Hepatosplenic gamma delta T-cell lymphoma in a boy with visceral leishmaniasis: a case report

Abstract

IntroductionHepatosplenic gamma delta T-cell lymphoma is a rare peripheral T-cell lymphoma of cytotoxic T-cell origin with an aggressive clinical course. Chronic immunosuppression has been proposed as a possible pathogenetic mechanism. No association of hepatosplenic gamma delta T-cell lymphoma with visceral leishmaniasis has been described in the past. We describe a case of an adolescent boy with hepatosplenic gamma delta T-cell lymphoma with leukemic presentation, who was diagnosed to have visceral leishmaniasis, 9 months prior to presentation at our center. To the best of our knowledge this is the first report of hepatosplenic gamma delta T-cell lymphoma with a prior history of visceral leishmaniasis in the medical literature.Case presentationA 13-year-old Indian boy presented to the hematology out-patient department with a history of progressive abdominal distension of 9 months’ duration and low grade fever of 2 months’ duration. He was a known case of visceral leishmaniasis and was treated with some clinical improvement in the past. However, his symptoms recurred and he was diagnosed to have hepatosplenic gamma delta T-cell lymphoma at our center. Cytogenetic analysis showed characteristic karyotype of isochromosome 7.ConclusionsChronic antigen stimulation due to visceral leishmaniasis may have led to an expansion of gamma delta T cells in our patient, and immunophenotypic analysis of bone marrow aspirate and characteristic karyotype helped to achieve the diagnosis. The aim of this case report is to highlight the rare association of hepatosplenic T-cell lymphoma with visceral leishmaniasis.

Hepatosplenic γδ T‐cell lymphoma of two adolescents: Case report and retrospective literature review in children, adolescents, and young adults

“…Although HSTCL can present at any age, it has been most frequently reported in teenagers and young adults, with a strong male predominance . Our retrospective literature review (1992‐2016) identified 68 pediatric, adolescent, and young adults (less than or equal to age 21 years) with the diagnosis of HSTCL . Similar to these other case series, our retrospective pediatric review validated similar male predominance, with a male to female ratio of 3.5:1 .…”

“…Immunocompromise status due to immune suppression such as solid‐organ transplant and immune dysregulation secondary to malignancy or infection have been described as potential contributors to lymphomagenesis . Inflammatory bowel disease and the use of immunosuppressive agents (eg, antitumor necrosis‐α agents) and antimetabolite therapy (eg, 6TG, 6MP) had also been associated with HSTCL development.…”

“…Comorbidities and the use of some form of immunosuppressive therapy were identified in 28 (41%) of the 68 HSTCL cases reported in the literature. They included heart transplant (n = 1), renal transplant (n = 1), SLE (n = 1), Crohn's disease (n = 18), UC (n = 1), asthma (n = 2), autoimmune hepatitis (n = 2), common variable immune deficiency with MAS (n = 1), and visceral leishmaniasis (n = 1) . All of the patients with Crohn's disease and other immunosuppressed states died of HSTCL or complications related to treatment.…”

“…A literature search through PubMed for γδ HSTCL of children, adolescents, and young adults (age ≤21 years); treatment modalities (chemotherapy with or without HDC and blood and bone marrow transplant); and treatment outcomes resulted in 68 reported cases. The results of these cases are summarized in Tables and . Because of the retrospective nature of this report, descriptive statistical analysis was primarily used to report data.…”

“…HSTCL has a 20% association with the clinical setting of chronic immunosuppression and prolonged antigenic stimulation, particularly among solid organ transplant recipients, as well as patients with inflammatory bowel disease, notably Crohn's disease . HSTCL is known for its aggressive clinical course, poor response to chemotherapy, and a historically poor outcome with a 5‐year survival rate of 7% among all age‐groups .…”