Multiple nonleukemic myeloid sarcoma (MS) is a rare form of MS that is developed in multiple anatomic sites other than bone marrow at diagnosis, without a preceding myeloid neoplasm. The prevalence, prognosis, and optimal management of multiple nonleukemic MS have not been addressed. The role of allogenic or autologous hematopoietic cell transplantation (HCT) for nonleukemic MS is also less well defined. We present a case of MS characterized by systemic lymphadenopathies and multiple effusions, which presumably had a very poor prognosis. The patient was treated with acute myeloid leukemia-type induction chemotherapy and autologous peripheral blood stem cell transplantation, and, unexpectedly, she has remained disease free for more than 6 years. We also reviewed the literature on this rare disease, and found that multiple nonleukemic MS was associated with younger age and a worse prognosis when compared with the overall nonleukemic MS population. We suggest that autologous HCT represents a valid option for young patients with chemosensitive disease and should be performed at the status of minimal residual disease-negative complete remission.

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