Intravascular large B-cell lymphoma

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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]

Synonyms and keywords: Intravascular lymphomatosis; Malignant angioendotheliomatosis

Overview

Intravascular large B-cell lymphoma is a very rare subtype of diffuse large B-cell lymphoma (DLBCL). It is also considered a distinct type of non-Hodgkin lymphoma (NHL) in the World Health Organization (WHO) classification system. Intravascular large B-cell lymphoma affects small blood vessels.

Classification

Based on the clinical presentation, intravascular large B-cell lymphoma may be classified into either Western variant or Asian variant.

Intravascular large B-cell lymphoma classification
Name Description
Western variant
  • Western variant characterized by symptoms related to the main organ involved, predominantly neurological or cutaneous
Asian variant
  • Asian variant in which the patients present with multi-organ failure, hepatosplenomegaly, pancytopenia, and hemophagocytic syndrome.
  • This is an aggressive lymphoma which responds poorly to chemotherapy.
  • The poor prognosis reflects in part frequent delays in diagnosis because of a lack of detectable tumor masses.

Pathophysiology

Intravascular large B-cell lymphoma is a rare type of extranodal large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of vessels, particularly capillaries, with exception of larger arteries and veins.

Microscopic Pathology

Causes

There are no established causes for intravascular large B-cell lymphoma.

Differentiating type page name here from other Diseases

Intravascular large B-cell lymphoma must be differentiated from other diseases such as:

Epidemiology and Demographics

Age

The incidence of intravascular large B-cell lymphoma increases with age; the median age at diagnosis is 67 years.[2]

Gender

Intravascular large B-cell lymphoma affects men and women equally.[2]

Risk Factors

There are no established risk factors for intravascular large B-cell lymphoma.

Screening

According to the the U.S. Preventive Service Task Force (USPSTF), there is insufficient evidence to recommend routine screening for intravascular large B-cell lymphoma.[3]

Natural History, Complications and Prognosis

  • People with this type of lymphoma can also develop a very serious condition where certain parts of the immune system are activated (hemophagocytic syndrome).[2]
  • Hemophagocytic syndrome causes:


Diagnosis

Symptoms

Symptoms of the intravascular large B-cell lymphoma include:[2]

Physical Examination

Vitals

Skin

  • Skin rash

HEENT

Thorax

Abdomen

Extremities

Laboratory Findings

Laboratory tests for intravascular large B-cell lymphoma include:[2]

Biopsy

Lymph node biopsy is diagnostic of intravascular large B-cell lymphoma.

Other Imaging Findings

CT, MRI, and PET scan may be helpful in the diagnosis of intravascular large B-cell lymphoma.

Treatment

Medical Therapy

Treatment of intravascular large B-cell lymphoma[4]
Therapy Description
Chemotherapy
  • Combination therapy :
Radiation therapy

References

  1. 1.0 1.1 1.2 1.3 1.4 Intravascular Large B-Cell Lymphoma Presenting as Interstitial Lung Disease. Hindawi Publishing Corporation. http://www.hindawi.com/journals/cripa/2014/928065/. Accessed on March 10, 2016
  2. 2.0 2.1 2.2 2.3 2.4 Intravascular large B-cell lymphoma. Surveillance, Epidemiology, and End Results Program. http://seer.cancer.gov/seertools/hemelymph/51f6cf5ae3e27c3994bd54a7/. Accessed on March 09, 2016
  3. Recommendations. U.S Preventive Services Task Force. Accessed on March 9, 2016
  4. Intravascular large B-cell lymphoma. Canadian Cancer Society. http://www.cancer.ca/en/cancer-information/cancer-type/non-hodgkin-lymphoma/non-hodgkin-lymphoma/types-of-nhl/intravascular-large-b-cell-lymphoma/?region=nb. Accessed on March 9, 2016

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