Follicular lymphoma natural history, complications and prognosis: Difference between revisions

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==Overview==
==Overview==
Prognosis of follicular lymphoma is generally poor. The [[median]] survival is around 10 years.
Follicular lymphoma presents in the age group of 60-65 with systemic symptoms of [[fever]], [[weight loss]], [[anorexia]], [[night sweats]] and other organ dependent symptoms based on the [[Metastasis|metastatic]] spread of the lymphoma. Complications arise from the systemic effects of the lymphoma and metastasis. The prognosis is evaluated from follicular lymphoma international prognostic index which divides patients into 3 groups.
==Prognosis==
 
*Follicular lymphoma is regarded as incurable (although allogenic [[stem cell transplantation]] may be curative, the mortality from the procedure is too high to be a first line option). The [[median]] survival is around 10 years, but the range is wide, from less than one year, to more than 20 years. Some patients may never need treatment. The overall survival rate at 5 years is 72-77%.<ref>{{EMedicine|article|203268|Lymphoma, Follicular}}</ref>
== Natural history ==
*Follicular lymphoma is graded according to the proportion of large cells (centroblasts). Studies suggest this histologic grading predicts clinical outcome, with more large cells behaving more aggressively and having a higher likelihood of transformation to diffuse large cell lymphoma. When any area of diffuse large-B-cell lymphoma is present in a follicular lymphoma the disease should be reported as diffuse large B-cell lymphoma.<ref name="seer">National Cancer Institute. Surveillance, Epidemiology, and End Results Program 2015. http://seer.cancer.gov</ref>.  
* Follicular lymphoma presents in older age group people of age 60-65<ref name="pmid23023713">{{cite journal| author=Kridel R, Sehn LH, Gascoyne RD| title=Pathogenesis of follicular lymphoma. | journal=J Clin Invest | year= 2012 | volume= 122 | issue= 10 | pages= 3424-31 | pmid=23023713 | doi=10.1172/JCI63186 | pmc=3461914 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=23023713  }} </ref><ref name="pmid9166827">{{cite journal| author=| title=A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project. | journal=Blood | year= 1997 | volume= 89 | issue= 11 | pages= 3909-18 | pmid=9166827 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9166827  }} </ref><ref name="pmid7028244">{{cite journal| author=Winberg CD, Nathwani BN, Bearman RM, Rappaport H| title=Follicular (nodular) lymphoma during the first two decades of life: a clinicopathologic study of 12 patients. | journal=Cancer | year= 1981 | volume= 48 | issue= 10 | pages= 2223-35 | pmid=7028244 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7028244  }} </ref><ref name="pmid21375524">{{cite journal| author=Fernández de Larrea C, Martínez-Pozo A, Mercadal S, García A, Gutierrez-García G, Valera A et al.| title=Initial features and outcome of cutaneous and non-cutaneous primary extranodal follicular lymphoma. | journal=Br J Haematol | year= 2011 | volume= 153 | issue= 3 | pages= 334-40 | pmid=21375524 | doi=10.1111/j.1365-2141.2011.08596.x | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21375524  }} </ref>.
*There was no difference in survival outcomes between patients with Grade 3A and 3B follicular lymphoma, whereas patients with FL3 with more than 50% diffuse component have an inferior survival similar to the survival of those with diffuse large cell lymphoma.  
* Patients may present with complaints of:
*FL3B with cytogenetic abnormalities of BCL6 (at 3q27) are thought to be genetically more akin to germinal center type diffuse large B-cell lymphoma than FL1-3A, and is associated with a more aggressive clinical course.
** Unexplained fever
*Patients with FL3B with BCL2 [[translocation]] appear to have a clinical course similar to patients with FL1-3A. Since FL3B is rare, the clinical behavior of FL3 in most studies is based mainly on FL3A cases.
** [[Weight loss]]
** Painless and progressive [[adenopathy]]
** Night sweats
** Nerve lesions
** Constant [[Fatigue (physical)|fatigue]]
** [[Pruritis|Itchy skin]]
** Reddened patches on the skin
** [[Cough]]
** [[Shortness of breath]]
* Organ-related symptoms develop depending on the metastatic spread of the lymphoma.
* Patients may have a history of:
** Viral disease ([[Human Immunodeficiency Virus (HIV)|HIV]], [[Hepatitis B virus|HBV]], [[Hepatitis C|HCV]] or [[Epstein Barr virus|EBV]])
** [[Autoimmune diseases]]
** [[Immunodeficiency|Immunocompromised]] state


1. The Follicular Lymphoma International Prognostic Index (FLIPI) includes<ref>{{cite web | title = Non-Hodgkin's Lymphomas (NCCN.org) | url = http://www.nccn.org/professionals/physician_gls/PDF/nhl.pdf }}</ref>
== Complications ==
:*Age
* Skin reactions
:*Ann Arbor stage
* [[Bone marrow]] suppression
:*Number of nodal sites involved
* Secondary cancers
:*Hemoglobin levels
* Gastric obstruction
:*Serum [[LDH]] levels
* Urethral obstruction
* [[Renal insufficiency|Renal failure]]
* [[Infertility]]
* Heart disease (Heart failure, valvular defects, pericarditis)
* Lung disease (Pleural effusion, lung mass)
* Mediastinal mass
* Immune system deficiency


2. The Follicular Lymphoma International Prognostic Index (FLIPI-2) includes
==Prognosis==
:*Age
* Follicular Lymphoma International Prognostic Index (FLIPI) is used for the evaluation of prognosis.<ref name="pmid15126323">{{cite journal| author=Solal-Céligny P, Roy P, Colombat P, White J, Armitage JO, Arranz-Saez R et al.| title=Follicular lymphoma international prognostic index. | journal=Blood | year= 2004 | volume= 104 | issue= 5 | pages= 1258-65 | pmid=15126323 | doi=10.1182/blood-2003-12-4434 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15126323  }} </ref><ref name="pmid7780151">{{cite journal| author=Martin AR, Weisenburger DD, Chan WC, Ruby EI, Anderson JR, Vose JM et al.| title=Prognostic value of cellular proliferation and histologic grade in follicular lymphoma. | journal=Blood | year= 1995 | volume= 85 | issue= 12 | pages= 3671-8 | pmid=7780151 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7780151  }} </ref>
:*Hemoglobin levels
:*Longest diameter of largest involved lymph node
:*[[Beta-2 microglobulin]] levels
:*Bone marrow involvement


Both the FLIPI-1 and FLIPI-2 predict for prognosis, but not yet useful for selecting treatment options.
* It includes the following:


3. Most recently,a simpler prognostic index includes
** Age >60 years
:*baseline serum beta 2-microglobulin  
** Bone marrow involvement
:*[[LDH]] levels 
** Hemoglobin level <12.0 g/dl
** Greatest diameter of the largest involved node >6 cm
** Elevated serum β-2 microglobulin level
* The FLIPI divides patients into 3 groups:
** Low (0-1 risk factor)
** Intermediate (2 risk factors)
** High (≥ 3 risk factors)
* The 10-year mortality of low group is 96%.
* The 10-year mortality of intermediate group is 71%.
* The 10-year mortality of high group is 37%.


==References==
==References==

Latest revision as of 16:34, 22 January 2019

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

Overview

Follicular lymphoma presents in the age group of 60-65 with systemic symptoms of fever, weight loss, anorexia, night sweats and other organ dependent symptoms based on the metastatic spread of the lymphoma. Complications arise from the systemic effects of the lymphoma and metastasis. The prognosis is evaluated from follicular lymphoma international prognostic index which divides patients into 3 groups.

Natural history

Complications

  • Skin reactions
  • Bone marrow suppression
  • Secondary cancers
  • Gastric obstruction
  • Urethral obstruction
  • Renal failure
  • Infertility
  • Heart disease (Heart failure, valvular defects, pericarditis)
  • Lung disease (Pleural effusion, lung mass)
  • Mediastinal mass
  • Immune system deficiency

Prognosis

  • Follicular Lymphoma International Prognostic Index (FLIPI) is used for the evaluation of prognosis.[5][6]
  • It includes the following:
    • Age >60 years
    • Bone marrow involvement
    • Hemoglobin level <12.0 g/dl
    • Greatest diameter of the largest involved node >6 cm
    • Elevated serum β-2 microglobulin level
  • The FLIPI divides patients into 3 groups:
    • Low (0-1 risk factor)
    • Intermediate (2 risk factors)
    • High (≥ 3 risk factors)
  • The 10-year mortality of low group is 96%.
  • The 10-year mortality of intermediate group is 71%.
  • The 10-year mortality of high group is 37%.

References

  1. ↑ Kridel R, Sehn LH, Gascoyne RD (2012). "Pathogenesis of follicular lymphoma". J Clin Invest. 122 (10): 3424–31. doi:10.1172/JCI63186. PMC 3461914. PMID 23023713.
  2. ↑ "A clinical evaluation of the International Lymphoma Study Group classification of non-Hodgkin's lymphoma. The Non-Hodgkin's Lymphoma Classification Project". Blood. 89 (11): 3909–18. 1997. PMID 9166827.
  3. ↑ Winberg CD, Nathwani BN, Bearman RM, Rappaport H (1981). "Follicular (nodular) lymphoma during the first two decades of life: a clinicopathologic study of 12 patients". Cancer. 48 (10): 2223–35. PMID 7028244.
  4. ↑ Fernández de Larrea C, Martínez-Pozo A, Mercadal S, García A, Gutierrez-García G, Valera A; et al. (2011). "Initial features and outcome of cutaneous and non-cutaneous primary extranodal follicular lymphoma". Br J Haematol. 153 (3): 334–40. doi:10.1111/j.1365-2141.2011.08596.x. PMID 21375524.
  5. ↑ Solal-Céligny P, Roy P, Colombat P, White J, Armitage JO, Arranz-Saez R; et al. (2004). "Follicular lymphoma international prognostic index". Blood. 104 (5): 1258–65. doi:10.1182/blood-2003-12-4434. PMID 15126323.
  6. ↑ Martin AR, Weisenburger DD, Chan WC, Ruby EI, Anderson JR, Vose JM; et al. (1995). "Prognostic value of cellular proliferation and histologic grade in follicular lymphoma". Blood. 85 (12): 3671–8. PMID 7780151.
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