Myasthenia Gravis after Bone-Marrow Transplantation

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

Section: Discussionmentioning

confidence: 99%

“…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. …”

mentioning

confidence: 99%

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

Saunders

Silverman

et al. 1999

“…Levite et al 22 demonstrated that SLE autoantibody production in mice is determined by bone marrow-derived cells, and not by other extramedullary immune cells. This provides an acceptable explanation for the different experimental findings such as transfer of both PAAS and SLE from affected mice to healthy recipients, 23 which has also been reported in humans for MG, 14 AT 15 and some auto-antibodies related to SLE. 16 It is difficult to elucidate whether these observations are merely due to high-intensity chemo-radiotherapy or whether there is an additional role for immune reactions after transplantation.…”

Section: Discussionmentioning

confidence: 99%

“…Nevertheless, several authors have described selected cases in which, because of their severity, ABMT has been performed in these pathologies. [11][12][13] On the other hand, transferral of MG, 14 AT 15 and some autoimmune phenomena of SLE 16 from donors to recipients of a BMT has also been described, as if there were a direct correspondence between the activity of the disease and marrow immunocompetent cells.…”

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confidence: 99%

Disappearance of lupus anticoagulant after allogeneic bone marrow transplantation

Olalla

Ortín

Hermida

et al. 1998

Summary:Lupus anticoagulant antibodies have never been reported to disappear after either allogeneic or autologous bone marrow transplantation in humans. We report the first case of disappearance of lupus anticoagulant antibodies in a patient without systemic lupus erythematosus or clinical evidence of other autoimmune disorders, who received an allogeneic bone marrow transplant as treatment for chronic myeloid leukemia. Although marrow transplantation is not a recognized therapy for antiphospholipid syndrome, our observation should be considered another example of the capability of intensive chemo-radiotherapy followed by stem cell transplantation to ablate a pathologic marrow clone resulting in an autoimmune disorder and improve, or even cure, some severe autoimmune diseases.

“…Acute polyneuropathies, such as the Guillain-Barré syndrome and acute sensorimotor polyneuropathy (critical illness neuropathy), have frequently been reported as a common cause of weakness in patients in the ICU, and have been described after bone marrow transplantation. 7,8 The normal motor and sensory conduction velocities in this patient exclude a demyelinating polyneuropathy. The low or absent compound muscle action potentials upon supramaximal stimulation of the motor nerves indicate a preferential motor axonopathy.…”

Section: Discussionmentioning

confidence: 99%

Acute quadriplegic myopathy following autologous peripheral blood stem cell transplantation for breast cancer

Samuelsson¹,

Zackrisson

Tokics³

et al. 1999

Summary:Autologous peripheral blood stem cell transplantation (APSCT) is increasingly used in the treatment of breast cancer. We report a patient who experienced septic shock, and after treatment with antibiotics, high-dose corticosteroids and mechanical ventilation due to respiratory insufficiency, developed quadriplegia. Electroneurophysiological examination, as well as a muscle biopsy, showed a typical picture of acute quadriplegic myopathy with loss of thick filament proteins. This is, to the best of our knowledge, the first reported case of this complication following APSCT.