Original Research Article
Year: 2023 | Month: August | Volume: 10 | Issue: 8 | Pages: 823-831
DOI: https://doi.org/10.52403/ijrr.202308106
Exploring the Clinical Spectrum and Hematological Patterns of Hemophagocytic Lymphohistiocytosis: A Comprehensive Study
Dr Asima1, Dr Fahim Manzoor2, Dr Rohi3, Dr Nusrat Basir4, Dr Shareefa Akhtar5, Dr Sadaqat6, Dr S. Bilal7
1GMC Srinagar
2Associate Professor, Pathology, GMC Srinagar
3Associate professor, GMC Srinagar
4Associate Professor, GMC Srinagar
5Assistant Professor, GMC Srinagar
6Assistant Professor. Medicine, GMC Srinagar
7Professor and Head Pathology, GMC Srinagar
Corresponding Author: Dr Asima
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
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