Corticosteroid-responsive pure red cell aplasia in chronic lymphatic leukemia

Acquired pure red cell aplasia characterized by selective failure of erythropoiesis was seen in a 37-yr-old female patient with a diagnosis of chronic lymphatic leukemia (CLL). Though different immunosuppressive therapies have been tried in the past with variable response, there has been no case report of such favorable response to corticosteroids alone. We report this rare and unusual case of PRCA associated with CLL who responded to steroid therapy.     

Fenbufen induced pure red cell aplasia in rheumatoid arthritis

Pure red cell aplasia occurred after fenbufen administration in a patient with severe rheumatoid arthritis. In vitro studies were performed to determine the pathogenesis of the selective red cell aplasia. No cellular or humoral inhibitory mechanisms were demonstrated on growth of erythroid and multipotent bone marrow progenitors. Also, no direct effect of fenbufen alone or in combination with IgG and/or patient serum was found. It is possible that a metabolite of the drug formed from its metabolism was responsible for the aplasia and that the target marrow cell precursor affected is later than both erythroid bone marrow progenitors (BFU-E and CFU-E) and therefore not apparent in our studies. Recovery upon cessation of fenbufen suggests its implication in the pure red cell aplasia.     

Rheumatoid arthritis and pure red cell aplasia

Three patients with severe, deforming, and long-standing rheumatoid arthritis developed pure red cell aplasia that did not remit after withdrawal of medications, ran a chronic course, and in two patients remitted only after cytotoxic immunosuppressive treatment. An IgG inhibitor of autologous erythroid colony-forming and burst-forming unit growth in vitro was found in the serum of one patient. This specific erythropoietic inhibitor persisted in lower titer in the patient's serum even after an azathioprine-induced remission of pure red cell aplasia, indicating the possible need for maintenance immunosuppressive therapy. Chronic pure red cell aplasia may be another extra-articular manifestation of rheumatoid arthritis and should be considered when severe anemia develops in the absence of blood loss or hemolysis.     

Pure red cell aplasia in rheumatoid arthritis

We report a patient with longstanding rheumatoid arthritis (RA) who developed pure red cell aplasia. This condition is a rare complication of RA. Our patient recovered, apparently in response to treatment with corticosteroids and cyclophosphamide followed by azathioprine.     

Rheumatoid arthritis with eosinophilic fasciitis and pure red cell aplasia

A case of longstanding rheumatoid arthritis with concurrent development of eosinophilic fasciitis and pure red cell aplasia is described. A simultaneous occurrence of all 3 disorders has not been reported. Treatment with moderate dosages of prednisone resulted in a prompt and complete remission of both the fasciitis and the selective marrow aplasia.     

First case report of acquired pure red cell aplasia associated with micafungin

A 70-year-old Japanese man with chronic kidney disease under treatment with oral prednisolone for organizing pneumonia developed pulmonary aspergilloma. The patient was started on micafungin (MCFG), with no addition of any other new drug. About 5 weeks later, aggravation of his normocytic anemia associated with a low reticulocyte count was observed. Bone marrow puncture and biopsy revealed intense hypoplasia of the erythroblasts. As there was no evidence of malignancy, human parvovirus B19 infection, autoimmune diseases or hemorrhage, the patient was diagnosed as having acquired pure red cell aplasia (PRCA). The anemia improved along with an increase of the reticulocyte count to the normal level within 12 weeks of discontinuation of the MCFG therapy. The patient showed no evidence subsequently of any recurrence of the normocytic normochromic anemia or relapse of the PRCA. This is the first reported case of PRCA associated with MCFG.     

Acquired pure red cell aplasia in Japan

Abstract: We reviewed the clinical features of 150 patients with acquired pure red cell aplasia (PRCA) in Japan. There were 35 patients with acute type and 115 with chronic type PRCA. Of the acute PRCA patients, 17 had human parvovirus B19 infection. Drug-induced PRCA was demonstrated in 7 patients. Of the 115 patients with chronic PRCA, 51 patients were classified as primary and 64 cases were associated with miscellaneous diseases such as thymoma, a variety of hematological disorders and collagen diseases. Among the hematological disorders, PRCA was most frequently seen in granular lymphocyte proliferative disorders (GLPD). The erythroid colony growth patterns from bone marrow were variable. The serum erythropoietin level was high in most patients. Various kinds of treatment were tried for the chronic PRCA cases. Cyclosporin A (CyA) was the most effective form of treatment and the response rate was 82% (31/38). Twenty-three of 37 patients (62%) responded to bolus methylprednisolone therapy. The largest number of patients were treated with oral prednisolone, and the therapy was effective in 27 of the 55 (49%). The response rate to cyclophosphamide was only 29% (5/17), but in combination with prednisolone, half of the patients (7/14) responded to the therapy. CyA is recommended as the first-line therapy for acquired chronic PRCA.     

Acquired pure red cell aplasia due to treatment with clopidogrel: first case report

A 62-year-old woman with hypertension, type 2 diabetes mellitus, hyperlipemia and coronary heart disease started taking clopidogrel, with no addition of any other new drugs. However, with the addition of the drug, the patient was diagnosed as having acquired pure red cell aplasia (PRCA), and no any other inducing factors were detected from the patient. Furthermore, with the withdrawal of clopidogrel from the treatment, the patient recovered from the PRCA and did not recur. Therefore, we report PRCA as a rare side effect of clopidogrel for the first time.     

纯红细胞再生障碍性贫血2例报道----附文献复习

纯红细胞再生障碍性贫血(pure red cell aplasia,PRCA)是以骨髓红系造血衰竭为突出表现的临床综合征,部分患者还伴有白细胞和(或)血小板轻度减少,但骨髓粒系和巨核系比例不减少,仅红系比例显著减低,甚至缺如。PRCA分为先天性(如Diamond-Blackfan综合征)和后天获得性,而后天获得性又有原发性和继发性之分。本文报道我院二例获得性PRCA患者,并文献复习提高大家对此病的认识。     

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.