[The 5q- syndrome associated with marked erythroid hypoplasia and Coombs test positive hemolysis].
Rinsho Ketsueki
; 40(6): 499-504, 1999 Jun.
Article
em Ja
| MEDLINE
| ID: mdl-10422288
ABSTRACT
The 5q- syndrome is a myelodysplastic disorder characterized by macrocytic anemia, hypolobulated micromegakaryocytic hyperplasia, and an interstitial deletion of chromosome 5 as a solitary cytogenetic abnormality. The majority of patients with this syndrome are elderly women exhibiting red blood cell transfusion-dependent refractory anemia with a normal-to-increased number of platelets and modest granulocytopenia. The prognosis is relatively favorable with a low incidence of leukemic transformation. We report on a patient with 5q- syndrome associated with autoimmune hemolytic anemia (AHIA) and severe erythroid hypoplasia mimicking pure red cell aplasia (PRCA). A 65-year-old woman was admitted because of severe anemia. Elevated serum levels of LDH and indirect bilirubin, and a positive direct Coombs' test suggested AIHA associated with a huge ovarian dermoid cyst. However, lack of peripheral reticulocytes and bone marrow eryhroblasts, characteristic megakaryocytic morphology, and solitary 5q- anomaly favored a diagnosis of 5q- syndrome complicated by PRCA-like feature. Underlying immunological abnormalities ascribed to aberrant lymphoid clones intrinsic to MDS may be responsible for red cell aplasia and autoantibodies against red blood cells.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Síndromes Mielodisplásicas
/
Cromossomos Humanos Par 5
/
Deleção Cromossômica
/
Aplasia Pura de Série Vermelha
/
Anemia Hemolítica Autoimune
Tipo de estudo:
Prognostic_studies
/
Risk_factors_studies
Limite:
Aged
/
Female
/
Humans
Idioma:
Ja
Revista:
Rinsho Ketsueki
Ano de publicação:
1999
Tipo de documento:
Article