Orta Zarifa, Benli Aysun, Çağatay Arif Atahan, Başaran Seniha, Erdem Simge, Beşişik Sevgi
Department of Infectious Diseases and Clinical Microbiology, Tekirdag Dr.İsmail Fehmi Cumalıoglu City Hospital, Tekirdag, Türkiye.
Istanbul Faculty of Medicine, Department of Infectious Diseases and Clinical Microbiology, Istanbul University, Istanbul, Türkiye.
Int J STD AIDS. 2025 Jul 23:9564624251355834. doi: 10.1177/09564624251355834.
BackgroundKaposi sarcoma in people living with HIV and AIDS usually involves slowly progressive skin lesions. Cases of Kaposi sarcoma complicated by hemophagocytic lymphohistiocytosis are rare. In this report, we present the first series of hemophagocytic lymphohistiocytosis cases from Türkiye associated with AIDS and Kaposi sarcoma.MethodsCases of hemophagocytic lymphohistiocytosis in people living with HIV and AIDS and Kaposi sarcoma who met the HLH-2004 diagnostic criteria recommended by the "Histiocyte Society" were included in the study. The patient's demographic, clinical, and laboratory characteristics were recorded retrospectively.ResultsFour patients presented with fever, cytopenia, peripheral lymphadenomegaly, splenomegaly, and hepatomegaly. Fever persisted throughout their hospitalization. At the time of HLH diagnosis, Kaposi sarcoma was detected in the lymph node biopsies of one patient and in the skin biopsies of three patients. Hemophagocytosis was observed in bone marrow aspirations from all patients. Three patients received dexamethasone and etoposide for hemophagocytic lymphohistiocytosis treatment, while one received dexamethasone alone. All four patients died.ConclusionsCases of hemophagocytic lymphohistiocytosis associated with Kaposi sarcoma and AIDS are rare in the literature. In conclusion, high fever may appear in the early stages of Kaposi sarcoma in people living with HIV and AIDS. Since hemophagocytic lymphohistiocytosis has a high mortality rate, early diagnosis and prompt initiation of appropriate treatment are crucial.
背景
感染人类免疫缺陷病毒（HIV）和获得性免疫综合征（AIDS）的人群中，卡波西肉瘤通常表现为缓慢进展的皮肤病变。卡波西肉瘤合并噬血细胞性淋巴组织细胞增生症的病例较为罕见。在本报告中，我们呈现了土耳其首例与艾滋病和卡波西肉瘤相关的噬血细胞性淋巴组织细胞增生症病例系列。
方法
本研究纳入了符合“组织细胞协会”推荐的HLH - 2004诊断标准的HIV/AIDS合并卡波西肉瘤且患有噬血细胞性淋巴组织细胞增生症的患者。回顾性记录患者的人口统计学、临床和实验室特征。
结果
4例患者出现发热、血细胞减少、外周淋巴结肿大、脾肿大和肝肿大。发热在整个住院期间持续存在。在HLH诊断时，1例患者的淋巴结活检及3例患者的皮肤活检中检测到卡波西肉瘤。所有患者的骨髓穿刺均观察到噬血细胞现象。3例患者接受地塞米松和依托泊苷治疗噬血细胞性淋巴组织细胞增生症，1例仅接受地塞米松治疗。所有4例患者均死亡。
结论
文献中卡波西肉瘤与艾滋病相关的噬血细胞性淋巴组织细胞增生症病例罕见。总之，HIV/AIDS患者的卡波西肉瘤早期可能出现高热。由于噬血细胞性淋巴组织细胞增生症死亡率高，早期诊断并及时开始适当治疗至关重要。
