Hepatosplenic T-cell lymphoma

Hepatosplenic T-cell lymphoma is a rare and generally incurable form of lymphoma.[1]
Contents
1 Synonyms
2 ICD-O Code
3 Definition
4 Epidemiology
5 Clinical features ...

Diagnosis of hepatosplenic T-cell lymphoma can be complicated. Unlike most forms of NHL, there is not an easily accessible swollen lymph node to biopsy.

Gamma/delta hepatosplenic T-cell lymphoma. This is an aggressive form of peripheral T-cell lymphoma that involves the liver and spleen. It occurs most often in male adolescents and young men.

» FDA Drug Safety Communication: Safety Review update on reports of Hepatosplenic T-Cell Lymphoma in adolescents and young adults receiving tumor necrosis factor (TNF) blockers, azathioprine and/or mercaptopurine ...

[57] Though very rare, hepatosplenic T-cell lymphoma is associated with children and adolescents who have Crohn disease and has been fatal in all cases.[58] Optimal therapy for PTCL is unclear, even for adult patients.

The patient has symptoms like vomiting, weight loss, stomach pain, bowel obstruction, anemia, abdominal mass, low serum albumin and high serum alkaline phosphatase. Another type in this category is hepatosplenic T-cell lymphoma which involves the ...

Lymphomatoid granulomatosis (angiocentric pulmonary B-cell lymphoma).
6.
Angioimmunoblastic T-cell lymphoma.
7.
Peripheral T-cell lymphoma, unspecified.1.
Subcutaneous panniculitis-like T-cell lymphoma.
2.
Hepatosplenic T-cell lymphoma.

[160,161] An unusual type of peripheral T-cell lymphoma occurring mostly in young men, hepatosplenic T-cell lymphoma, appears to be localized to the hepatic and splenic sinusoids, with cell surface expression of the T-cell receptor gamma/delta.

Combination therapy with azathioprine/6-mercaptopurine and biologics increases the risk of a particularly rare type of lymphoma called hepatosplenic T-cell lymphoma. As with all medications, you should discuss the risks and benefits with your doctor.

See also: Lymphoma, T-cell lymphoma, Cancer, Symptom, Fusion