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• W4386086918 abstract "Topic: 23. Hematopoiesis, stem cells and microenvironment Background: Hemophagocytic Lymphohistiocytosis (HLH) is an often-fatal syndrome of uncontrolled immune activation caused by dysregulation of macrophages and lymphocytes resulting in increased levels of inflammatory cytokines and significant tissue damage. Here we present 20 cases of adult secondary HLH that were identified and treated at Nashik Hematology Services in a period of two years. Aims: To highlight the presentation and etiology of secondary HLH and the importance of prompt recognition and early treatment. Methods: Between Jan 2020 to January 2023 20 adult patients >18 years old were identified at Nashik Hematology Services that met criteria and were treated for adult secondary HLH. All 20 patients had 5 out of 8 of the following findings established in the HLH-2004 guidelines: fever ≥ 38.5 C°, splenomegaly, cytopenias affecting 2 of 3 lineages in the peripheral blood (hemoglobin <9 g/dL, platelets <100x103/mL, neutrophils <1x103/mL), hypertriglyceridemia (fasting >265 mg/dL) and/or hypofibrinogenemia (<150 mg/dL), hemophagocytosis in the bone marrow, spleen, lymph nodes or liver, low or absent NK cell activity, ferritin >500 ng/mL and elevated sIL2R. Patients were treated with immunotherapy mainly dexamethasone and Ivig based on the previous experience of treatment of secondary HLH, as well as treatment targeted at an underlying malignancy or infection. Patient demographics and clinical characteristics, underlying etiology of secondary HLH and treatment modalities were compared. Results: All 20 patients included in this study met criteria for secondary HLH as defined above. The median age at diagnosis was 30 (range 22-68 years). Five out of 20 (25%) patients had Dengue fever. The median ferritin at diagnosis was 3000 ng/mL (range 1,000-10,500 ng/mL). Patient clinical manifestations are summarized in Table 2. All patients presented with fevers, elevated ferritin, cytopenias and splenomegaly and 15/20(75%) patients had evidence of hemophagocytosis in the bone marrow. 19 patients (95%) had secondary HLH due to an infection and 1(5%) had primary HLH. All patients received Dexamethasone and treatment for underling infection. At the time of this report, 5 patients had died (25 %), 14 were alive and 1 patient was lost to follow up. Summary/Conclusion: Diagnosis of the adult secondary HLH remains a challenge due to overlap in symptoms and lack of specificity of laboratory findings. Treatment is often postponed due to delayed recognition of this deadly disease. A high degree of medical suspicion in the appropriate clinical context should prompt early treatment with an Dexamethazone and Etoposide-containing regimen. Prospective studies are warranted in this area.Keywords: Hemophagocytic Lymphohistiocytosis (HLH)" @default.
• W4386086918 created "2023-08-24" @default.
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• W4386086918 date "2023-08-01" @default.
• W4386086918 modified "2023-09-26" @default.
• W4386086918 title "PB2487: SECONDARY HLH: RETROSPECTIVE DATA FROM TERTIARY CARE CENTRE." @default.
• W4386086918 doi "https://doi.org/10.1097/01.hs9.0000976652.16479.87" @default.
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