M-component with reactivity against actin associated with thrombotic thrombocytopenic purpura
Article Abstract:
In some disorders, the number of B lymphocytes (a type of white blood cell) that manufacture monoclonal immunoglobulins (MIgs) increases dramatically, a phenomenon known as clonal expansion. MIgs are directed against antigens (proteins) on the surfaces of red blood cells, myelin, cell constituents, and bacterial products. One MIg target is actin, a component of muscle. In such cases, MIgs are said to have smooth muscle antibody (SMA) activity; however, a clinical picture associated with SMA MIgs has not been described. In this report, a case study is presented of a patient with an MIgG (an immunoglobulin of the G class) with SMA activity directed against actin. The patient also suffered three episodes of a syndrome similar to thrombotic thrombocytopenic purpura (TTP), a rare condition in which tiny clots form in the smallest blood vessels of all organs, platelet levels fall, and anemia develops. During these episodes, the levels of antiactin antibody rose. The patient had Bence Jones proteinuria (excretion of the light-chain component of the immunoglobulin molecule) and later developed joint pain and inflammation. SMA activity was detected and other symptoms (ocular and mental changes, and fever) prompted the diagnosis of TTP. While many of the patient's symptoms were similar to those of patients with multiple myeloma, a malignant disorder of bone marrow cells in which one immunoglobulin clone is manufactured in excessive amounts, all of the diagnostic criteria for this disease were not fulfilled. SMA activity was detected in the abnormal immunoglobulin (such immunoglobulins are called the M-component) and the autoantibody was further investigated. It was found to be reactive to actin. The patient's symptoms were relieved and the size of the M-component reduced by the administration of steroid drugs. It is likely that the MIgG and the TTP-like disorder were related. Possible links between the physiological abnormalities and the clinical symptoms are discussed. One interpretation of this case is that certain autoimmune reactions against structural proteins in smooth muscle cells of blood vessels can trigger blood clotting and the TTP-like syndrome. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: American Journal of Medicine
Subject: Health care industry
ISSN: 0002-9343
Year: 1991
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Interleukin-1: biology, pathophysiology, and clinical prospects
Article Abstract:
Interleukin-1 (IL-1) is a peptide produced by many different types of cells in response to injury. It was first identified in 1972, and was shown to have several biologic functions, including metabolic, endocrine, immunologic, and hematologic effects. The production of the two forms of IL-1, IL-1-alpha and IL-1-beta, is controlled by genes on chromosome 2. Although the two forms of IL-1 differ in structure, they have similar biologic properties and share a common receptor, which is a specific binding site on the cell membrane. IL-1 is produced mainly by macrophages, a type of immune cell, and to a lesser extent by other cell types of the immune and central nervous system. The production of IL-1 is activated by various factors, including immune reactions, endotoxins (toxic compounds produced by bacteria), ultraviolet radiation, bacteria, and viruses. IL-1 is the main activator of the acute-phase response to injury, characterized by fever, activation of immune responses, release of amino acids from muscle, and the induction of slow-wave sleep. IL-1 has demonstrated an antitumor effect and an ability to enhance recovery of blood cells in cancer patients after chemotherapy. The biologic effects of IL-1 and its role in the development of certain disorders are reviewed. The potential use of IL-1 in the treatment of cancer is also discussed. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: American Journal of Medicine
Subject: Health care industry
ISSN: 0002-9343
Year: 1990
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Thrombotic thrombocytopenic purpura as the first manifestation of human immunodeficiency virus infection
Article Abstract:
Thrombotic thrombocytopenia purpura (TTP) is a rare disorder that results in blood and nerve abnormalities. TTP is characterized by bleeding caused by a decreased number of platelets (cells essential for clotting) accompanied by microemboli (small clots), neurological abnormalities and kidney failure. These case reports describe two patients with human immunodeficiency virus (HIV) infection who exhibited TTP as the initial symptom of acquired immunodeficiency syndrome (AIDS). A 51-year-old patient without a history of drug abuse or homosexuality came to the hospital with diarrhea, severe weight loss and dehydration. The patient was found to have TTP and was treated without symptomatic relief. Another patient, a 52-year-old intravenous drug abuser, had nausea, vomiting and lethargy. Both patients tested positive for HIV and developed TTP as the first symptom of the infection. Only the second patient was in an AIDS risk group. The first patient eventually had infections with organisms not normally harmful to healthy people (opportunistic infections, a characteristic of AIDS). The second patient had no other symptoms of AIDS. These cases support the theory that there is a relationship between AIDS and TTP. Thus, all patients with TTP should be evaluated for HIV infection. (Consumer Summary produced by Reliance Medical Information, Inc.)
Publication Name: American Journal of Medicine
Subject: Health care industry
ISSN: 0002-9343
Year: 1989
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