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===Pathogenesis===
__NOTOC__
Waldenström Macroglobulinemia is uncontrolled clonal proliferation of terminally differentiated B lymphocytes, which are normally involved in humoral immunity.<ref name="HP">Waldenström's macroglobulinemia. Wikipedia (2015)https://en.wikipedia.org/wiki/Waldenström%27s_macroglobulinemia#Pathophysiology Accessed on November 6, 2015</ref>
{{Waldenström's macroglobulinemia}}
In Waldenström Macroglobulinemia, peripheral B lymphocyte are stimulated to undergo somatic hypermutation of the immunoglobulin heavy chain gene in the germinal center, without class switching.
{{CMG}}{{AE}} {{MGS}}
==Overview==
==Electrophoresis and Immunofixation==
===Serum protein electrophoresis===
*Screening test for monoclonal protein<ref name="LR">Waldenström's macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=machineLearning&search=Waldenström%27s+macroglobulinemia&selectedTitle=1%7E80&sectionRank=3&anchor=H29#H18 Accessed on November 10, 2015</ref>
*The key diagnostic criterion for Waldenström's macroglobulinemia is the detection of monoclonal IgM protein.
*Sharp, narrow spike or dense band of monoclonal IgM


===Genetics===
===Serum immunofixation===
*Development of Waldenström Macroglobulinemia is the result of multiple gene mutations.<ref name="HP">Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=see_link Accessed on November 9, 2015</ref>
*Confirmatory test for presence of monoclonal proteins and to determine it's subtype.
*Genes involved in pathogenesis of Waldenström Macroglobulinemia are:
*The quantitative IgM levels observed should be 2 or 3g/dL more than that found in the serum protein electrophoretic spike.
:*MYD88 L265P in chromosome 3p22.2  
:*CXCR4
::*MYD88: has a role in toll-like receptor and interleukin-1 receptor signaling causing activation of transcription factors of the NF-kB family. Thus, activating point mutation of MYD88 augments growth and survival of both normal and neoplastic B cells by preventing apoptosis. MYD88 also has role in BTK signaling which also helps in B cell growth and survival. Point mutation of MYD88 leads to leucine (L) to proline (P) substitution in codon 265 (L265P) of MYD88 and produces constantly overactive protein causing proliferation of malignant cells that should normally undergo apoptosis.<ref name="HP">Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=see_link Accessed on November 9, 2015</ref><ref name="Genetics">Waldenström macroglobulinemia. Genetics Home Reference (2015)http://ghr.nlm.nih.gov/condition/waldenstrom-macroglobulinemia Accessed on November 9, 2015</ref>
::* Patients with Waldenström Macroglobulinemia with co-existing mutation of MYD88 & CXCR4 are more likely to have hyperviscosity syndrome and bone marrow involvement.


:*40-60 percent of patients with Waldenström Macroglobulinemia are associated with deletions of 6q21.<ref name="HP">Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=see_link Accessed on November 9, 2015</ref>
===Associated Conditions===
===Pathology===
====Gross pathology====
====Microscopic pathology====
====Immunohistochemistry====
Malignant cells in Waldenström Macroglobulinemia express IgM surface immunoglobulin and lack IgD.<ref name="HP">Epidemiology, pathogenesis, clinical manifestations and diagnosis of Waldenström macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=see_link Accessed on November 9, 2015</ref>
Lymphocytic component: expresses pan B cell antigens
Plasma cell component: expresses CD138
Majority of malignant cells express:
*Adhesion molecules including:
:*L-selectin
:*ICAM-1
:*CD44
:*CD11c
*Lymphocyte function associated antigen 1 (LFA-1)


References:
References:
{{Reflist}}
{{Reflist|2}}

Revision as of 15:57, 10 November 2015

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Mirdula Sharma, MBBS [2]

Overview

Electrophoresis and Immunofixation

Serum protein electrophoresis

  • Screening test for monoclonal protein[1]
  • The key diagnostic criterion for Waldenström's macroglobulinemia is the detection of monoclonal IgM protein.
  • Sharp, narrow spike or dense band of monoclonal IgM

Serum immunofixation

  • Confirmatory test for presence of monoclonal proteins and to determine it's subtype.
  • The quantitative IgM levels observed should be 2 or 3g/dL more than that found in the serum protein electrophoretic spike.


References:

  1. ↑ Waldenström's macroglobulinemia. UpToDate (2015)http://www.uptodate.com/contents/epidemiology-pathogenesis-clinical-manifestations-and-diagnosis-of-waldenstrom-macroglobulinemia?source=machineLearning&search=Waldenström%27s+macroglobulinemia&selectedTitle=1%7E80&sectionRank=3&anchor=H29#H18 Accessed on November 10, 2015
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