Histiocytic sarcoma associated with idiopathic myelofibrosis.

Archives of Pathology & Laboratory Medicine
Masaharu Fukunaga, Hiroyuki Kato

Abstract

We describe the case of a 39-year-old man with idiopathic myelofibrosis, who developed histiocytic sarcoma (true histiocytic lymphoma) 6 months after diagnosis. The patient developed generalized lymphadenopathy. A lymph node biopsy showed pronounced distension of the sinuses in the medulla and periphery, caused by the accumulation of large tumor cells. The tumor cells had abundant clear or eosinophilic cytoplasm. The nuclei were of various sizes and shapes, with condensed chromatin and prominent nucleoli. Some tumor cells displayed erythrophagocytosis. Immunohistochemically, the tumor cells were positive for CD68, alpha(1)-antitrypsin, CD45, CD45RO, and S100 protein, and were negative for B- and T-cell markers, CD30, CD1a, lysozyme, myeloperoxidase, factor VIII-related antigen, CAM 5.2, and HMB-45. Despite multiagent chemotherapy, the patient died of disease 25 months after diagnosis. Although histiocytic sarcomas are very rare, their recognition may be important for clinical and prognostic reasons.

References

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Citations

May 10, 2011·Clinical Nuclear Medicine·William MakisRobert Lisbona
Jul 19, 2015·Diagnostic Pathology·Jiangning ZhaoQuanyi Lu
Jun 11, 2008·Pathology, Research and Practice·Xiaohong ZhangConrad Schuerch
Feb 2, 2010·Journal of Korean Medical Science·Byeong Seok SohnSang-Oh Lee

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