Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS
BackgroundHemophagocytic lymphohistiocytosis (HLH) is a fatal immunological syndrome resulting from excessive production of inflammatory cytokines. The conventional therapies for HLH, which are based on cytotoxic agents, are not always efficacious and safe, especially in patients with severe immunod...
| Main Authors: | , , , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Frontiers Media S.A.
2022-10-01
|
| Series: | Frontiers in Immunology |
| Subjects: | |
| Online Access: | https://www.frontiersin.org/articles/10.3389/fimmu.2022.1012643/full |
| _version_ | 1811205088626081792 |
|---|---|
| author | Xiang Liu Xueling Zhu Xiaotang Zhou Yirui Xie Dairong Xiang Zhikai Wan Ying Huang Biao Zhu |
| author_facet | Xiang Liu Xueling Zhu Xiaotang Zhou Yirui Xie Dairong Xiang Zhikai Wan Ying Huang Biao Zhu |
| author_sort | Xiang Liu |
| collection | DOAJ |
| description | BackgroundHemophagocytic lymphohistiocytosis (HLH) is a fatal immunological syndrome resulting from excessive production of inflammatory cytokines. The conventional therapies for HLH, which are based on cytotoxic agents, are not always efficacious and safe, especially in patients with severe immunodeficiency. Ruxolitinib, a strong inhibitor of Janus kinase (JAK) 1/2, has already been evaluated as salvage and first-line therapy for HLH. Despite its promising efficacy and tolerability in the treatment of secondary HLH, the efficacy and safety of ruxolitinib in HLH patients with HIV infection remain to be investigated.Case presentationTwo men (ages: 45 and 58 years) both presented at our hospital with a high fever. They were found to be HIV-positive with severe immunodeficiency and opportunistic infections. Their laboratory tests showed severe pancytopenia, hypofibrinogenemia, hypertriglyceridemia, and increased levels of inflammatory factors and ferritin. Hemophagocytosis was found in the bone marrow, and abdominal computed tomography or ultrasonography showed splenomegaly. Both patients were diagnosed with infection-induced HLH due to severe immunodeficiency. Given they were both highly immunocompromised, we chose ruxolitinib as a first-line treatment alternative to cytotoxic chemotherapy. Rapid remission of clinical symptoms and normalization of laboratory parameters were achieved after ruxolitinib therapy. Neither patient had any associated adverse drug reactions or other laboratory abnormalities. Both patients were eventually discharged and ruxolitinib was discontinued as their disease alleviated, and they did not show signs of relapse during the 3- and 5-month of follow-up examinations.ConclusionWe described two cases of AIDS-related secondary HLH treated with ruxolitinib. Our cases highlight the feasibility of using ruxolitinib as a first-line therapy in patients with HIV infection and secondary HLH. Nevertheless, the safety and efficacy of this novel treatment need to be evaluated in large clinical trials in the future. |
| first_indexed | 2024-04-12T03:24:43Z |
| format | Article |
| id | doaj.art-17bda0bd7ede42d18400db87f2b45dea |
| institution | Directory Open Access Journal |
| issn | 1664-3224 |
| language | English |
| last_indexed | 2024-04-12T03:24:43Z |
| publishDate | 2022-10-01 |
| publisher | Frontiers Media S.A. |
| record_format | Article |
| series | Frontiers in Immunology |
| spelling | doaj.art-17bda0bd7ede42d18400db87f2b45dea2022-12-22T03:49:44ZengFrontiers Media S.A.Frontiers in Immunology1664-32242022-10-011310.3389/fimmu.2022.10126431012643Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDSXiang LiuXueling ZhuXiaotang ZhouYirui XieDairong XiangZhikai WanYing HuangBiao ZhuBackgroundHemophagocytic lymphohistiocytosis (HLH) is a fatal immunological syndrome resulting from excessive production of inflammatory cytokines. The conventional therapies for HLH, which are based on cytotoxic agents, are not always efficacious and safe, especially in patients with severe immunodeficiency. Ruxolitinib, a strong inhibitor of Janus kinase (JAK) 1/2, has already been evaluated as salvage and first-line therapy for HLH. Despite its promising efficacy and tolerability in the treatment of secondary HLH, the efficacy and safety of ruxolitinib in HLH patients with HIV infection remain to be investigated.Case presentationTwo men (ages: 45 and 58 years) both presented at our hospital with a high fever. They were found to be HIV-positive with severe immunodeficiency and opportunistic infections. Their laboratory tests showed severe pancytopenia, hypofibrinogenemia, hypertriglyceridemia, and increased levels of inflammatory factors and ferritin. Hemophagocytosis was found in the bone marrow, and abdominal computed tomography or ultrasonography showed splenomegaly. Both patients were diagnosed with infection-induced HLH due to severe immunodeficiency. Given they were both highly immunocompromised, we chose ruxolitinib as a first-line treatment alternative to cytotoxic chemotherapy. Rapid remission of clinical symptoms and normalization of laboratory parameters were achieved after ruxolitinib therapy. Neither patient had any associated adverse drug reactions or other laboratory abnormalities. Both patients were eventually discharged and ruxolitinib was discontinued as their disease alleviated, and they did not show signs of relapse during the 3- and 5-month of follow-up examinations.ConclusionWe described two cases of AIDS-related secondary HLH treated with ruxolitinib. Our cases highlight the feasibility of using ruxolitinib as a first-line therapy in patients with HIV infection and secondary HLH. Nevertheless, the safety and efficacy of this novel treatment need to be evaluated in large clinical trials in the future.https://www.frontiersin.org/articles/10.3389/fimmu.2022.1012643/fullruxolitinibhemophagocytic lymphohistiocytosisacquired immune deficiency syndromecytokinesinflammationtherapeutics |
| spellingShingle | Xiang Liu Xueling Zhu Xiaotang Zhou Yirui Xie Dairong Xiang Zhikai Wan Ying Huang Biao Zhu Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS Frontiers in Immunology ruxolitinib hemophagocytic lymphohistiocytosis acquired immune deficiency syndrome cytokines inflammation therapeutics |
| title | Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS |
| title_full | Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS |
| title_fullStr | Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS |
| title_full_unstemmed | Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS |
| title_short | Case report: Ruxolitinib as first-line therapy for secondary hemophagocytic lymphohistiocytosis in patients with AIDS |
| title_sort | case report ruxolitinib as first line therapy for secondary hemophagocytic lymphohistiocytosis in patients with aids |
| topic | ruxolitinib hemophagocytic lymphohistiocytosis acquired immune deficiency syndrome cytokines inflammation therapeutics |
| url | https://www.frontiersin.org/articles/10.3389/fimmu.2022.1012643/full |
| work_keys_str_mv | AT xiangliu casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT xuelingzhu casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT xiaotangzhou casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT yiruixie casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT dairongxiang casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT zhikaiwan casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT yinghuang casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids AT biaozhu casereportruxolitinibasfirstlinetherapyforsecondaryhemophagocyticlymphohistiocytosisinpatientswithaids |