Bilineage hematopoietic inhibitor and T lymphocyte dysfunction in a patient with pure red cell aplasia, myasthenia gravis and thymoma.
The case history, laboratory findings and clinical course of a patient with pure red cell aplasia (PRCA) combined with myasthenia gravis and thymoma are reported herein. In vitro study revealed bilineage complement-dependent IgG inhibitor(s) in both the granulocyte-macrophage and erythroblastic progenitor cells. His serum showed high anti-acetylcholine receptor antibody levels associated with activity of myasthenia gravis as well as PRCA. Patient history of thymectomy 7 years previously followed by extensive cutaneous candidiasis with abnormal T lymphocyte subsets (decreased T4/T8 ratio and increased number of activated T lymphocytes) in both the bone marrow and peripheral blood suggested primary T lymphocyte dysfunction, whereas the erythropoiesis was not inhibited by T lymphocytes. This case is of interest in the context of a possible immunological pathogenesis for other hematopoietic disorders, including some cases of aplastic anemia.
Murase
T
T
Department of Internal Medicine, Second Tokyo National Hospital, Japan.
D000906:Antibodies / Q000097:blood; D000911:Antibodies, Monoclonal / Q000032:analysis / Q000276:immunology; D016516:CD4-CD8 Ratio; D003165:Complement System Proteins / Q000502:physiology; D005434:Flow Cytometry; D006098:Granulocytes / Q000166:cytology; D006410:Hematopoiesis / Q000502:physiology*; D006412:Hematopoietic Stem Cells / Q000187:drug effects; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008264:Macrophages / Q000166:cytology; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D011950:Receptors, Cholinergic / Q000032:analysis / Q000276:immunology; D012010:Red-Cell Aplasia, Pure / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D013234:Stem Cells / Q000187:drug effects; D016176:T-Lymphocyte Subsets / Q000276:immunology / Q000473:pathology / Q000502:physiology; D013601:T-Lymphocytes / Q000473:pathology / Q000502:physiology* / Q000648:ultrastructure; D013945:Thymoma / Q000150:complications / Q000473:pathology / Q000503:physiopathology*; D013953:Thymus Neoplasms / Q000150:complications / Q000473:pathology / Q000503:physiopathology*
D000906:Antibodies; D000911:Antibodies, Monoclonal; D007074:Immunoglobulin G; D011950:Receptors, Cholinergic; D003165:Complement System Proteins