ABSTRACT
Pure red cell aplasia (PRCA) is a rare hematologic syndrome, characterized by an isolated normocytic anemia with severe reticulocytopenia, and defined by absence or near absence of erythroid precursors in the bone marrow. First described in 1922, PRCA may be a primary autoimmune or clonal myeloid or lymphoid disorder, but may also be secondary to other disorders of immune dysregulation/autoimmunity, to infections, to neoplasms, or to drugs. Insights from the study of PRCA have helped illuminate the understanding of the regulation of erythropoiesis. This review summarizes the classification, diagnostic, and therapeutic approach to PRCA as it begins its second century, with a particular focus on opportunities and challenges provided by new developments in the role of T-cells and T-cell regulatory mutations; the role of clonal hematopoiesis; and new developments in therapy for refractory PRCA and PRCA associated with ABO incompatible stem cell transplantation.
Subject(s)
Anemia , Hematopoietic Stem Cell Transplantation , Red-Cell Aplasia, Pure , Humans , Red-Cell Aplasia, Pure/diagnosis , Red-Cell Aplasia, Pure/therapy , Red-Cell Aplasia, Pure/complications , Anemia/complicationsSubject(s)
Osteoporosis , Thalassemia , Humans , Erythropoiesis , Thalassemia/complications , Erythrocytes , Osteoporosis/etiologySubject(s)
Anaphylaxis , Drug Hypersensitivity , Humans , Anaphylaxis/chemically induced , Iron/adverse effectsABSTRACT
Discussion of the hematologic complications of vaccination for severe acute respiratory syndrome coronavirus-2 (COVID-19) has primarily focused on the development of vaccine-associated immune thrombosis with thrombocytopenia (VITT). Other hematologic complications are uncommon. We report the case of a patient who developed immunoglobulin G (IgG)-mediated autoimmune hemolytic anemia (AIHA) after the Moderna COVID-19 messenger ribonucleic acid (mRNA) vaccine.
Subject(s)
Anemia, Hemolytic, Autoimmune , COVID-19 , Vaccines , Anemia, Hemolytic, Autoimmune/chemically induced , COVID-19 Vaccines , Humans , RNA, Messenger/genetics , SARS-CoV-2ABSTRACT
A normal pregnancy consumes 500-800 mg of iron from the mother. Premenopausal women have a high incidence of marginal iron stores or iron deficiency (ID), with or without anemia, particularly in the less developed world. Although pregnancy is associated with a "physiologic" anemia largely related to maternal volume expansion; it is paradoxically associated with an increase in erythrocyte production and erythrocyte mass/kg. ID is a limiting factor for this erythrocyte mass expansion and can contribute to adverse pregnancy outcomes. This review summarizes erythrocyte and iron balance observed in pregnancy; its implications and impact on mother and child; and provides an overview of approaches to the recognition of ID in pregnancy and its management, including clinically relevant questions for further investigation.
Subject(s)
Anemia, Iron-Deficiency , Child Development , Fetal Development , Iron Deficiencies , Iron, Dietary/administration & dosage , Iron/metabolism , Nutritional Physiological Phenomena , Nutritional Requirements , Pregnancy Complications , Adolescent , Adult , Anemia, Iron-Deficiency/diagnosis , Anemia, Iron-Deficiency/therapy , Child , Child, Preschool , Female , Humans , Infant , Male , Pregnancy , Pregnancy Complications/diagnosis , Pregnancy Complications/therapy , Pregnancy Outcome , Premenopause/metabolism , Young AdultSubject(s)
Anti-Infective Agents , Bacteremia , Hematology , Neoplasms , Blood Culture , Humans , InpatientsABSTRACT
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents. The therapeutic approach to PRCA typically involves immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. Cyclosporine A, with or without concurrent corticosteroids, appears to be the single most effective immunosuppressive agent.
Subject(s)
Adrenal Cortex Hormones/therapeutic use , Anemia, Diamond-Blackfan , Autoimmune Diseases , Cyclosporine/therapeutic use , Immunosuppressive Agents/therapeutic use , Anemia, Diamond-Blackfan/drug therapy , Anemia, Diamond-Blackfan/immunology , Anemia, Diamond-Blackfan/pathology , Autoimmune Diseases/drug therapy , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , Humans , Leukemia, Large Granular Lymphocytic/drug therapy , Leukemia, Large Granular Lymphocytic/immunology , Leukemia, Large Granular Lymphocytic/pathology , Leukemia, Lymphocytic, Chronic, B-Cell/drug therapy , Leukemia, Lymphocytic, Chronic, B-Cell/immunology , Leukemia, Lymphocytic, Chronic, B-Cell/pathology , Myelodysplastic Syndromes/drug therapy , Myelodysplastic Syndromes/etiology , Myelodysplastic Syndromes/immunology , Myelodysplastic Syndromes/pathology , Parvoviridae Infections/drug therapy , Parvoviridae Infections/immunology , Parvoviridae Infections/pathology , Parvovirus B19, Human/immunology , Thymoma/drug therapy , Thymoma/immunology , Thymoma/pathology , Vasculitis/drug therapy , Vasculitis/immunology , Vasculitis/pathologyABSTRACT
Pure red cell aplasia (PRCA) is a syndrome defined by a normocytic normochromic anemia with severe reticulocytopenia and marked reduction or absence of erythroid precursors from the bone marrow. Diamond-Blackfan anemia is a congenital form of PRCA. Acquired PRCA may be either a primary disorder or secondary to some other disorder or agent. Primary acquired PRCA is an autoimmune disorder that is frequently antibody-mediated. Myelodysplastic syndromes may also present with the morphologic appearance of PRCA. Secondary acquired PRCA may be associated with collagen vascular/autoimmune disorders such as systemic lupus erythematosus; lymphoproliferative disorders such as chronic lymphocytic leukemia or large granular lymphocyte leukemia; infections, particularly B19 parvovirus; thymoma and other solid tumors; or a variety of other disorders, drugs, or toxic agents. The therapeutic approach to PRCA typically involves immunosuppression, but specific pathogenic subtypes are associated with specific therapeutic approaches. Cyclosporine A, with or without concurrent corticosteroids, appears to be the single most effective immunosuppressive agent.