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Query: UMLS:C0002874 (aplastic anemia)
 5,905 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 16 year old boy underwent allogeneic bone marrow transplantation (BMT) from an human leukocyte antigen (HLA)-identical sibling for severe aplastic anaemia. He was symptomatic for 7 years before transplantation and had received multiple red blood cell and platelet transfusions. Conditioning for BMT consisted of cyclophosphamide, antilymphocyte globulin and total lymphoid irradiation. Engraftment was rapid, there was no evidence of rejection despite the history of multiple blood product transfusions and he did not develop acute or chronic graft versus host disease. He was well for the first 8 months after transplantation but then developed fevers, interstitial pneumonia, herpes simplex infections and cytomegalovirus enteritis. Serological studies revealed antibodies to human immunodeficiency virus (HIV) and he was considered to have acquired immune deficiency syndrome (AIDS). Retrospective analysis of the serum samples showed that he was seronegative for HIV until approximately 10 months before transplantation when his serum became HIV positive. Lymphocyte function studies done after transplantation suggested immunologic recovery at 3 months post-transplant with a brisk though subnormal response to phytohaemagglutinin stimulation. T cell subset analysis performed subsequently showed complete absence of CD4 positive cells indicating immune incompetence which was associated with clinical features of AIDS. Bone marrow transplantation had failed to produce sustained immunologic reconstitution and prevent the progression of HIV to which he ultimately succumbed.
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PMID:Failure of allogeneic bone marrow transplantation to benefit HIV infection. 149 64

Bone marrow transplantation from a human leukocyte antigen (HLA)-identical sibling for treatment of severe aplastic anemia among women of reproductive age is becoming more common. Successful pregnancy has been reported to occur in several such patients. A woman delivered a healthy, term, female infant 18 months after a transplant from her HLA-identical sister. Her pregnancy was complicated by disseminated herpes zoster, treated with intravenous acyclovir at 14 weeks' gestation, before the diagnosis of pregnancy. While there have been several case reports involving the use of acyclovir in the third trimester, primarily in the treatment of varicella infections, there have been no previous reports of such an early utilization of this antiviral drug.
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PMID:Early-second-trimester use of acyclovir in treating herpes zoster in a bone marrow transplant patient. A case report. 156 15

A 19-month-old girl with idiopathic severe aplastic anemia refractory to multi-agent immunosuppressive therapy developed severe platelet alloimmunization following several months of platelet transfusions. She became refractory to human leukocyte antigen (HLA)-matched platelet transfusions and experienced frequent episodes of bleeding. She was treated with intravenous vincristine administered weekly for three doses and showed marked improvement in both clinical and laboratory response to platelet transfusions. When vincristine was held for 3 weeks, she again became refractory to HLA-matched platelet transfusion. Reinstitution of vincristine resulted in cessation of clinical bleeding and improved response to platelet transfusion. The mechanism of response likely involves selective delivery of cytotoxic drug to macrophages. To our knowledge this is the first reported case of alloimmune thrombocytopenia responsive to vincristine.
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PMID:Vincristine therapy for severe platelet alloimmunization. 179 56

We have performed bone marrow transplants on four children with severe aplastic anemia who lacked an human leukocyte antigen (HLA)-identical sibling donor. Patients were prepared with cyclophosphamide and 600 cGy fractionated total body irradiation, and then received marrow from a parent donor mismatched for one (two patients), two (one patient), or three (one patient) HLA antigens. All four patients engrafted. One died early of acute graft-versus-host disease. The three others showed sustained complete hematopoietic reconstitution. Two are alive and hematologically normal 43-87 months after transplant. Both have had acute and chronic graft-versus-host disease (CGVHD), and one of the two remains on immunosuppressive drugs. The fourth died at 48 months after transplant of CGVHD. The previous experience with HLA-incompatible marrow transplants is reviewed, and the rationale for this preparative regimen is discussed. Cyclophosphamide and 600 cGy fractionated total body irradiation is an effective preparative regimen for children with severe aplastic anemia receiving transplants from HLA-nonidentical parental donors, allowing engraftment and full hematologic reconstitution.
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PMID:Successful treatment of severe aplastic anemia by bone marrow transplantation from HLA nonidentical family members: preliminary results utilizing cyclophosphamide and 600 cGY fractionated total body irradiation. 202 71

We treated 14 patients by transplantation of marrow from unrelated volunteer donors. Eight patients had severe aplastic anemia, 3 had chronic granulocytic leukemia, and 3 had Fanconi's anemia. The results are compared with those of a group of 14 similar patients transplanted concurrently from human leukocyte antigen (HLA)-mismatched family members: Sustained engraftment was achieved in 8 of 14 patients in both groups; one additional patient survived with autologous marrow reconstitution following an unrelated donor transplant. In the unrelated donor group, 6 of 9 evaluable patients developed grade III through IV acute graft-v-host disease, as compared with 4 of 9 patients after family-mismatched transplants. Overall survival was similar in the two groups. In the unrelated donor group 4 of 14 (29%) patients survived (median survival 1,299 days) as compared with 5 of 14 (36%) in the mismatched-family donor group (median survival 808 days). In both groups, patients with HLA phenotypically matched donors fared better than those with donors who were mismatched for one or more HLA antigen. Of the patients transplanted from HLA phenotypically matched donors 6 of 12 patients (50%) survived, as compared with 3 of 16 patients (19%) transplanted from HLA-mismatched donors. We conclude that unrelated donor bone marrow transplantation (BMT) should be considered in those cases of leukemia or bone marrow failure in which the chance of cure using conventional therapy is remote and a HLA genotypically or phenotypically matched family donor is not available.
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PMID:Histocompatible unrelated volunteer donors compared with HLA nonidentical family donors in marrow transplantation for aplastic anemia and leukemia. 353 29

Bone marrow transplantation is now an accepted form of therapy for many hematologic disorders including aplastic anemia, genetically determined diseases and malignant diseases, particularly leukemia, and for rescue of patients given intensive chemoradiotherapy for malignant disease. The donor may be a healthy identical twin, a family member or even an unrelated person. Selection is made on the basis of human leukocyte antigen tissue typing. Intensive chemoradiotherapy is used to suppress patients' immune systems to facilitate engraftment and destroy diseased marrow. Transfusion of platelets, erythrocytes and granulocytes (or all of these), antibiotic coverage and protection from infection are necessary during the pancytopenic period. Use of a Hickman catheter facilitates maintenance of adequate nutritional intake and provides easy access for drawing blood and intravenous administration. Survival rates vary considerably depending on a patient's disease, clinical state and age. Patients with aplastic anemia transplanted early in the course of their disease have a survival rate of approximately 80%. Patients with acute lymphoblastic leukemia are usually transplanted in a second or subsequent remission and have a survival rate of 25% to 40%. Patients with acute nonlymphoblastic leukemia in remission have survivals ranging from 45% to 70%. More than 200 patients in the chronic phase of chronic granulocytic leukemia have been transplanted with survival ranging from 50% to 70%. Complications of marrow transplantation include marrow graft rejection, graft-versus-host disease, immunologic insufficiency and the possibility of recurrence of the leukemia. The risk of death from these complications must be balanced against the possibility of cure.
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PMID:Overview of marrow transplantation. 391 95

The association of granulocyte transfusions with cytomegalovirus infection and interstitial pneumonitis in allogeneic marrow transplant recipients was reviewed. Data from 387 patients with aplastic anemia or acute leukemia receiving transplants from human leukocyte antigen-matched siblings were analyzed. Acquisition of cytomegalovirus infection was higher in recipients of prophylactic and therapeutic granulocytes than in control subjects. The incidence was significantly different, however, only in cytomegalovirus seronegative patients who received granulocytes from seropositive granulocyte donors. There was no significant increase in the total incidence of or mortality from all forms of interstitial pneumonitis.
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PMID:The effect of granulocyte transfusions on the incidence of cytomegalovirus infection after allogeneic marrow transplantation. 627 15

One hundred ten of 175 patients with aplastic anemia conditioned by cyclophosphamide had sustained engraftment of marrow from human leukocyte antigen (HLA)-identical siblings and lived for more than 6 months. Forty-nine of the 110 patients developed chronic graft-versus-host disease between 85 and 464 days. Ninety-seven patients are alive from 1.4 to 11 years after engraftment; 13 died between 208 and 726 days. Twenty of the 36 surviving patients with chronic graft-versus-host disease have Karnofsky performance scores of 100%, 7 of 90%, 5 of 80%, 1 of 70%, 2 of 60%, and 1 of 40%. Our analysis, using a binary logistic regression model, identified three factors predicting chronic graft-versus-host disease: moderate to severe acute graft-versus-host disease with an estimated relative risk of 11.65; increasing patient age; and the use of viable donor buffy coat cells in addition to the marrow to prevent graft rejection. The last two factors were significant only in patients without acute graft-versus-host disease.
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PMID:Predictive factors in chronic graft-versus-host disease in patients with aplastic anemia treated by marrow transplantation from HLA-identical siblings. 634 May 76

Children with severe aplastic anemia (SAA) are treated with bone marrow transplantation (BMT) if a human leukocyte antigen (HLA) compatible sibling donor is available, or alternatively with immunosuppressive therapy (IST). Three retrospective trials examining BMT vs IST in pediatric patients treated from 1970-1988 found BMT resulted in a superior survival rate. Advances have been made in general supportive care and in the approach to each of these treatment modalities in the last decade. To compare survival following BMT and IST in a more recent era, we retrospectively analyzed the results of 48 consecutively treated children with SAA presenting to Memorial Sloan-Kettering Cancer Center (MSKCC) between 1983 and 1992. In contrast to the previous studies, the estimated survival of the BMT and IST groups at 120 months are equivalent, 75.6% and 73.8%, respectively. The IST results in our series are superior to the 42-48% (2-10 year) survival previously published, but similar to survival data observed in more recent IST trials employing more intensive immunosuppression (antithymocyte globulin and cyclosporine). The overall BMT survival rates are similar to those previously published, although BMT results improved dramatically during the latter five years of this analysis, with all 11 patients transplanted surviving with a minimum follow-up of 3 years. No surviving BMT patient has extensive chronic graft-versus-host disease (GvHD), a major cause of long-term mortality post-BMT. Therefore, it is likely the BMT survival curve will remain stable. In contrast, the survival curve of the IST patients is likely unstable, since patients are still at risk for relapse or development of clonal disease. Thus, despite overall similar survival rates, we continue to recommend BMT as first-line therapy in pediatric SAA patients with matched sibling donors.
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PMID:Comparison of long-term outcome of children with severe aplastic anemia treated with immunosuppression versus bone marrow transplantation. 920 37

This article describes a gingival squamous cell carcinoma that developed in a 21-year-old woman who received a bone marrow transplant at the age of 16 from her human leukocyte antigen-identical sister as treatment for severe aplastic anemia. Thirty days after transplantation, she presented with cutaneous erythema as a result of acute graft-versus-host disease, and this subsequently evolved into chronic graft-versus-host disease. A lichenoid white plaque of the gingiva developed shortly thereafter, and it began to increase in size rapidly 4 years posttransplantation. Biopsy indicated squamous cell carcinoma arising in this region, apparently associated with chronic graft-versus-host disease. Few reports have described a secondary solid malignancy involving the oral cavity of young adults after bone marrow transplantation.
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PMID:Gingival squamous cell carcinoma in a patient with chronic graft-versus-host disease. 926 19


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