Autoimmune hemolysis accompanied by thrombopenia: Consumption or myelodysplasia?

Universitätsklinik für Innere Medizin III mit Hämatologie, internistischer Onkologie, Hämostaseologie, Infektiologie, Rheumatologie und Onkologisches Zentrum, Landeskrankenhaus Salzburg, Universitätsklinikum der Paracelsus Medizinischen Privatuniversität.
DMW - Deutsche Medizinische Wochenschrift (Impact Factor: 0.54). 10/2013; 138(41):2093-2095. DOI: 10.1055/s-0033-1349586
Source: PubMed


A 77-year-old patient with a known autoimmune hemolysis for years was treated with steroids, rituximab, cyclophosphamid, cyclosporin A. Because of accompanying thrombopenia he received eltrombopag and underwent splenectomy but without lasting effect. After 3 years he presented with decreased leukocytes and worsening of thrombopenia.

A peripheral blood count showed moderate pancytopenia without blasts or left shift. Bone marrow biopsy revealed myelodysplasia with excessive blasts, cytogenetics showed partial trisomy 18q.

Treatment and course:
Because of the high risk of transformation to acute myeloid leukemia treatment with 5-azacytidine was started. Thrombopenia rapidly improved, but after an infectious complication treatment was paused for several months due to patient wish. Treatment was started again after 11 months because of progressive thrombopenia and resulted in a rapid hematological improvement.

The new diagnosis of a myelodysplastic syndrome should be considered in the case of new cytopenia even in the presence of an already established hematological disease.

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