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DOI: 10.4103/wjnm.WJNM_73_19
Rapidly lethal secondary hemophagocytic lymphohistiocytosis predicted by fluorodeoxyglucose positron-emission tomography/computed tomography

Abstract
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a rare disease with either an indolent or aggressive course. A 29-year-old male presented with fever, polyarthralgias, splenomegaly, retroperitoneal adenopathy, and laboratory findings consistent with Epstein—Barr-mediated sHLH. Consistent with a prior survival analysis by Kim et al., splenic maximum standardized uptake value (SUVmax) >2.52 and bone marrow SUVmax>3.13 on 18F-fuorodeoxyglucose positron emission tomography/computed tomography (18FDG-PET/CT) predicted an aggressive disease with poor treatment response. Despite optimal treatment, the patient rapidly progressed to death within 3 months of symptom onset. This case underscores the potential lethal nature of sHLH, and the evolving role of 18FDG-PET/CT in predicting disease severity and treatment response.
Keywords
Fluorodeoxyglucose positron emission tomography/computed tomography - maximum standardized uptake value - rapidly lethal - secondary hemophagocytic lymphohistiocytosis - survival analysisFinancial support and sponsorship
Nil.
Publication History
Received: 27 September 2019
Accepted: 06 October 2019
Article published online:
19 April 2022
© 2020. Sociedade Brasileira de Neurocirurgia. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commecial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/)
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