Hepatosplenic T-cell lymphoma

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Hepatosplenic T-cell lymphoma
Other nameshepatosplenic γδ T-cell lymphoma[1]
SpecialtyHematology and oncology

Hepatosplenic T-cell lymphoma is a rare form of

stem cell transplant.[2][3]
It is a systemic
T-cells that show significant sinusoidal infiltration in the liver, spleen, and bone marrow.[1]

Signs 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

T-cell clonally expressing the γδ T-cell receptor. The disease is seen more often in immunosuppressed recipients of solid organ transplants, an association that has led to the hypothesis that long-term immune stimulation in the setting of immunosuppression is the causative agent.[citation needed
]

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

nucleoli.[5]

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

portal triads.[1]

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.

chromosomal abnormalities such as trisomy 8.[10]

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]

Autologous bone marrow transplantation is currently being investigated.[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

External links