Hepatosplenic T-cell lymphoma

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Hepatosplenic T-cell lymphoma
Classification and external resources
ICD-O: 9716/3
DiseasesDB 34970

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

Synonyms[edit]

Hepatosplenic γδ T-cell lymphoma[2]

ICD-O Code[edit]

9716/3[2]

Definition[edit]

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[edit]

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[edit]

Etiology[edit]

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[edit]

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

Laboratory Findings[edit]

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[edit]

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

Morphology[edit]

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

Spleen and liver[edit]

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

Bone marrow[edit]

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[edit]

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

Molecular Findings[edit]

Immunophenotype[edit]

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[edit]

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[edit]

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[edit]

References[edit]

  1. ^ Mackey AC, Green L, Liang LC, Dinndorf P, Avigan M (February 2007). "Hepatosplenic T cell lymphoma associated with infliximab use in young patients treated for inflammatory bowel disease". J. Pediatr. Gastroenterol. Nutr. 44 (2): 265–7. doi:10.1097/MPG.0b013e31802f6424. PMID 17255842. 
  2. ^ a b c d e f g h i j Elaine Sarkin Jaffe, Nancy Lee Harris, World Health Organization, International Agency for Research on Cancer, Harald Stein, J.W. Vardiman (2001). Pathology and genetics of tumours of haematopoietic and lymphoid tissues. World Health Organization Classification of Tumors 3. Lyon: IARC Press. ISBN 92-832-2411-6. 
  3. ^ a b c d e Cooke CB, Krenacs L, Stetler-Stevenson M, et al. (December 1996). "Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin". Blood 88 (11): 4265–74. PMID 8943863. 
  4. ^ FDA Warning concerning azathioprine and Hepatosplenic T-cell lymphoma http://www.fda.gov/Safety/MedWatch/SafetyInformation/ucm258794.htm
  5. ^ Ross CW, Schnitzer B, Sheldon S, Braun DK, Hanson CA (September 1994). "Gamma/delta T-cell posttransplantation lymphoproliferative disorder primarily in the spleen". Am. J. Clin. Pathol. 102 (3): 310–5. PMID 8085554. 
  6. ^ Macon WR, Levy NB, Kurtin PJ, et al. (March 2001). "Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas". Am. J. Surg. Pathol. 25 (3): 285–96. doi:10.1097/00000478-200103000-00002. PMID 11224598. 
  7. ^ Farcet JP, Gaulard P, Marolleau JP, et al. (June 1990). "Hepatosplenic T-cell lymphoma: sinusal/sinusoidal localization of malignant cells expressing the T-cell receptor gamma delta". Blood 75 (11): 2213–9. PMID 2140703. 
  8. ^ Alonsozana EL, Stamberg J, Kumar D, et al. (August 1997). "Isochromosome 7q: the primary cytogenetic abnormality in hepatosplenic gammadelta T cell lymphoma". Leukemia 11 (8): 1367–72. doi:10.1038/sj.leu.2400742. PMID 9264394.