Erschienen in:
01.10.2010 | Letter to the Editor
Hemophagocytic lymphohistiocytosis associated with myelodysplastic syndromes
verfasst von:
Takahiro Tsuji, Hiroshi Yamasaki, Nobuyuki Arima, Hiroyuki Tsuda
Erschienen in:
International Journal of Hematology
|
Ausgabe 3/2010
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Excerpt
Hemophagocytic lymphohistiocytosis/hemophagocytic syndrome (HLH/HPS) is a reactive disorder of the mononuclear phagocytic system, characterized by generalized histiocytic proliferation with marked hemophagocytosis in the bone marrow [
1]. It comprises two different categories: a primary (genetic) and a secondary (acquired) form. Malignant neoplasm-associated HPS (MAHS) is categorized as a secondary HLH. MAHS is mainly associated with lymphoma and rarely with other carcinomas [
2]. To our knowledge, HLH associated with myelodysplastic syndromes (MDS) has not been described, although MDS with hemophagocytosis has been observed. Here, we describe a case of HLH associated with MDS, which presented with abundant CD8
+ T cells in the bone marrow and elevated plasma-soluble interleukin 2 receptor (sIL-2R). …