Hepatosplenic T-cell lymphoma
Hepatosplenic T-cell lymphoma | |
---|---|
Other names | hepatosplenic γδ T-cell lymphoma[1] |
Specialty | Hematology and oncology |
Hepatosplenic T-cell lymphoma is a rare form of
It is a systemicSigns and symptoms
The typical clinical finding in a patient with hepatosplenic T-cell lymphoma is hepatosplenomegaly.[4]
The spleen and liver are always involved, and bone marrow involvement is common. Nodal involvement is exceedingly rare.[1][5]
Cause
The cell of origin for this disease is an immature cytotoxic
Cases of hepatosplenic T-cell lymphoma have been reported in patients treated with the immunosuppressants azathioprine, infliximab, and adalimumab. The majority of cases occurred in patients with inflammatory bowel disease. Adolescents and young adult males were most frequently affected. They presented with a very aggressive disease course, and all but one died. The Food and Drug Administration required changes to the drugs' labeling to inform users and clinicians of the risk.[6][7][8]
Diagnosis
The neoplastic cells in hepatosplenic T-cell lymphoma show a monotonous appearance, with a small amount of
Laboratory findings
The constellation of thrombocytopenia, anemia, and leukopenia is common in patients with hepatosplenic T-cell lymphoma.[9]
Spleen and liver
The disease shows a distinct
Bone marrow
While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to a diffuse, interstitial pattern. Immunohistochemistry can aid in diagnosis.[1]
Peripheral blood
Cells of a similar morphology observed in solid organs are observed in peripheral blood.[1]
Immunophenotype
The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.[1][5]
Status | Antigens |
Positive | TIA-1
|
Negative | CD4, CD5, CD8 |
Genetic findings
Clonal rearrangement of the γ gene of the T-cell receptor is the hallmark of hepatosplenic T-cell lymphoma. A few cases have shown rearrangement of the T-cell receptor β gene.
Treatment
The CHOP chemotherapy regimen frequently induces remission but has proven weak compared to treatments that integrate cytarabine, with Hyper-CVAD being particularly effective. When treated solely with chemotherapy, most patients relapse and die within two years. Treatment solely with doxorubicin can make the disease worse.[citation needed]
Epidemiology
Hepatosplenic lymphoma is rare, comprising less than 5% of all lymphoma cases, and is most common in young adults and adolescents. A distinct male gender preference has been described.[5]
See also
References
- ^ ISBN 92-832-2411-6.)
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: CS1 maint: multiple names: authors list (link - ^ PMID 26047388.
- PMID 17255842.
- PMID 2140703.
- ^ PMID 8943863.
- ^ FDA Warning concerning azathioprine and Hepatosplenic T-cell lymphoma https://www.fda.gov/Safety/MedWatch/SafetyInformation/ucm258794.htm
- PMID 8085554.
- PMID 11224598.
- PMID 22379294.
- PMID 9264394.