Rapidly lethal secondary hemophagocytic lymphohistiocytosis predicted by fluorodeoxyglucose positron-emission tomography/computed tomography

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...

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Detalles Bibliográficos
Autores principales: Makusha, Lovemore Peter, Pucar, Darko, Young, Colin R.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7745859/
https://www.ncbi.nlm.nih.gov/pubmed/33354187
http://dx.doi.org/10.4103/wjnm.WJNM_73_19
Descripción
Sumario: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 (SUV(max)) >2.52 and bone marrow SUV(max) >3.13 on (18)F(-) fuorodeoxyglucose positron emission tomography/computed tomography ((18)FDG-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 (18)FDG-PET/CT in predicting disease severity and treatment response.

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