Lymphoplasmacytic lymphoma diagnostic study of choice: Difference between revisions

Jump to navigation Jump to search
 
(6 intermediate revisions by 2 users not shown)
Line 3: Line 3:
{{CMG}}; {{AE}}{{S.M.}}
{{CMG}}; {{AE}}{{S.M.}}
== Overview ==
== Overview ==
The [[diagnosis]] of [[lymphoplasmacytic lymphoma]]  is [[Based on Symptoms|based]] on [[bone marrow aspiration]] and [[biopsy]] and [[serum]] [[protein]] [[analysis]] [[Study design|studies]] such as [[immunohistochemistry|immunohistochemistry,]]  [[flow cytometry]] and [[cytogenetics]] to distinguish LPL from other types of [[B-cell]] [[lymphomas]].
The [[diagnosis]] of [[lymphoplasmacytic lymphoma]]  is [[Based on Symptoms|based]] on [[bone marrow aspiration]] and [[biopsy]] and [[serum]] [[protein]] [[analysis]] [[Study design|studies]] such as [[immunohistochemistry|immunohistochemistry,]]  [[flow cytometry]] and [[cytogenetics]] to [[Differentiate|distinguish]] [[Lymphoplasmacytic lymphoma|LPL]] from other types of [[B-cell]] [[lymphomas]]. [[CSF]] [[flow cytometry]], [[protein electrophoresis]] and [[immunofixation]] is [[done]] for the [[diagnosis]] of [[Bing-Neel syndrome]] (a late, but severe, [[rare]] [[Complication (medicine)|complication]]).


== Diagnostic Study of Choice ==
== Diagnostic Study of Choice ==
 
*There is no single [[diagnostic study of choice]] for the [[diagnosis]] of [[lymphoplasmacytic lymphoma]] (LPL), but '''[[bone marrow aspiration]]''' and '''[[biopsy]]''' is considered to be [[Mandatory labelling|mandatory]] for the [[Assessment and Plan|assessment]] of [[patients]] with LPL and further [[Support|supported]] by [[monoclonal]] [[protein]]/[[Immunophenotyping|immunophenotypic studies]] such as [[immunohistochemistry]], [[flow cytometry]] and [[cytogenetics]] to distinguish LPL from other types of [[B-cell]] [[lymphomas]].<ref name="pmid15735132">{{cite journal| author=Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP| title=Diagnosis and management of Waldenstrom's macroglobulinemia. | journal=J Clin Oncol | year= 2005 | volume= 23 | issue= 7 | pages= 1564-77 | pmid=15735132 | doi=10.1200/JCO.2005.03.144 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15735132  }} </ref><ref name="pmid26980727">{{cite journal| author=Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R et al.| title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms. | journal=Blood | year= 2016 | volume= 127 | issue= 20 | pages= 2375-90 | pmid=26980727 | doi=10.1182/blood-2016-01-643569 | pmc=4874220 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26980727  }} </ref>
* There is no single [[diagnostic study of choice]] for the [[diagnosis]] of [[lymphoplasmacytic lymphoma]] (LPL), but '''[[bone marrow aspiration]] and [[biopsy]]''' is considered to be [[Mandatory labelling|mandatory]] for the [[Assessment and Plan|assessment]] of [[patients]] with LPL and further [[Support|supported]] by [[monoclonal]] [[protein]]/[[Immunophenotyping|immunophenotypic studies]] such as [[immunohistochemistry]], [[flow cytometry]] and [[cytogenetics]] to distinguish LPL from other types of [[B-cell]] [[lymphomas]].<ref name="pmid15735132">{{cite journal| author=Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP| title=Diagnosis and management of Waldenstrom's macroglobulinemia. | journal=J Clin Oncol | year= 2005 | volume= 23 | issue= 7 | pages= 1564-77 | pmid=15735132 | doi=10.1200/JCO.2005.03.144 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15735132  }} </ref><ref name="pmid26980727">{{cite journal| author=Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R et al.| title=The 2016 revision of the World Health Organization classification of lymphoid neoplasms. | journal=Blood | year= 2016 | volume= 127 | issue= 20 | pages= 2375-90 | pmid=26980727 | doi=10.1182/blood-2016-01-643569 | pmc=4874220 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26980727  }} </ref>
*Not all the [[diagnostic]] [[Test|tests]] mentioned are [[Performance status|performed]] in a [[patient]] with [[lymphoplasmacytic lymphoma]]. A [[Doctor of Medicine|doctor]] takes into account the following factors before choosing [[diagnostic]] [[Test|tests]] in a particular [[patient]]:
 
* Not all the [[diagnostic]] [[Test|tests]] mentioned are [[Performance status|performed]] in a [[patient]] with [[lymphoplasmacytic lymphoma]]. A [[Doctor of Medicine|doctor]] takes into account the following factors before choosing [[diagnostic]] [[Test|tests]] in a particular [[patient]]:
 
** Suspected type of [[cancer]]
** Suspected type of [[cancer]]
** [[Signs and Symptoms|Signs]] and [[symptoms]]
** [[Signs and Symptoms|Signs]] and [[symptoms]]
Line 20: Line 17:
====Diagnostic criteria presented in second International Workshop, Greece, 2002====
====Diagnostic criteria presented in second International Workshop, Greece, 2002====


In September 26-30, 2002, in Athens, Greece,the '''Second International Workshop''' was held in which a '''[[diagnostic criteria]]''' for [[Waldenström's macroglobulinemia|Waldenstrom's Macroglobulinemia]] was [[Proposition|proposed]]. According to this [[criteria]], the following findings on [[Performance status|performing]] [[Bone marrow examination|bone marrow biopsy]] and '''[[Serum protein electrophoresis|serum protein analysis]]''' are [[Confirmatory factor analysis|confirmatory]] of [[Waldenström macroglobulinemia]] and [[Exclusion criteria|exclude]] other small [[B cell]] [[lymphoid]] [[neoplasms]] with plasmacytic [[differentiation]]:<ref name="pmid15735132" />
* In September 26-30, 2002, in Athens, Greece,the Second International Workshop was held in which a [[Diagnosis|diagnostic]] criteria for [[Waldenström's macroglobulinemia|Waldenstrom's Macroglobulinemia]] was [[Proposition|proposed]]. According to this [[criteria]], the following findings on [[Performance status|performing]] [[Bone marrow examination|bone marrow biopsy]] and [[serum]] [[protein]] analysis are [[Confirmatory factor analysis|confirmatory]] of [[Waldenström macroglobulinemia]] and [[Exclusion criteria|exclude]] other small [[B cell]] [[lymphoid]] [[neoplasms]] with plasmacytic [[differentiation]]:<ref name="pmid15735132" /><ref name="pmid12720118">{{cite journal| author=Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML et al.| title=Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia. | journal=Semin Oncol | year= 2003 | volume= 30 | issue= 2 | pages= 110-5 | pmid=12720118 | doi=10.1053/sonc.2003.50082 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12720118  }}</ref>


{| class="wikitable"
{| class="wikitable"
Line 44: Line 41:
|}
|}
====mSMART guidelines for diagnosis of Waldenstrom macroglobulinemia and associated disorders====
====mSMART guidelines for diagnosis of Waldenstrom macroglobulinemia and associated disorders====
[[Mayo Clinic|Mayo]] [[Stratification]] of [[Macroglobulinemia]] and [[RiskMetrics|Risk]]-[[Adapted process|Adapted]] [[Therapy]] (mSMART) [[Medical guideline|Guidelines]] 2016 for [[diagnosis]] of [[Waldenstrom macroglobulinemia]] and [[Association (statistics)|associated]] [[disorders]] are as follows:<ref name="AnsellKyle2010">{{cite journal|last1=Ansell|first1=Stephen M.|last2=Kyle|first2=Robert A.|last3=Reeder|first3=Craig B.|last4=Fonseca|first4=Rafael|last5=Mikhael|first5=Joseph R.|last6=Morice|first6=William G.|last7=Bergsagel|first7=P. Leif|last8=Buadi|first8=Francis K.|last9=Colgan|first9=Joseph P.|last10=Dingli|first10=David|last11=Dispenzieri|first11=Angela|last12=Greipp|first12=Philip R.|last13=Habermann|first13=Thomas M.|last14=Hayman|first14=Suzanne R.|last15=Inwards|first15=David J.|last16=Johnston|first16=Patrick B.|last17=Kumar|first17=Shaji K.|last18=Lacy|first18=Martha Q.|last19=Lust|first19=John A.|last20=Markovic|first20=Svetomir N.|last21=Micallef|first21=Ivana N.M.|last22=Nowakowski|first22=Grzegorz S.|last23=Porrata|first23=Luis F.|last24=Roy|first24=Vivek|last25=Russell|first25=Stephen J.|last26=Short|first26=Kristen E. Detweiler|last27=Stewart|first27=A. Keith|last28=Thompson|first28=Carrie A.|last29=Witzig|first29=Thomas E.|last30=Zeldenrust|first30=Steven R.|last31=Dalton|first31=Robert J.|last32=Rajkumar|first32=S. Vincent|last33=Gertz|first33=Morie A.|title=Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines|journal=Mayo Clinic Proceedings|volume=85|issue=9|year=2010|pages=824–833|issn=00256196|doi=10.4065/mcp.2010.0304}}</ref>
 
* [[Mayo Clinic|Mayo]] [[Stratification]] of [[Macroglobulinemia]] and [[RiskMetrics|Risk]]-[[Adapted process|Adapted]] [[Therapy]] (mSMART) [[Medical guideline|Guidelines]] 2016 for [[diagnosis]] of [[Waldenstrom macroglobulinemia]] and [[Association (statistics)|associated]] [[disorders]] are as follows:<ref name="AnsellKyle2010">{{cite journal|last1=Ansell|first1=Stephen M.|last2=Kyle|first2=Robert A.|last3=Reeder|first3=Craig B.|last4=Fonseca|first4=Rafael|last5=Mikhael|first5=Joseph R.|last6=Morice|first6=William G.|last7=Bergsagel|first7=P. Leif|last8=Buadi|first8=Francis K.|last9=Colgan|first9=Joseph P.|last10=Dingli|first10=David|last11=Dispenzieri|first11=Angela|last12=Greipp|first12=Philip R.|last13=Habermann|first13=Thomas M.|last14=Hayman|first14=Suzanne R.|last15=Inwards|first15=David J.|last16=Johnston|first16=Patrick B.|last17=Kumar|first17=Shaji K.|last18=Lacy|first18=Martha Q.|last19=Lust|first19=John A.|last20=Markovic|first20=Svetomir N.|last21=Micallef|first21=Ivana N.M.|last22=Nowakowski|first22=Grzegorz S.|last23=Porrata|first23=Luis F.|last24=Roy|first24=Vivek|last25=Russell|first25=Stephen J.|last26=Short|first26=Kristen E. Detweiler|last27=Stewart|first27=A. Keith|last28=Thompson|first28=Carrie A.|last29=Witzig|first29=Thomas E.|last30=Zeldenrust|first30=Steven R.|last31=Dalton|first31=Robert J.|last32=Rajkumar|first32=S. Vincent|last33=Gertz|first33=Morie A.|title=Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines|journal=Mayo Clinic Proceedings|volume=85|issue=9|year=2010|pages=824–833|issn=00256196|doi=10.4065/mcp.2010.0304}}</ref>
{| class="wikitable"
{| class="wikitable"
|+
|+
Line 72: Line 70:
* Findings [[Suggestion|suggestive]] of [[lymphoplasmacytic lymphoma]] include:<ref name="pmid18555588">{{cite journal| author=Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J et al.| title=Waldenstrom macroglobulinemia. | journal=Cancer Lett | year= 2008 | volume= 270 | issue= 1 | pages= 95-107 | pmid=18555588 | doi=10.1016/j.canlet.2008.04.040 | pmc=3133633 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18555588  }} </ref>
* Findings [[Suggestion|suggestive]] of [[lymphoplasmacytic lymphoma]] include:<ref name="pmid18555588">{{cite journal| author=Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J et al.| title=Waldenstrom macroglobulinemia. | journal=Cancer Lett | year= 2008 | volume= 270 | issue= 1 | pages= 95-107 | pmid=18555588 | doi=10.1016/j.canlet.2008.04.040 | pmc=3133633 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18555588  }} </ref>
** Hypercellular [[bone marrow]] [[aspirate]]
** Hypercellular [[bone marrow]] [[aspirate]]
** Lymphoplasmacytic [[Infiltration (medical)|infiltrate]] with [[Characteristic function (probability theory)|characteristic]] [[immunophenotype]]
**[[Lymphoplasmacytic lymphoma|Lymphoplasmacytic]] [[Infiltration (medical)|infiltrate]] with [[Characteristic function (probability theory)|characteristic]] [[immunophenotype]]


{|
{|
|
|
[[File:Wm bm aspirate.png|thumb|250px|none| Bone marrow aspirate. Lymphocytes with lymphoplasmacytoid appearance (arrows).[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3894394_br-48-300-g001&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=91 Source: D'Angelo G. et al, Laboratorio di Chimica-Clinica, Ematologia e Microbiologia (Ematologia/Coagulazione), Azienda Ospedaliera "S. Antonio Abate" di Gallarate, Varese, Italy.]]]
[[File:Wm bm aspirate.png|thumb|250px|none| [[Bone marrow]] [[aspirate]]. [[Lymphocytes]] with [[Lymphoplasmacytic lymphoma|lymphoplasmacytic]] appearance (arrows).[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3894394_br-48-300-g001&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=91 Source: D'Angelo G. et al, Laboratorio di Chimica-Clinica, Ematologia e Microbiologia (Ematologia/Coagulazione), Azienda Ospedaliera "S. Antonio Abate" di Gallarate, Varese, Italy.]]]
|}
|}


Line 87: Line 85:
*Three [[Pattern|patterns]] of [[Bone marrow|marrow]] involvement are described, as follows:
*Three [[Pattern|patterns]] of [[Bone marrow|marrow]] involvement are described, as follows:
**Lymphoplasmacytoid [[Cells (biology)|cells]] (lymphoplasmacytic and small [[lymphocytes]]) in a [[nodular]] [[pattern]]
**Lymphoplasmacytoid [[Cells (biology)|cells]] (lymphoplasmacytic and small [[lymphocytes]]) in a [[nodular]] [[pattern]]
**Lymphoplasmacytic [[Cells (biology)|cells]] (small [[lymphocytes]], mature [[plasma cells]], [[mast cells]]) in an [[interstitial]]/[[nodular]] [[pattern]]
**[[Lymphoplasmacytic lymphoma|Lymphoplasmacytic]] [[Cells (biology)|cells]] (small [[lymphocytes]], mature [[plasma cells]], [[mast cells]]) in an [[interstitial]]/[[nodular]] [[pattern]]
**A polymorphous [[Infiltration (medical)|infiltrate]] (small [[lymphocytes]], [[plasma cells]], [[plasmacytoid]] [[Cells (biology)|cells]], [[Immunoblast|immunoblasts]] with [[mitotic]] figures)
**A polymorphous [[Infiltration (medical)|infiltrate]] (small [[lymphocytes]], [[plasma cells]], [[plasmacytoid]] [[Cells (biology)|cells]], [[Immunoblast|immunoblasts]] with [[mitotic]] figures)


Line 100: Line 98:
{|
{|
|
|
[[File:Serum immunofixation electrophoresis.png|thumb|250px|none| Serum immunofixation electrophoresis. (A) There is a slightly dense band with IgM, kappa antisera, suggestive of monoclonal gammopathy (B) After the treatment, a dense band with IgM was disappeared.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3102879_jkms-26-824-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=41 Source: Kim YL. et al, Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea.]]]
[[File:Serum immunofixation electrophoresis.png|thumb|250px|none| Serum [[immunofixation]] electrophoresis. (A) There is a slightly dense band with [[IgM]], kappa [[antisera]], suggestive of [[Monoclonal gammopathy|monoclonal]] gammopathy (B) After the treatment, a dense band with [[IgM]] was disappeared.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3102879_jkms-26-824-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=41 Source: Kim YL. et al, Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea.]]]
|}
|}


Line 109: Line 107:
{|
{|
|
|
[[File:Brain biopsy gif.gif|thumb|250px|none| Stereostactic brain biopsy showing diffuse infiltration of atypical plasmacytoid lymphocytes into the dural fibrous tissue (A) Hematoxylin & eosin (original magnification ×200); (B) Positive immunohistochemical staining for CD20 (original magnification ×40). [https://openi.nlm.nih.gov/detailedresult.php?img=PMC2694623_jkms-22-1079-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=29 Source: Kim HD. et al, Department of Internal Medicine, Yeoungnam University College of Medicine, Daegu, Korea.]]]
[[File:Brain biopsy gif.gif|thumb|250px|none| Stereotactic brain [[biopsy]] showing diffuse infiltration of atypical plasmacytoid [[lymphocytes]] into the dural [[fibrous tissue]] (A) [[Hematoxylin and eosin stain|Hematoxylin]] & eosin (original magnification ×200); (B) Positive [[immunohistochemical]] staining for [[CD20]] (original magnification ×40). [https://openi.nlm.nih.gov/detailedresult.php?img=PMC2694623_jkms-22-1079-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=29 Source: Kim HD. et al, Department of Internal Medicine, Yeoungnam University College of Medicine, Daegu, Korea.]]]
|
|
[[File:Csf plasmacytoid cells.png|thumb|250px|none| Plasmacytoid cells found on cytospin of the cerebrospinal fluid confirming cellular infiltration of the central nervous system.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC4837273_CRIHEM2016-3931709.004&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=71 Source: Halperin D. et al, Whipps Cross Hospital, London E11 1NR, UK.]]]
[[File:Csf plasmacytoid cells.png|thumb|250px|none| Plasmacytoid cells found on cytospin of the cerebrospinal fluid confirming cellular infiltration of the central nervous system.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC4837273_CRIHEM2016-3931709.004&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=71 Source: Halperin D. et al, Whipps Cross Hospital, London E11 1NR, UK.]]]

Latest revision as of 14:44, 29 October 2019

Lymphoplasmacytic lymphoma Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Lymphoplasmacytic Lymphoma from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Lymphoplasmacytic lymphoma diagnostic study of choice On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Lymphoplasmacytic lymphoma diagnostic study of choice

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Lymphoplasmacytic lymphoma diagnostic study of choice

CDC on Lymphoplasmacytic lymphoma diagnostic study of choice

Lymphoplasmacytic lymphoma diagnostic study of choice in the news

Blogs on Lymphoplasmacytic lymphoma diagnostic study of choice

Directions to Hospitals Treating Psoriasis

Risk calculators and risk factors for Lymphoplasmacytic lymphoma diagnostic study of choice

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]

Overview

The diagnosis of lymphoplasmacytic lymphoma is based on bone marrow aspiration and biopsy and serum protein analysis studies such as immunohistochemistry, flow cytometry and cytogenetics to distinguish LPL from other types of B-cell lymphomas. CSF flow cytometry, protein electrophoresis and immunofixation is done for the diagnosis of Bing-Neel syndrome (a late, but severe, rare complication).

Diagnostic Study of Choice

Diagnostic Criteria:

Diagnostic criteria presented in second International Workshop, Greece, 2002

Diagnostic criteria presented in second International Workshop, Greece, 2002
1:Presence of IgM monoclonal gammopathy of any concentration on serum protein analysis Necessary criteria
2:A bone marrow biopsy demonstrating more than 10% infiltration by small lymphocytes, plasmacytoid lymphocytes, and plasma cells, (with variable numbers of admixed immunoblasts), with an intertrabecular pattern consistent with lymphoplasmacytic lymphoma Proliferation centers (pathognomonic of CLL/SLL) and paler-appearing marginal zone type differentiation (seen in marginal zone lymphoma) are absent Necessary criteria
IgM concentration widely varies in WM, and it is not possible to define a concentration that reliably distinguishes WM from other lymphoproliferative disorders. Hence, a diagnosis of WM can be made irrespective of IgM concentration if there is an evidence of bone marrow infiltration by lymphoplasmacytoid lymphoma as defined by the Revised European-American Lymphoma classification and WHO criteria. This is a tumor of small lymphocytes showing evidence of plasmacytoid or plasma cell differentiation.
A recent study found that, in 39% of patients, the bone marrow aspirate contained a spectrum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells; in 39% of patients, there was a predominance of small lymphocytes with fewer plasmacytoid lymphocytes or plasma cells, and 22% of the patients contained a mixture of small lymphocytes and plasma cells, with rare plasmacytoid cells. Mast cells were increased in 26% of patients.
3:Intertrabecular pattern of bone marrow infiltration Supportive criteria
4:Immunophenotype of the lymphoplasmacytic infiltrate consistent with Waldenstrom's macroglobulinemia. This includes: IgM+, CD5-, CD10-, CD11c-, CD19+, CD20+, CD22+, CD23-, CD25+, CD27+, FMC7+, CD103- and CD138+ Supportive criteria

mSMART guidelines for diagnosis of Waldenstrom macroglobulinemia and associated disorders

mSMART guidelines 2016 for diagnosis of Waldenstrom macroglobulinemia and associated disorders
Waldenström macroglobulinemia IgM monoclonal gammopathy (regardless of the size of the M protein) with >10% bone marrow lymphoplasmacytic infiltration (usually intertrabecular) by small lymphocytes that exhibit plasmacytoid or plasma cell differentiation and a typical immunophenotype (surface IgM+, CD5–, CD10–, CD19+, CD20+, CD23–) that satisfactorily excludes other lymphoproliferative disorders, including chronic lymphocytic leukemia and mantle cell lymphoma
IgM MGUS Serum IgM monoclonal protein level <3 g/dL, bone marrow lymphoplasmacytic infiltration <10%, and no evidence of anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly
Smoldering Waldenström macroglobulinemia (indolent /asymptomatic Waldenström macroglobulinemia) Serum IgM monoclonal protein level ≥3 g/dL and/or bone marrow lymphoplasmacytic infiltration ≥10% and no evidence of end-organ damage, such as anemia, constitutional symptoms, hyperviscosity, lymphadenopathy, or hepatosplenomegaly, that can be attributed to a lymphoplasmacytic proliferative disorder

Definitive Diagnostic Tests

Bone Marrow Aspirate:

Bone marrow aspirate. Lymphocytes with lymphoplasmacytic appearance (arrows).[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3894394_br-48-300-g001&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=91 Source: D'Angelo G. et al, Laboratorio di Chimica-Clinica, Ematologia e Microbiologia (Ematologia/Coagulazione), Azienda Ospedaliera "S. Antonio Abate" di Gallarate, Varese, Italy.
]

Bone Marrow Biopsy:

Electrophoresis and Immunofixation

Serum immunofixation electrophoresis. (A) There is a slightly dense band with IgM, kappa antisera, suggestive of monoclonal gammopathy (B) After the treatment, a dense band with IgM was disappeared.[https://openi.nlm.nih.gov/detailedresult.php?img=PMC3102879_jkms-26-824-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=41 Source: Kim YL. et al, Department of Internal Medicine, Eulji University College of Medicine, Seoul, Korea.
]

CSF flow cytometry, protein electrophoresis and immunofixation for diagnosis of Bing-Neel syndrome:

Stereotactic brain biopsy showing diffuse infiltration of atypical plasmacytoid lymphocytes into the dural fibrous tissue (A) Hematoxylin & eosin (original magnification ×200); (B) Positive immunohistochemical staining for CD20 (original magnification ×40). [https://openi.nlm.nih.gov/detailedresult.php?img=PMC2694623_jkms-22-1079-g002&query=waldenstrom+macroglobulinaemia&it=xg&req=4&npos=29 Source: Kim HD. et al, Department of Internal Medicine, Yeoungnam University College of Medicine, Daegu, Korea.
]
Plasmacytoid cells found on cytospin of the cerebrospinal fluid confirming cellular infiltration of the central nervous system.Source: Halperin D. et al, Whipps Cross Hospital, London E11 1NR, UK.

References

  1. 1.0 1.1 Dimopoulos MA, Kyle RA, Anagnostopoulos A, Treon SP (2005). "Diagnosis and management of Waldenstrom's macroglobulinemia". J Clin Oncol. 23 (7): 1564–77. doi:10.1200/JCO.2005.03.144. PMID 15735132.
  2. Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R; et al. (2016). "The 2016 revision of the World Health Organization classification of lymphoid neoplasms". Blood. 127 (20): 2375–90. doi:10.1182/blood-2016-01-643569. PMC 4874220. PMID 26980727.
  3. Owen RG, Treon SP, Al-Katib A, Fonseca R, Greipp PR, McMaster ML; et al. (2003). "Clinicopathological definition of Waldenstrom's macroglobulinemia: consensus panel recommendations from the Second International Workshop on Waldenstrom's Macroglobulinemia". Semin Oncol. 30 (2): 110–5. doi:10.1053/sonc.2003.50082. PMID 12720118.
  4. Ansell, Stephen M.; Kyle, Robert A.; Reeder, Craig B.; Fonseca, Rafael; Mikhael, Joseph R.; Morice, William G.; Bergsagel, P. Leif; Buadi, Francis K.; Colgan, Joseph P.; Dingli, David; Dispenzieri, Angela; Greipp, Philip R.; Habermann, Thomas M.; Hayman, Suzanne R.; Inwards, David J.; Johnston, Patrick B.; Kumar, Shaji K.; Lacy, Martha Q.; Lust, John A.; Markovic, Svetomir N.; Micallef, Ivana N.M.; Nowakowski, Grzegorz S.; Porrata, Luis F.; Roy, Vivek; Russell, Stephen J.; Short, Kristen E. Detweiler; Stewart, A. Keith; Thompson, Carrie A.; Witzig, Thomas E.; Zeldenrust, Steven R.; Dalton, Robert J.; Rajkumar, S. Vincent; Gertz, Morie A. (2010). "Diagnosis and Management of Waldenström Macroglobulinemia: Mayo Stratification of Macroglobulinemia and Risk-Adapted Therapy (mSMART) Guidelines". Mayo Clinic Proceedings. 85 (9): 824–833. doi:10.4065/mcp.2010.0304. ISSN 0025-6196.
  5. 5.0 5.1 5.2 Leleu X, Roccaro AM, Moreau AS, Dupire S, Robu D, Gay J; et al. (2008). "Waldenstrom macroglobulinemia". Cancer Lett. 270 (1): 95–107. doi:10.1016/j.canlet.2008.04.040. PMC 3133633. PMID 18555588.
  6. 6.0 6.1 Riches PG, Sheldon J, Smith AM, Hobbs JR (1991). "Overestimation of monoclonal immunoglobulin by immunochemical methods". Ann Clin Biochem. 28 ( Pt 3): 253–9. doi:10.1177/000456329102800310. PMID 1872571.
  7. O'Neil DS, Francescone MA, Khan K, Bachir A, O'Connor OA, Sawas A (2018). "A Case of Bing-Neel Syndrome Successfully Treated with Ibrutinib". Case Rep Hematol. 2018: 8573105. doi:10.1155/2018/8573105. PMC 6136466. PMID 30228918.
  8. Minnema MC, Kimby E, D'Sa S, Fornecker LM, Poulain S, Snijders TJ; et al. (2017). "Guideline for the diagnosis, treatment and response criteria for Bing-Neel syndrome". Haematologica. 102 (1): 43–51. doi:10.3324/haematol.2016.147728. PMC 5210231. PMID 27758817.
  9. Tallant A, Selig D, Wanko SO, Roswarski J (2018). "First-line ibrutinib for Bing-Neel syndrome". BMJ Case Rep. 2018. doi:10.1136/bcr-2018-226102. PMID 30279255.

Template:WH Template:WS