Cold agglutinin induced hemolysis in a newly diagnosed systemic lupus erythematosus.
ABSTRACT Systemic lupus erythematosus (SLE) is a well-known autoimmune chronic inflammatory disease, which can virtually affect any organ system in the body. Although hemolytic anemia has been known to occur in <10% of SLE patients, they are usually mediated through warm antibodies. It is extremely rare to see cold antibody-mediated hemolytic anemia in SLE, and only few cases have been reported in literature to our knowledge. This is a unique case report of SLE associated with cold agglutinin hemolytic anemia in a patient presented with generalized tender lymphadenopathy and typical B-symptoms including fever, night sweats, and significant weight loss.
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Article: Cold agglutinin disease.[Show abstract] [Hide abstract]
ABSTRACT: Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We review the clinical and pathologic features, prognosis, and management in the literature, and we describe our institutional experience to improve strategies for accurate diagnosis and treatment. Retrospective analysis identified 89 patients from our institution with cold agglutinin disease from 1970 through 2012. Median age at symptom onset was 65 years (range, 41-83 years), whereas the median age at diagnosis was 72 years (range, 43-91 years). Median survival of all patients was 10.6 years, and 68 patients (76%) were alive 5 years after the diagnosis. The most common symptom was acrocyanosis (n=39 [44%]), and many had symptoms triggered by cold (n=35 [39%]) or other factors (n=20 [22%]). An underlying hematologic disorder was detected in 69 patients (78%). Thirty-six patients (40%) received transfusions during their disease course and 82% received drug therapy. Rituximab was associated with the longest response duration (median, 24 months) and the lowest proportion of patients needing further treatment (55%). Our institution's experience and review of the literature confirms that early diagnostic evaluation and treatment improves outcomes in cold agglutinin disease.Blood 06/2013; · 9.78 Impact Factor
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ABSTRACT: Exact diagnosis of the subtype has essential therapeutic consequences in autoimmune hemolytic anemia. Cold-antibody types include primary chronic cold agglutinin disease (CAD) and rare cases of cold agglutinin syndrome (CAS) secondary to cancer or acute infection. Primary CAD is a clonal lymphoproliferative disorder. Not all patients require pharmacological therapy, but treatment seems indicated more often than previously thought. Corticosteroids should not be used to treat primary CAD. Half of the patients respond to rituximab monotherapy; median response duration is 11 months. The most efficient treatment to date is fludarabine and rituximab in combination, resulting in responses in 75%, complete responses in 20% and median response duration of more than 66 months. Toxicity may be a concern, and an individualized approach is discussed. Erythrocyte transfusions can be given provided specific precautions are undertaken. No evidence-based therapy exists in secondary CAS, but optimal treatment of the underlying disorder is essential when feasible.Blood reviews 02/2012; 26(3):107-15. · 7.19 Impact Factor