ArticleUpsala journal of medical sciences2015
Secondary haemophagocytic lymphohistiocytosis: Experience from the Uppsala University Hospital.
Article in Upsala journal of medical sciences, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
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Who cites it
21 citing papers in PubMed, 36 citations in OpenAlex.
- Crimean-Congo Hemorrhagic Fever in Children: Secondary Hemophagocytic Lymphohistiocytosis and Time-course of Biomarkers in a Single-Center Cohort (2019-25).Journal of tropical pediatrics · 2026Article
- Consensus recommendations for the diagnosis and management of hemophagocytic lymphohistiocytosis in the Gulf Cooperation Council: a modified Delphi approach.Frontiers in immunology · 2026Article
- Article
- Hemophagocytic Lymphohistiocytosis Associated With T-cell Lymphoma in Pregnancy: Diagnostic Conundrum Unveiled.Cureus · 2024Article
- Unique presentation of T-cell/histiocyte-rich large B cell lymphoma complicated with hemophagocytic lymphohistiocytosis: Case report and review of the literature.Journal of medical biochemistry · 2024Article
- Impaired STING Activation Due to a Variant in the E3 Ubiquitin Ligase AMFR in a Patient with Severe VZV Infection and Hemophagocytic Lymphohistiocytosis.Journal of clinical immunology · 2024Article
- Article
- Into the storm: the imbalance in the yin-yang immune response as the commonality of cytokine storm syndromes.Frontiers in immunology · 2024Review
- COVID-19 Complications: Oxidative Stress, Inflammation, and Mitochondrial and Endothelial Dysfunction.International journal of molecular sciences · 2023Review
- Article
- Review
- Article
- Hemophagocytic Lymphohistiocytosis in the Setting of Therapy-Induced Acute Myeloid Leukemia: An Autopsy Report.Diseases (Basel, Switzerland) · 2022Article
- B-cell lymphoma-associated hemophagocytic lymphohistiocytosis: A case report.Oncology letters · 2022Article
- Effective Natural Killer Cell Degranulation Is an Essential Key in COVID-19 Evolution.International journal of molecular sciences · 2022Observational
- COVID-19-A Trigger Factor for Severe Immune-Mediated Thrombocytopenia in Active Rheumatoid Arthritis.Life (Basel, Switzerland) · 2022Article
- A mild, self-resolving case of Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis.IDCases · 2022Article
- Psychological and neuropsychiatric implications of COVID-19.European archives of psychiatry and clinical neuroscience · 2021Review
- Intensive care unit complications and outcomes of adult patients with hemophagocytic lymphohistiocytosis: A retrospective study of 16 cases.World journal of critical care medicine · 2018Article
- Rituximab, etoposide, methylprednisolone, high-dose cytarabine, and cisplatin in the treatment of secondary hemophagocytic lymphohistiocytosis with classical Hodgkin lymphoma: a case report and review of the literature.Journal of medical case reports · 2016Review
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1 author at 1 institution in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
backgroundHaemophagocytic lymphohistiocytosis (HLH) is a rare clinical syndrome characterized by fever, hepatosplenomegaly, cytopenia, and progressive multiple-organ failure. HLH in adults is often secondary to autoimmune diseases, cancer, or infections in contrast to familial HLH. Treatment of secondary HLH is directed against the triggering disease in addition to immunosuppressive therapy, the latter commonly according to the HLH-2004 protocol.
methodsWe conducted a retrospective study to identify triggering diseases, disease-specific and immunosuppressive therapy administered, and prognosis in adult patients with secondary HLH. Patient data were collected from October 2010 to January 2015.
resultsTen adult patients with secondary HLH were identified. Seven were men, and the median age at diagnosis was 62 years. Five cases were triggered by malignant disease and five by infection. The median patient fulfilled five of the eight HLH-2004 diagnostic criteria. All patients fulfilled the criteria fever, cytopenia, and ferritin >500 µg/L. Median time from hospital admission to HLH diagnosis was 20 days. Four patients received immunosuppressive therapy according to the HLH-2004 protocol. The prognosis was dismal, especially for the patients with malignancy-associated HLH, of whom all died.
conclusionHLH should be suspected in patients who present with fever, cytopenia, and ferritin >500 µg/L. Secondary HLH has a dismal prognosis. None of the patients with HLH triggered by malignancy survived. Achieving remission of the triggering disease seems to be important for a favourable outcome as, in all surviving patients, the haemophagocytic syndrome resolved after remission of the underlying infection.
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