[Intense neutropenia of 14 years duration as the only manifestation of a myelodysplastic syndrome].
Myelodysplastic syndromes (MDS) are a group of acquired hemopathies characterized by peripheral cytopenias due to ineffective hematopoiesis and a high risk of transformation into acute non lymphoblastic leukemia (ANLL) which, in most cases, usually occurs from 6 months to 4 years after diagnosis. A patient with extreme neutropenia with intense dysgranulopoiesis as the only manifestations of MDS is described. The patient was controlled over 14 years and presented multiple infectious episodes, 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 (oxymetholone, prednisone and lithium carbonate) with no hematologic response being observed. The leukocyte count remained around 3 x 10(9)/L with a mean proportion of neutrophils of 12% with no variations being found in the bone marrow aspirates carried out throughout the evolution (total of 9). At 14 years the diagnosis of MDS evolved to ANLL. The patient died shortly after the acute transformation due to respiratory failure secondary to bilateral pneumonia. In this case three peculiar features are of note: the almost exclusive involvement of the granulopoietic series without 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 were of note.
Las Heras
G
G
Servicio de Hematología, Hospital Universitario Germans Trias i Pujol, Badalona.
Marti
J M
JM
Villamor
N
N
Ribera
J M
JM
Feliu
E
E
Rozman
C
C
D000368:Aged; D002908:Chronic Disease; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005500:Follow-Up Studies; D016905:Gram-Negative Bacterial Infections / Q000209:etiology; D006801:Humans; D008297:Male; D009190:Myelodysplastic Syndromes / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D009503:Neutropenia / Q000097:blood / Q000150:complications / Q000175:diagnosis*; D012008:Recurrence
Neutropenia intensa de 14 años de duración como única manifestación de un síndrome mielodisplásico.