levoleucovorin and Ataxia-Telangiectasia

Children with ataxia-telangiectasia (A-T) and cancer have a poorer prognosis due in part to increased treatment-related toxicity. We piloted a curative intent approach in five children with A-T who presented with advanced stage (III, n = 2; IV, n = 3) B-NHL (diffuse large B-cell lymphoma, n = 4; Burkitt leukemia, n = 1) using a modified LMB-based protocol. Two achieved sustained CCR (one, CCR at 6 years; one, pulmonary death after 3 years in CCR). Two died from toxicity during induction and 1 failed induction with progressive disease. Novel therapeutic approaches which overcome drug resistance and are less toxic are needed for children with A-T and B-NHL.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Ataxia Telangiectasia; Child; Cyclophosphamide; Cytarabine; Doxorubicin; Etoposide; Female; Follow-Up Studies; Humans; Hydrocortisone; Leucovorin; Lymphoma, B-Cell; Male; Methotrexate; Neoplasm Staging; Pilot Projects; Prednisone; Prognosis; Prospective Studies; Vincristine; Young Adult

Ataxia-telangiectasia (AT) is a hereditary disorder characterized by progressive neurological dysfunction, oculocutaneous telangiectasia, immunodeficiency, cancer susceptibility, and radiation sensitivity. Pediatric patients may develop acute lymphoblastic leukemia (ALL). However development of ALL in an adult patient with AT is a rare occurrence. Here we report such a patient who presented with hyperleukocytosis and were treated with leukapheresis. A 25years old male patient, who were diagnosed with AT and mental retardation, was admitted to the emergency department due to fatigue, nausea and headache. On admission he had a moderate general condition and was fully cooperated. His white blood cell (WBC) count were 466×10(9)/l. Blastic cells were observed in peripheral blood smear. Flow cytometry (FC) of peripheral blood showed T-ALL. Two sessions of large volume leukapheresis was performed. Symptoms due to hyperleukocytosis markedly improved after leukapheresis. Patients with AT should be closely monitored due to risk of malignancy. Leukapheresis may improve the prognosis of high risk ALL patients presenting with hyperleukocytosis.

Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Asparaginase; Ataxia Telangiectasia; Doxorubicin; Flow Cytometry; Humans; Leucovorin; Leukapheresis; Leukocytosis; Male; Methotrexate; Precursor Cell Lymphoblastic Leukemia-Lymphoma; Prognosis; Steroids; Vincristine