Hemophagocytic Lymphohistiocytosis: A Case Report

The hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH) is a syndrome with apoptosis deficiency that results in the impairment of a regulatory pathway with consequent immune and inflammatory responses. Fever, cytopenias, splenomegaly, and hemophagocytosis are cardinal signs. It...

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Bibliographic Details
Published in:Curēus (Palo Alto, CA) CA), 2024-05, Vol.16 (5), p.e61015
Main Authors: Costa E Silva, Teresa, Jorge Alves, Hugo, Horta Antunes, João, Noronha, Carla, Sousa Picado, Bárbara
Format: Article
Language:English
Subjects:
Antibiotics
Blood
Blood platelets
Bone marrow
Case reports
Cytokines
Disease
Emergency Medicine
Fever
Hematology
Hemoglobin
Hypertension
Infections
Internal Medicine
Laboratories
Leukocytes
Lymphatic system
Neutrophils
Patients
Sepsis
Triglycerides
Urine
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Summary:The hemophagocytic syndrome (HS) or hemophagocytic lymphohistiocytosis (HLH) is a syndrome with apoptosis deficiency that results in the impairment of a regulatory pathway with consequent immune and inflammatory responses. Fever, cytopenias, splenomegaly, and hemophagocytosis are cardinal signs. It may be familial or secondary to infection, autoimmunity, or neoplasia. Impaired natural killer (NK)-cell cytotoxicity is the hallmark of HLH. All genetic defects in familial HLH are related to granule-dependent cytotoxicity. The authors present a 50-year-old black female patient with a history of drepanocytosis who attended the emergency department due to fever, asthenia, lethargy, and hypogastric pain. Her laboratory workup on admission revealed severe pancytopenia. She was ultimately diagnosed with HLH due to sepsis of urinary origin, with a fatal outcome. HLH is a rare and life-threatening syndrome. The delay in its diagnosis due to the variability of the clinical and laboratory findings constitutes the main obstacle to a successful prognosis, as illustrated in this case report.
ISSN:2168-8184
2168-8184
DOI:10.7759/cureus.61015