Pure red-cell aplasia and autoimmune hemolytic anemia in a patient with acute hepatitis A

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.

Síndrome de Good y aplasia pura de la serie roja, reporte de un caso y revisión de la literatura: Report of one case / Association of Good syndrome with pure red cell aplasia

Background: The association between Good's syndrome (hypogammaglobulinemia and thymoma) with pure red aplasia is very uncommon. We report a 70-year-old male, who had a thymoma excised nine years before. Afterwards, he suffered frequent respiratory infections, which were attributed to a humoral immunodeficiency. Nine years later, he developed a pure red cell aplasia. He received prednisone and cyclosporine, resulting in a progressive rise of hemoglobin level, after one month of treatment. The patient died shortly thereafter due to infection, complicating a domestic accident.

A Case of Severe Acute Hepatitis A Complicated with Pure Red Cell Aplasia / 대한소화기학회지

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.

Acquired Amegakaryocytic Thrombocytopenia after Thymectomy in a Case of Pure Red Cell Aplasia Associated with Thymoma / 대한진단검사의학회지

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.

Myelodysplastic syndrome with erythroid hypoplasia: a rare and distinct clincopathological entity--a report of two cases.

Erythroid hypoplasia in myelodysplastic syndrome (MDS) happens to be a rare association and is being recognized as a distinct clinico pathological entity. We report here two such cases diagnosed as Refractory anaemia (RA) and Refractory anaemia with excess blast (RAEB) who had marked suppression of the erythroid cell lines. Both patients presented with severe transfusion dependent anaemia. Recognition of these cases is important as alternative modalities of treatment such as immunosuppressives may be considered for these patients.

Myelodysplastic Syndrome with Erythroid Aplasia following Pure Red Cell Aplasia

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.

Pure red cell aplasia--report of 11 cases from eastern Nepal.

There were eleven cases of pure red cell aplasia diagnosed over a period of 2 years (January 2000-December 2001). All the patients had anemia with pallor and weakness being the presenting complaints. Hematological profile depicted normocytic normochromic anemia, reticulocytopenia and marked paucity of erythroid precursors on bone marrow aspiration and biopsy studies. In the present study, one case was of congenital pure red cell aplasia, in one other case of pyrexia of unknown origin, no definitive diagnosis could be made. Other associated diseases seen with pure red cell aplasia were thymoma, septicemia, protein energy malnutrition, non-Hodgkin's lymphoma, juvenile rheumatoid arthritis, acute myeloid leukemia, tuberculosis and hepatitis C. The association of pure red cell aplasia with haematologic malignancies is rare. There are very few case reports on pure red cell aplasia with hepatitis C.

A Case of Relapsing Polychondritis Associated with Myelodysplastic Syndrome with Erythroid Hypoplasia/Aplasia

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.

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.

Successful Treatment of Pure Red Cell Aplasia with Plasmapheresis in a Patient with Systemic Lupus Erythematosus

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.

Non-Hodgkin's lymphoma with pure red cell aplasia.

Pure red cell aplasia (PRCA) associated with non-Hodgkin's lymphoma, is an extremely rare condition, with few cases reported worldwide. More commonly PRCA is associated with thymoma or viral infections. Steroids and other immunosuppressive drugs are the preferred treatment of PRCA.

Aplasia pura de células vermelhas e lúpus eritematoso sistêmico / Pure red-cell aplasia and systemic lupus erythematosus

A aplasia pura de células vermelhas (APCV) é uma forma relativamente rara de anemia, a qual pode associar-se com desordens linfo e mieloproliferativas, timoma, infecçöes virais, uso de determinados medicamentos e várias doenças de auto-imunidade. A coexistência de APCV com lúpus eritematoso sistêmico (LES), embora claramente reconhecida, é considerada uma associaçäo rara. Säo descritos aqui dois casos de LES associados com APCV, com o intuito de alertar o clínico para o reconhecimento desta associaçäo.(au)

Pure red cell aplasia and non-Hodgkin's lymphoma: a case report and brief literature review.

Pure red cell aplasia associated with lymphoproliferative malignancies other than thymoma is an uncommon occurrence. In the present paper we report a rare case of nodular non-Hodgkin's lymphoma with pure red cell aplasia who presented with symptoms related to anemia rather than the lymphoma and responded well to combination chemotherapy.

Candidiasis en el Hospital Santo Tomás / Candidiasis at the Hopsital Santo Tomás

Se estudió la incidencia, sintomatológica y tratamiento de Candidiasis en el Hospital Santo Tomás. En 41 ocasiones se obtuvo el cultivo de un hongo patógeno, del género Candida, en material del tracto digestivo, de la sangre, de la piel y o de la orina de 20 pacientes. Todos ellos sufrían también de una enfermedad subyacente (leucemia, neoplasia sólida, lupus eritematoso sistémico o aplasia medular). La fiebre y las ulceraciones orofaríngeas fueron las hallazgos clínicos más frecuentemente observados. Los pacientes fueron tratados con Ketoconazole y o con Anfotericina B. Se observó que el uso de Ketoconazole produjo más casos de candidemia y mayor mortalidad que el uso de Anfotericina B