Exploring the Clinical Spectrum and Hematological Patterns of Hemophagocytic Lymphohistiocytosis: A Comprehensive Study

Abstract

Background: Hemophagocytic Lymphohistiocytosis (HLH) presents a challenging disorder characterized by complex manifestations resulting from dysregulated immune responses. This study aims to enhance our understanding of the clinical and hematological spectrum of HLH, focusing on adult patients. Methods: A retrospective-prospective observational study was conducted involving 64 adult HLH patients meeting HLH-2004 criteria. Comprehensive hematopathological assessments were performed, encompassing clinical parameters, hematological markers, and mortality rates. Data analysis utilized SPSS Version 20.0 for descriptive statistics. Results: The most frequent clinical manifestations were fever (100%), splenomegaly (87.5%), bicytopenia (73.4%), hypertriglyceridemia (60.9%), hyperferritenemia (48.4%), hypofibrinogenemia (32.8%), and pancytopenia (26.6%). We observed that leucopenia was present in majority of patients (62.5%), and leukocytosis was present in 17.2% patients. About 65.6% had mild to moderate thrombocytopenia, followed by 14.1% patients with severe thrombocytopenia, 14.1% had normal platelet count and 6.3% had thrombocytosis. Conclusion: The study revealed a diverse clinical spectrum within adult HLH patients, including universal fever, varied hematological irregularities, and mortality rates of 7.8%. These findings emphasize the complexity of HLH and the importance of early diagnosis and tailored interventions for improved outcomes. This study contributes valuable insights to the understanding of HLH's intricate clinical and hematological aspects, informing more informed diagnostic and therapeutic strategies for this complex disorder. Keywords: Hemophagocytic lymphohistiocytosis (HLH), Clinical characteristics, Laboratory tests, Prognosis