Iraqi Journal of Hematology (Jan 2024)
Lymphoblastic lymphoma presenting as soft-tissue swelling – A single-center experience
Abstract
BACKGROUND: Lymphoblastic lymphomas (LBL) are a rare, aggressive type of non-Hodgkin lymphomas and constitute 2% of all lymphomas. It is classified as T-lymphoblastic lymphoma (T-LBL) and B lymphoblastic lymphoma (B-LBL) and resembles acute lymphoblastic leukemia (ALL), with no or limited bone-marrow involvement. LBL rarely present as soft-tissue swellings. Soft-tissue involvement is more common in B-LBL than in T-LBL. They occur predominantly in adolescents and young adults and have a prognosis similar to ALL. OBJECTIVE: p>The aim was to study the clinical characteristics, treatment response, and survival of patients with lymphoblastic lymphoma presenting with soft-tissue swelling. SETTINGS AND DESIGN: This was a retrospective study of eight adolescent and adult patients with lymphoblastic lymphoma presenting with soft-tissue swelling conducted in the Department of Medical Oncology at a tertiary cancer center in India. STATISTICAL ANALYSIS: The baseline patient characteristics, treatment details, and response assessment were analyzed using descriptive statistics. Overall survival (OS) and progression-free survival were obtained by the Kaplan–Meier method, using the SPSS version. 11. MATERIALS AND METHODS: We present the clinical features, imaging, diagnosis, treatment, and outcome of eight cases of lymphoblastic lymphoma presenting with soft-tissue swellings. Patients above 14 years of age treated over 12 years were included in the study. RESULTS: There were five males and three females. The median age at diagnosis was 24 years. Common presenting complaints were swelling, pain, and paraparesis. The sites of involvement were the epidural mass, thigh, calf, breast, and anterior chest wall. The diagnosis was confirmed by immunohistochemistry. Seven patients were diagnosed with B-LBL and one with T-LBL. They were treated with intensive chemotherapy (six patients with Berlin–Frankfurt–Munster protocol and two with hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone). Three patients received consolidation radiotherapy and one received palliative radiotherapy. Seven patients attained remission after induction chemotherapy and four of them are currently alive at a median follow-up of 48 months. The 2-year OS was 71.4%. CONCLUSION: Even though rare, lymphoblastic lymphoma should be considered a differential diagnosis in patients presenting with soft-tissue swelling and should be managed with systemic chemotherapy similar to ALL.
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