Gamma-Delta T Cell Acute Lymphoblastic Leukemia: A Single-Center Experience

Abstract

Gamma-delta (γδ) T cell neoplasms are a rare disease entity characterized by an aggressive clinical course [1,2]. The management of these neoplasms associated with high incidence of induction failures and poor clinical outcomes [3]. Here we present two cases of gamma-delta T cell acute lymphoblastic leukemia (γδ TALL) successfully treated with chemotherapy and allogeneic stem cell transplant at our institution. We also review the literature and summarize what is known about this disease. In our experience, induction chemotherapy followed by allogeneic stem cell transplantation has been an effective strategy in producing durable remissions.

A Challenging Case of Gamma Delta T-Cell Lymphoma with Precursor T-Cells and Marked Eosinophilia: A Case Report

“…T-cell acute lymphoblastic leukemia/lymphoma (T-ALL/LBL) is rare and infrequently shows T-cell differentiation or TCR expression [ 12 ]. γδ T-cell lymphoblastic leukemia is rare representing a small percentage of T-ALL cases [ 4 , 13 ].…”

“…γδ T-cell neoplasms including HSTL, PCγδTCL, γδ T-LGL, and γδ T-ALL/LBL subtypes differ in their presentation, clinical course, response to treatment, and prognosis [ 13 ].…”

“…Nevertheless, the appropriate induction regimen and consolidation policy for this exclusive entity remain unidentified. Despite aggressive therapy for patients diagnosed with this unique disease, the prognosis is poor and long-term survival is hardly achieved [ 13 ].…”

Gamma/Delta (γδ) T Cells: The Role of the T-Cell Receptor in Diagnosis and Prognosis of Hematologic Malignancies

“…For example, rare cases of MF 6 or T-ALL. 8 with γδ expression have not been demonstrated to have poorer prognosis.…”

“…Entities that commonly derive from γδ T cells include primary cutaneous γδ T-cell lymphoma (PCGDTCL), 4 hepatosplenic T-cell lymphoma (HSTCL), and monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL). 5 Very rare cases of mycosis fungoides (MF), 6 large granulocytic leukemia (LGL), 7 and T-cell acute lymphocytic leukemia (T-ALL) 8 may demonstrate expression of the γδ TCR, although these are generally malignancies of αβ T cells. Rare γδ T cells have also recently been identified in pityriasis lichenoides (PL) 9 and lymphomatoid papulosis (LyP), although their presence is of unclear significance.…”