Hemophagocytic lymphohistiocytosis complicated by central nervous system lesions in a patient with dermatomyositis: a case presentation and literature review

Modern Rheumatology
Hiroyuki YamashitaAkio Mimori

Abstract

We report a case of dermatomyositis (DM) and hemophagocytic lymphohistiocytosis (HLH) complicated by central nervous system (CNS) lesions and review eight literature cases of DM and HLH. A 17-year-old woman, admitted to our hospital because of severe muscle weakness and high fever, was diagnosed with DM based on elevated serum levels of muscle enzymes and a typical skin rash. Pancytopenia, high serum ferritin and soluble interleukin (IL)-2 receptor, and hepatosplenomegaly were also noted. Bone-marrow examination was negative for hemophagocytosis. Steroid therapy combined with immunoglobulin i.v. was ineffective against the DM, pancytopenia, hepatic dysfunction, and hyperferritinemia. On the 27th hospital day, seizures and acute respiratory failure occurred. In the course of improving muscle enzyme levels after starting adjunctive treatment with cyclosporine, the patient suffered disturbed consciousness, dyskinesia, and tremor. Brain magnetic resonance imaging (MRI) revealed T2 hyperintense lesions in the pons. Additional cyclophosphamide pulse therapy successfully decreased serum ferritin. Unfortunately, the diffuse alveolar damage (DAD) confirmed by biopsy progressed and the patient died. Autopsy findings revealed DAD througho...Continue Reading

References

Jan 1, 1986·Dermatologica·C CooperJ H Barth
Oct 1, 1995·American Journal of Hematology·S KumakuraS Kobayashi
Jan 1, 1997·The American Journal of Medicine·S KumakuraS Kobayashi
Feb 11, 1999·British Journal of Rheumatology·S YasudaT Koike
May 28, 2003·Pediatric Radiology·Nancy E Fitzgerald, Kenneth L MacClain
Dec 1, 1952·Archives of Disease in Childhood·J W FARQUHAR, A E CLAIREAUX
Jun 4, 2005·Arthritis and Rheumatism·Avril A FitzgeraldCharles A Dinarello
Apr 6, 2006·Nihon Rinshō Men'eki Gakkai kaishi = Japanese journal of clinical immunology·Takahiko SugiharaTetsushi Sakurai
Apr 22, 2009·Therapeutic Apheresis and Dialysis : Official Peer-reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy·Hyogo NakakuraHiroshi Tamai
Jan 18, 2012·Annual Review of Medicine·G E Janka

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Citations

Nov 12, 2015·Clinical and Molecular Allergy : CMA·Paola ParronchiCristina Scaletti
Aug 11, 2015·Clinical Imaging·Tara L AndersonTimothy J Kaufmann
Jan 29, 2014·Journal of Medical Case Reports·Ilse Gradwohl-MatisMartin W Dünser
Sep 19, 2019·Rheumatology International·Dimitri Poddighe, Kaisar Dauyey

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