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

Angioimmunoblastic T-cell lymphoma (AILT) is a peripheral T-cell lymphoma often complicated autoimmune phenomena such as autoimmune cytopenia, and is a truly rare type of NHL. In order to investigate the clinical features, pathological manifestation of this lymphoma, and to explore its therapy protocol, a 37-years old patient with AILT was investigated. The routine blood examination, bone marrow smear, lymphonodus biopsy, Coombs test, flow cytometry for bone marrow mononuclear cells, serological test, immunochemistry method etc were performed for this patient. The results showed that the systemic lymphadenectasis and hepatosplenomegaly were seen in patient, the cervical lymphonode biopsy revealed AITL. The hematoglobin level and number of reticulocytes were very low. Coombs test was positive. Simultaneously, the bone marrow aspirate revealed erythroid aplasia. The warm type autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were co-existed. After one course of chemotherapy with CHOP-E, infiltration sign of AITL patient with AIHA and PRCA disappeared. In conclusion, the AITL patient complicated with AIHA and PRCA was successfully diagnosed, the lymphonode biopsy and bone marrow smear showed more significant, the chemotherapy protocol of CHOP-E can give some effect to cure such angioimmunoblastic T cell lymphoma.
Adult ; Anemia, Hemolytic, Autoimmune ; complications ; diagnosis ; Humans ; Immunoblastic Lymphadenopathy ; complications ; diagnosis ; Lymphoma, T-Cell ; complications ; diagnosis ; Male ; Red-Cell Aplasia, Pure ; complications ; diagnosis

OBJECTIVETo analyze the characteristics of T-cell large granular lymphocyte leukemia (T-LGLL).

METHODSRetrospectively analyze the clinical and laboratory data of 27 patients with T-LGLL diagnosed between 1999 and 2007 in our hospital.

RESULTSThe median age at diagnosis was 48 years. All patients were symptomatic, mainly complaining of fatigue. Of the 27 patients, 14 (51.9%) had splenomegaly, and 4(14.8%) hepatomegaly. Rheumatoid arthritis was not present in any patients. The most frequent hematological abnormality was anemia (24 patients, 88.9%) with a median Hb level of 57.5 g/L. Pure red cell aplasia was found in 18 patients (66.67%). The median WBC count was 4.24 x 10(9)/L and 19 cases were neutropenia (ANC < 1.5 x 10(9)/L). The median LGL count in peripheral blood was 1.45 x 10(9)/L and most of them (77.8%) were less than 2.0 x 10(9)/L. Twenty-two patients (81.5%) showed the CD3+ CD8+ CD57+ CD56(-) LGL phenotype. With immunosuppressive therapy, 91.3% of patients responded and complete hematological remission rate was 65.2%.

CONCLUSIONT-LGLL mainly presented with anemia and complete hematological remission rate was 65.2%. Pure red cell aplasia was commonly associated with the disease. The patients had a good response to immunosuppressive therapy.

Adolescent ; Adult ; Aged ; Female ; Humans ; Immunophenotyping ; Immunosuppression ; Leukemia, Large Granular Lymphocytic ; complications ; diagnosis ; immunology ; Male ; Middle Aged ; Red-Cell Aplasia, Pure ; etiology ; Retrospective Studies ; Young Adult

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