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We reported a case of secondary hemophagocytic lymphohistiocytosis (HLH), a rare and life-threatening condition, which was suspected to have been triggered by a severe case of coronavirus disease 2019 (COVID-19). A 50-year-old man with a past medical history of ulcerative colitis with recent pancolitis status post colectomy and ileostomy two weeks before presentation presented to the emergency department with one week of subjective fevers, weakness, watery diarrhea, and decreased oral intake. A CT scan showed fluid in the rectum and post-surgical changes from his recent colectomy along with diffuse reticulonodular opacities of the lungs. His COVID-19 reverse transcriptase-polymerase chain reaction (RT-PCR) test was positive. Over the subsequent days, the patient's condition worsened as he developed worsening acute hypoxic respiratory failure with diffuse lymphadenopathy, splenomegaly, worsening cytopenias, and increased ferritin of >100,000 ng/ml on hospital day six. Hematology oncology was consulted and he was started on empiric steroid therapy followed by etoposide. However, his condition continued to worsen, and eventually, the patient passed away on hospital day eight.
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http://dx.doi.org/10.7759/cureus.34022 | DOI Listing |
Front Immunol
September 2025
Department of Hematology, Qingdao Women and Children's Hospital, Qingdao, China.
Familial hemophagocytic lymphohistiocytosis type 2 (FHL2), caused by perforin 1 (PRF 1), is a rare and fatal autosomal recessive disorder characterized by a hyperinflammatory syndrome and the accumulation of activated T lymphocytes and histiocytes in the reticuloendothelial system. Autoimmune lymphoproliferative syndrome (ALPS) is an autoimmune disease that typically presents in children with lymphadenopathy, splenomegaly, and cytopenias or lymphomas. We report a case of a 9-year-old boy who was newly diagnosed with FHL, carrying a new type of compound heterozygous mutations (c.
View Article and Find Full Text PDFJ Med Virol
September 2025
Department of Pathology, West China Hospital, Sichuan University, Chengdu, China.
Systemic chronic active Epstein-Barr virus disease (sCAEBVD) can primarily involve skeletal muscle to form CAEBV-myopathy (CAEBV-M), which may resemble idiopathic inflammatory myopathies (IIMs). This study reports an 11-patient, 10-year cohort of CAEBV-M to summarize clinicoseropathologic features. CAEBV-M typically affects young adults (median: 29 years), with universal limb swelling, frequent disseminated muscle involvement (73%), and systemic symptoms like fever (82%), splenomegaly (82%), and hemophagocytic lymphohistiocytosis (55%).
View Article and Find Full Text PDFFront Immunol
September 2025
Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Chongqing, China.
Background: UNC13D deficiency is the most common form of familial hemophagocytic lymphohistiocytosis (FHL) in Asia. Hypogammaglobulinemia is a rare phenotype observed in both patients with FHL3 and sporadic hemophagocytic lymphohistiocytosis (HLH). Our observations suggest that UNC13D deficiency with hypogammaglobulinemia presents a distinct clinical phenotype compared to other HLH patients.
View Article and Find Full Text PDFIndian J Nephrol
August 2024
Department of Nephrology, Government Stanley Medical College & Hospital, Chennai, Tamil Nadu, India.
A syndrome of exaggerated lymphocytic proliferation and activation, called hemophagocytic lymphohistiocytosis (HLH) can occur primarily due to genetic mutation, in children and secondary to infection, malignancy or autoimmunity in adults. It is characterized by a misdirected activation of immune system, which causes cytokine release from macrophages and cytotoxic cells, in an uncontrolled fashion. Most treatment protocols are formulated for primary hemophagocytic histiocytosis, which occurs in children, whereas awareness and therapeutic guidelines for the secondary form of the disease which affects predominantly the adults is limited.
View Article and Find Full Text PDFCureus
July 2025
Transplant Surgery, Willis Knighton Health, Shreveport, USA.
Hemophagocytic lymphohistiocytosis (HLH) is a rare disease more commonly found in neonates and adolescents. It can be classified as primary/genetic or acquired following viral infection, lymphomas, or autoimmune diseases. There is not enough evidence of the prevalence of this disease in adults, but, when diagnosed, it is most commonly secondary to viruses, such as Epstein-Barr virus (EBV).
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