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{{SK}} Waldenstrom/Waldenstrom's macroglubulinemia (WM); Plasmacytoid lymphocytic lymphoma; Familial Waldenstrom's Macroglobulinaemia; Waldenström macroglobulinaemia; 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.The term "macroglobulinemia" refers to the production of excess IgM monoclonal protein that occurs in certain clonal lymphoproliferative disorders and plasma cell dyscrasias. This broad definition includes patients with monoclonal gammopathy of undetermined significance of the IgM type (IgM MGUS), smoldering Waldenström macroglobulinemia, Waldenström macroglobulinemia (WM), and a number of related disorders in which an IgM monoclonal protein is detected, such as chronic lymphocytic leukemia (CLL), a number of lymphoma variants, and primary (AL) amyloidosis. According to new 2016 WHO classification, when hyperviscosity occurs in LPL patients. it is termed as Waldenström macroglobulinemia (WM). Hence, now WM is considered as a rare distinct subtype/clinicopathologic entity demonstrating lymphoplasmacytic lymphoma (LPL), with symptoms associated with presence of a serum [[Immunoglobulin M|IgM]] [[paraprotein]] due to infiltration of the hematopoietic tissues and the effects of monoclonal IgM in the blood. 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 Lymphoplasmacytic lymphoma 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. Less common but more severe complications include [[Schnitzler syndrome]], [[Richter syndrome]], and [[Bing-Neel syndrome]]. 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 lymphoplasmacytic lymphoma depend on the degree of tissue infiltration by malignant tumor cells, hyper-viscosity syndrome, and accumulation of paraprotein. The diagnosis of lymphoplasmacytic lymphoma is based on [[bone marrow biopsy]] and serum [[protein]] analysis. [[Risk stratification tools|Risk stratification]] determines the protocol of management used for lymphoplasmacytic lymphoma 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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