Resumen

GAMEZ PEREZ, Anadely et al. Leucemia mielomonocítica aguda y drepanocitosis no tratada con hidroxiurea: A propósito de un casoAcute Melanocytic Leukemia and Drepanocytosis non-treated with hydroxyurea a case report. Rev Ciencias Médicas [online]. 2009, vol.13, n.2, pp. 241-246. ISSN 1561-3194.

Malignant diseases related to sickle-cell anemia have been previously reported in a reduced number of patients, the risk of suffering from cancer in those patients have increased with the advent of the hydroxyurea treatment. In this case no evidence of being taken this medication was proved. A 25-years old male presenting hepatic crisis, jaundice, hyperbilirubinemia, high levels of transaminase, hepatomegaly and low red blood cell counts (40 g/l), blast cells of 25 µm in diameter, granular cell presenting nucleus of monocytic-like cells (some of them), monocytic component in periphery equal to 15 %, leucocytes counts 120x10⁹/l, medullogram: 80 % of blast cells, monocytic component greater than 20 %, concluded as Acute Myelomonocytic Leukemia (AML M4) diagnostic cyto-chemical studies showed an increase of lysozyme and muramidase higher than 13 mg lis/ml of plasma (a positive Na acetatoesterases). A plasmapheresis and ex-transfusion and two rechanges, deep rehydration. No satisfactory response was observed and the patient died on the third day with a diagnose of acute respiratory insufficiency. Because of this unsual disorder and no other cases described by the medical literature as an Acute Myeloproliferative Syndrome in association to Drepanocytosis non-treated with Hydroxyurea motivated the authors to present this case report.

Palabras clave : Hemoglobinopathies; drepanocytosis; acute myeloproliferative syndrome.

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