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PubMed1999

[Chronic erythremic myelosis (or the chronic form of Di Guglielmo's disease). A case report].

The chronic erythromyelosis (or chronic type of Di Guglielmo's disease) is a rare illness of uncertain classification. This illness shows characteristics that remember on the one hand the myeloproliferative and on the other hand the myelodysplastic diseases. The therapy is quite unsatisfactory. As a presence of erythrocytic antibodies has been detected, a therapy with steroids, danazol, splenectomy, immunosuppressive agents, plasmapheresis is advised. We have tried a cycle of therapy with high doses of intravenous gammaglobulin (0.4 g/kg/day for 4 days) also in the attempt of reduce, even if temporarily, the hemolytic component due to the reticuloendothelial system's hyperplasia. We conclude that this therapy, in our patient, has not yielded good outcomes immediately but carried out settled levels of haemoglobin as well as reduction of bilirubin and LDH after a month.

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