Platelet dysfunction as the presenting feature of atypical myelodysplastic syndrome with monosomy 7, normal blood counts and no bleeding tendency |
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Authors: | Dragomir Marisavljevi Peter Antunovi Predrag Milji and Milena Panti |
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Institution: | (1) Clinical Centre of Serbia, Institute of Haematology, Koste Todorovića 2, 11000 Belgrade, Yugoslavia |
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Abstract: | A 71-year-old male patient with atypical myelodysplastic syndrome showing monosomy 7 is described. He presented with severe
foot pains, trophic skin and nail changes, loss of distal pulses, all compatible with peripheral arterial occlusive disease.
He had completely normal blood counts and no bleeding tendency. Prolonged bleeding time was disclosed by chance, during routine
haemostatic studies. An acquired platelet dysfunction was considered, with prolonged bleeding time and large platelets that
failed to aggregate in response to arachidonic acid and that had impaired response to collagen and adrenaline. The bone marrow
was hypercellular, with numerous dysplastic megakaryocytes and two other slightly dysplastic myeloid lines. Cytogenetic analyses
of the bone marrow cells showed a mosaic karyotype: 46,XY/45,XY,-7. On angiography, bilateral thrombosis of the iliac, superficial
femoral and popliteal arteries was disclosed. The patient was prepared with platelet transfusions. Arterial thrombectomy and
amputation of the left calf were performed. Ten months later, his blood counts showed mild pancytopenia. He died at home.
The authors discuss some clinical and pathogenetical aspects of such presentations of myelodysplastic syndromes. |
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Keywords: | myelodysplastic syndromes platelet dysfunction normal blood counts monosomy 7 |
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