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)

Severe respiratory distress appeared in a 14-year-old girl with acute lymphoblastic leukemia 2 months after receiving syngeneic bone marrow transplantation (BMT) with a conditioning regimen of a high-dose of busulfan, etoposide and nimustine. Rapid body-weight gain and edema, especially in eyelids and lower-limbs, were also observed. Without any findings of heart failure nor GVHD, pulmonary edema was recognized on the chest roentgenogram. As soon as the diagnosis of pulmonary edema due to 'capillary leak syndrome' was suspected, the patient was treated with intravenous administration of diuretics, albumin and bolus methylprednisolone in combination of mechanical ventilation. Although the clinical manifestations were improved by the treatment, the disease recurred 5 weeks later. The patient was successfully treated by the same medications, and there has been no recurrence as of the sixth month after discontinuance of the therapy. At present, the mechanism of capillary leak syndrome is still undefined. In this case, however, we speculate that the conditioning regimen for BMT intensified the capillary disturbance initially caused by intensive chemotherapy since remission induction. Furthermore hypoalbuminemia due to severe anorexia might have enhanced the occurrence of the disease.
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PMID:[Recurrent pulmonary edema in a patient with acute lymphoblastic leukemia after syngeneic bone marrow transplantation]. 157 40

A murine IgG1 antibody specific for the IL-2-binding site on the human lymphocyte IL-2 receptor beta chain (CD25) was evaluated in 11 patients who developed acute graft-versus-host disease following allogeneic marrow transplantation. All patients had received cyclosporine and methotrexate for prophylaxis of GVHD, either alone (4 cases), or in combination with antithymocyte globulin (4 cases) or with prednisone (3 cases). Patients had developed GVHD at 7-53 days (median 12) after transplantation and had failed treatment with corticosteroids for 3-44 days (median 19). Residual GVHD was of grade II severity in 4 patients, grade III in 5 patients, and grade IV in 2 patients. Sequential patients received monoclonal antibody in escalating doses from 0.1 mg/kg/day to 1.0 mg/kg/day for 7 days. Side effects were fever, respiratory distress, hypertension, hypotension, and chills occurring in 11 of 72 (14%) antibody infusions. Trough antibody levels greater than 6 micrograms/ml were achieved in patients treated with 0.5 or 1.0 mg/kg/day. Four of eight evaluable patients had an IgM antibody response, and one had an IgG response to the murine immunoglobulin. Clinical response of GVHD was evaluated in 10 patients who received the entire course of the antibody treatment. Among 7 patients treated within 40 days from transplantation, one patient had a complete response in the skin as the only involved organ, and 3 patients had a partial response, 2 in the skin and one in the gastrointestinal tract. No responses were achieved with liver disease at anytime or in any organ in patients treated beyond 40 days after transplantation. Since administration of this antibody was well tolerated and some efficacy was observed in patients with acute GVHD treated early after transplantation, there is a rationale for testing this antibody as an agent for prophylaxis of GVHD.
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PMID:A phase I-II study evaluating the murine anti-IL-2 receptor antibody 2A3 for treatment of acute graft-versus-host disease. 236 50

Thirty patients with chronic granulocytic leukemia (CGL), were given cyclophosphamide 60 mg/kg on each of 2 consecutive days, followed by total body irradiation (TBI) 10 Gy and an HLA-identical bone marrow transplant (BMT). Eleven patients were in the accelerated phase of their disease (CGLacc) or in second/secondary chronic phase (CGL-2CP), with a median age of 33 years: four patients died of transplant related complications, and four of recurrent leukemia; three patients are alive and well 19, 31, 33 months from BMT. The actuarial 33-month survival is 27%. The actuarial relapse rate is 50%. Nineteen patients were in their first chronic phase (1CP), with a median age of 32 years: three died of graft versus host disease (GvHD), two of infection, and two of acute respiratory distress syndrome (ARDS); 12 are alive and well 6 to 29 months post-BMT. The actuarial 29-month survival is 63%. The actuarial survival of patients younger than 30 years is 63%, compared to 62% for patients older than 30 (P = 0.1). The survival of patients grafted within or after 24 months from the onset of CGL is respectively 87% and 45% (P = 0.04). None of the patients grafted in 1CP had a true hematologic-cytogenetic relapse. The Ph' chromosome was detected on one occasion in two patients 12, 13 months post-BMT: they both remain hematologically normal and Ph1-negative 3 to 6 months later, after discontinuation of cyclosporin A. This study confirms that survival exceeding 60% can be obtained in CGL in the first chronic phase, whereas less than 30% of patients will survive if grafted in accelerated, second/secondary chronic phase, mainly because of leukemic relapse. The duration of the disease seems to be relevant to the outcome of the transplant. The effect of post-transplant immunosuppression, in our case cyclosporin A, on the interaction between normal and Ph1-positive hemopoietic cells, may deserve further attention.
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PMID:Bone marrow transplantation for chronic granulocytic leukemia. 353 Apr 28

A total of 29 consecutive patients with leukemia or aplastic anemia who received an HLA-identical marrow graft were given cyclosporin A (CyA) to prevent graft-versus-host disease (GvHD). These patients were compared with an historic group of 25 similar patients with leukemia or AA given methotrexate (MTX) for GvHD prophylaxis at this institution. Engraftment was faster in patients given CyA when compared with MTX patients, with less days of granulocytopenia (P = 0.04), a shorter interval before reaching a platelet count of 70 X 10(9)/l (P = 0.04), fewer major infections (P = 0.01), and fewer days on intravenous antibiotics (P = 0.02). There were no graft failures in CyA patients compared with four of 25 in MTX patients (P = 0.01). Early mortality was lower in CyA patients but not significantly (P = 0.06). The incidence of pulmonary complications was comparable, five of 29 and seven of 25 in CyA and MTX patients, respectively, but the clinical features of such complications differed. Interstitial pneumonia developing after day 30 was seen in MTX patients, whereas an acute respiratory distress syndrome developing between day +8 and day +18 was seen in CyA patients. Acute GvHD was less severe in CyA patients (P = 0.04), but chronic GvHD was comparable (P = 0.3). The actual one-year survival is currently 72% and 52% in CyA and MTX patients, respectively (P = 0.1). Although our initial experience with CyA is encouraging with regard to engraftment and acute GvHD, optimization of CyA protocols will probably be needed for it to be proven as having a definite advantage over MTX.
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PMID:Cyclosporin A in marrow transplantation for leukemia and aplastic anemia. 388 17

Of the 393 children who underwent BM autotransplantation in the pediatric oncology unit of the Institut Gustave Roussy between February 1979 and September 1991, 14 (3.56%) developed disseminated Candida infection within 3 months. This incidence was far lower than in other published series. Eleven of the 14 patients recovered from the infection, giving a far higher survival rate (78%) than among adult BM transplant recipients (usually < 30%). All 14 patients had four or more risk factors and persistent BM aplasia (median, 25 days); six had Candida tropicalis infection. Four cases of deep visceral involvement were documented, two of which were lethal. Clinical signs were relatively uniform and included secondary high-grade fever (> 40 degrees C) for 8 days; half the patients developed cardiovascular impairment, respiratory distress, neurological disturbances (altered consciousness or delirium) and severe diarrhoea, within as little as 10 days after transplantation. Blood cultures rapidly became positive after the onset of clinical signs and this permitted prompt treatment with a combination of amphotericin B and 5-fluorocytosine; in addition, central catheters were removed. Blood cultures became sterile within 4 days of treatment in 10 of the 14 cases. The generally favourable outcome in this series could be related to the young age of the patients, the absence of GVHD, the absence of total body irradiation in the conditioning regimen, and early antifungal treatment.
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PMID:Systemic Candida infection in pediatric BM autotransplantation: clinical signs, outcome and prognosis. 833 27

Allogeneic bone marrow transplantation (BMT) for advanced acute leukemia is associated with a high risk of relapse. It is postulated that interleukin-2 (IL-2) administered after BMT might induce or amplify a graft-versus-leukemia effect and thereby reduce the relapse rate. To identify an IL-2 regimen for testing this hypothesis, a phase I trial of IL-2 (Roche) was performed in children in complete remission (CR) without active graft-versus-host disease (GVHD) off immunosuppressive agents after unmodified allogeneic matched-sibling BMT for acute leukemia beyond first remission. Beginning a median of 68 days after BMT, 17 patients received escalating doses of induction IL-2 (0.9, 3.0, or 6.0 x 10(6) IU/m2/d representing levels I, II, and III) for 5 days by continuous intravenous infusion (CIV). After 6 days of rest, they received maintenance IL-2 (0.9 x 10(6) IU/m2/d) for 10 days by CIV infusion. Levels I and II were well-tolerated, but, of 6 patients at level III, 1 developed pulmonary infiltrates, 1 developed hypotension (both resolved), and 1 died of bacterial sepsis and acute respiratory distress syndrome. Grade II acute GVHD developed in 1 patient at level I and 1 at level III. The maximum tolerated dose of induction IL-2 was level II. IL-2 induced lymphocytosis, with an increase in CD56+ and CD8+ cells. Ten patients remain in CR at 5+ to 67+ months. Thus, a regimen of IL-2 has been identified that did not induce a high incidence of acute GVHD when administered to children after unmodified allogeneic BMT. Its clinical activity will be assessed in a phase II trial.
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PMID:Phase I trial of interleukin-2 after unmodified HLA-matched sibling bone marrow transplantation for children with acute leukemia. 860 12

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

An infant who received haploidentical BM for severe combined immunodeficiency (SCID) developed acute, reversible complete heart block in association with an exacerbation of GVHD. Respiratory distress and myocardial dysfunction were also seen with this and previous GVHD exacerbations. The patient had not received chemotherapy or radiation prior to BMT. The complete heart block resolved after 1 week of intensive immunosuppression. The association of complete heart block with GVHD is important because the heart block is potentially reversible with prompt, aggressive control of the GVHD.
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PMID:Complete heart block in association with graft-versus-host disease. 948

Patients with compromised immune function suffer a wide variety of lung insults. Infections are the most common causes of both acute and chronic lung diseases, but many noninfectious conditions affect the lungs. The clinical presentation of these noninfectious conditions often mimic infections, thus causing diagnostic dilemmas. The spectrum of noninfectious lung injury and response in the immunosuppressed host includes interstitial edema, interstitial fibrosis, diffuse idiopathic pneumonia, acute respiratory distress syndrome, and obliterative bronchiolitis. Alveolar hemorrhage may complicate any of these conditions. Lung injury in the immunosuppressed host is associated with a diversity of etiologies: sepsis, irradiation, graft rejection, reperfusion injury, graft-versus-host disease, and chemotherapeutic agents and other drug reactions. These injuries most often present as diffuse pulmonary infiltrates on chest radiograph. Establishing a specific diagnosis and etiology for the injury is often problematic. From a pragmatic standpoint, excluding the possibility of infection is the principal aim of diagnostic testing.
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PMID:Noninfectious lung disease in the immunocompromised host. 1051 34

We report the occurrence of tuberculosis in a 10-year-old Taiwanese boy, approximately 4 months after he received a matched-related bone marrow transplantation from his sister for acute T-cell lymphoblastic leukemia. After transplantation, grade III acute graft-versus-host disease developed and the patient was treated with prednisolone and cyclosporine. Marrow failure was noted on day 77 post-transplantation, however, after an episode of herpes zoster infection. Interstitial pneumonia, diagnosed on the basis of chest x-ray and computed tomography findings, occurred on day 120. Histologic examination of an open-lung biopsy specimen showed caseating granulomas and a few acid-fast bacilli. The patient died of acute respiratory distress syndrome, despite immediate implementation of antituberculosis therapy. Sputum cultures grew Mycobacterium tuberculosis 5 weeks later. This report demonstrates that the possibility of tuberculosis needs to be considered in immunocompromised patients, and that appropriate prophylaxis should be instituted in areas where tuberculosis is endemic.
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PMID:Acute respiratory distress syndrome due to tuberculosis in a child after allogeneic bone marrow transplantation for acute lymphoblastic leukemia. 1057 42


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