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. 2020 Apr 3;33(3):326-330.
doi: 10.1080/08998280.2020.1740052. eCollection 2020 Jul.

Hemophagocytic lymphohistiocytosis in adults

Yadav Pandey  1 , Dinesh Atwal  1 , Manojna Konda  1 , Milan Bimali  2 , Derek Middleton  1 , Naveen Yarlagadda  1 , Belal Firwana  3 , Appalanaidu Sasapu  1
Affiliations

Hemophagocytic lymphohistiocytosis in adults

Yadav Pandey et al. Proc (Bayl Univ Med Cent). .

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an underrecognized disorder due to the variability of its presentation and the fact that in adults, its diagnosis is based on cumbersome, pediatric-based criteria. Data regarding demographics, underlying causes, clinical features, laboratory results, complications, treatments received, and clinical outcomes were collected and analyzed in 41 patients who were diagnosed and treated at University of Arkansas for Medical Sciences between 2007 and 2019. In this group, 51% were male, the median age at diagnosis was 47 years, and 85% (35/41) met the HLH-2004 diagnostic criteria (5/8 variables). When evaluating seven extended variables easily obtained by routine laboratory test, 93% (38/41) of patients met 8 out of 15 criteria. The overall mortality in our patient population was 54% (22/41). The 30-day and 1-year overall survival estimates were 0.73 (95% confidence interval 0.56, 0.84) and 0.46 (95% confidence interval 0.29, 0.62), respectively. Thirty-five patients (85.4%) received HLH-directed therapy, and 19 patients (46.3%) achieved remission. The most common regimen for treating HLH was dexamethasone plus etoposide (53.7%). The patients with malignancy-related HLH had a worse prognosis than those without underlying malignancy, with a 73.33% (11/15) vs 34.62% (9/26) mortality (P = 0.02). In conclusion, despite increasing recognition, HLH remains an enigmatic disorder with increased mortality, even more so with malignancy-associated HLH.

Keywords: Cytopenia; hemophagocytic lymphohistiocytosis; hemophagocytosis.

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Figures

Figure 1.
Figure 1.
Plot of number of criteria vs patients. The plot represents the number of patients (y axis) against ≥ total number of criteria. The numbers within the plot represents the number of patients who experienced the exact number of criteria.
Figure 2.
Figure 2.
(a) Distribution of overall survival. (b) Distribution of overall survival restricted to 365 days.
See this image and copyright information in PMC

References

    1. Filipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow Transplant. 2010;16(1):S82–S89. doi:10.1016/j.bbmt.2009.11.014. - DOI - PubMed
    1. George MR. Hemophagocytic lymphohistiocytosis: review of etiologies and management. J Blood Med. 2014;5:69–86. doi:10.2147/JBM.S46255. - DOI - PMC - PubMed
    1. Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118(15):4041–4052. doi:10.1182/blood-2011-03-278127. - DOI - PMC - PubMed
    1. Machaczka M, Vaktnas J, Klimkowska M, Hagglund H. Malignancy-associated hemophagocytic lymphohistiocytosis in adults: a retrospective population-based analysis from a single center. Leuk Lymphoma. 2011;52(4):613–619. doi:10.3109/10428194.2010.551153. - DOI - PubMed
    1. Clementi R, Emmi L, Maccario R, et al. . Adult onset and atypical presentation of hemophagocytic lymphohistiocytosis in siblings carrying PRF1 mutations. Blood. 2002;100(6):2266–2267. doi:10.1182/blood-2002-04-1030. - DOI - PubMed

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