Severe acute granulomatous myositis and fasciitis in graft-versus-host disease

Polymyositis associated with chronic graft-versus-host disease (GVHD occurs in 3–7% of patients after allogeneic hematopoietic stem cell transplantation (HSCT). The few reported cases with acute onset of polymyositis are mainly related to donor lymphocyte infusion (DLI). Fasciitis along with other manifestations of GVHD has been obeserved in 3% of allogeneic HSTC patients. We report a 58-year-old man, who was diagnosed with chronic idiopathic myelofibrosis (CIMF) and associated anemia in July 2002 and received allogeneic HSCT from his HLA-identical brother in December 2005. Three months later immunosuppressive treatment was tapered and hydryurea was given because of CIMF relapse. A single DLI was followed by acute GVHD of the skin. In August 2006, the patient presented with progressive muscle weakness, myalgia, fever and markedly elevated serum creatine kinase. MRI scan revealed generalized edema in both quadriceps muscles. Biopsy of the left vastus medialis muscle disclosed a severe granulomatous necrotizing myositis and fasciitis with severe destruction of the parenchyma and inflammatory infiltrates of lymphocytes, numerous macrophages and scattered multinucleated giant cells resembling Langhans cells. The morphological pattern was strongly suggestive of tuberculous myositis. However, caseous necrosis or acid-fast bacilli on Ziel-Neelsen staining were not observed. Furthermore, mycobacterium tuberculosis genome could not be detected by polymerase chain reaction (PCR). In addition, there was no clinical hint for tuberculosis. Initiation of steroid therapy resulted in complete remission of myositis. To our knowledge, this unusual myopathological pattern after an interval of almost 4 months following DLI has not been described before.