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

Hematologic complications of systemic lupus erythematosus (SLE) usually involve peripheral destruction of blood elements. We report a case of SLE-associated aplastic anemia in which an IgG complement-dependent antibody, obtained from the patient's disease-phase serum but not remission-phase serum, suppressed growth of granulocyte-macrophage progenitor cells from bone marrow of normal donors in vitro. Therapy with plasmapheresis and immunosuppression resulted in lasting remission.
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PMID:Serum inhibitor in systemic lupus erythematosus associated with aplastic anemia. 673 7

We studied the role of ABH antigens in determining graft outcome in 104 patients who received HLA-identical bone marrow transplants for aplastic anaemia and acute leukaemia. ABH compatibility had no significant effect on incidence of graft rejection or graft-versus-host disease. Fifteen recipients ahd pre-transplant antibodies against donor ABH antigens. In 14, large volume plasma exchange and transfusion of donor-type erythrocytes was successful in reducing the antibody titre to low or undetectable levels. In one patient, plasma exchange was unsuccessful and red cells were removed from the marrow inoculum by unit gravity sedimentation. This approach prevented transfusion reaction, and permitted engraftment of all haematopoietic cell lines despite persistently elevated antibody titres. Parallel in vitro studies revealed that antibodies to ABH antigens failed to inhibit the growth of progenitor cells committed to both granulocyte-macrophage (CFU-C) and erythroid (BFU-E) development. These findings indicate that ABH-antigens are not clinically important transplantation antigens and suggest that ABH antigens are not operationally present on hematopoietic stem cells.
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PMID:ABH antigens and bone marrow transplantation. 699 Sep 58

Colony-forming capacities were studied in three Japanese children with Shwachman's syndrome (chronic neutropenia and exocrine pancreatic insufficiency). Bone marrow granulocyte-macrophage colony-forming cells assayed in a soft agar culture were markedly reduced in all three cases. The cytochemical examination of granulocyte-macrophage colonies by a new technique revealed that 90% of the colonies by a new technique revealed that 90% of the colonies consisted exclusively of granulocytes. Erythroid colony-forming cells assayed in a plasma clot culture were significantly reduced in two of the three cases. Bone marrow phagocytic cells did not suppress granulopoiesis in contrast to the cases of idiopathic aplastic anemia. Moreover, the patient serum did not inhibit granulopoiesis of normal bone marrow cells. These results have been discussed with the possibility of involving the hemopoietic stem cells and other additional factors.
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PMID:Hemopoietic colony-forming cells in Shwachman's syndrome. 711 96

Colonies of fibroblast-like cells have been grown from the mononuclear cell fractions of bone marrow aspirated from normal individuals and patients with aplastic anaemia. Some of the characteristics of the fibroblastoid cells have been determined and their granulocyte-macrophage colony-stimulating activity (CSA) in semi-solid agar culture has been used as a functional test of their influence on granulopoiesis. The incidence and growth rates of fibroblastoid colony-forming cells (F-CFC) from aplastic patients' bone marrows were markedly different form normal either before or after treatment by allogeneic bone marrow transplantation or with antilymphocyte globulin (ALG). Confluent monolayers of fibroblastoid cells grown from normal marrow were, on the whole, poor stimulators of granulocyte-macrophage colony-forming cells (GM-CFC) and CSA was not detected in the supernatant medium. Fibroblastoid monolayers derived from many of the aplastic bone marrows studied were efficient stimulators of GM-CFC but, like the monolayers grown from normal cells, did not release CSA into the culture medium. Addition of methylprednisolone (MP) to the cultures had little effect on fibroblastoid cell growth, induced fat-accumulation by some of the fibroblastoid colonies comprising the monolayer and reduced the abilities of the monolayers to stimulate GM-CFC.
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PMID:Bone marrow fibroblastoid colony-forming cells (F-CFC) in aplastic anaemia: colony growth and stimulation of granulocyte-macrophage colony-forming cells (GM-CFC). 729 89

Thirty-six patients with aplastic anemia and three with aplastic anemia-PNH syndrome in eight institutes were studied for the presence of peripheral blood mononuclear cells having a suppressive effect on granulocyte-macrophage (G-M) and erythroid colony formation using a uniform protocol. In 11 cases of 29 (38%) and 9 cases of 29 (31%), the presence of mononuclear cells with a suppressive effect on G-M and erythroid colony formation, respectively, was demonstrated. However, the presence of mononuclear cells suppressive both to G-M and erythroid colony formation was demonstrated only in 3 of 19 cases (16%).
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PMID:Effect of peripheral blood mononuclear cells from aplastic anemia patients on the granulocyte-macrophage and erythroid colony formation in samples from normal human bone marrow in vitro--a cooperative work. 737 84

To investigate whether phagocytic cells play a role in the pathogenesis of aplastic anaemia, we studied the effect of the mononuclear phagocytic cells in the peripheral blood from patients with idiopathic aplastic anaemia on granulocyte-macrophage colony (GM-colony) formation. There was no apparent infection in the studied patients. In nine out of 20 cases the mononuclear cells suppressed the formation of GM-colonies by normal bone marrow. Removal of the phagocytic cells by carbonyl iron powder caused a loss of this suppressive effect. These results suggest the requirement for phagocytic cells in this suppressive effect and the possible involvement of phagocyte-mediated immunological mechanism in the pathogenesis of some cases of idiopathic aplastic anaemia.
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PMID:Suppression of in vitro granulocyte-macrophage colony formation by the peripheral mononuclear phagocytic cells of patients with idiopathic aplastic anaemia. 745 79

A 6-year-old girl with post-hepatitic severe aplastic anaemia was referred to our hospital. Haematological examination showed a haemoglobin level of 5.2 g/dl, platelet count of 8,000/microliters, and white blood cell count of 130/microliters with 17% neutrophils. She was treated with recombinant human granulocyte-colony stimulating factor (15 micrograms/kg/day i.v.) and cyclosporin A (6 mg/kg/day p.o.). The absolute neutrophil count gradually increased, but Hb and platelets were not improved. The intravenous administration of recombinant human erythropoietin (100 U/kg three times a week) was started, and the reticulocyte count reached 20,000/microliters on day 12. The platelets increased to 81,000/microliters after 16 months of combined administration of recombinant human granulocyte-colony stimulating factor, recombinant human erythropoietin and cyclosporin A. After 20 months of combined administration, the haematological results were: Hb, 13.1 g/dl; platelets 80,000/microliters; WBC, 9500/microliters with 40% neutrophils. After recombinant human granulocyte-colony stimulating factor treatment, the myeloid elements of the bone marrow and the number of granulocyte-macrophage colony forming units increased. Bone marrow erythropoiesis and erythroid colonies also increased after recombinant human erythropoietin administration. The clinical course suggested a beneficial effect of haemopoietic growth factors and cyclosporin A in post-hepatitic aplastic anaemia.
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PMID:Haematological improvement by long-term administration of recombinant human granulocyte-colony stimulating factor and recombinant human erythropoietin in a patient with severe aplastic anaemia. 751 92

Interferon-gamma (IFN-gamma), an immunoregulatory cytokine produced by activated T cells and natural killer cells in response to viral infection or other stimuli, is generally recognized as a suppressor of hematopoiesis. IFN-gamma inhibited in vitro colony formation by granulocyte-macrophage (GM), erythroid and multipotential progenitors. This cytokine exerted direct suppression on the proliferation process, but not on the commitment, of GM progenitors. The antiproliferative effects of IFN-gamma may, in part, result from the prolongation of the doubling time of GM progenitors. Clinically, IFN-gamma may play an important role in the pathogenesis of pancytopenia in aplastic anemia and in the hemophagocytic syndrome. However, as well as showing inhibitory effects, IFN-gamma increased the number of pure and mixed megakaryocyte colonies formed by post-5-fluorouracil treated bone marrow cells and, moreover, the addition of IFN-gamma to culture containing stem cell factor resulted in a synergistic effect on the development of both primitive hematopoietic progenitors and mature populations. These findings suggest that IFN-gamma has bifunctional activity in hematopoiesis.
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PMID:Hematopoietic progenitors and synergism of interferon-gamma and stem cell factor. 752 87

Effects of recombinant human interleukin (IL)-13 on in vitro haemopoiesis from non-adherent mononuclear cells (NAMC) or highly enriched CD34+ cells of human cord blood (CB) were studied. IL-13 significantly increased megakaryocyte (MK) colony formation from either NAMC or CD34+ cells cultured in a plasma clot system supplemented with aplastic anaemia serum (AAS) and phytohaemagglutinin-stimulated human peripheral blood leucocyte-conditioned medium (PHA-LCM) in a dose-dependent manner. Experiments using a modified plasma clot culture, in which normal AB serum and various cytokines were added to replace AAS and PHA-LCM, demonstrated an increased MK colony number in the presence of IL-13, especially in combination with IL-3. However, IL-13 had no stimulatory effect, but rather a slight inhibitory effect in some cases on granulocyte-macrophage (GM) colony formation in both plasma clot cultures. Furthermore, the growth of GM progenitor cells in a methylcellulose culture system in the presence of IL-3, GM-CSF, Epo, G-CSF or in combination was significantly inhibited by the addition of IL-13. On the other hand, high concentrations (100 ng/ml) of IL-13 were needed to cause a slight inhibition on the growth of BFU-E-derived colonies under the same methylcellulose culture. These results indicate that IL-13, alone and synergistically with the effect of IL-3, promotes MK colony formation, but it inhibits the growth of GM and erythroid progenitor cells in vitro.
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PMID:Differential effects of recombinant human interleukin-13 on the in vitro growth of human haemopoietic progenitor cells. 766 73

The mechanism of therapeutic effect of anti-lymphocyte globulin (ALG) and cyclosporin A (CyA) on patients with aplastic anemia was studied. When peripheral CD8 positive cells obtained before therapy were cocultured with bone marrow CD34 positive cells obtained after hematological recovery, the number of colony forming unit granulocyte-macrophage (CFU-GM) and burst forming unit erythroid (BFU-E) were decreased. This result indicates that ALG and CyA inhibits CD8 positive cells which suppress the growth of progenitor cells, resulting in the recovery of hematopoiesis. Next we investigated the plasma concentrations of cytokines including G-CSF, GM-CSF, IL-6, IL-1 alpha and IL-1 beta after ALG treatment. Although the elevation of plasma concentrations of G-CSF and GM-CSF after ALG treatment were found in 11 of 13 patients and in 2 of 13 patients respectively, cytokine production by ALG appeared to be unrelated to the therapeutic effect of ALG for aplastic anemia.
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PMID:[Mechanism of immunosuppressive therapy for aplastic anemia]. 768 63


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