1/1051. Chrysotherapy-induced aplastic anemia: a case report. A case of gold-induced aplastic anemia occurring in a 58-year-old male with rheumatoid arthritis who received a total dose of 635 mg of gold sodium thiomalate over a 16-week period is reported. The clinical features, treatment, prevention and pathogenesis of gold-induced aplastic anemia are reviewed. The ability to predict patients in whom this hematologic effect may occur is discussed. Chrysotherapy is beneficial in the treatment of rheumatoid arthritis, but gold-induced aplastic anemia may be fatal. Absolute identification of patients at risk of having this hematologic side effect is not possible, but dosage reduction and intense monitoring of laboratory and clinical signs may prevent its occurrence. ( info) |
2/1051. trisomy 6 associated with aplastic anemia. A clone with 47 chromosomes was observed in the bone marrow of a patient with aplastic anemia and found to be trisomic for chromosome 6. The abnormal clone was not observed in the peripheral blood. ( info) |
3/1051. Late graft rejection and second infusion of bone marrow in children with aplastic anaemia. Late graft rejection following allogeneic bone marrow transplantation (BMT) for aplastic anaemia is a significant clinical problem and is associated with a high risk of mortality. We report two children with severe aplastic anaemia (SAA) who developed very late graft rejection 2 years and 4 months and 10 years respectively after allogeneic BMT from HLA-identical siblings. Following a second BMT from their initial donors, engraftment has been sustained in both cases. The patients are alive with full donor chimaerism, 18 and 19 years from initial transplant. These cases illustrate that graft failure can be an extremely late event after allogeneic BMT for SAA, and that long-term sustained engraftment can be achieved in these patients with second BMT from the original donors. ( info) |
4/1051. Transient hypoplastic anemia caused by primary human parvovirus B19 infection in a previously untreated patient with hemophilia transfused with a plasma-derived, monoclonal antibody-purified factor viii concentrate. BACKGROUND: Modern plasma-derived clotting factor concentrates are produced using various virus-inactivation protocols and are assumed to be safer than they were previously with regard to the risk for transmitting viral infections such as human immunodeficiency virus, hepatitis b, and hepatitis c. The risks from viruses that are relatively resistant to the current inactivation procedures remain uncertain. PATIENT: A 7-year-old with mild hemophilia a who had not been previously infused with any blood products was treated with a plasma-derived, monoclonal antibody-purified factor viii concentrate to cover orthopedic surgery after traumatic fracture of his left arm. RESULTS: A typical primary human parvovirus (HPV)-B19 infection was observed associated with transient hypoplastic anemia. retrospective studies including serologic examination and polymerase chain reaction analysis confirmed that the HPV-B19 infection was transmitted by the factor viii concentrate. CONCLUSIONS: Clotting factor concentrates for the treatment of hemophilia retain a risk for HPV-B19 contamination. HPV-B19 viral infection might induce hypoplastic anemia in these patients, particularly during enhanced hemopoiesis after acute blood loss. ( info) |
5/1051. Rapid autologous marrow recovery and eradication of infectious mononucleosis despite severe immunosuppression following second transplantation for aplastic anemia. A patient with aplastic anemia failed to respond to immunosuppressive therapy and first marrow transplantation (BMT). Recovery of autologous hematopoiesis was rapid following a second stem cell transplant with a non-myeloablative preparatory regimen. The autologous immune response to infectious mononucleosis (IM) 4 weeks post-transplant was normal despite recent and ongoing severe immunosuppression. ( info) |
6/1051. Successful cytokine treatment of aplastic anemia following living-related orthotopic liver transplantation for non-A, non-B, non-C hepatitis. The relationship between aplastic anemia and viral hepatitis is well recognized, and such patients usually have a high mortality. We successfully treated a case of aplastic anemia following living-related orthotopic liver transplantation (LROLT) for non-A, non-B, non-C hepatitis. A 2-yr-old boy with fulminant hepatic failure from non-A, non-B, non-C hepatitis received LROLT. Before transplantation, he had pancytopenia which was probably hepatitis associated, and viral suppression was suspected after bone marrow (BM) biopsy. After the transplantation, he developed progressive pancytopenia and a diagnosis of aplastic anemia was made via BM biopsy. With immunosuppressant agents (cyclosporine, methylprednisolone), cytokine therapy (granulocyte-colony stimulating factor (G-CSF), macrophage-colony stimulating factor (M-CSF), recombinant human erythropoietin (rhEPO)) was effectual and the patient recovered from pancytopenia. He was discharged from the hospital 57 d after the liver transplantation and remains well 1 yr after LROLT. Combined cytokine therapy with high doses of G-CSF, M-CSF and rhEPO appeared to be effective in the treatment of aplastic anemia following liver transplantation for non-A, non-B, non-C hepatitis. Since M-CSF activates macrophages, it may have contributed to the graft rejection. Careful consideration should be given to the use of high-dose M-CSF in liver transplant patients. ( info) |
| dyskeratosis congenita is recognized by its dermal lesions and constitutional aplastic anemia in some cases. We report successful allogeneic bone marrow transplantation in two siblings with this disease from their sister, and their long term follow-up. We used reduced doses of cyclophosphamide and busulfan for conditioning instead of total body irradiation. Also, we report late adverse effects of transplantation which are not distinguishable from the natural course of disease. ( info) |
8/1051. Severe aplastic anemia associated with human parvovirus B19 infection in a patient without underlying disease. Human parvovirus B19 (B19 virus) infection is known to induce aplastic crisis in patients with hemolytic anemia. In healthy subjects, B19 infection may sometimes cause mild pancytopenia, but these changes are transient and recovery is spontaneous. We report the first case of aplastic anemia in a previously healthy boy without any underlying diseases, following asymptomatic infection with the B19 virus. Laboratory examination initially showed thrombocytopenia, mild leukopenia in the peripheral blood, and severe hypoplastic bone marrow. Since pancytopenia developed and worsened progressively, immunosuppressive therapy was given, resulting in a complete remission. Despite the lack of an infectious prodrome, serological and histological analysis revealed an underlying infection with the B19 virus. Thus, B19 virus infection must be considered one of the causes of aplastic anemia in patients without underlying hemolytic anemia and an apparent episode of the viral infection. ( info) |
9/1051. fusarium infections in patients with severe aplastic anemia: review and implications for management. BACKGROUND AND OBJECTIVE: The prognosis of severe fungal infections, such as fusarium infections, in patients with aplastic anemia is directly related to the recovery of bone marrow functions. In this study, in vitro anti-fusarium activity of granulocytes was investigated, the case of disseminated infection in a child with very severe aplastic anemia is reported, and implications for management of such infective complications are discussed. DESIGN AND methods: The in vitro efficiency of PMNL from three untreated, normal blood donors and from two G-CSF-treated WBC donors in contrasting the growth of the fusarium sp strain isolated from the patient we present was measured by a 3H-glucose uptake inhibition assay and confirmed by microscopic examination. RESULTS: Basic growth inhibitory activity of unstimulated PMNL on fusarium cells was significantly enhanced in the presence of GM-CSF in all three blood donors tested. In one of the two G-CSF-treated donors, in vitro efficiency of PMNL in contrasting the growth of the fungus increased notably after G-CSF treatment. We report the case of a 3-year-old girl with very severe aplastic anemia unresponsive to conventional immunosuppressant therapy who developed a disseminated fusarium infection. The child initially responded to liposomal amphotericin b and granulocyte transfusions from G-CSF stimulated donors. Subsequently she was given a cord blood stem cell transplantation but died of disseminated infection. INTERPRETATION AND CONCLUSIONS: Including the present case, there are only ten reports of invasive infections caused by the genus fusarium in aplastic anemia patients and only two of the patients survived. in vitro data seem to suggest that in vivo treatment with rh-G-CSF could have a stimulatory effect on the anti-fusarium activity of neutrophils. Despite the efficacy of granulocyte transfusions by G-CSF-stimulated donors in the temporary control of fusarium infection, treatment of the underlying hematologic disease is required to cure the infection in patients with severe aplastic anemia. Granulocyte transfusions by G-CSF-stimulated donors while awaiting bone marrow recovery following the blood stem cell transplant should be considered. ( info) |
10/1051. A case of aplastic anaemia in pregnancy. Aplastic anaemia in pregnancy is an extremely rare condition with high maternal morbidity and mortality rates. Intensive haematological support remains the mainstay of therapy and a successful obstetric outcome can be best accomplished with the close clinical collaboration of the haematologist and the obstetrician as occurred with our patient reported here. ( info) |