No monoclonal spike is found on electrophoresis. In some cases, paroxysmal cold hemoglobinuria and infection-induced exacerbation of primary CAD will have to be ruled out as differential diagnoses. A few case reports have described chronic CA-mediated hemolysis in patients with systemic lupus erythematosus (SLE).

In one of these publications, the presence of a clonal disorder was considered but could not be confirmed.66 These very rare cases of SLE-associated CAS should not be confused with primary CAD. Several authors have reported the development of CA-mediated hemolysis after allogenic stem cell transplantation. In some of these patients, the AIHA seemed related to the transplantation per se; in other cases it was associated with virus infection. [64] and [67] CAS has also been described during pregnancy in one single patient. 68 Until a decade ago, pharmacological therapy Crizotinib price for primary CAD was largely ineffective.[6] and [69] Partly based on this fact and partly because the severity

of the clinical features have not been appreciated, counseling has been regarded the mainstay of management.[3], [6] and [36] However, documentation of efficacy AZD2281 manufacturer is mainly anecdotal.[15] and [70] Still, in our clinical experience, staying warm seems to alleviate the symptoms and can probably prevent severe exacerbations of hemolytic anemia. In particular, the head, face and extremities should be protected against cold exposure.[36], [69] and [71] Some patients experience increased Hgb levels and alleviation of circulatory symptoms after relocating to warmer regions during the cold season, but severely symptomatic CAD does exist even in the subtropics. Infusion of cold liquids should be avoided. Surgery under hypothermia requires specific

precautions, e.g. preoperative plasmapheresis.[72] and [73] Erythrocyte transfusions can safely be given provided appropriate precautions are undertaken.[31] and [69] In contrast to the compatibility problems characteristic for warm-antibody AIHA, it is usually easy to find compatible donor erythrocytes, and screening tests for irregular blood Interleukin-3 receptor group antibodies are most often negative. Antibody screening and, if required, compatibility tests should be performed at 37 °C. The patient and, in particular, the extremity chosen for infusion should be kept warm, and the use of an in-line blood warmer is recommended.72 Failure to observe required precautions has resulted in dismal or, very rarely, even fatal outcome.[72] and [74] Because complement proteins can exacerbate hemolysis, transfusion of blood products with a high plasma content should probably be avoided.39 In a population-based retrospective series on primary CAD we identified three splenectomised patients, none of whom had responded to the splenectomy.6 This observation is not surprising, since clearance of C3b-opsonized erythrocytes primarily occurs in the liver.