1/325. Pure red cell aplasia evolving through the hyperfibrotic myelodysplastic syndrome to the acute myeloid leukemia: some pathogenetic aspects.The authors report a 58-year-old female who originally presented with acquired pure red cell aplasia (PRCA). At diagnosis, the karyotype was normal, the serum erythropoietin level was highly elevated and no T-cell mediated inhibition of erythropoiesis was demonstrated in coculture studies. Conventional immunosuppressive therapy proved ineffective. A year later a diagnosis of hyperfibrotic myelodysplastic syndrome was assessed. The sequential bone marrow examinations in the course of the three years showed a progressive increase in bone marrow fibrosis, erythroid hyperplasia and dysmegakaryocytopoiesis, terminating in the acute myeloid leukemia. This sequence of the events included the appearance of del(5)(q13q33), four years after setting a diagnosis of PRCA. The authors suggest that the absence of both cytogenetic abnormality and the signs of dyshematopoiesis at the diagnosis of PRCA does not exclude ultimately a "clonal" category of the disease. Thus, repeated hematological and cytogenetical reevaluations are recommended.- - - - - - - - - - ranking = 1keywords = red (Clic here for more details about this article) |
2/325. Pure red cell aplasia as presentation of systemic lupus erythematosus: antibodies to erythropoietin.In this case report we describe two patients with pure red cell aplasia (PRCA) as an initial manifestation of systemic lupus erythematosus (SLE). antibodies to erythropoietin were determined, by an ELISA method developed in our laboratory, in frozen serum obtained from one of the patients. A high titer of antibodies to erythropoietin was detected in serum obtained before treatment with high dose intravenous immunoglobulin (IVIG). The antibody titer declined after successful treatment. This observation suggests that antibodies to erythropoietin may contribute to the pathogenesis of SLE associated PRCA.- - - - - - - - - - ranking = 0.83333333333333keywords = red (Clic here for more details about this article) |
3/325. Successful treatment of refractory acquired pure red cell aplasia (PRCA) by allogeneic bone marrow transplantation.This case describes a 16-year-old woman treated successfully by a bone marrow transplant from her HLA-identical brother for refractory acquired pure red cell aplasia. Conditioning was as for severe aplastic anaemia with cyclophosphamide 4 x 50 mg/kg and antithymocyte globulin. Complete donor type engraftment at 3 months reversed to full autologous reconstitution at 2 years with normal haemopoiesis. The potential implications on pathogenesis of the disease as well as on treatment of autoimmune disorders by stem cell transplantation are discussed.- - - - - - - - - - ranking = 1.6666666666667keywords = red (Clic here for more details about this article) |
4/325. Acute pure red cell aplasia associated with allopurinol therapy.Several investigators have reported patients with acute pure red cell aplasia (PRCA) caused by anticonvulsants, antibiotics, or antithyroid agents. allopurinol is known to be a causative agent of aplastic anemia, but there have been few reports of acute PRCA induced by allopurinol. We describe here a 15-year-old boy who suffered from anemia 6 weeks after initiation of allopurinol therapy; his anemia immediately improved after cessation of the drug. His bone marrow showed severe erythroid hypoplasia with a myeloid/erythroid ratio of 18.6 and low expression of glycophorin A detected on cell-surface antigen analysis. No morphological abnormalities were observed in myeloid series and megakaryocytes. The prolonged plasma iron disappearance rate and the decreased plasma iron turnover rate also indicated erythroid hypoplasia. He had been free from any infections, including parvovirus B19, before manifestation of PRCA. Taken together, these results suggest a diagnosis of acute PRCA. This side effect of allopurinol should be taken into consideration.- - - - - - - - - - ranking = 1keywords = red (Clic here for more details about this article) |
5/325. Treatment of aregeneratoric anemia following an ABO-incompatible allogeneic peripheral blood stem cell transplantation: a case report.Aregeneratoric anemia (AA) occurs rarely after ABO-incompatible allogeneic peripheral blood stem cell transplantation (alloPBSCT), and its management is generally difficult. Here, we present a 31-year-old white man with myelodysplastic syndrome who developed AA after receiving stem cells from his human leukocyte antigen (HLA) identical, but ABO-incompatible sibling. Because his anti-A antibody titers were high, therapy with conventional doses of erythropoietin and prednisolone failed to treat the AA. Following 8 cycles of plasma exchange and higher doses of erythropoietin and prednisolone as well as danazol administration, anti-A titers decreased, and his anemia improved significantly. In conclusion, to treat and obtain a low titer of antibodies in a patient with AA following an ABO-incompatible alloPBSCT, higher doses of erythopoietin and corticosteroids associated with plasma exchange have to be used.- - - - - - - - - - ranking = 0.33333333333333keywords = red (Clic here for more details about this article) |
6/325. Severe hepatitis and pure red cell aplasia in adult Still's disease: good response to immunosuppressive therapy.adult-onset Still's disease is a systemic inflammatory disorder with a highly variable clinical course. Mild hepatitis and anemia are common manifestations. We describe a patient with adult Still's disease who developed a severe hepatitis and a life-threatening pure red cell aplasia. The hepatitis developed after treatment with NSAIDs was started. The patient was successfully treated with a combination of prednisone, cyclosporin, and methotrexate. physicians should be aware that severe hepatitis and pure red cell aplasia can occur in adult Still's disease. We recommend a careful monitoring of liver functions in patients with adult Still's disease who are being treated with NSAIDs.- - - - - - - - - - ranking = 1.1666666666667keywords = red (Clic here for more details about this article) |
7/325. Clonal T cell proliferation in patients with pure red cell aplasia.Among the patients with idiopathic pure red cell aplasia (PRCA) who do not meet the diagnostic criteria of chronic lymphocytic leukemia, there are some cases which suggest an association with clonal T lymphocytic proliferation. The morphological characteristics and responses to treatment revealed two distinct groups among the present 13 patients. The lymphocytes in one group were typical granular lymphocytes of T cell phenotype which were treated effectively with cyclophosphamide rather than cyclosporine. The response to therapy in this group occurred after a perceptible reduction in lymphocyte mass, which took at least 8 weeks. The lymphocytes in the second group consisted mainly of non-granulated lymphocytes or some granulated lymphocytes with fine and indistinct granules and responded well to cyclosporine therapy. A reduction in lymphocytes mass was not a prerequisite for the development of the remission of red cell aplasia in this group, and responses occurred within 4 weeks. Clonal T cell proliferation was detected in some patients, which raised the possibility of idiopathic PRCA being associated with a clonal proliferation of T cells. Distinguishing lymphocytes in patients with PRCA could potentially be used to plan treatment strategy and assess prognosis.- - - - - - - - - - ranking = 1.6666666666667keywords = red (Clic here for more details about this article) |
8/325. Clonality of acquired primary pure red cell aplasia.Acquired primary pure red cell aplasia (PRCA) has frequently been shown to be associated with T cells that inhibit marrow erythropoiesis. A 41-year-old female presented with anemia in December 1985. bone marrow examination revealed 1.8% erythroid cells. A diagnosis of PRCA was made. She received prednisolone, and her hemoglobin level recovered to 12 g/dl. In February 1995, her hemoglobin level decreased to 5.8 g/dl, and acquired primary PRCA recurred. Surface markers of peripheral blood mononuclear cells demonstrated CD4/8 ratio inversion. The T-cell receptor (TCR)-beta chain gene showed germ line configuration by Southern blot analysis of the mononuclear cells in peripheral blood. However, stepdown polymerase chain reaction analysis revealed that the TCR-beta gene of peripheral blood mononuclear cells was rearranged. With highly sensitive polymerase chain reaction analysis, clonality of T cells was confirmed. We propose that some acquired primary PRCA patients have a T-cell clonal disorder, similar to some PRCA patients with large granular lymphocytes leukemia or thymoma.- - - - - - - - - - ranking = 2.5keywords = red (Clic here for more details about this article) |
9/325. parvovirus B19 infection causing red cell aplasia in renal transplantation on tacrolimus.parvovirus B19 infection is known to cause chronic anemia in immunocompromised hosts, including organ transplant recipients. Most reported cases of parvovirus B19-associated aplastic anemia in renal transplant recipients responded to intravenous immunoglobulin (IVIG) infusion. tacrolimus is of special interest; it was proposed to be associated with pure red cell aplasia (PRCA) on its own because resolution of anemia on withdrawal of tacrolimus was previously observed. Interaction between parvovirus B19 infection and tacrolimus has not been reported. We report a case of parvovirus B19-associated PRCA in a renal transplant recipient treated with tacrolimus who failed to clear the virus despite repeated courses of IVIG. She showed complete recovery promptly after tacrolimus was switched to cyclosporine A. A well-documented concomitant decrease in serum parvovirus dna polymerase chain reaction titer was also observed. This shows another mechanism by which tacrolimus can aggravate PRCA because of impaired clearance of parvovirus B19 infection in transplant recipients. For those patients receiving tacrolimus who have parvovirus B19 infection with refractory anemia and who fail to recover with IVIG, replacement of tacrolimus with cyclosporine A can be considered.- - - - - - - - - - ranking = 1.1666666666667keywords = red (Clic here for more details about this article) |
10/325. adult onset cyclic hematopoiesis in a patient with myelodysplastic syndrome.A 75-year-old woman presenting with myelodysplastic syndrome showed cyclic oscillations in her white blood cell and platelet counts. Each cycle lasted for 5 to 6 months, with 4 cycles occurring over the course of a 2-year period. During successive cycles, the white blood cell count fluctuated from 10.1 to 2.6; 13.8 to 1.8; 11.0 to 1.6, and 8.6 to 1.3 x 10(9)/L. The platelet count fluctuated from 242 to 38, 199 to 11, 110 to 5, and 75 to 3 x 10(9)/L. The patient underwent red blood cell transfusions because of red blood cell aplasia; the frequency of the transfusions and the erythropoietin concentration in serum were inversely correlated. The number of circulating granulocyte-macrophage colony-forming units and CD34-positive cells in peripheral blood oscillated in phase with the white blood cell and platelet counts. These patterns suggested a periodic influx of progenitor cells from hematopoietic stem cells. The ratio of neutrophils to mononuclear cells remained essentially constant throughout the clinical course. Lymphocyte subset assessments using monoclonal antibodies showed an inverse CD4/CD8 ratio (less than 1) and extreme B cell lymphopenia throughout the fourth cycle. The percentage of CD3-positive cells oscillated inversely, suggesting that the cyclic cytopenia had an immune mechanism involving T lymphocytes.- - - - - - - - - - ranking = 0.33333333333333keywords = red (Clic here for more details about this article) |
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