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Hepatosplenic T-cell lymphoma

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Hepatosplenic T-cell lymphoma

Hepatosplenic T-cell lymphoma
Classification and external resources
ICD-O: 9716/3
DiseasesDB provide an accessible overview of all important aspects of the article. (January 2011)

Hepatosplenic T-cell lymphoma is a rare and generally incurable form of lymphoma.[1]

Synonyms

Hepatosplenic γδ T-cell lymphoma[2]

ICD-O Code

9716/3[2]

Definition

Hepatosplenic T-cell lymphoma is a systemic neoplasm comprising medium-sized cytotoxic T-cells that show a significant sinusoidal infiltration in the liver, spleen, and bone marrow.[2]

Epidemiology

This lymphoma is rare, comprising less than 5% of all cases, and is most common in young adults and adolescents. A distinct male gender preference has been described.[3]

Clinical features

Etiology

The cell of origin for this disease is an immature cytotoxic T-cell clonally expressing the γδ T-cell receptor. This disease is seen more often in immunosuppressed solid organ transplant recipients, an association that has led to the hypothesis that long-term immune stimulation in the setting of immunosuppression is the causative agent.

Cases of hepatosplenic T-cell lymphoma (HSTCL) have been reported in patients treated with the immunosuppressant azathioprine. The majority occurred in patients with Inflammatory Bowel Disease. Adolescents and young adult males were the major cohort of cases. They presented with a very aggressive disease course and, with one exception, died of the lymphoma. The FDA has required changes to the labeling to inform users and clinicians of the issue.[4]

[5][6]

Clinical Presentation

The typical clinical finding in a patient with hepatosplenic T-cell lymphoma is hepatosplenomegaly.[7]

Laboratory Findings

The constellation of thrombocytopenia, anemia, and leukocytosis is common in patients with hepatosplenic T-cell lymphoma.[3] The discovery of neoplastic cells in peripheral blood is often a finding late in the clinical course.[2]

Sites of Involvement

The spleen and liver are always involved, with bone marrow involvement frequently present. Nodal involvement is exceedingly rare.[2][3]

Morphology

The neoplastic cells in this disorder show a monotonous appearance, with a small amount of cytoplasm and inconspicuous nucleoli.[3]

Spleen and liver

This disease shows a distinct sinusoidal pattern of infiltration which spares the splenic white pulp and hepatic portal triads.[2]

Bone marrow

While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern. Immunohistochemistry can aid in the detection of this lymphoma.[2]

Peripheral blood

Cells of a similar morphology observed in solid organs are observed in peripheral blood.[2]

Molecular Findings

Immunophenotype

The immunophenotype for hepatosplenic T-cell lymphoma is a post-thymic, immature T-cell.[2][3]

Status Antigens
Positive CD3, TCRδ1, TIA-1
Negative CD4, CD5, CD8

Genetic findings

Clonal rearrangement of the γ gene of the T-cell receptor is the hallmark of this disease. A few cases have shown rearrangement of the T-cell receptor β gene.[2] Isochromosome 7q has been observed in all cases described so far, sometimes in conjunction with other chromosomal abnormalities such as trisomy 8.[8]

Treatment

CHOP frequently induces remission initially, but most patients relapse and die within 2 years. Autologus bone marrow transplantion is currently being investigated in the treatment of hepatosplenic lymphoma. Allogeneic bone marrow transplant has been proven to attain remission for over five years and possibly cure hepatosplenic lymphoma.

See also

  • List of hematologic conditions

References

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