Partial splenectomy in children with sickle cell disease and repeated episodes of splenic sequestration.

Twenty-five children with sickle cell disease and repeated episodes of splenic sequestration crises underwent partial splenectomy. They have been followed for 12 to 75 months (median 48 months) after surgery. During this period we have not observed any episode of splenic sequestration and the need for hospitalization and transfusions has been greatly reduced. We believe this is a useful and safe procedure for treating patients with this condition.

[Sickle-cell anemia in the 1st 5 years of life]. / La drepanocitosis en los cinco primeros años de vida.

PURPOSE: A programme for antenatal diagnosis of sickle-cell anaemia (SA) and SC haemoglobinopathy (SC) was started in Cuba in 1983. The purpose of this paper is to report the clinical and haematological data of a group of children diagnosed before birth or in the first few months of life. PATIENTS AND METHODS: Between June 1986 and December 1992, 78 children under 5 years of age were studied at the Haemoglobinpathies Clinic of the Instituto de Hematología e Inmunologia in La Habana; of them, 53 had SA and 25 had SC, there were 41 girls and 37 boys. A comprehensive questionnaire was carried out during the first visit to the clinic, about the onset of the clinical manifestations, as well as a careful physical examination plus peripheral blood study and Hb A2 and Hb F assay. Parents were informed, and folate and prophylactic penicillin were given orally to the children. RESULTS: The yearly average admissions to hospital were 1.6 +/- 3.8 for SA and 0.1 +/- 0.6 for SC. The average number of veno-occlusive crises was 0.2 +/- 0.6 in SA and 0.1 +/- 0.6 in SC (p < 0.04); the average hand-foot crises were 0.3 +/- 1.1. for SA and nil for SC. The average number of infections per year was 0.3 +/- 0.6 in SA and 0.1 +/-0.3 in SC, without any significant difference. Significant differences were found in the haemoglobin rates and reticulocyte counts between both haemoglobinopathies. Hb F rate was 7.80 +/- 4.78% in SA and 2.83 +/- 3.45% in SC. CONCLUSIONS: Admission to hospital, hand-foot crises, infections and splenic sequestration are the commonest clinical events during the first 5 years of life. The number of infections episodes decreases with prophylactic oral penicillin, and overacute pneumococcal infection is suppressed. Parent education allows one to early treat sequestration crises, and partial splenectomy avoids repetition of this complication, while preserving the splenic function.

[Treatment of promyelocytic leukemia with retinoic acid: results in Cuba]. / Tratamiento de la leucemia promielocítica con ácido retinoico: resultados en Cuba.

PURPOSE: To assess the results attained with all-trans-retinoic acid (ATRA) in a group of Cuban patients with acute promyelocytic leukaemia (PML). PATIENTS AND METHODS: Twenty-one patients with PML were studied. Their cytogenetic study was performed with G-band techniques. ATRA was given orally as a single dosis of 50 mg/m2 a day, or divided in two doses. After attaining complete remission (CR), ATRA was maintained for 1-3 months in association with minimal doses of Ara-C or alpha-interferon. A rotation of three therapeutic regimes (TRAP, POMP and DOAP) was subsequently administered. RESULTS: Twelve of the patients were women and 9 men; 15 were adults and 6 were children, the median age being 19 years (range: 6-60 years). Only two patients had leucocytosis, all others presented with leucopenia. Platelet count below 30 x 10(10)/L was found in 67% of the cases, while some sort of bleeding was present in 81% of them. Laboratory evidence of disseminated intravascular coagulation was seen in 52% of the cases, and t (15; 17) appeared in 67% of the evaluable cytogenetic studies. CR was attained in 17 patients (81%) within a mean of 40 days. Headache was the commonest untoward effect of the treatment. Eight patients developed leucocytosis during treatment, white-cell count being over 20 x 10(9)/L in six of them. Fever without infectious signs was present in 5 patients, and in 3 of them the temperature recovered with steroid therapy. Two patients had retinoic acid syndrome prior to achieving CR. Four patients relapsed and 13 (76%) have maintained CR after 1 to 24 months. CONCLUSIONS: The incidence of CR in this series is within the limits reported in the literature. The secondary effects of the treatment are the same than those reported by others, and they were transient and well tolerated. The response to steroids of those patients with fever secondary to ATRA is noteworthy. The efficacy of ATRA, in general terms, in the induction of CR in PML seems confirmed by these results.