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)

To investigate clinical characteristics of early central nervous system (CNS) complications after reduced-intensity stem cell transplantation (RIST), we reviewed the medical records of 232 patients who had undergone RIST for hematologic diseases at our institutions between September 1999 and June 2003. All patients had received purine analog-based preparative regimens. Stem cell sources comprised granulocyte colony-stimulating factor-mobilized blood from HLA-identical or 1 locus-mismatched related donors (n = 151), unrelated bone marrow (n = 44), or unrelated cord blood (n = 37). Graft-versus-host disease prophylaxis incorporated cyclosporine with or without methotrexate. Diagnosis of CNS complications was based on clinical, radiologic, and microbiological findings. CNS complications occurred in 18 patients (7.8%), with a median onset of 22 days, and were infectious (n = 1), metabolic (n = 15), or cerebrovascular (n = 2). Symptoms included seizures (n = 7), visual disturbance (n = 2), headache (n = 8), nausea (n = 8), vomiting (n = 6), impaired consciousness (n = 16), and hemiparesis (n = 3). Complications improved promptly in 10 patients, and 8 patients died without improvement within 30 days. Multivariate analysis with logistic regression identified umbilical cord blood transplantation as a significant risk factor for early CNS complications (odds ratio, 14.5; 95% confidence interval, 3.7-56.9; P <.0001). CNS complications are a significant problem after RIST, particularly with umbilical cord blood. Limbic encephalopathy is an unrecognized subtype of neurotoxicity after umbilical cord blood transplantation.
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PMID:Early central nervous system complications after reduced-intensity stem cell transplantation. 1528 34

Measles inclusion body encephalitis (MIBE) is a disease of the immunocompromised host and typically occurs within 1 year of acute measles infection or vaccination. We report a 13-year-old boy who had chronic granulomatous disease and presented 38 days after stem cell transplantation with afebrile focal seizures that progressed despite multiple anticonvulsants. After an extensive diagnostic evaluation, brain biopsy was performed, revealing numerous intranuclear inclusion bodies consistent with paramyxovirus nucleocapsids. Measles studies including reverse transcriptase-polymerase chain reaction and viral growth confirmed measles virus, genotype D3. Immunohistochemistry was positive for measles nucleoprotein. Despite intravenous ribavirin therapy, the patient died. MIBE has not been described in stem cell recipients but is a disease of immunocompromised hosts and typically occurs within 1 year of measles infection, exposure, or vaccination. Our case is unusual as neither the patient nor the stem cell donor had apparent recent measles exposure or vaccination, and neither had recent travel to measles-endemic regions. The patient had an erythematous rash several weeks before the neurologic symptoms; however, skin biopsy was consistent with graft-versus-host disease, and immunohistochemistry studies for measles nucleoprotein were negative. As measles genotype D3 has not been seen in areas where the child lived since his early childhood, the possibility of an unusually long latency period between initial measles infection and MIBE is raised. In addition, this case demonstrates the utility of brain biopsy in the diagnosis of encephalitis of unknown cause in the immunocompromised host.
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PMID:A new complication of stem cell transplantation: measles inclusion body encephalitis. 1552 95

Neurological complications may occur in BMT recipients (11-59%), frequently contributing to morbidity or mortality. They are the main causes of death in 10-15%. Life-threatening neurological complications were seen in 11 out of 113 (9.7%) children who underwent BMT from HLA-matched family (n=7) or mismatched donors (n=4) at our institution. Diagnoses of patients with neurological complications were acute myeloblastic leukemia (AML) (five), thalassemia major (two), Fanconi anemia (two), Omenn syndrome (one) and leukodystrophy (one), and the neurological events were seen between days +13 and +85 after transplantation. Minor symptoms including reversible, nonrepetitive seizures were excluded. Cyclosporine A toxicity was diagnosed in six children. The rest of the complications were brain abscess/meningoencephalitis (two), severe hypomagnesemia (one), busulfan toxicity (one), sustained hypertension (three), and intracranial hemorrhage (three). Six patients with neurological complications suffered from >grade II graft-versus-host disease (GvHD), and all were high risk for transplant-related complications. In this study, risk status of the underlying disease, mismatched transplantation, a diagnosis of AML (advanced stage), older age and >grade II GvHD were important adverse factors for the development of severe life-threatening neurological complications.
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PMID:Life-threatening neurological complications after bone marrow transplantation in children. 1553 98

A 46-year-old woman with Hodgkin's disease who underwent nonmyeloablative allogeneic stem cell transplantation developed cortical blindness, seizures, and left hemiparesis on day 100 while receiving tacrolimus (FK506) and prednisone for the treatment of graft-versus-host disease (GVHD). Magnetic resonance imaging revealed multiple changes, mainly in the bilateral occipital lobes, suggesting FK506-related leukoencephalopathy. These abnormalities improved after discontinuation of FK506. However, 3 days after the episode, cerebral hemorrhage in the left occipital lobe with perforation to the left subdural space occurred. Although FK506-induced leukoencephalopathy with cerebral hemorrhage is considered the more severe form of such leukoencephalopathy, the patient's neurological symptoms almost completely resolved and radiographic findings improved after discontinuation of FK506, tapering of methylprednisolone, and initiation of mycophenolate mofetil. FK506-related leukoencephalopathy is a rare complication after allogeneic stem cell transplantation. Although the symptoms usually subside after discontinuation of FK506, therapeutic intervention in many cases may result in severe complications, including GVHD and vascular disease. We consider it important to use immunosuppressive agents without vascular endothelial toxicity for preventing the development of fatal GVHD after discontinuation of FK506.
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PMID:Successful treatment of tacrolimus (FK506)-related leukoencephalopathy with cerebral hemorrhage in a patient who underwent nonmyeloablative stem cell transplantation. 1554 Sep 7

Hematopoietic cell transplantation (HCT) involves the intravenous infusion of hematopoietic progenitor cells from the patient (autologous) or a human leukocyte antigen-matched donor (allogeneic). Before transplantation, the recipient undergoes a conditioning regimen with high-dose chemotherapy or radiotherapy (or both) to destroy a defective bone marrow or residual cancer cells. After allogenic HCT chronic immunosuppression is needed to prevent graft rejection and graft-versus-host disease. The frequency and type of neurological complication depends on the type of HCT, the underlying disease, and the case ascertainment. In this review the neurological complications are presented according to the stage of HCT that they are most likely to occur: (1) conditioning: drug-related encephalopathies and seizures or complications secondary to medical procedures; (2) bone marrow depletion: metabolic and drug-related encephalopathies and seizures, septic cerebral infarctions, and hemorrhages; (3) chronic immunosuppression: infections by viruses and opportunistic organisms; and (4) late events: central nervous system relapses of the original disease, neurological complications of graft versus host disease, and second neoplasms.
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PMID:Neurological complications of hematopoietic cell transplantation. 1563 54

Two patients with a plausible diagnosis of central nervous system graft-versus-host disease (CNS-GVHD) are described. Both presented with neurological manifestations 6 and 18 months following allogeneic transplant with hemiparesis, seizure, encephalopathy and magnetic resonance findings of hyperintense white matter lesions on T-2 weighed images. Brain biopsy in one and autopsy in the other revealed profound perivascular lymphocytic infiltrates composed predominantly of T-lymphocytes that were of donor origin. Although an unequivocal diagnosis of CNS-GVHD is difficult to establish, the transplantation community should be aware of this controversial entity.
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PMID:Central nervous system graft-versus-host disease: report of two cases and literature review. 1792 40

An 18-year-old female with myelodysplastic syndrome underwent an allogeneic cord blood transplantation in May 2005. The conditioning regimen consisted of total body irradiation, cytarabine and cyclophosphamide. The day of the cord stem cell transfusion was counted as Day 0. For acute GVHD prophylaxis, cyclosporine A (CsA) and methotrexate were used. Engraftment was achieved on Day 30, acute GVHD grade II developed on Day 45 and treatment with methylprednisolone for acute GVHD was started. On Day 68 the patient had generalized seizures accompanied by loss of consciousness, hypertension and left hemiparesis, and was intubated. A cranial CT scan showed a mass effect on the right basal ganglia, and high signal intensities on the T2-weighted and FLAIR images of a MR examination were detected in the bilateral basal ganglia and posterior lobes, the findings of which were compatible with a brain tumor or infectious disease. Since an increased level of apparent diffusion coefficient (ADC) values on the bilateral basal ganglia was noted, we suspected that vasogenic edema had caused the mass effect. She went into remission immediately after CsA treatment was discontinued. Therefore, this case was diagnosed as atypical reversible posterior leukoencephalopathy syndrome (RPLS) associated with CsA. CsA-induced encephalopathy presenting a mass effect in clinical imaging findings is very rare, and an ADC map may be useful for the diagnosis of RPLS.
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PMID:[Myelodysplastic syndrome with cyclosporine A-induced encephalopathy presenting with a mass effect on the right basal ganglia after cord blood transplantation]. 1723 73

Immunosuppressive monoclonal antibodies directed to immune system cells may reduce rejection and graft versus host disease (GvHD) after allogeneic stem cell transplantation (SCT), but can increase the risks of viral infection. Here, we report human herpes virus-6 (HHV-6) encephalitis despite antiviral prophylaxis in 5 of 43 (11.6%) patients receiving alemtuzumab supported conditioning. Encephalitis occurred at 41-103 days (median 60 days) presenting with confusion in all patients, combined with amnesia (n=3) or seizures (n=2). MRI revealed non-specific white matter changes in two and a non-enhancing medial temporal lobe lesion in three patients. Cerebrospinal fluid (CSF) PCR amplification for HHV-6 was positive in all five patients, (600-2 25 000 (median 4700) copies/ml CSF), while analysis of peripheral blood revealed 100-22 500 (median 1200) viral copies/ml plasma. CSF protein was elevated in four patients, with minimal CSF pleocytosis. Intravenous foscarnet produced neurological improvement at 8-13 (median 11) days and negative plasma PCR at 30-66 (median 50) days. Four patients had complete neurological recovery, but one patient with persistent viral DNA in the CSF succumbed to progressive encephalopathy. Given this high incidence of HHV-6 and the possibility of successful outcome with prompt treatment, a high index of suspicion of this disorder is required in recipients of monoclonal antibody supported allografts.
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PMID:Human herpesvirus-6 encephalitis following allogeneic hematopoietic stem cell transplantation. 1740 92

The incidence of central nervous system (CNS) complications, their risk factors, and impact on outcome are not well defined in recipients of allogeneic hematopoietic stem cell transplant (aHSCT). We reviewed the medical records of 302 consecutive patients who underwent aHSCT for malignant and nonmalignant hematologic diseases at Princess Margaret Hospital between 2002 and 2005. The cumulative incidences of all CNS complications and posterior reversible encephalopathy syndrome (PRES) at days 100 and 365 were 18% (95% confidence interval [CI]: 14-22) and 23% (95% CI: 19-29), and 6% (95% CI: 4-9) and 7% (95% CI: 5-11), respectively. Seizures occurred in 37% of all CNS events in the first 100 days and 73% of PRES episodes. Female gender and high-dose total-body irridiation (TBI) were identified as independent risk factors for CNS complications in the first 100 days posttransplant. Survival at 1-year was significantly inferior in patients who developed CNS complications within 100 days of transplant (28% [95% CI: 17-41] versus 72% [95% CI: 66-77]) and PRES (27% [95% CI: 10-47] versus 67% [95% CI: 62-73]) compared to those who did not (P < .0001). Death from graft-versus-host disease (GVHD) was more common in patients with CNS complications in the first 100 days (P = .006) and PRES (P = .01) compared to patients without complications.
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PMID:Central nervous system complications after allogeneic hematopoietic stem cell transplantation: incidence, manifestations, and clinical significance. 1795 Sep 23

Posterior reversible encephalopathy syndrome (PRES) is one of the serious adverse side effects of calcineurin inhibitors, which are used for the prophylaxis of graft-versus-host disease (GVHD) after allogeneic stem cell transplantation (allo-SCT). We retrospectively analyzed 12 patients who developed PRES after allo-SCT aiming to clarify the clinical features, risk factors, and prognosis of PRES. Median onset of PRES is 17 days after allo-SCT. The most frequent primary symptom was high blood pressure, followed by headache and visual disturbance. Nine of our patients subsequently developed systemic seizure. Sites of PRES by MRI were detected in the frontal, temporal, and parietal lobes, basal ganglia, and brain stem in addition to occipital lobe. Serum creatinine that had increased two-fold from the baseline value was identified as the only risk factor for developing PRES after allo-SCT. The incidence of acute GVHD (grade II-IV) in patients with PRES and those without were 88.9% and 48.7%; respectively (P<0.001), and most of these patients died of GVHD or GVHD-related causes. The 2-year overall survival of patients with PRES and those without were 16.7% and 72.4%, respectively (P<0.001). These data suggested the importance of early intervention for PRES and exploitation of optimal GVHD prophylaxis after developing PRES.
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PMID:[Retrospective analysis of posterior reversible encephalopathy syndrome after allogeneic stem cell transplantation]. 1922 23


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