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Fanconi's anemia is a rare autosomal recessive disorder associated with variable clinical manifestations. So far, eight complementation groups have been identified, and the genes of four of them have been cloned. Early-onset progressive bone marrow failure and a predisposition to malignancies, particularly acute myelogenous leukemia, liver tumors, and mucocutaneous squamous cell carcinomas, result in a poor prognosis.
View Article and Find Full Text PDF[Clonal myelodysplasia: prognostic stratification and therapeutic possibilities].
Rev Clin Esp
October 1993
Servicio de Hematología y Hemoterapia, Fundación Jiménez Díaz, Facultad de Medicina, Universidad Autónoma, Madrid.
Myelodysplastic syndromes represent an entire group of clonal panmyelopathies with very distinct evolutionary pathways. Their common denominator, however, is a self-maintained functional failure of the myeloid hemopoiesis which tends to evolve into severe non-lymphoid leukemia (SNLL) in 20-30% of the cases. First, the prognostic value of each the following is reviewed: the morphological classification F.
View Article and Find Full Text PDF[The borderlines --closed, open, or blurred-- of panmyelopathies].
An Med Interna
August 1993
Servicio de Hematología, Fundación Jiménez Díaz, Facultad de Medicina, Universidad Autónoma, Madrid.
Bone marrow scintigraphy was performed in 40 patients with haematological disorders (lymphomas, multiple myeloma, myeloid leukaemia, panmyelopathy and others). Routine skeletal scintigraphy was available for comparison in 18 patients. In agreement with the literature it became evident that marrow scintigraphy can show infiltrations of the bone marrow in haematological disorders earlier than skeletal scintigraphy.
View Article and Find Full Text PDFFrom the viewpoint of a territorial haematological centre with a certainly preselected number of patients the initial symptom of bi- or pancytopenia in 3/4 of the cases proves as first reference to a myeloproliferative or myelodysplastic syndrome. Of these cases 62.5% transferred into a crisis of blasts; that is, they must be regarded as real preleukemia.
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