Pure red cell aplasia evolving through the hyperfibrotic myelodysplastic syndrome to the acute myeloid leukemia: some pathogenetic aspects.
The authors report a 58-year-old female who originally presented with acquired pure red cell aplasia (PRCA). At diagnosis, the karyotype was normal, the serum erythropoietin level was highly elevated and no T-cell mediated inhibition of erythropoiesis was demonstrated in coculture studies. Conventional immunosuppressive therapy proved ineffective. A year later a diagnosis of hyperfibrotic myelodysplastic syndrome was assessed. The sequential bone marrow examinations in the course of the three years showed a progressive increase in bone marrow fibrosis, erythroid hyperplasia and dysmegakaryocytopoiesis, terminating in the acute myeloid leukemia. This sequence of the events included the appearance of del(5)(q13q33), four years after setting a diagnosis of PRCA. The authors suggest that the absence of both cytogenetic abnormality and the signs of dyshematopoiesis at the diagnosis of PRCA does not exclude ultimately a "clonal" category of the disease. Thus, repeated hematological and cytogenetical reevaluations are recommended.
Suvajdzic
N
N
Clinical Center of Serbia, Institute of Hematology, Belgrade, Yugoslavia.
Marisavljevic
D
D
Jovanovic
V
V
Pantic
M
M
Sefer
D
D
Colovic
M
M
D000208:Acute Disease; D005260:Female; D006801:Humans; D007951:Leukemia, Myeloid / Q000209:etiology*; D008875:Middle Aged; D009190:Myelodysplastic Syndromes / Q000503:physiopathology*; D012010:Red-Cell Aplasia, Pure / Q000503:physiopathology*