title: Hemosiderin deposits in chronic graft-vs.-host disease related myopathy.
abstract: Chronic graft-vs.-host disease (cGVHD) occurs in 20-50% of patients who survive for at least 100 d after allogeneic stem cell transplantation (SCT). cGVHD includes scleroderma-like skin changes, chronic cholangitis, obstructive lung disease and general wasting syndrome. Polymyositis or myopathy are rare manifestations of cGVHD with approximately 40 reported cases. Polymyositis accompanied by hemosiderin deposits in cGVHD has been reported only once, and there are no reports on lipofuscin deposits in skeletal muscle cells in cGVHD. We report here on a 56-yr-old male who underwent allogeneic SCT in 1999 for osteomyelofibrosis and progressive hematopoietic insufficiency. In February 2004, the patient was hospitalized for progressive muscular weakness with loss of the ability to walk. Laboratory tests demonstrated normal values for serum creatine kinase, aldolase and lactic dehydrogenase; the ferritin level was highly elevated. The femoral muscle biopsy showed mostly perifascicular atrophy as well as numerous subsarcolemmal hemosiderin and lipofuscin deposits. Intravenous administration of the chelating agent deferoxamine was ineffective. Three weeks later the patient died of aspiration pneumonia. Interestingly, autopsy disclosed moderate hemosiderin deposits in the liver, the organ usually involved in hemosiderosis.
authors.affiliation: Medizinische Klinik III (Hämatologie, Onkologie und Transfusionsmedizin), Charité- Campus, Benjamin Franklin, Hindenburgdamm, Berlin, Germany. martin.schmidt-hieber@charite.de
authors.forename: Martin
authors.initials: M
authors.lastname: Schmidt-Hieber
authors.forename: Ali Fuat
authors.initials: AF
authors.lastname: Okuducu
authors.forename: Gisela
authors.initials: G
authors.lastname: Stoltenburg
authors.forename: Bruno-Marcel
authors.initials: BM
authors.lastname: Mackert
authors.forename: Nadia
authors.initials: N
authors.lastname: Benzian
authors.forename: Eckhard
authors.initials: E
authors.lastname: Thiel
authors.forename: Igor Wolfgang
authors.initials: IW
authors.lastname: Blau
mesh_terms: D001706:Biopsy; D002908:Chronic Disease; D006086:Graft vs Host Disease / Q000150:complications / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D006485:Hemosiderin / Q000378:metabolism*; D006486:Hemosiderosis / Q000378:metabolism / Q000473:pathology; D006801:Humans; D008062:Lipofuscin / Q000378:metabolism; D008099:Liver / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000378:metabolism / Q000473:pathology; D017285:Polymyositis / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D055728:Primary Myelofibrosis / Q000150:complications / Q000473:pathology / Q000628:therapy*; D012508:Sarcolemma / Q000378:metabolism / Q000473:pathology; D033581:Stem Cell Transplantation / Q000379:methods; D014184:Transplantation, Homologous
chemical_list: D008062:Lipofuscin; D006485:Hemosiderin