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Usual and Unusual Manifestations of Familial Hemophagocytic Lymphohistiocytosis and Langerhans Cell Histiocytosis. Pediatr Clin North Am 2017 Feb;64(1):91-109

Date

11/30/2016

Pubmed ID

27894453

DOI

10.1016/j.pcl.2016.08.006

Scopus ID

2-s2.0-84998773661   6 Citations

Abstract

Familial hemophagocytic lymphohistiocytosis (FHL) and Langerhans cell histiocytosis (LCH) are histiocytic diseases that occur most commonly in young children. Improvements in recognition and treatment have been substantial for both diseases in the past decade, although early and late morbidity continue to be major concerns. These two diagnoses behave differently, although the clinical spectra for both diseases are diverse and can lead to confusion and delays in diagnosis and treatment. This article focuses on the clinical and genetic spectrum of FHL as well as the clinical and treatment variations of LCH.

Author List

Erker C, Harker-Murray P, Talano JA

Authors

Paul D. Harker-Murray MD, PhD Associate Professor in the Pediatrics department at Medical College of Wisconsin
Julie-An M. Talano MD Professor in the Pediatrics department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Child
Delayed Diagnosis
Diagnosis, Differential
Diagnostic Errors
Histiocytosis, Langerhans-Cell
Humans
Lymphohistiocytosis, Hemophagocytic
Rare Diseases