Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series
Abstract Background Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory syndrome resulting from uncontrolled activation of T cells and macrophages, frequently leading to multiorgan failure. Severe lactic acidosis (lactate ≥ 10 mmol/L), a rare yet critical manifestation, poses...
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2025-08-01
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| Series: | Journal of Medical Case Reports |
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| Online Access: | https://doi.org/10.1186/s13256-025-05335-7 |
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| author | Tianqi Xu Ming Tang Meiqing Wu Xiaoyan Xue |
| author_facet | Tianqi Xu Ming Tang Meiqing Wu Xiaoyan Xue |
| author_sort | Tianqi Xu |
| collection | DOAJ |
| description | Abstract Background Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory syndrome resulting from uncontrolled activation of T cells and macrophages, frequently leading to multiorgan failure. Severe lactic acidosis (lactate ≥ 10 mmol/L), a rare yet critical manifestation, poses unique diagnostic and therapeutic challenges in the intensive care unit. Here, we report two cases of Chinese men with hemophagocytic lymphohistiocytosis presenting with extreme lactic acidosis, highlighting the necessity of early hemophagocytic lymphohistiocytosis screening in intensive care unit patients with unexplained hyperlactatemia. Case presentation Case 1: A 43-year-old Chinese male with diffuse large B cell lymphoma developed hemophagocytic lymphohistiocytosis, presenting with lactate 14.2 mmol/L, cytopenia, and hyperferritinemia. Etoposide therapy rapidly normalized lactate levels (within 24 hours) and led to complete recovery after autologous stem cell transplantation. Case 2: A 60-year-old Chinese male with chronic gout and soft tissue infection developed septic shock and reactive hemophagocytic lymphohistiocytosis (lactate 14.3 mmol/L). Despite initial response to etoposide, he succumbed to invasive aspergillosis due to treatment-related immunosuppression. Conclusion Extreme lactic acidosis in intensive care unit patients should prompt urgent hemophagocytic lymphohistiocytosis evaluation, particularly in Chinese populations. Etoposide is effective for malignancy-associated hemophagocytic lymphohistiocytosis, while reactive hemophagocytic lymphohistiocytosis may require tailored immunosuppression with strict infection prophylaxis. Routine reporting of patient ethnicity aids epidemiological understanding of rare diseases such as hemophagocytic lymphohistiocytosis. |
| format | Article |
| id | doaj-art-e6cf01d7b4f94981b82fb23a30791a39 |
| institution | Kabale University |
| issn | 1752-1947 |
| language | English |
| publishDate | 2025-08-01 |
| publisher | BMC |
| record_format | Article |
| series | Journal of Medical Case Reports |
| spelling | doaj-art-e6cf01d7b4f94981b82fb23a30791a392025-08-24T11:31:43ZengBMCJournal of Medical Case Reports1752-19472025-08-011911610.1186/s13256-025-05335-7Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case seriesTianqi Xu0Ming Tang1Meiqing Wu2Xiaoyan Xue3Intensive Care Medicine Department, Aerospace Center HospitalIntensive Care Medicine Department, Aerospace Center HospitalIntensive Care Medicine Department, Aerospace Center HospitalIntensive Care Medicine Department, Aerospace Center HospitalAbstract Background Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammatory syndrome resulting from uncontrolled activation of T cells and macrophages, frequently leading to multiorgan failure. Severe lactic acidosis (lactate ≥ 10 mmol/L), a rare yet critical manifestation, poses unique diagnostic and therapeutic challenges in the intensive care unit. Here, we report two cases of Chinese men with hemophagocytic lymphohistiocytosis presenting with extreme lactic acidosis, highlighting the necessity of early hemophagocytic lymphohistiocytosis screening in intensive care unit patients with unexplained hyperlactatemia. Case presentation Case 1: A 43-year-old Chinese male with diffuse large B cell lymphoma developed hemophagocytic lymphohistiocytosis, presenting with lactate 14.2 mmol/L, cytopenia, and hyperferritinemia. Etoposide therapy rapidly normalized lactate levels (within 24 hours) and led to complete recovery after autologous stem cell transplantation. Case 2: A 60-year-old Chinese male with chronic gout and soft tissue infection developed septic shock and reactive hemophagocytic lymphohistiocytosis (lactate 14.3 mmol/L). Despite initial response to etoposide, he succumbed to invasive aspergillosis due to treatment-related immunosuppression. Conclusion Extreme lactic acidosis in intensive care unit patients should prompt urgent hemophagocytic lymphohistiocytosis evaluation, particularly in Chinese populations. Etoposide is effective for malignancy-associated hemophagocytic lymphohistiocytosis, while reactive hemophagocytic lymphohistiocytosis may require tailored immunosuppression with strict infection prophylaxis. Routine reporting of patient ethnicity aids epidemiological understanding of rare diseases such as hemophagocytic lymphohistiocytosis.https://doi.org/10.1186/s13256-025-05335-7Hemophagocytic lymphohistiocytosisHyperlactatemiaICUCase series |
| spellingShingle | Tianqi Xu Ming Tang Meiqing Wu Xiaoyan Xue Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series Journal of Medical Case Reports Hemophagocytic lymphohistiocytosis Hyperlactatemia ICU Case series |
| title | Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series |
| title_full | Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series |
| title_fullStr | Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series |
| title_full_unstemmed | Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series |
| title_short | Lactic acidosis in hemophagocytic syndrome: diagnostic and therapeutic challenges in the intensive care unit—a case series |
| title_sort | lactic acidosis in hemophagocytic syndrome diagnostic and therapeutic challenges in the intensive care unit a case series |
| topic | Hemophagocytic lymphohistiocytosis Hyperlactatemia ICU Case series |
| url | https://doi.org/10.1186/s13256-025-05335-7 |
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