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{{CMG}}; {{AE}} {{S.M.}}
{{CMG}}; {{AE}} {{S.M.}}


{{SK}} 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|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), [[Waldenström macroglobulinemia|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 [[World Health Organization|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 (medical)|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]], malabsorptive [[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|Signs and symptoms]] of patients with lymphoplasmacytic [[lymphoma]] depend on the degree of tissue [[Infiltration (medical)|infiltration]] by [[malignant]] [[Tumor cell|tumor]] cells, [[hyperviscosity 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]]. [[Ibrutinib]] with or without concurrent [[rituximab]], is considered as a [[drug]] of choice for treatment of [[Bing-Neel syndrome]].
 
==[[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 [[patient]] with lymphoplasmacytic [[lymphoma]]. A doctor takes into account the following factors before choosing [[diagnostic]] tests in a particular [[patient]]:
**Suspected type of [[cancer]].
**[[Signs]] and [[symptoms]].
**Age.
**[[Medical condition]] of the [[patient]].
**Results of earlier [[medical]] tests.


==References==
[[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]]
{{reflist|2}}
 
==Treatment==
[[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]]
 
==Case Studies==
[[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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