Adult; Anemia, Aplastic/therapy; Bone Marrow Transplantation/adverse effects; Graft vs Host Disease/etiology/immunology; Humans; Male; Myasthenia Gravis/etiology/immunology; Transplantation, Homologous
Abstract :
[en] A 20-year-old man with aplastic anemia developed myasthenia gravis (MG) 7 months after bone marrow transplantation (BMT) from an HLA one locus-mismatched sister. Proximal muscle weakness (predominant in the lower limbs) and dysphagia occurred without any other sign of graft-versus-host disease (GVHD), 1 month after cessation of immunosuppression with cyclosporine. The diagnosis of MG was based on clinical symptoms and on neurophysiologic investigations showing a significant increase of the Jitter in single-fiber electromyography and a significant decremental response during repetitive stimulation at slow rates, but antibodies against the acetylcholine receptor (AchRab) were negative. All clinical and neurophysiological signs normalized within 1 month of treatment with low-dose prednisolone and pyridostigmine, and the patient is perfectly well 1 year after cessation of all therapy. All cases of BMT-associated MG previously published are reviewed in comparison with ours. The originality of this new observation is that this case is the only one not associated with chronic GVHD and negative for AchRab. Alternatively, MG may have been the sole manifestation of chronic GVHD in this patient.
Disciplines :
Hematology
Author, co-author :
Baron, Frédéric ; Centre Hospitalier Universitaire de Liège - CHU > Hématologie clinique
Sadzot, Bernard ; Centre Hospitalier Universitaire de Liège - CHU > Neurologie Sart Tilman
Wang, François-Charles ; Centre Hospitalier Universitaire de Liège - CHU > Médecine de l'appareil locomoteur
Beguin, Yves ; Centre Hospitalier Universitaire de Liège - CHU > Hématologie clinique
Language :
English
Title :
Myasthenia gravis without chronic GVHD after allogeneic bone marrow transplantation.
Smith CI, Aarli JA, Biberfeld P et al. Myasthenia gravis after bone-marrow transplantation. Evidence for a donor origin. New Engl J Med 1983; 309: 1565-1568.
Seely E, Drachman D, Smith BR et al. Post bone marrow transplantation (BMT) myasthenia gravis: evidence for acetyl- choline receptor (AChR) abnormality. Blood 1984; 64 (Suppl. 1): 221a.
Bolger GB, Sullivan KM, Spence AM et al. Myasthenia gravis after allogeneic hone marrow transplantation: relationship to chronic graft-versus-host disease. Neurology 1986; 36: 1087-1091.
Lefvert AK, Bolme P, Hammarström L et al. Bone marrow grafting selectively induces the production of acetylcholine receptor antibodies, immunoglobulins bearing related idiotypes, and anti-idiotypic antibodies. Ann NY Acad Sci 1987; 505: 825-827.
Atkinson K, Bryant D, Delprado W, Biggs J. Widespread pulmonary fibrosis as a major clinical manifestation of chronic graft-versus-host disease. Bone Marrow Transplant 1989; 4: 129-132.
Grau JM, Casademont J, Monforte R et al. Myasthenia gravis after allogeneic bone marrow transplantation: report of a new case and pathogenetic considerations. Bone Marrow Transplant 1990; 5: 435-137.
Abecassis MM. Complicaçoes pouco habituais da transplantacao de medula ossea (TMO). Experiência da Unidade de TMO do Institue Português de Oncologia de Francisco Gentil, Centro de Lisboa. Acta Med Portuguesa 1991; 4 (Suppl 1): 37-38.
Melms A, Faul C, Sommer N et al. Myasthenia gravis after BMT: identification of patients at risk? Bone Marrow Transplant 1992; 9: 78-79.
Haslam PJ, Proctor SJ, Goodship TH, Zouvani J. Immune complex glomerulonephritis. myasthenia gravis and compensated hypothyroidism in a patient following allogeneic bone marrow transplantation. Nephrol Dial Transplant 1993; 8: 1390-1392.
Shimoda K, Gondo H, Harada M et al. Myasthenia gravis after allogeneic bone marrow transplantation. Bone Marrow Transplant 1994; 14: 155-156.
Zaja F, Russo D, Silvestri F et al. Myasthenia gravis after allogeneic bone marrow transplantation: a case report. Bone Marrow Transplant 1995; 15: 649-650.
Adams C, August CS, Maguire H, Sladky JT. Neuromuscular complications of bone marrow transplantation. Pediat Neurol 1995; 12: 58-61.
Drachman DB. Myasthenia gravis. New Engl J Med 1994; 330: 1797-1810.
Smith CI, Hammarström L, Lefvert AK. Bone marrow grafting induces acelylcholine receptor antibody formation. Lancet 1985; i: 978.
Lefvert AK, Bjorkholm M. Antibodies against the acetylcholine receptor in hematologic disorders: implications for the development of myasthenia gravis after bone marrow grafting. New Engl J Med 1987; 317: 170.
