Hematopoietic stem cell transplantation (HSCT) in patients with primary granulocytic sarcoma (GS)

Abstract

16529 Background: GS preceding acute myeloid leukemia (AML) is extremely rare and the prognosis is poor. Patients usually undergo systemic chemotherapy (CT) but the role of HSCT is not sufficiently defined. Methods: We report on 3 cases of non-leukemic GS, all of those treated with inductive CT followed by either autologous or allogeneic SCT. Furthermore, the literature on the role of HSCT in GS is reviewed. Results: Case 1: A 55-year-old man presented with a mediastinal mass and right pleural effusion. Fluid cytology revealed hematopoietic cells. Mediastinoscopy showed GS but peripheral blood and bone marrow were free of blasts. He underwent 2 cycles of anthracyline-cytarabine (AC) containing induction CT followed by myeloablative conditioning and autologous SCT in 1st remission. Radiotherapy to the mediastinum was applied afterwards. He remained in CR for 6 months when a leukemic relapse was diagnosed. He is currently undergoing reinduction CT and will proceed to receive an unrelated donor (URD) SCT. Case 2: A 51-year old man was diagnosed with GS of the right testicle. After orchiectomy he was followed by observation only. 8 months later a leukemic relapse [karyotype 46 XY, del (9)(q22), der(13) t(8;13)] occurred. The patient received 2 courses of AC-based induction CT followed by myeloablative conditioning and an HLA-matched URD transplant in 1st CR. However, 27 months after HSCT another leukemic relapse was diagnosed. He received a 2nd URD transplant but died of severe graft versus host disease (GvHD) and veno-occlusive disease 10 weeks later. Case 3: A 37 year-old woman was diagnosed with non-leukemic GS located at multiple bone sites. She underwent 4 courses of AC-containing CT resulting in a CR. 6 months later she presented with multiple recurrent GS confined to the skeleton. She received myeloablative conditioning followed by an allogeneic SCT from her HLA-mismatched brother. Another CR was attained. However, she suffered from severe GvHD and died of severe sepsis 3 months after HSCT. Conclusions: The outcome of patients with primary GS appears to be poor even after treatment with HSCT. However, allogeneic SCT has curative potential as indicated by some cases of successful transplants reported in the literature. No significant financial relationships to disclose.