Nam Hohyung, Min Gi-June, Kim Tong Yoon, Jeon Youngwoo, Cho Seok-Goo
Department of Hematology, Seoul St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Department of Hematology, Yeouido St. Mary's Hematology Hospital, College of Medicine, The Catholic University of Korea, Seoul, Republic of Korea.
Front Oncol. 2025 Feb 24;15:1505737. doi: 10.3389/fonc.2025.1505737. eCollection 2025.
Histiocytic sarcoma (HS) is a rare and aggressive hematologic malignancy with a poor prognosis. HS can present with either isolated organ involvement or multi-systemic disease. This case series reports on nine patients with diverse clinical presentations and outcomes.
Diagnoses of HS were confirmed using immunohistochemistry, with markers such as CD68 and lysozyme. Treatment primarily involved anthracycline-based chemotherapy with autologous hematopoietic stem cell transplantation (auto-HSCT) consolidation, and with salvage therapies for resistant or relapsed cases including allogeneic HSCT (allo-HSCT).
Despite intensive treatment, long-term remission was rare. Among the nine patients, three achieved complete remission but relapsed, three had stable disease, two experienced progressive disease, and one is under treatment. No patient maintained complete remission for at least three years, indicating the chemo-resistant nature of HS. Moreover, of three cases in our cohort that achieved complete remission, two declined auto-HSCT owing to the intensity of first-line chemotherapy, and one relapsed shortly after achieving remission. To overcome chemo-resistance, four patients underwent allo-HSCT, and two of them achieved long-term remission.
These findings highlight the importance of early diagnosis and suggest potential benefits of either autologous or allogeneic transplantation, while emphasizing the need for further research on treatment protocols.
组织细胞肉瘤（HS）是一种罕见且侵袭性强的血液系统恶性肿瘤，预后较差。HS可表现为孤立器官受累或多系统疾病。本病例系列报告了9例临床表现和预后各异的患者。
采用免疫组织化学方法，使用CD68和溶菌酶等标志物确诊HS。治疗主要包括以蒽环类为基础的化疗并联合自体造血干细胞移植（auto-HSCT）巩固治疗，对于耐药或复发病例采用挽救性治疗，包括异基因HSCT（allo-HSCT）。
尽管进行了强化治疗，但长期缓解很少见。9例患者中，3例达到完全缓解但复发，3例病情稳定，2例病情进展，1例正在接受治疗。没有患者维持完全缓解至少三年，这表明HS具有化疗耐药性。此外，在我们队列中达到完全缓解的3例患者中，2例因一线化疗强度而拒绝auto-HSCT，1例在缓解后不久复发。为克服化疗耐药性，4例患者接受了allo-HSCT，其中2例实现了长期缓解。
这些发现突出了早期诊断的重要性，并提示自体或异基因移植的潜在益处，同时强调需要对治疗方案进行进一步研究。
