Intravascular large B-cell lymphoma
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:
Overview
Classification
Intravascular large B-cell lymphoma may be classified into either
| Name | Description |
|---|---|
Pathophysiology
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:
- Hepatosplenic T-cell lymphoma
- B-cell chronic lymphocytic leukemia
- Mantle cell lymphoma
- Splenic marginal zone lymphoma
Epidemiology and Demographics
Age
The incidence of intravascular large B-cell lymphoma increases with age; the median age at diagnosis is 67 years.[1]
Gender
Intravascular large B-cell lymphoma affects men and women equally.[1]
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.[2]
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).[1]
- Hemophagocytic syndrome causes:
- Fever
- Enlarged liver and spleen (hepatosplenomegaly)
- A lower number of red blood cells, white blood cells and platelets in the blood (pancytopenia)
Diagnosis
Symptoms
Symptoms of the intravascular large B-cell lymphoma include:[1]
- Fever
- Weight loss
- Night sweats
- Painless swellings in the neck, axilla, groin, thorax, and abdomen
- Pain in the chest, abdomen, or bones
Physical Examination
Vitals
- Fever is often present
Skin
- Skin rash
HEENT
Thorax
- Thoracic masses suggestive of central lymphadenopathy
Abdomen
- Abdominal masses suggestive of central lymphadenopathy
- Hepatosplenomegaly
Extremities
Laboratory Findings
Laboratory tests for intravascular large B-cell lymphoma include:[1]
- Complete blood count (CBC): pancytopenia
- Blood chemistry studies:
- Cytogenetic analysis
- Flow cytometry
- Immunohistochemistry
- Immunophenotyping
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
References
Related Chapters