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case_images: The clinical course of HLH case 1: A 21-year-old woman was admitted for persistent high fever and diagnosed with HLH based on the HLH-2004 guidelines. Soon after corticosteroid (CS) therapy treatment was initiated, her fever resolved and laboratory abnormalities improved. CS was reduced and the treatment was completed in two months.
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case_images: HLH Case 1 received only corticosteroid treatment and improved within a week (Figure 1).
case_images: The clinical course of HLH case 2: A 40-year-old woman was admitted for persistent high fever and diagnosed with HLH based on the HLH-2004 guidelines. Corticosteroid therapy resulted in only partial fever relief and no improvement of laboratory findings. Cyclosporine, Acyclovir, recombinant thrombomodulin, antimicrobial agents, and repeated mPSL pulse therapy were unsuccessful. The patient died from a cerebral hemorrhage on day 18.
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case_images: HLH Case 2 was treated with corticosteroid, cyclosporine, etoposide, and several antimicrobial agents including ceftriaxone, meropenem, sitafloxacin, and acyclovir; however, she died 18 days after presentation (Figure 2).
case_images: Flow cytometric analysis of lymphocytes in bone marrow (A) CD45 gating. The population with the highest CD45 fluorescence intensity corresponds to lymphocytes. (B) CD38+ cells were dominant in both cases. (C) Both CD4+ cells and CD8+ cells existed in case 1; however, CD8+ cells were dominant in case 2. (D) CD5 expression was reduced significantly in case 2, but not in case 1. These findings suggest clonal proliferation of CD5-CD8+ T lymphocytes in case 2.
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case_images: The CD38+ lymphocytes in the bone marrow of the HLH patients were analyzed with FCM (CD45-SSC gating; Figure 3).