Spontaneous remission in pure red cell aplasia associated with thymoma.

A 57-year-old woman with thymoma-associated pure red cell aplasia (PRCA) manifested spontaneous recovery of erythropoiesis. Anemia recurred before the surgical removal of her thymoma, however, and the second anemic remission following surgery did not occur promptly. In this report, we describe a rare occurrence of spontaneous remission in PRCA.     

Pure red cell aplasia associated with thymoma

A case of thymoma with mixed spindle and lymphocytic variety along with acquired, secondary, chronic pure red cell aplasia is an uncommon entity. The presented case is a case of anterior mediastinal tumour with marked anemia. On histopathologic and hematological examinations, it proved to be a case of thymoma along with pure red cell aplasia. High index of suspicion, bone marrow examination, radiological including CT scan examination, total thymectomy after preparation with repeated blood transfusion remains the mainstay of treatment. Prolonged corticosteroid therapy leads to remission for upto ten months after operative intervention.     

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.     

Autoreactive erythroid progenitor-T suppressor cells in the pure red cell aplasia associated with thymoma and panhypogammaglobulinemia

In vitro erythroid culture studies and lymphocyte markers were performed in a patient with a spindle cell thymoma who developed red cell aplasia, panhypogammaglobulinemia, and multiple opportunistic infections. At the time of presentation, erythroid progenitor cells (CFUe, BFUe) were markedly reduced when cultured from marrow mononuclear cells. Removal of T cells from bone marrow mononuclear cells by E-rosetting or complement-mediated lysis with OKT3 pan T cell monoclonal antibody increased growth of erythroid progenitor cells in vitro. Readdition of bone marrow or pleural fluid T cells derived from the thymoma suppressed autologous, but not allogenic, erythroid progenitor cell (CFUe, BFUe) proliferation in vitro. The erythroid progenitor suppressor T cells were predominantly OKT11+, OKT3+, OKT8+ and Ia+ consistent with activated suppressor T cells. Treatment of the patient with cyclophosphamide and corticosteroids reduced marrow lymphocytes fourfold, and a prompt reticulocytosis ensued. After recovery, erythroid progenitor cells were easily detectable. These studies provide new evidence for T cell-mediated suppression of erythropoiesis in a unique subset of patients with red cell aplasia associated with thymoma and hypogammaglobulinemia.     

Successful corticosteroid therapy of a recurrent thymoma with pure red cell aplasia]

A 71-year-old man underwent extended thymomectomy with partial resection of the upper lobe of the left lung and pericardium for stage III invasive thymoma in March, 2000. Postoperative chemotherapy and radiation therapy were carried out. The patient did well until June 2005, when a mediastinal mass and pleural dissemination were detected. The recurrence of the thymoma was strongly suspected radiographically. Combination chemotherapy of carboplatin and paclitaxel achieved partial remission. About three months after the last chemotherapy, the patient had a severe anemia in February 2006. Based on the blood data and the bone marrow examination, pure red cell aplasia was diagnosed. Prednisolone treatment (50 mg daily) resulted in dramatic regression of recurrent mediastinal and pleural tumors, as well as improvement of pure red cell aplasia.     

Cyclosporin improved pure red cell aplasia associated with thymoma and tended to decrease thymoma size: a case report]

The case was a 56-year-old man who underwent extended thymothymectomy in 1993 because of an invasive thymoma classified as Masaoka IIa. The tumor was disseminated through the right thoracic cavity in 1994. Although the chemotherapy was repeated, the disseminated thymoma tended to increase in size. The patient was suffering from severe anemia, and pure red cell aplasia was diagnosed in September 2002. The administration of cyclosporin was initiated, and not only brought about improvement of pure red cell aplasia but also tended to shrink the thymoma. Cyclosporin may be an effective drug for the reduction of thymoma as well as for pure red cell aplasia.     

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。     

Demonstration of three distinct immunological disorders on erythropoiesis in a patient with pure red cell aplasia and autoimmune haemolytic anaemia associated with thymoma

A patient with pure red cell aplasia (PRCA) and autoimmune haemolytic anaemia (AIHA), associated with a thymoma which had already been removed, was studied in order to investigate the pathogenesis of PRCA and AIHA. The autoantibody eluted from the surface of the patient's red blood cells (RBC) reacted with the large E antigen of the Rh complex. Immunoglobulin-G (IgG) purified from the patient's serum suppressed CFU-E and BFU-E but not CFU-GM colony formation in the presence of complement. This antibody was not adsorbed with large E antigen. T-lymphocytes in the bone marrow suppressing autologous CFU-E and BFU-E colonies were demonstrated. Thus, three distinct immunological disorders on erythropoiesis were present in this patient with PRCA and AIHA associated with thymoma in a thymectomized state.     

Steroid-responsive pure red cell aplasia associated with natural killer cell lymphocytosis

A patient with pure red cell aplasia and expansion of the peripheral blood natural killer (NK) cell population is described. Despite normal absolute and differential leukocyte counts, NK cells were increased at diagnosis and at relapse. Furthermore, these cells were not morphologically recognizable on the peripheral blood smear examination. A favorable clinical response to glucocorticoid therapy was accompanied by a decrease in NK cells.     

Chronic T-cell lymphoproliferative disorder associated with pure red cell aplasia

A 42-year-old woman was admitted to our hospital because of easy fatigability in Jan. 1976. Laboratory examination revealed severe macrocytic anemia and slight lymphocytosis. She was diagnosed as having pure red cell aplasia (PRCA). She went into hematological remission 6 weeks following 40 mg/day treatment with prednisolone, but the anemia relapsed frequently when the dosage was lessened. She was then treated with 50 mg/day of cyclophosphamide, 50 mg/day of azathioprine, splenectomy, and methylprednisolone pulse therapy, but the recovery from anemia was temporary after each treatment. Since 1984, peripheral lymphocyte counts were 1-30,000/microliters, and reticulocyte counts were 0. She died of sepsis of Listeria in Sep. 1986. Peripheral lymphocytes had large azurophilic granules and an immunophenotype of OKT3+8+11+Ia1+Leu7+.     

Intrahepatic cholestasis and pure red cell aplasia associated with ticlopidine

A 77-year-old woman developed jaundice and anemia 3 and 8 weeks, respectively, after starting ticlopidine (100 mg daily) for cerebral infarction. From the laboratory findings, including histological study of the liver and bone marrow specimen, ticlopidine-induced intrahepatic cholestasis and pure red cell aplasia were highly suspected. Jaundice slowly improved after the withdrawal of ticlopidine. Anemia immediately improved with steroid therapy. These are very rare adverse effects of ticlopidine; nevertheless, periodic laboratory examinations are recommended.