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{{CMG}}; {{AE}} {{S.M.}}
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{{SK}} Waldenstrom's macroglobulinemia; Waldenstrom's disease; Primary macroglobulinemia; Hyperviscosity syndrome; Lymphoplasmacytoid lymphoma
{{SK}} Plasmacytoid lymphocytic [[lymphoma]]; Familial Waldenström's macroglobulinemia; Primary macroglobulinemia; Hyperviscosity syndrome; Lymphoplasmacytoid [[lymphoma]]


==Overview==
==[[Lymphoplasmacytic lymphoma overview|Overview]]==
Lymphoplasmacytic lymphoma (LPL, previously termed lymphoplasmacytoid lymphoma) is an uncommon mature B cell lymphoma usually involving the bone marrow and, less commonly, the spleen and/or lymph nodes.<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> Waldenström macroglobulinemia is a clinicopathologic entity associated with an IgM monoclonal gammopathy in the blood that is virtually always a manifestation of LPL.[[Waldenström macroglobulinemia|Waldenström macroglobulinemia (WM)]] is a rare [[Lymphoproliferative disorders|lymphoproliferative]] disorder characterized by the presence of a serum, [[Immunoglobulin M|IgM]] [[paraprotein]], associated with infiltration of the bone marrow by [[Lymphoplasmacytic lymphoma|lymphoplasmacytic]] lymphoma. Waldenström macroglobulinemia is a type of [[lymphoproliferative disease]] involving [[lymphocytes]] with IgM as the main attributing antibody and shares clinical characteristics with the indolent [[non-Hodgkin lymphoma]]s.  Waldenström's macroglobulinemia was first discovered by Jan G. Waldenström and represents 1% of all hematological cancers. Common causes of this disease include genetic, environmental, and [[Autoimmune|autoimmune factors]]. While common risk factors include [[monoclonal gammopathy of undetermined significance]], age >50 year old, white ethnicity, heredity, [[hepatitis C]], and immune disorders. Genes involved in the pathogenesis of Waldenström macroglobulinemia include: [[MYD88]]-L265P, [[CXCR4]] and chromosomes 6q, [[13q deletion syndrome|13q]], 3q, 6p and [[18q syndrome|18q]]. The hallmark of Waldenström's macroglobulinemia is [[hyperviscosity syndrome|hyper-viscosity syndrome]]. If left untreated, patients with asymptomatic Waldenström's macroglobulinemia may progress to develop a [[symptomatic]] disease. Common complications of Waldenström's macroglobulinemia include: [[hyperviscosity syndrome]], cold [[Hemagglutinin|haemagglutinin]] disease, [[cryoglobulinemia]], [[peripheral neuropathy]], [[venous thromboembolism]], [[primary amyloidosis]], mal-absorptive diarrhea, and bleeding manifestations. Prognosis varies depending on the multiple factors involved. Five year survival rate is 87% for low-risk disease and 36% for high-risk disease. Signs and symptoms of patients with Waldenström's macroglobulinemias depend on the degree of tissue infiltration by malignant tumor cells, hyper-viscosity syndrome, and accumulation of paraprotein. The diagnosis of Waldenström macroglobulinemia is based on [[bone marrow biopsy]] and serum [[protein]] analysis. [[Risk stratification tools|Risk stratification]] determines the protocol of management used for Waldenström macroglobulinemia patients. Watchful waiting is recommended for asymptomatic Waldenström's macroglobulinemia. Symptomatic Waldenström's macroglobulinemia is treated with [[Rituximab]] +/- [[Chemotherapy]].
 
==[[Lymphoplasmacytic lymphoma historical perspective|Historical Perspective]]==
 
==[[Lymphoplasmacytic lymphoma classification|Classification]]==
 
==[[Lymphoplasmacytic lymphoma pathophysiology|Pathophysiology]]==
 
==[[Lymphoplasmacytic lymphoma causes|Causes]]==
 
==[[Lymphoplasmacytic lymphoma differential diagnosis|Differentiating Lymphoplasmacytic lymphoma from other Diseases]]==
 
==[[Lymphoplasmacytic lymphoma epidemiology and demographics|Epidemiology and Demographics]]==
 
==[[Lymphoplasmacytic lymphoma risk factors|Risk Factors]]==
 
==[[Lymphoplasmacytic lymphoma screening|Screening]]==
 
==[[Lymphoplasmacytic lymphoma natural history, complications and prognosis|Natural History, Complications and Prognosis]]==


==Diagnosis==
==Diagnosis==
*Not all the diagnostic tests mentioned are performed in a WM patient. A doctor takes into account the following factors before choosing diagnostic tests in a particular patient:
 
**Suspected type of cancer.
[[Lymphoplasmacytic lymphoma diagnostic study of choice|Diagnostic study of choice]] | [[Lymphoplasmacytic lymphoma history and symptoms|History and Symptoms]] | [[Lymphoplasmacytic lymphoma physical examination|Physical Examination]] | [[Lymphoplasmacytic lymphoma laboratory findings|Laboratory Findings]] | [[Lymphoplasmacytic lymphoma electrocardiogram|Electrocardiogram]] | [[Lymphoplasmacytic lymphoma x ray|X-Ray Findings]] | [[Lymphoplasmacytic lymphoma echocardiography and ultrasound|Echocardiography and Ultrasound]] | [[Lymphoplasmacytic lymphoma CT scan|CT-Scan Findings]] | [[Lymphoplasmacytic lymphoma MRI|MRI Findings]] | [[Lymphoplasmacytic lymphoma other imaging findings|Other Imaging Findings]] | [[Lymphoplasmacytic lymphoma other diagnostic studies|Other Diagnostic Studies]]
**Signs and symptoms.
 
**Age.
==Treatment==
**Medical condition of the patient.
[[Lymphoplasmacytic lymphoma medical therapy|Medical Therapy]] | [[Lymphoplasmacytic lymphoma surgery|Surgery]] | [[Lymphoplasmacytic lymphoma primary prevention|Primary Prevention]] | [[Lymphoplasmacytic lymphoma secondary prevention|Secondary Prevention]] | [[Lymphoplasmacytic lymphoma cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Lymphoplasmacytic lymphoma future or investigational therapies|Future or Investigational Therapies]]
**Results of earlier medical tests.
 
==References==
==Case Studies==
{{reflist|2}}
[[Lymphoplasmacytic lymphoma case study one|Case #1]]
 
[[Category: (name of the system)]]

Latest revision as of 20:45, 19 August 2020

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

Synonyms and keywords: Plasmacytoid lymphocytic lymphoma; Familial Waldenström's macroglobulinemia; Primary macroglobulinemia; Hyperviscosity syndrome; Lymphoplasmacytoid lymphoma

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 Findings | Echocardiography and Ultrasound | CT-Scan Findings | MRI Findings | 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

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