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

Abstract

HSTCL is a highly aggressive malignancy with a poor prognosis. Case series and accounts have reported the use of different chemotherapy regimens with diverse patient outcomes. Most long-term survivors had undergone high-dose chemotherapy with autologous or allogeneic HCT. We describe two pediatric patients with HSTCL who were treated with chemotherapy followed by allogeneic HCT. Both patients are alive and in complete remission 2 and 8 years after therapy. Multiagent chemotherapy followed with allogeneic HCT seems to provide patients who have chemotherapy-sensitive disease a long-term disease-free survival.

Rare non‐Hodgkin lymphoma of childhood and adolescence: A consensus diagnostic and therapeutic approach to pediatric‐type follicular lymphoma, marginal zone lymphoma, and nonanaplastic peripheral T‐cell lymphoma

“…For advanced or disseminated stages, low‐intensity chemo‐ and immunotherapy, such as anthracycline‐free but rituximab‐containing mature B‐NHL courses, R‐COP, or R‐CVP, should be considered 32‐35 . Current treatment recommendations for nonanaplastic PTCL are more complex and range from a moderately intense block‐like ALCL‐derived regimen (due to a better tolerability compared to LBL/ALL‐derived regimens) for PTCL‐NOS and SPTCL to a block‐like mature B‐NHL‐ or ALCL‐derived regimen followed by allogeneic/autologous HSCT in CR1 for HSTCL and AITL 27‐30,99‐101,107–109 …”

Pralatrexate‐based therapy induced response in an adolescent with refractory hepatosplenic T‐cell lymphoma

“…Poorly responsive to chemotherapy, the estimated 5‐year overall survival (OS) of pediatric patients with HSTL is less than 10% 1 . Patients are treated with diverse chemotherapy regimens to achieve a maximum response preceding allogeneic stem cell transplant (Allo‐SCT) 2,3 . Pralatrexate (PRX) is a potent antimetabolite approved by the Food and Drug Administration (FDA) for use in adult patients with relapsed/refractory peripheral T‐cell lymphoma (r/rPTCL), including HSTL 4 …”

Treatment and prognosis of mature (non‐anaplastic) T‐ and NK‐cell lymphomas in childhood, adolescents, and young adults

“…The typical HSTL clinical presentation is of systemic symptoms, cytopenias and marked hepatosplenomegaly without adenopathy. Although bone marrow involvement is present in most patients, overt leukaemia presentation is uncommon, and patients are at high risk of developing haemophagocytic syndrome (Table ) (McThenia et al , ).…”

“…Treatment outcome of patients with HSTL is very guarded (Mellgren et al , ; Sorge et al , ). HSTL is not responsive to anthracycline‐ or platinum‐based chemotherapy (Belhadj et al , ; McThenia et al , ). SCT is suggested to provide some benefit.…”

“…SCT is suggested to provide some benefit. In a retrospective review of 68 cases of paediatric SCT, only 4 patients (6%) not treated with autologous or allogeneic SCT survived versus 15 cases (22%) (McThenia et al , ). Similar to ENKL cells, HSTL cells overexpress pGP, encoded by the ABCB1 gene (previously termed MDR1 ), which probably explains the resistance to conventional chemotherapy.…”