Parvovirus B19-induced Pure Red Cell Aplasia in a Liver Transplant Recipient / 대한진단검사의학회지

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.

Persistent Anemia in a Patient with Diffuse Large B Cell Lymphoma: Pure Red Cell Aplasia Associated with Latent Epstein-Barr Virus Infection in Bone Marrow

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.

Pure red cell aplasia-a single centre experience

To determine the frequency of Pure Red Cell Aplasia [PRCA] in patients referred to Haematology Department of Holy Family Hospital, Rawalpindi for bone marrow examination. Single center descriptive study. The study was conducted at the Haematology Department of Holy Family Hospital Rawalpindi from 1st January 1999 to 31[st] September 2004. All patients referred to this department for bone marrow were included in this study. They comprised both male and female subjects of all ages. After taking a detailed history and conducting a complete physical examination blood samples were taken for complete blood picture. Bone marrow aspiration was done on all patients and trephine biopsies were taken only where indicated. Total of 1724 bone marrow aspirations biopsies were performed during this period, of which. 13 [0.75%] were diagnosed as Pure Red Cell Aplasia. 08/13 [61.5%] were males and 05/13 [38.4%] were females. The age range was from 1-52 years. Maximum number of patients belonged to 15-30 years age group. 01/13 [7.7%] was diagnosed as Diamond Blackfan Anaemia CDBA[2], 03/13 [23%] were associated with viral hepatitis. 04/13 [30.7%] cases had developed after some viral infection other than viral hepatitis. 01/13 [7.7%] had immune mediated PRCA, developing during the course of DAT positive hemolytic anaemia. 04/13 [30.7%] were idiopathic in origin. DBA patients initially responded well to corticosteroids but later developed resistance to steroids and became transfusion dependent. 02/13 [15.4%] patients also showed a good initial response to corticosteroid therapy and thus were labelled as Acute PRCA. 02/13 [15.4%] recovered spontaneously after diagnosis. Both these cases were children and thus diagnosed as cases of Transient Erythroblastopenia of Childhood [TEC]. PRCA is a rare disorder with a frequency of 0.75%. It is most commonly encountered in young adults secondary to a viral infection. Hepatitis is a major health hazard in our society and may play a significant role in the etiology of PRCA our patients

Acquired pure red cell aplasia in a child.

Primary acquired pure red cell aplasia is a rare occurrence in childhood. An eleven-year old boy presented to us with pallor, which required multiple packed red cell transfusions. He did not have hepatosplenomegaly, jaundice or lymphadenopathy. Bone marrow examination revealed the diagnosis of pure red cell aplasia. All possible investigations were done to exclude secondary causes of pure red cell aplasia. No secondary cause was found on investigations. Rheumatoid factor and anti-nuclear antibodies were positive. He was started on oral steroids, to which he did not respond. He was then given cyclosporine A. Response to cyclosporine was dramatic and the child now does not require any transfusions.

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)

Mesotelioma y aplasia pura de serie roja / Malignant mesothellioma and pure red cell aplasia

La aplasia pura de serie roja (APSR) es un síndrome clínico definido por la disminución de precursores eritroides maduros. Los pacientes con esta enfermedad presentan reticulopenia pero su cuenta de plaquetas y granulocitos es normal. La patofisiología es heterogénea y puede ser congénita o adquirida. En su forma secundaria, se encuentra asociada con distintos tipos de tumores como los timomas, las leucemias y los linfomas. El interés del caso que presentamos es la presencia de una aplasia pura de serie roja en un paciente con un mesotelioma. El tratamiento utilizado para esta aplasia consistió en el uso de prednisona y eritropoyetina recombinante humana. Ninguno de los tratamientos utilizados mejoró la anemia, por lo que el paciente fue mantenido a base de transfusiones periódicas. Por otra parte, el mesotelioma es una neoplasia muy agresiva con una vida media de tan solo dos a ocho meses. El paciente presentó una sobrevida de 18 meses y un estado físico aceptable.

Aplasia pura roja : infrecuente manifestación hematológica de mielopatía autoinmune / Pure red aplasia : infrequent hematological manifestation of autoimmune myelopathy

Se presenta una paciente de 65 años de edad, con imagen tomográfica tumoral en lóbulo anterosuperior de pulmón izquierdo, que al resecarse se comprobó que era un nódulo de mediastino de 5 cm de diámetro, sin atipías, pero con infiltración linfoide. A continuación presentó linfocitosis de 10 meses de evolución seguida de anemia hemolítica. Luego de 2 años de remisión clínica se presentó con anemia aguda diagnosticada como Aplasia Pura Roja. Sus patologías revirtieron con corticosteroides. Se hace una actualización acerca de los vínculos entre las enfermedades de la médula ósea y la inmunopatología.

Aplasia selectiva congénita de la serie roja: estudio de 13 casos / Congenital pure red cell aplasia: report of 13 cases

Se estudiaron 13 niños con aplasia selectiva congénita de la serie roja (ASCSR); nueve furon del sexo masculino. La mayoría de los pacientes iniciaron sus manifestaciones clínicas antes de los seis meses de edad, caracterizadas principalmente por: palidez; hepatomegalia, soplo cardíaco y malformaciones congénitas diversas. En todos los casos se documentó anemia moderada a severa, con cuenta de reticulocitos baja (promedio 0.6%) y de leucocitos y plaquetas normales. En todos los casos el estudio de médula ósea mostró disminución marcada de los precursores de la serie roja. Tres pacientes presentaron remisión completa de la anemia después del tratamiento con prednisona; en otros seis casos se observó respuesta parcial al tratamiento corticosteroide, requiriendo la administración prolongada de dosis bajas de prednisona. Finalmente, los cuatro pacientes restantes no presentaron respuesta al tratamiento, requiriendo transfusiones frecuentes