CLINICAL AND PARACLINICAL CHARACTERISTICS OF HEMOPHAGOCYTIC SYNDROME IN ADULTS AT BACH MAI HOSPITAL

Ha Le Thu1, , Huong Nguyen Thi Lan1, Huong Bui Thu1, Vinh Pham Quang1, Nga Van Thi1
1 Trung tâm Huyết học và Truyền máu, Bệnh viện Bạch Mai

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Abstract

Objectives: To describe the clinical and paraclinical characteristics of patients diagnosed with hemophagocytic lymphohistiocytosis at Bach Mai Hospital and to explore related factors of hemophagocytic lymphohistiocytosis. Participants and Methods: The study was conducted on 34 patients with hemophagocytic lymphohistiocytosis (HCTBM) diagnosed and treated at the Center for Hematology and Blood Transfusion, Bach Mai Hospital. Results: The average age of the study group was 42 years old, in which the most common age group was from 20 to 50 years old, the male/female ratio was 1.6/1. The most common clinical symptoms were fever (100%), anemia (94.1%), splenomegaly (73.5%), and bleeding (52.9%). Peripheral blood cell characteristics mainly showed thrombocytopenia and neutropenia accounting for over 60%, 70.1% of patients had decreased Fibrinogen below 1.5 g/l, 100% of patients had increased Ferritin, LDH. 18/34 patients had 5/8 diagnostic criteria for phagocytic syndrome, 11/34 patients had 6/8 criteria and 5/34 patients had 7/8 criteria. The rate of infectious agents related to HCTBM was the highest, accounting for 58.8%, viral infection accounted for 26.5%. Among the 29.1% of patients with malignant blood diseases and HCTBM, 70% of patients had T-cell non-Hodgkin lymphoma and 30% had T-cell acute lymphoblastic leukemia. Conclusion: Hemophagocytic Lymphohistiocytosis (HLH) in adults at Bach Mai Hospital predominantly affects middle-aged males, presenting with characteristic manifestations of fever, anemia, and splenomegaly. The condition is associated with distinct hematological abnormalities, and is frequently linked to bacterial infections, with a significant proportion of cases concurrent with T-cell lymphoid malignancies.

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References

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