Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018133 (graft-versus-host disease)
 18,032 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

BMT has become an important therapy for many hematologic disorders. Following BMT, the recipient may develop GVHD when it appears that immunocompetent donor lymphocytes react to host antigens. Acute and chronic GVHD represent two distinct syndromes. Acute GVHD has not been associated with primary neurologic involvement. Polymyositis has been reported in 12 patients with chronic GVHD, with the most common underlying illness being aplastic anemia. The clinical, serologic, and muscle biopsy features of the myositis in GVHD have been similar to those observed in idiopathic polymyositis. Weakness was moderate to severe and responded to prednisone, sometimes with the addition of azathioprine. Prognosis depended upon the underlying disease and not on the severity of the myositis. MG occurs rarely in chronic GVHD. Most patients with MG and GVHD have had aplastic anemia; those with aplastic anemia are more likely to have anti-AchR prior to BMT. The clinical manifestations of GVHD MG have not differed from classic autoimmune MG; each patient had elevated antiacetylcholine receptor antibodies titers. All patients have responded well to cholinesterase inhibitors but have received other immunosuppressants. These observations suggest that aplastic anemia is an important host factor in the development of the autoimmune disorders seen with chronic GVHD, certainly of myositis and MG. Herpes zoster peripheral nerve infections have occurred in patients with chronic GVHD. One patient had mononeuritis multiplex. In both acute and chronic GVHD, CNS impairment is usually caused by metabolic encephalopathy or infection. Primary CNS involvement has not been recognized.
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PMID:Neurologic complications of graft-versus-host disease. 304 48

A 13-year-old girl with chronic graft versus host disease (GVHD) developed severe proximal weakness. Muscle biopsy showed necrotic fibers with anti-IgG deposits and interstitial inflammation. Steroid treatment helped, but her weakness recurred. Repeat biopsy showed persistent inflammation and possible steroid myotoxicity. Numerical ratio of capillaries to myofibers and capillary numerical density were higher than childhood dermatomyositis (CD), and capillaries had no tubuloreticular inclusions. Absence of quantitative histologic, ultrastructural, or immunocytochemical abnormalities of capillaries differentiated our patient from CD. We conclude that myositis of chronic GVHD can present as a severe myopathy that responds to steroids and differs from CD.
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PMID:Myositis of chronic graft versus host disease. 635 Sep 24

A 37-year-old Japanese man with chronic myeloid leukemia (CML) developed myasthenia gravis 29 months after bone marrow transplantation (BMT) from an HLA one locus-mismatched brother. Proximal muscle weakness and bilateral ptosis occurred along with the exacerbation of chronic GVHD shortly after sudden cessation of cyclosporine (CYA) and prednisolone. The diagnosis of myasthenia gravis was made based on clinical symptoms and elevation of an anti-acetylcholine receptor antibody titer and all symptoms related to myasthenia gravis promptly diminished with the start of treatment for chronic GVHD. In most previously reported cases, the underlying disease was aplastic anemia (6 of 7 cases) and donors were of the opposite sex (6 of 7 cases). The haplotypes HLA B7 (3 of 5 cases), B35 (3 of 5 cases), and DR2 (3 of 3 cases) were common. All cases suffered from chronic GVHD. The present case had only chronic GVHD and HLA B7 as a background for myasthenia gravis after BMT. The abrupt cessation of immunosuppressive therapy may also be related to the development of myasthenia gravis after BMT.
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PMID:Myasthenia gravis after allogeneic bone marrow transplantation. 795 Nov 5

A syndrome indistinguishable from idiopathic polymyositis occurred in 11 patients as a manifestation of chronic GVHD. All patients had elevation of creatine phosphokinase (CPK). Immunohistology demonstrated the effector cells in the muscle infiltrates as cytotoxic T cells, a finding similar to idiopathic polymyositis. Polymyositis is a rarely reported complication of chronic graft-versus-host disease (GVHD) with only 8 cases described in the literature. We encountered this syndrome in a small but significant percentage of our patients with chronic GVHD. Polymyositis associated with chronic GVHD does not affect the overall prognosis for the patient. Moreover, polymyositis can be the only manifestation of chronic GVHD. Awareness of this complication is important because it can be confused with other causes of muscle weakness after bone marrow transplantation. Finally, prompt initiation of corticosteroid therapy results in a rapid improvement of the associated symptoms.
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PMID:Polymyositis as a manifestation of chronic graft-versus-host disease. 886 49

Myasthenia gravis (MG) is a rare complication of allogeneic bone marrow transplantation (BMT). We present the 11th case in the medical literature, a 23-year-old female 100 months post-allogeneic bone marrow transplantation for acute myelogenous leukemia (AML). After discontinuation of immunosuppression for chronic graft-versus-host disease (GVHD) involving skin, gastrointestinal tract and lacrimal glands, the patient developed severe, progressive dysphagia initially attributed to esophageal candidiasis. With the development of muscle weakness, ptosis, and dysphonia the diagnosis of generalized myasthenia gravis was suspected, and confirmed by elevated anti-acetylcholine receptor antibody titer and a positive edrophonium challenge. Prednisone and pyridostigmine produced improvement, and thymectomy was performed without pathologic evidence of thymoma. Recurrent post-operative respiratory distress required transient mechanical ventilation. Twenty-seven months after diagnosis, the patient requires maintenance prednisone to control symptoms of myasthenia gravis. The clinical features of all reported cases of MG post-allogeneic BMT are reviewed, and universal features include an association with decreasing immunosuppression, the presence of other manifestations of chronic GVHD, anti-acetylcholine receptor antibodies, and the absence of an associated thymoma. HLA Cw1, Cw7 and DR2 were identified at frequencies significantly above that expected from HLA antigen prevalance studies, and may be markers for increased risk of developing MG post-allogeneic BMT. No statistically significant associations with HLA A2, B7, B35 or donor-recipient sex mismatch were present. Reinstitution of immunosuppression and standard therapies for myasthenia gravis were effective in the majority of cases. The role of thymectomy in this population remains unclear.
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PMID:Myasthenia gravis in association with allogeneic bone marrow transplantation: clinical observations, therapeutic implications and review of literature. 915 70

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.
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PMID:Myasthenia gravis without chronic GVHD after allogeneic bone marrow transplantation. 970 30

A 31-year-old female with lymphoblastic lymphoma developed myasthenia gravis (MG) 26 months after receiving an allogeneic bone marrow transplant (BMT) from an HLA-identical sister. She presented with classic symptoms and electromyographic evidence of the disorder approximately 2 weeks after electing to abruptly discontinue her immunosuppressive medications. She initially responded to steroids and acetylcholinesterase inhibitors. Her subsequent course has been characterized by episodes of moderately severe weakness that respond to intravenous immunoglobulin and prednisone. This case of post-transplant MG is only the second reported to have occurred in association with BMT for lymphoblastic lymphoma. Potential risk factors for the development of post-transplant MG are discussed including underlying hematological disorder, HLA phenotype, family history of MG, the presence of chronic GVHD, and recent cessation of immune suppression.
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PMID:Myasthenia gravis after allogeneic bone marrow transplantation for lymphoblastic lymphoma. 1062 49

We report a 22-year-old man who had myositis in the course of chronic graft-versus-host disease after bone marrow transplantation for acute monocytic leukemia. The distribution of muscular involvement was different from idiopathic polymyositis. Muscular atrophy and weakness were noted in the distal muscles as well as in the proximal muscles of the upper extremities but there was little weakness in the proximal muscles of the lower extremities. However, histological and immunohistochemical study of the biceps brachii muscle showed findings similar to those of idiopathic polymyositis. It was suggested that myositis can be a manifestation of chronic GVHD caused by a cellular immune reaction by donor T cells.
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PMID:Myositis as a manifestation of chronic graft-versus-host disease. 1085 58

We experienced a case of demyelinating, inflammatory cervical myelopathy after bone marrow transplantation for chronic myelocytic leukemia (CML). A 28 years-old man who had been having skin and liver graft versus host disease (GVHD), developed paresthesia in the legs, and then, difficulty in walking. At the time of admission, weakness of the hands also appeared. There was no evidence of CML recurrence after bone marrow transplantation. The myelopathy was characterized by multiple abnormal spotty signal intensities in the cervical spinal cord on MRI and these were in part Gd-enhanced. A course of pulse-dose methylprednisolone was given, followed by prednisolone. The neurological deficits were improved to the degree of full recovery. The inflammatory myelopathy together with a plaque in the cerebral hemisphere, moderately delayed p-100 latency of VEP and elevation of myelin basic protein of the spinal fluid, is difficult to distinguish from that of multiple sclerosis. Although the precise mechanism of GVHD-myelopathy is not known, it is likely that the donor myelin-reactive T-lymphocytes were non-specifically activated with GVHD reaction and directed to a central nervous system. Tacrolimus might have precipitated the focal immune reaction by way of cytotoxic effects on brain capillaries. The "GVHD-myelopathy" presented here may thus be akin to multiple sclerosis in its immune mechanism.
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PMID:[A case of inflammatory demyelinative myelopathy after bone marrow transplantation]. 1108 92

An unusual case of respiratory failure and dropped head syndrome as a complication of severe chronic graft-versus-host disease (GVHD)-related polymyositis is described. The patient required tracheostomy and mechanical ventilation but recovered following treatment with aggressive immunosuppression and intensive rehabilitation. The differential diagnoses of muscle weakness in the bone marrow transplant (BMT) patient and the dropped head syndrome are both discussed. To our knowledge, this is the first reported case of respiratory failure requiring mechanical ventilation occurring as a complication of GVHD-related polymyositis.
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PMID:Chronic graft-versus-host disease-related polymyositis as a cause of respiratory failure following allogeneic bone marrow transplant. 1110 14


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