doi:10.5858/arpa.2014-0079-RS. ISSN 0003-9985.
  29. ↑ Alsohaibani FI, Abdulla MA, Fagih MM (2011). "Hepatosplenic T-cell lymphoma". Indian J Hematol Blood Transfus. 27 (1): 39–42. doi:10.1007/s12288-010-0051-1. PMC 3102508. PMID 22379294.
  30. ↑ Delabie, J.; Holte, H.; Vose, J. M.; Ullrich, F.; Jaffe, E. S.; Savage, K. J.; Connors, J. M.; Rimsza, L.; Harris, N. L.; Muller-Hermelink, K.; Rudiger, T.; Coiffier, B.; Gascoyne, R. D.; Berger, F.; Tobinai, K.; Au, W. Y.; Liang, R.; Montserrat, E.; Hochberg, E. P.; Pileri, S.; Federico, M.; Nathwani, B.; Armitage, J. O.; Weisenburger, D. D. (2011). "Enteropathy-associated T-cell lymphoma: clinical and histological findings from the International Peripheral T-Cell Lymphoma Project". Blood. 118 (1): 148–155. doi:10.1182/blood-2011-02-335216. ISSN 0006-4971.
  31. ↑ Bautista-Quach MA, Ake CD, Chen M, Wang J (September 2012). "Gastrointestinal lymphomas: Morphology, immunophenotype and molecular features". J Gastrointest Oncol. 3 (3): 209–25. doi:10.3978/j.issn.2078-6891.2012.024. PMC 3418529. PMID 22943012.
  32. ↑ Enteropathy-associated T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd5315/. Accessed on January 26, 2016
  33. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 19, 2016
  34. ↑ Extranodal NK-/T-cell lymphoma, nasal type. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd530f/. Accessed on February 02, 2016
  35. ↑ Extranodal Natural-Killer/T-Cell Lymphoma, Nasal Type. Hindawi Publishing Corporation. http://www.hindawi.com/journals/ah/2010/627401/. Accessed on February 18, 2016
  36. ↑ Swerdlow, S.H.; Campo, E.; Harris, N.L.; Jaffe, E.S.; Pileri, S.A.; Stein, H.; Thiele, J.; Vardiman, J.W (2008). "11 Mature T- and NK-cell neoplasms: Angioimmunoblastic T-cell lymphoma". WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. IARC WHO Classification of Tumours. 2 (4th ed.). IARC. ISBN 9283224310.
  37. ↑ [1] Quintanilla-Martinez L, Fend F, Moguel LR, Spilove L, Beaty MW, Kingma DW, Raffeld M, Jaffe ES. "Peripheral T-cell lymphoma with Reed-Sternberg-like cells of B-cell phenotype and genotype associated with Epstein-Barr virus infection." Am J Surg Pathol. 1999 Oct;23(10):1233-40. PMID: 10524524
  38. ↑ Angioimmunoblastic T-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf56e3e27c3994bd52dc/ Accessed on November 27, 2015
  39. ↑ Lemonnier F, Couronné L, Parrens M, Jaïs JP, Travert M, Lamant L, Tournillac O, Rousset T, Fabiani B, Cairns RA, Mak T, Bastard C, Bernard OA, de Leval L, Gaulard P (August 2012). "Recurrent TET2 mutations in peripheral T-cell lymphomas correlate with TFH-like features and adverse clinical parameters". Blood. 120 (7): 1466–9. doi:10.1182/blood-2012-02-408542. PMID 22760778.
  40. ↑ Jha, KunalKishor; Gupta, SureshK; Saluja, Harpreet; Subedi, Nuwadatta (2017). "Peripheral T-cell lymphoma, not otherwise specified". Journal of Family Medicine and Primary Care. 6 (2): 427. doi:10.4103/jfmpc.jfmpc_323_16. ISSN 2249-4863.
  41. ↑ 41.0 41.1 41.2 Falchook GS, Vega F, Dang NH, Samaniego F, Rodriguez MA, Champlin RE; et al. (2009). "Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment". Ann Oncol. 20 (6): 1080–5. doi:10.1093/annonc/mdn751. PMC 4092251. PMID 19237479.
  42. ↑ Yabe M, Medeiros LJ, Tang G, Wang SA, Ahmed S, Nieto Y; et al. (2016). "Prognostic Factors of Hepatosplenic T-cell Lymphoma: Clinicopathologic Study of 28 Cases". Am J Surg Pathol. 40 (5): 676–88. doi:10.1097/PAS.0000000000000614. PMID 26872013.
  43. ↑ International Non-Hodgkin's Lymphoma Prognostic Factors Project (1993). "A predictive model for aggressive non-Hodgkin's lymphoma". N Engl J Med. 329 (14): 987–94. doi:10.1056/NEJM199309303291402. PMID 8141877.
  44. ↑ Saito T, Matsuno Y, Tanosaki R, Watanabe T, Kobayashi Y, Tobinai K (2002). "Gamma delta T-cell neoplasms: a clinicopathological study of 11 cases". Ann Oncol. 13 (11): 1792–8. PMID 12419753.
  45. ↑ Boulland ML, Kanavaros P, Wechsler J, Casiraghi O, Gaulard P (1997). "Cytotoxic protein expression in natural killer cell lymphomas and in alpha beta and gamma delta peripheral T-cell lymphomas". J Pathol. 183 (4): 432–9. doi:10.1002/(SICI)1096-9896(199712)183:4<432::AID-PATH942>3.0.CO;2-4. PMID 9496260.
  46. ↑ Jonveaux P, Daniel MT, Martel V, Maarek O, Berger R (1996). "Isochromosome 7q and trisomy 8 are consistent primary, non-random chromosomal abnormalities associated with hepatosplenic T gamma/delta lymphoma". Leukemia. 10 (9): 1453–5. PMID 8751461.
  47. ↑ Simon A, Peoch M, Casassus P, Deconinck E, Colombat P, Desablens B; et al. (2010). "Upfront VIP-reinforced-ABVD (VIP-rABVD) is not superior to CHOP/21 in newly diagnosed peripheral T cell lymphoma. Results of the randomized phase III trial GOELAMS-LTP95". Br J Haematol. 151 (2): 159–66. doi:10.1111/j.1365-2141.2010.08329.x. PMID 20738307.
  48. ↑ Han X, Zhang W, Zhou D, Ruan J, Duan M, Zhu T; et al. (2017). "Autologous stem cell transplantation as frontline strategy for peripheral T-cell lymphoma: A single-centre experience". J Int Med Res. 45 (1): 290–302. doi:10.1177/0300060516676725. PMC 5536587. PMID 28222648.


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