| Title: |
Hemophagocytic lymphohistiocytosis in the context of hepatitis-associated severe aplastic anemia: A case report |
| Authors: |
Xu, Jianli; Yuan, Hailong; Chen, Gang; Qu, Jianhua; Wang, Huan; Chen, Rong; Jiang, Ming |
| Contributors: |
the Xinjiang Uygur Autonomous Region Natural Science Fund; the National Natural Science Foundation of China; the Translational Research Project of the National Clinical Research Center for Hematological Diseases |
| Source: |
Medicine ; volume 104, issue 44, page e45430 ; ISSN 0025-7974 1536-5964 |
| Publisher Information: |
Ovid Technologies (Wolters Kluwer Health) |
| Publication Year: |
2025 |
| Description: |
Rationale: Hemophagocytic lymphohistiocytosis (HLH) is a severe immune dysregulation syndrome. Hepatitis-associated severe aplastic anemia (SAA) is a specific subtype of acquired aplastic anemia characterized by concomitant hepatitis, leading to a challenging diagnosis and poor prognosis. Here, we present a rare case of HLH in the context of hepatitis-associated SAA, emphasizing the need for early recognition and individualized management. Patient concerns: The patient, a 19-year-old male, presented with recurrent fever, jaundice, and cytopenias, indicative of hepatitis. Diagnoses: Bone marrow aspiration and biopsy suggested SAA, while peripheral blood next-generation sequencing detected Candida tropicalis infection on 2 separate occasions. Despite combination antifungal therapy with amphotericin B and posaconazole, the bilirubin level continued to rise. Additional tests revealed an elevated soluble CD25 level of 3659 U/mL, decreased natural killer cell activity of 1.8%, and a serum ferritin level > 2000 μg/L. The final diagnosis was HLH. Interventions: Dexamethasone and ruxolitinib were administered to control the fever. Subsequently, the patient underwent HLA-matched allogeneic bone marrow and peripheral blood hematopoietic stem cell transplantation for SAA. Outcomes: The patient’s symptoms, including fever and fatigue, resolved. Lessons: This case highlights that HLH should be considered in SAA patients with unexplained fever, poor response to anti-infectives, and elevated bilirubin. A treatment strategy addressing both bone marrow failure and hyperinflammation is essential. In this patient, allogeneic hematopoietic stem cell transplantation served as a successful curative intervention by targeting the shared pathophysiology of HLH and SAA. However, given the inherent limitations of a single case, the generalizability of our findings and the efficacy of allogeneic hematopoietic stem cell transplantation require validation through larger, controlled studies. |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| DOI: |
10.1097/md.0000000000045430 |
| DOI: |
10.1097/MD.0000000000045430 |
| Availability: |
https://doi.org/10.1097/md.0000000000045430; https://journals.lww.com/10.1097/MD.0000000000045430 |
| Rights: |
http://creativecommons.org/licenses/by/4.0/ |
| Accession Number: |
edsbas.315AFEA9 |
| Database: |
BASE |