Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association
Pure red cell aplasia (PRCA) is a rare bone marrow failure syndrome defined by a progressive normocytic anaemia and reticulocytopenia without leukocytopenia and thrombocytopenia. Secondary PRCA can be associated with various haematological disorders, such as chronic lymphocytic leukaemia (CLL) or no...
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doaj-97e026bf3ede419e94d242b7e6b3a4262020-11-25T02:19:12ZengHindawi LimitedThe Scientific World Journal1537-744X2012-01-01201210.1100/2012/475313475313Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent AssociationEfthymia Vlachaki0Michael D. Diamantidis1Philippos Klonizakis2Styliani Haralambidou-Vranitsa3Elizabeth Ioannidou-Papagiannaki4Ioannis Klonizakis5Department of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, GreeceDepartment of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, GreeceDepartment of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, GreeceDepartment of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, GreeceDepartment of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, GreeceDepartment of Haematology, Second Department of Internal Medicine, Aristotle University of Thessaloniki, Hippokration General Hospital, 546 42, Thessaloniki, GreecePure red cell aplasia (PRCA) is a rare bone marrow failure syndrome defined by a progressive normocytic anaemia and reticulocytopenia without leukocytopenia and thrombocytopenia. Secondary PRCA can be associated with various haematological disorders, such as chronic lymphocytic leukaemia (CLL) or non-Hodgkin lymphoma (NHL). The aim of the present review is to investigate the infrequent association between PRCA and lymphoproliferative disorders. PRCA might precede the appearance of lymphoma, may present simultaneously with the lymphoid neoplastic disease, or might appear following the lymphomatic disorder. Possible pathophysiological molecular mechanisms to explain the rare association between PRCA and lymphoproliferative disorders are reported. Most cases of PRCA are presumed to be autoimmune mediated by antibodies against either erythroblasts or erythropoietin, by T-cells secreting factors selectively inhibiting erythroid colonies in the bone marrow or by NK cells directly lysing erythroblasts. Finally, focus is given to the therapeutical approach, as several treatment regimens have failed for PRCA. Immunosuppressive therapy and/or chemotherapy are effective for improving anaemia in the majority of patients with lymphoma-associated PRCA. Further investigation is required to define the pathophysiology of PRCA at a molecular level and to provide convincing evidence why it might appear as a rare complication of lymphoproliferative disorders.http://dx.doi.org/10.1100/2012/475313 |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Efthymia Vlachaki Michael D. Diamantidis Philippos Klonizakis Styliani Haralambidou-Vranitsa Elizabeth Ioannidou-Papagiannaki Ioannis Klonizakis |
spellingShingle |
Efthymia Vlachaki Michael D. Diamantidis Philippos Klonizakis Styliani Haralambidou-Vranitsa Elizabeth Ioannidou-Papagiannaki Ioannis Klonizakis Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association The Scientific World Journal |
author_facet |
Efthymia Vlachaki Michael D. Diamantidis Philippos Klonizakis Styliani Haralambidou-Vranitsa Elizabeth Ioannidou-Papagiannaki Ioannis Klonizakis |
author_sort |
Efthymia Vlachaki |
title |
Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association |
title_short |
Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association |
title_full |
Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association |
title_fullStr |
Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association |
title_full_unstemmed |
Pure Red Cell Aplasia and Lymphoproliferative Disorders: An Infrequent Association |
title_sort |
pure red cell aplasia and lymphoproliferative disorders: an infrequent association |
publisher |
Hindawi Limited |
series |
The Scientific World Journal |
issn |
1537-744X |
publishDate |
2012-01-01 |
description |
Pure red cell aplasia (PRCA) is a rare bone marrow failure syndrome defined by a progressive normocytic anaemia and reticulocytopenia without leukocytopenia and thrombocytopenia. Secondary PRCA can be associated with various haematological disorders, such as chronic lymphocytic leukaemia (CLL) or non-Hodgkin lymphoma (NHL). The aim of the present review is to investigate the infrequent association between PRCA and lymphoproliferative disorders. PRCA might precede the appearance of lymphoma, may present simultaneously with the lymphoid neoplastic disease, or might appear following the lymphomatic disorder. Possible pathophysiological molecular mechanisms to explain the rare association between PRCA and lymphoproliferative disorders are reported. Most cases of PRCA are presumed to be autoimmune mediated by antibodies against either erythroblasts or erythropoietin, by T-cells secreting factors selectively inhibiting erythroid colonies in the bone marrow or by NK cells directly lysing erythroblasts. Finally, focus is given to the therapeutical approach, as several treatment regimens have failed for PRCA. Immunosuppressive therapy and/or chemotherapy are effective for improving anaemia in the majority of patients with lymphoma-associated PRCA. Further investigation is required to define the pathophysiology of PRCA at a molecular level and to provide convincing evidence why it might appear as a rare complication of lymphoproliferative disorders. |
url |
http://dx.doi.org/10.1100/2012/475313 |
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