ABSTRACT
Weekly monitoring of absolute neutrophil count (ANC) under deferiprone therapy in thalassemia patients is recommended to avoid agranulocytosis adverse event. Actually, this recommendation may not be applicable in clinical setting. Our study aimed to establish incidence of neutropenia under deferiprone (DFP) monotherapy when it was monitored bimonthly due to socioeconomic conditions effecting local and refugee thalassemic patients including Syrian origin (SYR; n = 26) and Turkish origin (TR; n = 26) groups. Patients on DFP were followed up for 12 months. Fifteen neutropenic episodes were seen in 5 patients. All 5 patients (4 from SYR group and 1 from TR group) had splenomegaly and hypersplenism, and neutropenia ceased in 4 patients after splenectomy despite continuation of deferiprone. In the TR group, the frequency of patients who have neutropenia (absolute neutrophil count [ANC] <1500/mm(3)) was 3.8% (n = 1) in the 1st month, no patients in TR group had neutropenia until 10th month when again there was 1 patient with mild neutropenia. In SYR group, the frequency of patients who have neutropenia was 3.8% (n = 1), 7.7% (n = 2), and 11.5% (n = 3) in the 1st, 2nd, and 3rd months, respectively, and was found to be 3.8% (n = 1) between 6 and 12 months. Whether or not DFP therapy should be interrupted in case of mild neutropenia and the frequency of monitoring ANC in real-life conditions should be documented with further studies. Other causes of neutropenia in DFP-treated patients should also be kept in mind.
Subject(s)
Blood Transfusion , Neutropenia , Pyridones , Thalassemia , Adolescent , Anemia, Aplastic/blood , Anemia, Aplastic/epidemiology , Anemia, Aplastic/etiology , Anemia, Hemolytic/blood , Anemia, Hemolytic/epidemiology , Anemia, Hemolytic/etiology , Bone Marrow Diseases/blood , Bone Marrow Diseases/epidemiology , Bone Marrow Diseases/etiology , Bone Marrow Failure Disorders , Child , Child, Preschool , Deferiprone , Female , Hemoglobinuria, Paroxysmal/blood , Hemoglobinuria, Paroxysmal/epidemiology , Hemoglobinuria, Paroxysmal/etiology , Humans , Male , Neutropenia/blood , Neutropenia/epidemiology , Neutropenia/etiology , Pyridones/administration & dosage , Pyridones/adverse effects , Retrospective Studies , Socioeconomic Factors , Syria/epidemiology , Thalassemia/blood , Thalassemia/epidemiology , Thalassemia/therapy , Turkey/epidemiologyABSTRACT
Myelosuppression is a serious complication during treatment of acute lymphoblastic leukemia and the duration of myelosuppression is affected by underlying bone marrow failure syndromes and drug pharmacogenetics caused by genetic polymorphisms. Mutations in the thiopurine S-methyltransferase (TPMT) gene causing excessive myelosuppression during 6-mercaptopurine (MP) therapy may cause excessive bone marrow toxicity. We report the case of a 15-year-old girl with T-ALL who developed severe pancytopenia during consolidation and maintenance therapy despite reduction of the dose of MP to 5% of the standard dose. Prednisolone therapy produced a remarkable but transient bone marrow recovery. Analysis of common TPMT polymorphisms revealed TPMT *3A/*3C.
