A rare disease in the community hospital: Hemophagocytic lymphohistiocytosis (HLH) needs immediate local attention for early recognition and individualized treatment
Oncology Research and Treatment
; 44(SUPPL 2):167, 2021.
Article
in English
| EMBASE | ID: covidwho-1623608
ABSTRACT
Purpose:
HLH is a severe hyperinflammatory syndrome characterized by highly active cytotoxic T-cells, NK-cells, and macrophages. If undiagnosed, HLH can lead to multiorgan damage and death. Conditions triggering HLH are infections, malignancies and autoimmune/-inflammatory (MAS-HLH) disorders. Immunosuppressive patients are prone to develop infection triggered HLH. The incidence in the European community hospital is unknown, as is the number of unrecorded cases. HLH-patients, diagnosed at a single communal hospital with an adjacent catchment area of 500,000 citizens, were reviewed in the context of national and international guidelines.Methods:
From 08/2016 to 11/2020, 13 HLH patients were analysed retrospectively. Both HLH-2004 criteria and the web-based Hscore were used to diagnose HLH. The collected data depicts clinical presentation, underlying disease, laboratory findings, and treatment.Results:
This Study includes 13 HLH-patients (10 male). Median age at diagnosis was 53, ranging from 27-80 years. Most common triggers in our cohort were infections (n=7) and malignancies(n=4). MAS-HLH (n=1) was seen in a Still's disease patient. HLH-related gene mutation was identified (n=1) with a heterozygote mutation in Perforin (PrfA91). Lymphomas of B-as well as T-cell origin (n=2) and AML (n=3) represented main cause in malignancy associated HLH. Viral infections i.e., COVID-19(n=1), RSV (n=1) and EBV (n=1), also bacterial infections like M. tuberculosis (n=1), and the attenuated strain BCG (n=2) were seen in infection associated HLH. Most patients presented with fever (n=9) and splenomegaly (n=4). HLH patients show pancytopenia, peak ferritin levels ranging 1352-185000 ng/ml (median=21600), peak soluble IL-2 receptor levels ranging 2571-21660 U/ml (median=6606), and peak triglyceride levels ranging 175-610 mg/ml (median=227). Hemophagocytosis in bone marrow was found in 6 patients. First line therapy was glucocorticoids (n=12) combined with polyvalent immunoglobulins. Etoposide (n=5) and chemotherapy (n=4) were given to malignancy triggered HLH. Rituximab was applied in EBV-triggered HLH. Anakinra (n=3) and Ruxolitinib (n=4) was given to selected patients. Two patients received cytokine-depletion using adsorption columns Cytosorb®. Multiorgan failure (n=5) was the most common cause of death.Conclusion:
This data provides incidence estimation of HLH in adult patients. Institutional and national measures will be presented to prevent death due to HLH.
anakinra; cytokine; endogenous, compound; etoposide; ferritin; glucocorticoid; immunoglobulin; interleukin, 2, receptor, alpha; perforin; rituximab; ruxolitinib; adsorption; adult; attention; bacterial, strain; bone, marrow; cancer, patient; catchment, area; cause, of, death; clinical, article; cohort, analysis; community, hospital; conference, abstract; controlled, study; coronavirus, disease, 2019; drug, combination; drug, therapy; erythrophagocytosis; European, Union; female; ferritin, blood, level; fever; gene, expression; gene, mutation; genetic, association; hemophagocytic, syndrome; heterozygote; human; incidence; lymphoma; male; middle, aged; multiple, organ, failure; Mycobacterium, bovis, BCG; nonhuman; pancytopenia; practice, guideline; prevention; protein, expression; rare, disease; retrospective, study; splenomegaly; systemic, juvenile, idiopathic, arthritis; T, lymphocyte; triacylglycerol, level; virus, infection
Full text:
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Collection:
Databases of international organizations
Database:
EMBASE
Language:
English
Journal:
Oncology Research and Treatment
Year:
2021
Document Type:
Article
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