Showing papers on "Pure red cell aplasia published in 1994"

Parvovirus B19 as a cause of acquired chronic pure red cell aplasia.

Abstract: Parvovirus B19 infection causes chronic anaemia in immunodeficient individuals by selective suppression of erythropoiesis. The bone marrow morphology is characteristic of pure red cell aplasia (PRCA). To determine the frequency of B19-induced PRCA we retrospectively analysed a series of 57 PRCA patients. B19 DNA was present in serum of eight patients (14%) and could be extracted from bone marrow aspirate slides from five of these patients. Recent exposure to the virus was confirmed by the presence of anti-B19 IgM in sera from four and by the finding of giant pronormoblasts in marrow aspirates from five of the B19 DNA-positive patients. The sensitivities of anti-B19 IgM and of giant pronormoblasts were only 50% and 63%, respectively; specificities were 90% and 92%. Unexpectedly, PRCA in two B19 DNA-positive patients remitted after antilymphocyte globulin or cyclosporin A therapy, suggesting that the clinical course of B19-induced PRCA may be indistinguishable from other forms of PRCA. As therapy with immunoglobulin is uniformly effective for treatment of B19-associated anaemia, our data suggest that all patients with acquired PRCA should be evaluated for evidence of B19 infection. B19 DNA analysis is the most reliable method to demonstrate infection.

Pure red cell aplasia following peripheral stem cell transplantation: complete response to a short course of high-dose recombinant human erythropoietin.

Abstract: We studied a patient who developed pure red cell aplasia (PRCA) following peripheral stem cell transplantation for non-Hodgkin lymphoma. Serum erythropoietin was appropriate for the degree of anemia. Corticosteroid treatment was ineffective. Four months after transplantation rHuEpo was administered subcutaneously at a dose of 150 U/Kg per day, five days a week for 8 weeks. Treatment induced an erythropoietic response and corrected anemia. Response was maintained following discontinuation of rHuEpo. This study and previous reports indicate that high doses of rHuEpo given over a short time can resolve PRCA following autologous or allogeneic stem cell transplantation.

Treatment of pure red cell aplasia after major ABO-incompatible bone marrow transplantation resistant to erythropoietin. Bone Marrow Transplantation Team.

Abstract: A case of pure red cell aplasia after major ABO-incompatible bone marrow transplantation with long duration of up to 482 days is presented. The patient exhibited resistance to treatment using intravenous gamma globulin, prednisolone and 8 week administration of erythropoietin. The patient finally responded to treatment using erythropoietin and methylprednisolone simultaneously. Because of its safety and efficacy, it might be worthwhile to try erythropoietin with methylprednisolone, if necessary, for pure red cell aplasia complicating major ABO-incompatible bone marrow transplantation.

Pure red cell aplasia associated with malignant thymoma, myasthenia gravis, polyclonal large granular lymphocytosis and clonal thymic T cell expansion.

Abstract: A case with the triad of pure red cell aplasia (PRCA), myasthenia gravis, and malignant thymoma is reported. There was a clonal proliferation of T cells within the thymoma, as demonstrated by a T cell antigen receptor (TCR) delta chain gene rearrangement. However, despite a large granular lymphocytosis, clonality could not be shown in the peripheral blood either before or after thymectomy. There was no evidence of human T cell lymphotrophic virus type 7 (HTLV1) infection. It is postulated that the clonal thymic T cell population secreted cytokine(s), which stimulated the polyclonal proliferation of large granular lymphocytes, which in turn suppressed erythropoiesis. Thymectomy removed the stimulus to the large granular lymphocytes and hence there was a resurgence of erythropoiesis.

T-Cell Large Granular Lymphocytic Leukemia and Pure Red Cell Aplasia in a Patient With Type I Autoimmune Polyendocrinopathy: Response to Immunosuppressive Therapy

Abstract: Clonal proliferations of large granular lymphocytes (LGLs) of T-cell origin characterize T-cell LGL leukemia. This disorder has been described in association with rheumatoid arthritis and other autoimmune phenomena. The presence of endocrinologic abnormalities in patients with T-cell LGL leukemia has not been previously reported, nor has T-cell LGL leukemia been described in patients with endocrinologic abnormalities. Herein we describe a young woman with type I autoimmune polyendocrinopathy, in whom pure red cell aplasia developed in association with clonal proliferation of LGLs. Immunosuppressive therapy with cyclophosphamide resulted in remission of pure red cell aplasia, transient improvement in hypocalcemia, and disappearance of the LGL clone. Clonal proliferation of LGLs may be associated with autoimmune endocrinopathies. Clinicians who are responsible for the care of such patients should be aware of this possible association.

Pure red cell aplasia: further evidence of T cell clonal disorder

Abstract: Summary. We report a patient with pure red cell aplasia and type I autoimmune polyglandular syndrome who also had an expansion of suppressor T lymphocytes in the peripheral blood. Southern blotting of DNA from these cells suggested T cell receptor (TCR) γ gene rearrangement. We confirmed true clonality of this by amplification of the gene rearrangement using the polymerase chain reaction and subsequent analysis of the product by gene cloning and DNA sequencing.

Aplastic anemia and pure red cell aplasia.

Abstract: The role of known hematopoietic growth factors in the pathogenesis of aplastic anemia and congenital hypoplastic anemia has been extensively studied and no evidence has been obtained that deficiency of these factors contributes to the hypoproliferative state in these disorders. Clonal hematopoiesis seems to be present at least in a small percentage of cases of aplastic anemia, a finding that needs further investigation. Androgens were shown to be beneficial only for women with aplastic anemia treated with antilymphocyte globulin. Unrelated-donor bone marrow transplantation is becoming a realistic approach for children and very young adults with aplastic anemia, but in older groups the survival is very poor. New observations on abnormalities of lymphokines and cytokines in Fanconi's anemia have been described, but their pathogenetic significance remains unknown. A large number of studies have excluded the possibility that abnormalities of c-kit/SCF genes and their expression are responsible for the erythroid aplasia in Diamond-Blackfan syndrome. Cyclosporine was found to be an effective treatment for pure red cell aplasia associated with chronic lymphocytic leukemia. The cell membrane receptor for B19 parvovirus has been identified as the P antigen. Long-term studies showed that in 20% of patients with homozygous sickle cell disease, infection by B19 does not cause erythroid aplasia.

Pure Red Cell Aplasia After Chemotherapy for Hodgkin's Lymphoma: In Vitro Evidence for T Cell Mediated Suppression of Erythropoiesis and Response to Sequential Cyclosporin and Erythropoietin

Abstract: Acquired pure red cell aplasia (PRCA) has been associated with various lymphoproliferative conditions but its occurrence with Hodgkin's disease is rare. We report a case of PRCA occurring immediately following the completion of induction chemotherapy in a patient with Stage IIIB nodular sclerosing Hodgkin's disease. In vitro erythroid colony studies documented evidence for T cell mediated suppression of erythropoiesis and lack of a serum inhibitor. Addition of cyclosporin to the in vitro cultures stimulated erythroid colony growth. Following in vivo treatment with cyclosporin peripheral blood CD4/CD8 ratios returned to normal. However, serum erythropoietin levels were inappropriately low. Subsequent treatment with erythropoietin induced a reticulocytosis and transfusion independence. Since discontinuing the erythropoietin, the patient has been able to maintain a hemoglobin of 100 g/L. This case illustrates that red cell aplasia occurring in the setting of Hodgkin's disease may be due to T cell mediated suppression of erythropoiesis. A response to cyclosporin may be masked by inappropriately low erythropoietin levels. © 1994 Wiley-Liss, Inc.

Successful treatment of refractory pure red cell aplasia secondary to chronic lymphocytic leukaemia with cyclosporine A: correlation between clinical and in vitro effects

Abstract: This report presents the case of a patient with PRCA in CLL where in vitro culture studies correlated well with successful CS-A treatment. Before initiating CS-A therapy, coculture studies showed that T-cells from peripheral blood of the patient suppressed the formation of CFU-E and BFU-E colonies by normal bone marrow cells. Normal erythropoiesis reappeared in the bone marrow of the patient 3 weeks after the start of CS-A therapy. At this time, cocultures demonstrated that peripheral blood T-cells no longer inhibited the growth of normal BFU-E, although there was persistent suppression of CFU-E. Six months later the patient was in stable remission from PRCA on maintenance therapy with CS-A. Moreover, cocultures showed no T-cell inhibition of normal BFU-E or CFU-E colony formation. The strong correlation between in vitro culture studies and a beneficial clinical outcome observed in this case suggests that in vitro cultures could be used to monitor CS-A treatment in patients with PRCA in CLL.

Pure red cell aplasia associated with angioimmunoblastic lymphadenopathy with dysproteinemia.

Abstract: Angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) can best be described as a disorder of T-cells resulting in amplification of the B-cell response and clinical symptoms of lymphadenopathy, fever, hepatosplenomegaly, and a variety of blood abnormalities. Pure red cell aplasia (PRCA), an autoimmune disorder resulting in selective aplasia of the erythroid series, has only rarely been associated with AILD. Herein we report three cases of AILD and PRCA. Serum from one patient was available for study and contained a dose-dependent inhibitor of the CFU-E but not CFU-GM cultures from normal bone marrow. This activity was found in the globulin fraction after ammonium sulfate precipitation. Patients with AILD are known to make antibodies to many autologous epitopes, and the most well-characterized mechanism of PRCA involves antibodies to red cell precursors. Our serum data are consistent with the hypothesis that such an antibody existed in our patient. Aggressive treatment of these patients resulted in transient improvement in two; however, all three died without achieving a durable complete remission with two dying of infectious complications.

Acquired idiopathic pure red cell aplasia in a hemodialyzed patient with inactive systemic lupus erythematosus

Abstract: A male patient suffered chronic renal failure due to lupus nephritis and was undergoing hemodialysis. Six years after beginning hemodialysis, anemia developed, which improved by erythropoietin. Unresponsiveness to erythropoietin gradually appeared, and with a suspicion of pure red cell aplasia, he was treated with a high-dose corticosteroid but the unresponsiveness did not improve. Neither his serum nor lymphocytes inhibited erythropoiesis of either normal bone marrow stem cells or his own in vitro. These observations suggest an impaired hematopoietic microenvironment in his bone marrow.

Chronic idiopathic pure red cell aplasia: successful treatment during pregnancy and durable response to intravenous immunoglobulin

Abstract: A patient with chronic idiopathic pure red cell hypoplasia responded to intravenous immunoglobulin therapy. No other treatment was administered. During treatment, the patient was taken through two pregnancies without haematological problems. After 22 months of therapy, the intravenous immunoglobulin was discontinued; a durable remission was subsequently maintained. Intravenous immunoglobulin may be the treatment of choice for females of child-bearing potential.

A case of pure red cell aplasia: follow-up on different immunosuppressive regimens

Abstract: A 66-year-old patient was admitted to our hospital in January 1992 for further evaluation of severe normocytic anemia. Hemoglobin (Hb) was 3.5 g/dl, reticulocyte count 1ℵ. Bone marrow showed a nearly complete lack of red cell precursors, thus favoring the diagnosis of acquired pure red cell aplasia (PRCA). Immunosuppressive therapy with prednisolone was started but had to be supplemented with azathioprine because of a further rapid decrease in Hb to 3.7 g/dl after an initial transfusion of 6 U red blood cells. However, with this regimen a renewed decrease in Hb to 6.6 g/dl was noted, and further transfusions were required. Therefore therapy was switched to cyclosporine A (CyA) while tapering off prednisolone. Four months after the initial diagnosis a positive parvovirus B19 IgM antibody was found. After the failure of hematological remission with three immunosuppressive regimens a course of high-dose intravenous immunoglobulins (IVIG) was administered in July 1992. Six weeks after IVIG therapy a peak hemoglobin concentration of 12.3 g/dl was noted, and further transfusion was not required. CyA was tapered off in October 1992. One month later CyA was reinstituted because of a relapse of PRCA but was unsuccessful until January 1993. At this time immunosuppressive CyA therapy was discontinued because of a periodontal abscess. In February 1993 a second IVIG infusion was given, and a second remission of PRCA was noted, showing an increase in hemoglobin up to 14.5 g/dl by November 1993. At the last follow-up visit in February 1994 our patient was still in complete hematological remission.

Malignant fibrous histiocytoma in a patient with Blackfan-Diamond anemia.

Abstract: Blackfan-Diamond anemia, a chronic, pure red cell aplasia, is rare. Malignant fibrous histiocytoma is also uncommon; it can arise in pre-existing bone lesions. The authors describe a 23-year-old man with Blackfan-Diamond anemia complicated by hemochromatosis in whom malignant fibrous histiocytoma developed in the distal femur.

[Parvovirus B19-induced aplastic crisis in a patient with iron deficiency anemia].

Abstract: A 38-year-old female was referred to Takaoka City Hospital for treatment of common-cold-like symptom and an episode of transient unconsciousness. Physical examination on admission revealed severe anemia and an ejection heart murmur. Complete blood count revealed microcytic hypochromic anemia (Hb 4.1 g/dl), leukocytopenia (2.600/microliters), thrombocytopenia (7.1 x 10(4)/microliters) and reticulocytopenia (17,000/microliters). The bone marrow cellularity was within normal limits. Cells in the erythroid series were decreased to 5% of total bone marrow nucleated cells with maturation arrest at the level of proerythroblasts. Giant proerythroblasts were observed in 0.2% of marrow nucleated cells. No stainable iron was seen. Both anti-parvovirus B19 IgM antibody and IgG antibody were positive in the serum and parvovirus B19 DNA was detected in the bone marrow cells by polymerase chain reaction. From these results, iron deficiency anemia complicated with pure red cell aplasia secondary to parvovirus B19-induced infection was diagnosed. The anemia gradually improved with administration of sodium ferrous citrate one month after admission. Parvovirus B19 has been reported to cause an aplastic crisis in the patients who has a rapid red cell turn over such as hemolytic anemia or acute blood loss. This report suggested that severe aplastic crisis is also induced in patients with iron deficiency anemia by parvovirus B19-induced infection and warns that careful observation is necessary for the follow up of patients with iron deficiency anemia.

[Pure red cell aplasia induced by erythromycin and furosemide effects on in vitro erythroid colony forming unit (CFU-E)].

Abstract: A 84-year-old man was treated with antibiotics including erythromycin and a diuretic (furosemide) because of acute heart failure and pneumonia. During the treatment, he developed moderate anemia (Hb 8.7g/dl). His anemia improved after the treatment. He again developed marked anemia (Hb 6.3g/dl) during the second treatment with erythromycin and furosemide and received blood transfusions. Bone marrow aspiration study revealed severe erythroid hypoplasia (0.2%). He was referred to our hospital, but he was not treated because his hemoglobin levels and reticulocyte count increased (80%) and his bone marrow showed increased erythroblasts (41.5%). His anemia gradually improved without any treatment. We diagnosed the case as drug-induced pure red cell aplasia (PRCA). We cultured bone marrow cells obtained from the present case and four normal healthy volunteers by a plasma clot method to determine the effects of two drugs on the number of erythroid colony forming unit (CFU-E). Furosemide strongly inhibited the CFU-E colony formation in the patient, but the inhibition effect of erythromycin was moderate. Furthermore, CFU-E was markedly suppressed by a combination of erythromycin and furosemide in both patient and control materials. These results indicate that both furosemide and erythromycin were related to the occurrence of PRCA in this patient.

Pure red cell anemia with erythropoietic maturation arrest and spontaneous recovery.

Abstract: We report the case of a 21-year-old male who presented with severe anemia and hypercellular bone marrow, without known cause. The dominant feature of the bone marrow was that of a maturation arrest in the erythroid series, with absolute reticulocytopenia in the peripheral blood. Immunosuppressive therapy was withheld, but transfusions were given to maintain his hemoglobin prior to full recovery. The patient had features of both pure red cell anemia and of transient erythroblastopenia of childhood.

A case of pure red cell aplasia associated with sodium valproate therapy

Abstract: We reported a 14-year-old female with petit mal epilepsy who suffered from pure red cell aplasia 12 weeks after sodium valproate (VPA) therapy started. On admission she presented anemia but no jaundice. Her hemoglobin was 5.4g/dl and bone-marrow biopsy revealed hypocellularity with erythroid hypoplasia. The Direct Coombs' test and anti-nucleotide antibody were positive. Reticulocytosis occurred 10 days after discontinuation of VPA. The Direct Coombs' test became negative one year after discontinuation of VPA. In general, drug-induced PRCA is caused by toxic or allergic agents, but this case suggests the immune mechanism of VPA-induced PRCA.

[Pure red cell aplasia with thymoma, myasthenia gravis and normal pressure hydrocephalus].

Abstract: Cases of pure red cell aplasia with thymoma and myasthenia gravis are rare. We described a patient who had concomitant pure red cell aplasia, thymoma, myasthenia gravis and a normal pressure hydrocephalus. A 63-year-old man with disturbances of gait, left blepharoptosis and anemia was presented to our hospital. Laboratory examination on admission revealed severe anemia. Bone marrow aspirates showed erythroid hypoplasia of marked degree. Chest x-ray and chest CT revealed a tumor to the right of cardiac wall. The tensilon chloride test and antiacetylcholine receptor antibody were positive. A hydrocephalus was demonstrated with brain CT. Cerebrospinal fluid pressure was in the normal range. He was diagnosed as having pure red cell aplasia with thymoma, myasthenia gravis and a normal pressure hydrocephalus. This appears to be a fairly rare case. It seems important to consider that a normal pressure hydrocephalus may have immunological disorders.