Search results for "Aplastic anemia"

Allogeneic Stem Cell Transplantation for Acquired Aplastic Anemia: Better Outcome with Bone Marrow as Compared to Peripheral Blood in HLA-Matched Sib…

2009

Abstract Abstract 876 Background: Transplantation of bone marrow (BM) from a HLA-matched sibling donor is an effective treatment for severe aplastic anemia (AA) with long-term survival in excess of 80%. In the recent years there were two trends in allogeneic stem cell transplantation (SCT) for AA: (1) increasing proportion of transplants performed from matched unrelated donors (MUD) and (2) increasing proportion of transplants using peripheral blood progenitor cells (PBSC) as stem cell source instead of BM. A similar switch to PBSC over BM grafts is reported in leukemia transplants. PBSC grafts for leukemia are associated with higher rates of chronic graft-versus-host disease (cGVHD). This …

Editorial for “Quantification of Bone Marrow Fat Fraction and Iron by MRI for Distinguishing Aplastic Anemia and Myelodysplastic Syndromes”

2021

Development Of a Disease Specific Quality Of Life Questionnaire For Patients With Aplastic Anemia and/Or Paroxysmal Nocturnal Hemoglobinuria (QLQ-AA/…

2013

Abstract Introduction Acquired Aplastic Anemia (AA) and Paroxysmal Nocturnal Hemoglobinuria (PNH) are ultra-rare diseases with a yearly incidence 2 to 4 per million (AA) and of 1.3 to 2 per million (PNH. While much is known about pathophysiology and treatment of these interrelated diseases, less is known about patients (pts.) psycho-social issues. Quality of life (QoL) evaluation tools used in all studies for AA and PNH are rather unspecific and were initially designed for cancer patients (e.g. the European Organization of Research and Treatment (EORTC) QLQ-C30). Given the complexity of AA and PNH, the variation in symptoms and different treatment approaches (immunosuppression, bone marrow …

Cytomegalovirus inhibits the engraftment of donor bone marrow cells by downregulation of hemopoietin gene expression in recipient stroma

1998

ABSTRACT Cytomegalovirus (CMV) disease after bone marrow (BM) transplantation is often associated with BM graft failure. There are two possible reasons for such a correlation. First, a poor hematopoietic reconstitution of unrelated etiology could promote the progression of CMV infection by the lack of immune control. Alternatively, CMV infection could interfere with the engraftment of donor BM cells in recipient BM stroma. Evidence for a causative role of CMV in BM aplasia came from studies in long-term BM cultures and from the murine in vivo model of CMV-induced aplastic anemia. A deficiency in the expression of essential stromal hemopoietins, such as stem cell factor (SCF), has indicated …

Transient Pulmonary Infiltrates during Treatment with Anti-Thymocyte Globulin

1999

We report the case of a 54-year-old woman with aplastic anemia, who developed transient pulmonary infiltrates following intravenous infusion of rabbit antithymocyte globulin (ATG) for 3 days. There was no other explanation than the infusion of ATG for the infiltrates. Rechallenge with ATG induced the recurrence of opacities on the chest radiograph. Although rarely involved with only 4 previous reports, ATG should be included in the list of drugs capable of inducing pulmonary infiltrates.

Polypeptides controlling hematopoietic blood cell development and activation

1989

Colony-stimulating factors (CSFs) have entered the clinical arena. Several investigators have explored, in first clinical phase I studies, different routes of administration to define the optimum biological dose, maximum tolerated dose, toxicity, and pharmacokinetics of these reagents. It has been demonstrated that recombinant human (rh) granulocyte-macrophage CSF (GM-CSF) and granulocyte CSF (G-CSF) can be safely administered over a broad dose range to increase number of circulating granulocytes in man. More recently, GM-CSF and G-CSF have been involved in phase Ib/II studies to assess the granulopoietic responses of patients with granulocytopenia due to various underlying disease states i…

Lymphocyte subpopulations in solvent-exposed workers.

1986

To estimate the cellular immune response of workers highly exposed to mixtures of organic solvents, subpopulations of peripheral blood lymphocytes (PBLs) were analyzed. For this, the PBLs of nine floorers (aged 25–58 years, exposure time 8–35 years) were subsequently labelled with monoclonal antibodies OKT 4, OKT 8, OKT 11, anti-Leu 7 and anti-Leu 12. Analysis was made by a FACS IV cell sorter (Becton-Dickinson, USA). The control group consisted of matched pairs of healthy donors. In the exposed group we found a decrease in the OKT 11 (all) T cell fraction, a decrease in the OKT 4 helper cells, an increase in the anti-Leu 7 positive cells, mostly natural killer cells, an important increase …

Bone marrow failure by cytomegalovirus is associated with an in vivo deficiency in the expression of essential stromal hemopoietin genes.

1997

Bone marrow (BM) failure associated with cytomegalovirus (CMV) infection is a feared complication after clinical BM transplantation. Experiments in long-term BM cultures have indicated that BM stromal cells (BMSC) are targets of productive CMV infection, but an in situ infection of BM stroma remained to be documented, and the pathomechanism is open to question. Here we describe a murine in vivo model of lethal CMV aplastic anemia (CMV-AA). The reconstitution of hematopoietic progenitor cells expressing stem cell factor (SCF) receptor was found to be defective in CMV-AA. While murine CMV replication in permissive parenchymal tissues is cytolytic, the hematopoietic cord was found to be a site…

Partial T Cell-Depleted Peripheral Blood Stem Cell Transplantation from HLA-Identical Sibling Donors for Patients with Severe Aplastic Anemia

2019

We analyzed the outcomes of 26 consecutive patients with acquired severe aplastic anemia (SAA) undergoing peripheral blood stem cell transplantation (PBSCT) with partial ex vivo T cell depletion with a targeted T cell dose from HLA-identical sibling donors. The median patient age was 37 years (range, 3 to 63 years). Four patients with uncontrolled pneumonia at the time of transplantation died, on days +1, +2, +21, and +26. All evaluable patients engrafted, with a median time to neutrophil recovery of 11 days (range, 10 to 14 days) and a median time to platelet recovery of 19 days (range, 8 to 53 days). Two patients had transient grade I acute graft-versus-host disease (GVHD) with skin invol…

Posttransfusional, LKM-1-autoantibody-positive hepatitis C virus infection, cryoglobulinemia, and aplastic anemia.

1995

Aplastic anemia is occasionally caused by viral hepatitis, hepatitis C virus being the most important factor. Pathogenetically, decreased bone marrow function, abnormalities of the bone marrow microenvironment, and immune-mediated suppression of hematopoiesis are important. Hepatitis C virus infection is associated with a variety of extrahepatic manifestations including autoimmune features like cryoglobulinemia, Sjogren's syndrome, and autoimmune hepatitis. Here we report the case of a 42-year-old man with aplastic anemia due to posttransfusional hepatitis C virus infection associated with cryoglobulinemia and LKM-1 autoantibodies. Following a triple immunosuppressive therapy, there was a c…