Hepatosplenic γ/δ T-Cell Lymphoma With Isochromosome 7q, Translocation t(7;21), and Tetrasomy 8 in a 9-Year-Old Girl

Abstract

The authors report a child younger than age 15 years with a rare hepatosplenic gamma/delta T-cell lymphoma, which is highly aggressive and primarily seen in young men. A 9-year-old girl presented with thrombocytopenia and hepatosplenomegaly. Bone marrow analysis revealed a metastatic pleomorphic lymphoma of peripheral T-cell phenotype, with rearrangement of the T-cell receptor gamma/delta and expression of CD3 and CD16/56. Instead of the previously reported primary, nonrandom, chromosomal abnormalities, isochromosome 7q and trisomy 8, this patient had four copies each of chromosome 7q, including isochromosome 7[i(7)(q10)] and der(21)t(7;21), as well as chromosome 8. This entity needs to be considered in women and children with lymphoma. Conventional therapy appears to be inadequate for cure.

Hepatosplenic T-cell lymphoma is a rare clinicopathologic entity with poor outcome: report on a series of 21 patients

“…Therefore, in the context of antigen-driven stimulation of reactive ␥␦ T cells, it is tempting to speculate that neoplastic transformation could result from a multistep process involving impairment of the immune system (as in the patients receiving immunosuppressive therapy) or additional genetic alterations such as isochromosome arm 7q. The latter aberration was present in 9 of 13 documented cases, further confirming the association between HS␥␦TCL and isochromosome arm 7q, which was reported as a hallmark not only of HS␥␦TCL 12,14,25,[28][29][30][31][32] but also of its unusual ␣␤ immunophenotypic variant. 33,35 Although the mechanism by which it might contribute to the pathogenesis of HSTCL is unknown, its previously reported accumulation in forms with features of cytologic progression suggests that it benefits the outgrowth of malignant clones.…”

“…The identification of this lymphoma subtype, recognized as a provisional entity in the Revised European American Lymphoma (REAL) classification, 26 has been further supported by its cytotoxic phenotype 11,27 and its strong association with the isochromosome arm 7q cytogenetic abnormality. 12,14,25,[28][29][30][31][32] More recently, a few cases of HSTCL with sinusoidal infiltration and an ␣␤ T-cell receptor phenotype have been reported. [33][34][35] This is now considered an immunophenotypic variant of the same disease entity in the World Health Organization (WHO) classification.…”

Hepatosplenic γδ T‐cell lymphoma in a 10‐year‐old boy successfully treated with hematopoietic stem cell transplantation

“…Most of the reported cases were in young adult males. Only three children under 15 years old were reported: 2 boys and 1 girl [3][4][5]. The 2 boys failed to respond to multi-agent chemotherapy and survived less than 24 months.…”

Gamma/Delta T-cell Lymphoma as a Recurrent Complication after Transplantation

“…Although gd T-cell lymphoma has been previously reported in children [30,31], the occurrence of this lymphomatous subset in a 6 year-old child is an exceptional event.…”