Myasthenia gravis and polymyositis as manifestations of chronic graft-versus-host-disease

Abstract

Summary:Myasthenia gravis and polymyositis are each a rare manifestation of immune dysregulation in chronic graftversus-host disease (cGVHD). We report a 4-year-old boy with idiopathic acquired aplastic anemia who developed myasthenia gravis 22 months and polymyositis 69 months after an allogeneic BMT (5/6 matched, MLC-nonreactive). The occurrence of both syndromes in one patient is unique. Autoimmune dysfunction may be associated with the development of cGVHD as demonstrated by the high incidence of prior aplastic anemia in BMT patients presenting with myasthenia gravis and polymyositis. Recognition of these neurologic manifestations is important in the diagnosis and treatment of cGVHD. Keywords: chronic graft-versus-host disease; myasthenia gravis; polymyositis Chronic graft-versus-host disease (cGVHD) is a complication of allogeneic BMT which is associated with a high degree of morbidity and mortality. cGVHD commonly involves the skin, eyes, mouth, bowel, liver, lung and immune system. 1 Myasthenia gravis (MG) and polymyositis (PM) have been reported as rare manifestations of cGVHD. [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15] This report illustrates a case of MG and PM developing sequentially after an allogeneic BMT in a single patient. Case reportIn December 1990, a 4-year-old boy received an allogeneic BMT from his 5/6 serologically HLA-matched, MLC-nonreactive sister (mismatch A3/29) for treatment of severe idiopathic acquired aplastic anemia. He was conditioned with CY (200 mg/kg) and total body irradiation (300 cGy, single dose). MTX and CsA were given as GVHD prophylaxis. Engraftment of all three lines occurred by day 28 and

Chronic graft-versus-host disease manifesting as polymyositis: an uncommon presentation

“…Sensory and motor nerve conduction are preserved. 11,14,18,22,23,26 The diagnosis of polymyositis can be established on the basis of characteristic clinical and EMG findings. In atypical presentations, a muscle biopsy may be required to confirm the diagnosis.…”

“…2,12,[14][15][16][17][18][19][20][21][22][23][24][25][26] The onset of GVHD-associated polymyositis has been reported to occur between 100 days and 69 months after allogeneic HSCT, and about half of the patients do not have a history of acute GVHD. 21,23 Clinical manifestations of GVHD-associated polymyositis are indistinguishable from those of the idiopathic form polymyositis. The most common presenting symptoms are moderate to severe proximal muscle weakness and myalgias.…”

Fasciitis and myositis: an analysis of muscle-related complications caused by chronic GVHD after allo-SCT

“…Patients with chronic GVHD have decreased performance status, impaired quality of life (QOL) and an increased risk of mortality. 5,6 Muscle-related complications, fasciitis [7][8][9] and myositis [10][11][12][13] are relatively rare chronic GVHD manifestations, and their clinical features resemble autoimmune eosinophilic fasciitis and idiopathic polymyositis. The new chronic GVHD diagnostic guidelines proposed fasciitis as diagnostic, and myositis as a distinctive sign and symptom of chronic GVHD manifestation.…”

Successful treatment of thrombocytopenia and hemolytic anemia with IvIG in a patient with lupus-like syndrome after mismatched related PBSCT

“…[4][5][6][7][8][9][10] Cytopenia after allogeneic SCT is a common phenomenon and can occur secondary to various immune or non-immune mechanisms. In this case a non-immune mechanism such as graft failure, peripheral consumption due to bleeding, sepsis or relapse of the underlying disease could be excluded.…”