title: Usual and Unusual Manifestations of Familial Hemophagocytic Lymphohistiocytosis and Langerhans Cell Histiocytosis.
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.
authors.affiliation: Division of Pediatric Hematology/Oncology/Blood and Marrow Transplant, Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA.
authors.forename: Craig
authors.initials: C
authors.lastname: Erker
authors.affiliation: Division of Pediatric Hematology/Oncology/Blood and Marrow Transplant, Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA.
authors.forename: Paul
authors.initials: P
authors.lastname: Harker-Murray
authors.affiliation: Division of Pediatric Hematology/Oncology/Blood and Marrow Transplant, Medical College of Wisconsin, 8701 Watertown Plank Road, MFRC 3018, Milwaukee, WI 53226, USA. Electronic address: jtalano@mcw.edu.
authors.forename: Julie-An
authors.initials: JA
authors.lastname: Talano
mesh_terms: D002648:Child; D057210:Delayed Diagnosis; D003937:Diagnosis, Differential; D003951:Diagnostic Errors; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D035583:Rare Diseases / Q000175:diagnosis*
keywords: Familial hemophagocytic lymphohistiocytosis (FHL); Hemophagocytic lymphohistiocytosis (HLH); Histiocytic disorders; Langerhans cell histiocytosis (LCH)