Hepatosplenic Gamma Delta T-Cell Lymphoma (HSGDTCL): Two Rare Case Reports from Western India

  • Irappa Madabhavi Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat, India
  • Gaurang Modi Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat, India
  • Harsha Panchal Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat, India
  • Apurva Patel Department of Radiotherapy, Government Medical College, Haldwani, Uttarakhand, India
  • Swaroop Revannasiddaiah Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat, India
  • Asha Anand Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat, India
  • Sonia Parikh Department of Medical and Pediatric Oncology, GCRI, Ahmedabad, Gujarat, India
  • Kshitij Joshi Department of Pulmonary Medicine, IGMC, Shimla, Himachal Pradesh, India
  • Malay Sarkar Department of Pulmonary Medicine, IGMC, Shimla, Himachal Pradesh, India
Keywords: Hepatosplenic gamma delta T cell lymphoma (HSGDTCL), Immuno competent patients, CHOP

Abstract

Peripheral T cell lymphomas are a heterogeneous group of post-thymic, mature lymphoid malignancies, accounting for approximately 10-15% of all non-Hodgkin's lymphomas. Hepatosplenic T-cell lymphoma (HSGDTCL) is a rare entity, which is characterized by primary extra nodal disease with typical sinusoidal or sinusal infiltration of the liver and the spleen, respectively by expression of the T cell receptor   chain, and by a number of other frequent clinicopathologic features, including aggressive course of disease. Secondary involvement of liver by hematopoietic malignancies is much more common as compared to primary liver involvement. Primary involvement of liver by non- Hodgkin’s lymphoma (NHL) is documented and mostly DLBCL (diffuse large B cell lymphoma) type.  But, T cell lymphoma primarily arising from liver is very rare. It occurred commonly in immunocompromised patients and prognosis is very poor. Here, we present two case reports of Hepatosplenic gamma-delta T-cell lymphoma (HSGDTCL) and both are immunocompetent patients. Liver biopsy from the mass and subsequent IHC (immunohistochemistry) were performed for the purpose of diagnosis, which were positive for LCA (leukocyte common antigen), CD2 and negative for CD5, CD20 and CD79a. First patient was a 63-year-old female with hepatitis C virus seropositivity presented with liver mass simulating hepatocellular carcinoma.  Second patient was a 60-year- old male, chronic alcoholic patient, presented with liver mass and lytic bony lesion in pelvis. Both patients were managed with conventional CHOP (cyclophosphamide, hydroxydaunorubicin, vincristine, and prednisolone) and showed complete response after 4 cycles of chemotherapy. After completion of 6 cycles of chemotherapy, both patients remained under 6-month surveillance period for any recurrence of the disease.

References

Khan WA, Yu L, Eisenbrey AB , et al. Hepatosplenic gamma/delta T-Cell Lymphoma in Immunocompromised Patients. Am J Clin Pathol. 2001; 116(1):41-50

Falchook GS, Vega F, Dang NH, et al. Hepatosplenic gamma-delta T-cell lymphoma: clinicopathological features and treatment. Ann Oncol. 2009; 20(6):1080–5.

Cooke CB, Krenacs L, Stetler-Stevenson M, et al. Hepatosplenic T-cell lymphoma: a distinct clinicopathologic entity of cytotoxic gamma delta T-cell origin. Blood. 1996; 88 (11): 4265–74.

Salhany KE, Feldman M, Kahn MJ, et al. Hepatosplenic gammadelta T-cell lymphoma: ultrastructural, immunophenotypic, and functional evidence for cytotoxic T lymphocyte differentiation. Hum Pathol. 1997; 28(6): 674-85.

Iannitto E, Tripodo C. How I diagnose and treat splenic lymphomas. Blood. 2011; 117 (9):2585-95.

Ohno T, Komada F, Yamaguchi M, et al. Gamma/delta T-cell lymphoma with hepatosplenomegaly: report of a case. Int J Hematol. 1993 Jun; 57(3):269-76.

Jaffe ES. Classification of natural killer (NK) cell and NK- like T-cell malignancies. Blood. 1996; 87(4):1207-10.

Wang CC, Tien HF, Lin MT, et al. Consistent presence of isochromosome 7q in hepatosplenic T gamma/delta lymphoma : a new cytogenetic-clinicopathologic entity. Genes Chromosomes Cancer. 1995; 12(3):161-4.

Belhadj K, Reyes F, Farcet JP, et al. Hepatosplenic gammadelta T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients. Blood. 2003; 102(13):4261-9

Burg G, Dummer R, Wilhelm M, et al. A subcutaneous delta-positive T-cell lymphoma that produces interferon gamma. N Engl J Med. 1991; 325:1078-1081.

Wilhelm M, Meyer P, Batram C, et al. Gamma/ delta receptor-expressing T-cell clones from a cutaneous T-cell lymphoma suppress haematopoiesis. Ann Hematol. 1992; 65(3):111-5

Weidmann E. Hepatosplenic T-cell lymphoma: a review on 45 cases since the first report describing the disease as a distinct lymphoma entity in 1990. Leukemia. 2000; 14(6):991-7.

Published
2017-10-01
How to Cite
1.
Madabhavi I, Modi G, Panchal H, Patel A, Revannasiddaiah S, Anand A, Parikh S, Joshi K, Sarkar M. Hepatosplenic Gamma Delta T-Cell Lymphoma (HSGDTCL): Two Rare Case Reports from Western India. ijhoscr. 11(4):296-00.
Section
Articles