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.

Inflammatory myopathies after allogeneic stem cell transplantation

“…Serum CK is an important first line investigation but was not measured in 38% of the patients with weakness in our cohort. Weakness and CK elevation raise suspicion for IM; however, 1 of our patients with IM did not have an elevated CK, and 8 other cases of biopsy‐confirmed IM without an associated CK rise have been previously reported . Electromyography appeared to be underused in our cohort of patients.…”

“…Weakness and CK elevation raise suspicion for IM; however, 1 of our patients with IM did not have an elevated CK, and 8 other cases of biopsy-confirmed IM without an associated CK rise have been previously reported. 3,[10][11][12][13][14][15][16] Electromyography appeared to be underused in our cohort of patients.…”

Dermatomyositis‐like muscle pathology in patients with chronic graft‐versus‐host disease

“…One previous report7 described the typical clinical and histologic (including perifascicular atrophy) features of DM associated with cGVHD, and another study19 reported clinical and electromyography findings suggestive of DM but without muscle biopsy. Two additional cGVHD‐associated myositis patients have been categorized as either PM22 or just “myopathy,”23 despite muscle biopsies that revealed perifascicular atrophy. Three of these patients,7, 19, 23 like 2 of our patients, had normal CK levels, and all 7 had a similar clinical course.…”

“…Two additional cGVHD‐associated myositis patients have been categorized as either PM22 or just “myopathy,”23 despite muscle biopsies that revealed perifascicular atrophy. Three of these patients,7, 19, 23 like 2 of our patients, had normal CK levels, and all 7 had a similar clinical course. It is well appreciated that serum CK can be normal in DM, particularly in those with indolent presentations.…”