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Hydroxyurea therapy in paraparesisand cauda equina syndrome due to extramedullary haematopoiesis in thalassaemia: improvement of clinicaland haematological parameters
Author(s) -
Cianciulli P.,
Caravita di Toritto T.,
Sorrentino F.,
Sergiacomi L.,
Massa A.,
Amadori S.
Publication year - 2000
Publication title -
european journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.904
H-Index - 84
eISSN - 1600-0609
pISSN - 0902-4441
DOI - 10.1034/j.1600-0609.2000.9c165.x
Subject(s) - medicine , haematopoiesis , gastroenterology , fetal hemoglobin , extramedullary hematopoiesis , hemoglobinopathy , radiation therapy , surgery , fetus , hemolytic anemia , stem cell , biology , pregnancy , genetics
Patients with β‐globin disorders show amelioration of clinical condition by sustained synthesis of fetal haemoglobin in adult life. We report data on a patient with β°‐thalassaemia genotype and thalassaemia intermedia clinical phenotype. He received therapy with hydroxyurea (20 mg/kg/d) because of the presence of extramedullary masses causing paraparesis, neurogenic bladder and impotence. During therapy, the patient showed an improved clinical picture and a significant increase in total Hb (from 71.8 to 103.2 g/L) and a γ/α globin synthetic ratio (from 0.39 to 0.68). The myelosuppressive effect of hydroxyurea was revealed by a decrease in CFU‐GEMM, BFU‐E, and CFU‐GM. Therefore hydroxyurea can be effective in the treatment of patients with extramedullary haematopoiesis (EMH) who are not transfusion‐dependent and cannot be treated with radiotherapy.