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Since the first description of cryoglobulinemia in association with the clinical triad of skin purpura, joint pain, and weakness by Meltzer et al. in 1966, [4] [5] the percentage of cryoglobulinemic diseases described as essential cryoglobulinemia or idiopathic cryoglobulinemia (that is, cryoglobulinemic disease that is unassociated with an underlying disorder) has fallen.
Cryoglobulinemic vasculitis is a form of inflammation affecting the blood vessels caused by the deposition of abnormal proteins called cryoglobulins.These immunoglobulin proteins are soluble at normal body temperatures, but become insoluble below 37 °C (98.6 °F) and subsequently may aggregate within smaller blood vessels.
Cold sensitive antibodies (CSA) are antibodies sensitive to cold temperature. Some cold sensitive antibodies are pathological and can lead to blood disorder.These pathological cold sensitive antibodies include cold agglutinins, Donath–Landsteiner antibodies, and cryoglobulins which are the culprits of cold agglutinin disease, paroxysmal cold hemoglobinuria in the process of Donath ...
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Cryofibrinogenemia is also often associated with the inflammatory vasculitis that accompanies mixed Cryoglobulinemia#Classification, i.e. cryoglobulinemic vasculitis, particularly but not exclusively in instances where hepatitis C virus is an underlining disease. [6]
Cryoglobulinemic vasculitis (CryoVas) is a type of small-vessel vasculitis that primarily affects the kidneys, skin, joints, and peripheral nervous system. Monoclonal immunoglobulins associated with an underlying B-cell lymphoproliferative disorder are known as type I cryovalent vasculitis (CryoVas).
Inflammation-induced damage to the skin's blood vessels causes palpable purpura. Palpable purpura is the clinical manifestation of leukocytoclastic vasculitis, which can be idiopathic or linked to sepsis, reactions to drugs, connective tissue diseases, cryoglobulinemia, hepatitis C or B infection, or underlying cancers
Microvascular occlusions can be caused by heparin-induced thrombocytopenia, cryoglobulinemia, angioinvasive organisms, embolization, disseminated intravascular coagulation, livedoid vasculopathy, cell occlusion syndromes, and iatrogenic causes. [3]