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. 2016 Dec 20:7:36-40.
doi: 10.1016/j.bbacli.2016.12.002. eCollection 2017 Jun.

Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

Abdul Rashid Shah  1 , Tariq Muzzafar  2 , Rita Assi  1 , Dawid Schellingerhout  3 , Zeev Estrov  1 , Gevorg Tamamyan  4 , Hagop Kantarjian  1 , Naval Daver  1
Affiliations

Hemophagocytic lymphohistiocytosis in adults: An under recognized entity

Abdul Rashid Shah et al. BBA Clin. .

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation with macrophage and T-cell infiltration resulting in, multi organ damage. HLH may be primary or secondary in etiology. A high index of suspicion is essential for early diagnosis and treatment. Diagnostic criteria need to be refined and newer treatment options to be explored in order to improve survival especially in adult HLH and malignancy-associated HLH (M-HLH). We report a case of malignancy associated HLH (M-HLH) in adult treated on one of the only FDA-approved protocols for adult HLH to highlight the diagnostic and therapeutic challenges of this disease entity.

Keywords: Adults; Ferritin; Hemophagocytosis; Lymphohistiocytosis.

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Figures

Fig. 1
Fig. 1
(A) Fluid attenuated inversion recovery image demonstrates diffuse white matter signal involving the right temporal, occipital and parietal lobes. There is mass effect and midline shift to the left, with narrowing of the paramesencephalic cistern and compression of the uncus into the right cerebral peduncle. (B) Susceptibility image demonstrates a left anterior capsular/caudate focus of hemorrhage (arrow) with surrounding edema. (C) The equivalent FLAIR image shows surrounding edema.
Fig. 2
Fig. 2
(A) H&E section of clot shows an infiltrate of histiocytes (arrow). (B) Immunohistochemical studies for CD68 demonstrate markedly increased histiocytes in bone marrow. (C) & (D) Aspirate smears demonstrate scattered histiocytic cells containing phagocytized red blood cells.
Fig. 3
Fig. 3
Baseline and on treatment trends of ferritin (A), lactate dehydrogenase (B), fibrinogen (C) and triglycerides (D). Protocol treatment start date was 10/2/15.
See this image and copyright information in PMC

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