SEVERE NEUTROPENIA OF 14 YEARS AS THE ONL Y MANIFESTATION OF A MYELODYSPLASTIC SYNDROME

Myelodysplastic syndromes (MDS) are a group of acquired hemopathies ch aracterized by peripheral cytopenias due to ineffective hematopoiesis and a high risk of transformation into acute non lymphoblastic leukemi a (ANLL) which, in most cases, usually occurs from 6 months to 4 years after diagnosis, A patient with extreme neutropenia with intense dysg ranulopoiesis as the only manifestations of MDS is described, The pati ent was controlled over 14 years and presented multiple infectious epi sodes, in various locations, throughout the evolution, some being very severe and generally caused by gram-negative germs. Likewise, during this time the patient received different treatments (oximetholone, pre dnisone and lithium carbonate) with no hematologic response being obse rved. The leukocyte count remained around 3 x 10(9)/L with a mean prop ortion of neutrophils of 12% with no variations being found in the bon e marrow aspirates carried out throughout the evolution (total of 9). At 14 years the diagnosis of MDS evolved to ANLL, The patient died sho rtly after the acute transformation due to respiratory failure seconda ry to bilateral pneumonia, In this case three peculiar features are of note: the almost exclusive involvement of the granulopoietic series w ithout either anemia or thrombocytopenia, the long evolution of AREB, with acute transformation 14 years after diagnosis and the severity of the infections, among which recurrent lingual granulopenic ulcers wer e of note.