Cryoglobulinemia Flashcards
the presence of serum immunoglobulin (precipitates at low temperature and redissolves at 37°C) complexed with other immunoglobulins or proteins.
Cryoglobulinemia
Type 1-3
Type I Cryoglobulins: Monoclonal immu- noglobulins (IgM, IgG, IgA, light chains). Associated with plasma cell dyscrasias such as multiple myeloma, Waldenström macroglobu- linemia, lymphoproli erative disorders such as B cell lymphoma.
Type II Cryoglobulins: Mixed cryoglobulins: wo immunoglobulin components, one o
whichismonoclonal(usuallyIgG,lesso en IgM) and the other polyclonal. Associated with multiple myeloma, Waldenström macroglobu- linemia, chronic lymphocytic leukemia; rheu- matoid arthritis, systemic lupus erythematosus, and Sjögren syndrome.
Type III Cryoglobulins: Polyclonal immu- noglobulinsthat ormcryoprecipitatewith polyclonal IgG or a nonimmunoglobulin serum component occasionally mixed with comple- ment and lipoproteins. Represents immune complex disease. Associated with autoimmune diseases; connective tissue diseases; wide varietyo inectiousdiseases,i.e.,hepatitis
B, hepatitis C, Epstein–Barr virus in ection, cytomegalovirus in ection, subacute bacterial endocarditis, leprosy, syphilis, and streptococ- cal in ections.
Clinical manif
cold sensitivity in < 50% of cases. Chills,fever,dyspnea,and diarrhea mayoccur following cold exposure.
Noninflammatory purpura (usually type I), occurring at cold-exposed sites, e.g., helix, tip of nose
acrocyanosis and reynauds phenomenon
palapble purpura w bulla
livedo reticularis mostly on upper and low extremity
urticaria induced by cold
Diagnosis
Diagnosis is confrmed by determination
of cryoglobulins (blood drawn into
warmed syringe, RBC removed via warmed centrifuge; plasma refrigerated in a Wintrobe
tube at 4°C or 24 to 72 h, then centri uged and cryocrit determined) and diagnosis of underlying disease
Treatment
Treatment is that of the underlying disease. Idiopathic disease can be treated with plasmapheresis, oral corticosteroids, and mycophenolate mofetil
