Relapsing polychondritis (RP) is a rare multisystem disorder. Myelodysplastic syndrome (MDS) with erythroid hypoplasia/aplasia is a rare form of myelodysplasia. Several cases of RP associated with MDS have recently been described. However, RP associated with MDS with erythroid hypoplasia/aplasia has never been reported. There was only one case report of polymyalgia rheumatica associated with MDS with erythroid hypoplasia/aplasia. In this study, we report a 79-year-old patient with RP, who developed MDS subtype refractory anemia (RA) with erythroid hypoplasia/aplasia, a very characteristic subtype of MDS.
Aged ; Biopsy ; Human ; Male ; Myelodysplastic Syndromes/*complications ; Polychondritis, Relapsing/*complications/*diagnosis/pathology ; Red-Cell Aplasia, Pure/*complications/pathology

Myelodysplastic syndrome (MDS) with erythroid aplasia is a very rare disorder that has not been clearly defined. We experienced a case of pure red cell aplasia (PRCA), which evolved to MDS with erythroid aplasia. A 59-year-old male with transfusion-dependent PRCA was referred to our hospital for an evaluation of newly developed thrombocytopenia. Two years ago, PRCA was diagnosed by the laboratory findings and a bone marrow examination, which showed no evidence of any myelodysplastic features and thymoma. Upon admission, the bone marrow findings showed marked hypercellularity. with numerous dysplastic features in the three lineages including erythroid hypoplasia. These findings were compatible with a diagnosis of MDS with red cell aplasia. It is very interesting that the PRCA evolved to MDS with red cell aplasia, which strongly suggests an autoimmune mechanism for the development of MDS.
Erythroid Progenitor Cells/*pathology ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes/*complications/pathology ; Red-Cell Aplasia, Pure/*complications/pathology

Pure red cell aplasia (PRCA) is a rare cause of anemia associated with systemic lupus erythematosus (SLE), and fewer than 20 cases have been reported. The development of PRCA may be mediated by an autoimmune mechanism which is supported by the presence of antibodies that impair various stages and mechanisms of erythropoiesis, by the association with immunological disorders or lymphoma, and by a favorable response to immunosuppressive drugs, antilymphocyte globulin, thymectomy, and splenectomy. However, these therapies have not been successful in all patients with PRCA. We report our experience with a 31-year-old female patient with SLE who developed PRCA that did not respond to immunosuppressive therapies. However, complete normalization of erythropoiesis was achieved after the removal of the autoantibodies by plasmapheresis, and the patient has now maintained a normal hemoglobin level for more than eight months. We suggest that plasmapheresis might be tried in the treatment of PRCA cases before other more aggressive therapies are commenced.
Adult ; Bone Marrow/pathology ; Case Report ; Erythropoiesis ; Female ; Human ; Lupus Erythematosus, Systemic/*complications ; *Plasmapheresis ; Red-Cell Aplasia, Pure/*complications/pathology/physiopathology/*therapy

Pure red cell aplastic anemia (PRCA) is a group of heterogeneous disorders characterized by erythropoiesis failure or disorder of bone marrow function. PRCA can be classified as congenital and acquired types. The recent researches have shown that cellular immune abnormalities are main reasons to cause PRCA. This review will focus on the focus points: T lymphocytes abnormalities, change of T-cell subset phenotypes, abnormality of cellular clonality, and effects of related cytokines in PRCA.
Humans ; Immune System Diseases ; blood ; complications ; Immunity, Cellular ; immunology ; Red-Cell Aplasia, Pure ; etiology ; immunology ; Research ; trends ; T-Lymphocytes ; immunology ; pathology

We report a case of pure red cell aplasia (PRCA), which was initially suspected as a result of bone marrow involvement of diffuse large B cell lymphoma. Persistent anemia without an obvious cause was observed in a 47-yr-old man diagnosed with relapsed diffuse large B cell lymphoma. The bone marrow study showed only erythroid hypoplasia without the evidence of bone marrow involvement with lymphoma cells, thus PRCA was suggested. However, parvovirus infection was excluded as a potential cause of PRCA because of negative IgM anti-parvovirus B19 antibody and negative parvovirus PCR in the serum. Latent Epstein-Barr virus (EBV) infection of bone marrow was suggested by in situ hybridization with EBV-encoded small RNA (EBER) that showed a strong positive expression in bone marrow cells. Thus, PRCA was thought to be associated with latent EBV infection in bone marrow cells. Although the finding of unexplained anemia is a possible predictor of bone marrow involvement with lymphoma cells, PRCA as a result of a viral infection including EBV should be considered in lymphoma patients. This is the first report of the occurrence of PRCA associated with latent EBV infection in a patient with non-Hodgkin's lymphoma.
Adult ; Bone Marrow/pathology ; Bone Marrow Diseases/*complications/diagnosis/pathology ; Bone Marrow Neoplasms/pathology ; Diagnosis, Differential ; Epstein-Barr Virus Infections/*complications/diagnosis ; Humans ; Lymphoma, Large B-Cell, Diffuse/*complications/pathology ; Male ; Red-Cell Aplasia, Pure/diagnosis/*etiology

The association of thymoma with pure red cell aplasia has been well documented, but amegakaryocytic thrombocytopenia is not a recognized paraneoplastic syndrome complicating thymoma. We report a case of thymoma-complicated pure red cell aplasia and amegakaryocytic thrombocytopenia in a 73-yr-old woman. Pure red cell aplasia was diagnosed seven months after the detection of thymoma. One year after the diagnosis of pure red cell aplasia and seven months after thymectomy, bone marrow aspiration and biopsy showed an absence of megakaryocytes, marked erythroid hypoplasia with normal myeloid series. A diagnosis of amegakaryocytic thrombocytopenia and pure red cell aplasia was made. Oral steroid maintenance therapy resulted in recovery of platelet count. She has still transfusion-dependant anemia but platelet and neutrophil counts had been maintained in normal range for more than five months, until the last follow-up. We think that autoreactive T cells may induce a clinical autoimmune response even after eradication of thymoma, and aplastic anemia as a late complication following thymectomy was described in previous cases. This patient also has to be under a close observation because of the possibility to evolve into aplastic anemia.
Aged ; Bone Marrow/pathology ; Female ; Humans ; Imidazoles/therapeutic use ; Megakaryocytes/pathology ; Pregnadienetriols/therapeutic use ; Red-Cell Aplasia, Pure/complications/*diagnosis ; Thrombocytopenia/*diagnosis/drug therapy/*etiology ; Thymectomy/*adverse effects ; Thymoma/*complications/diagnosis/surgery ; Thymus Neoplasms/*complications/diagnosis/surgery

Hepatitis A is typically a self-limited acute illness that does not progress to chronic hepatitis. In rare cases, acute hepatitis A can be associated with serious complications (such as fulminant hepatitis or acute kidney injury) and may result in death or liver transplantation. Pure red cell aplasia (PRCA) is a rare hematologic disorder characterized by anemia, reticulocytopenia in the blood, and isolated erythroblastopenia with normal granulopoiesis and megakaryopoiesis in the bone marrow. PRCA is a rare hematopoietic complication of acute viral hepatitis, and few cases associated with hepatitis A virus infection have been reported. Recently, we experienced a case of severe hepatitis A complicated by fulminant hepatitis and acute kidney injury followed by PRCA which showed a favorable response to oral corticosteroids.
Acute Disease ; Acute Kidney Injury/etiology ; Adult ; Anti-Inflammatory Agents/therapeutic use ; Bone Marrow/pathology ; Female ; Hepatitis A/complications/*diagnosis ; Humans ; Prednisone/therapeutic use ; Red-Cell Aplasia, Pure/complications/*diagnosis/drug therapy

Pure red cell aplasia (PRCA) and autoimmune hemolytic anemia (AIHA) have rarely been reported as an extrahepatic manifestation of acute hepatitis A (AHA). We report herein a case of AHA complicated by both PRCA and AIHA. A 49-year-old female with a diagnosis of AHA presented with severe anemia (hemoglobin level, 6.9 g/dL) during her clinical course. A diagnostic workup revealed AIHA and PRCA as the cause of the anemia. The patient was treated with an initial transfusion and corticosteroid therapy. Her anemia and liver function test were completely recovered by 9 months after the initial presentation. We review the clinical features and therapeutic strategies for this rare case of extrahepatic manifestation of AHA.
Acute Disease ; Adult ; Anemia, Hemolytic, Autoimmune/*complications/*diagnosis/drug therapy ; Antineoplastic Agents, Hormonal/therapeutic use ; Bone Marrow/pathology ; Female ; Hepatitis A/*complications/*diagnosis ; Humans ; Male ; Middle Aged ; Prednisolone/therapeutic use ; Red-Cell Aplasia, Pure/*complications/*diagnosis/drug therapy ; Treatment Outcome ; Young Adult

Parvovirus B19 infection is known to cause chronic anemia in immunocompromised hosts, including organ transplant recipients. We report the first case of liver transplant recipient with parvovirus B19-induced pure red cell aplasia in Korea. A 57-yr-old female patient with hepatocellular carcinoma due to hepatitis C virus received a liver transplantation. Two months later, anemia developed and she received periodic red blood cell transfusions. However, chronic anemia persisted and bone marrow examination was performed 8 months after transplantation. Bone marrow aspiration smears showed markedly reduced erythroid precursors with atypical giant pronormoblasts and nuclear remnants with viral inclusions, and characteristic lantern cells were observed in biopsy sections. In addition, parvovirus B19 DNA PCR was positive. She was diagnosed as parvovirus B19-induced pure red cell aplasia and her anemia was improved following intravenous immunoglobulin therapy.
Blood Transfusion ; Bone Marrow/pathology ; Carcinoma, Hepatocellular/etiology/therapy ; DNA, Viral/analysis ; Female ; Hepatitis C/complications/diagnosis ; Humans ; Immunocompromised Host ; Immunoglobulins/therapeutic use ; Liver Neoplasms/etiology/therapy ; Liver Transplantation ; Middle Aged ; Parvoviridae Infections/complications/*diagnosis ; *Parvovirus B19, Human/genetics ; Red-Cell Aplasia, Pure/*diagnosis/therapy/virology

Anti-erythropoietin antibodies usually cross-react with all kinds of recombinant erythropoietins; therefore, erythropoiesis-stimulating agent (ESA)-induced pure red-cell aplasia (PRCA) is not rescued by different ESAs. Here, we present a case of ESA-induced PRCA in a 36-yr-old woman with chronic kidney disease, whose anemic condition improved following reintroduction of darbepoetin-alpha. The patient developed progressive, severe anemia after the use of erythropoietin-alpha. As the anemia did not improve after the administration of either other erythropoietin-alpha products or erythropoietin-beta, all ESAs were discontinued. Oxymetholone therapy failed to improve the transfusion-dependent anemia and a rechallenge with ESAs continuously failed to obtain a sustained response. However, her anemia improved following reintroduction of darbepoetin-alpha at 3 yr after the initial diagnosis. Interestingly, anti-erythropoietin antibodies were still detectable, although their concentration was too low for titration. In conclusion, darbepoetin-alpha can improve ESA-induced PRCA when the anti-erythropoietin antibody titer declines and its neutralizing capacity is lost.
Adult ; Anemia/drug therapy/etiology ; Antibodies/*blood/immunology ; Bone Marrow Cells/pathology ; Drug Hypersensitivity/immunology ; Erythropoietin/*analogs & derivatives/therapeutic use ; Erythropoietin, Recombinant/adverse effects/*immunology/therapeutic use ; Female ; Glomerulonephritis, IGA/complications ; Hematinics/adverse effects/immunology/*therapeutic use ; Humans ; Kidney Failure, Chronic/complications ; Oxymetholone/therapeutic use ; Red-Cell Aplasia, Pure/chemically induced/*drug therapy/immunology