Restriction in the repertoire of the immunoglobulin light chain subgroup in pathological cold agglutinins with anti‐Pr specificity

Leo, Albert B. De; Kreft, H; Hack, H.; Kempf, Tore; Roelcke, D.

doi:10.1111/j.0042-9007.2004.00401.x

Cited by 10 publications

(11 citation statements)

References 57 publications

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“…30 The monoclonal cold agglutinin IgM molecules are directed against the I/i carbohydrate antigens on the RBC surface. These cold agglutinins have heavy chains encoded by the immunoglobulin heavy variable (IGHV) 4-34 gene segment, 31 which suggests that certain amino acid sequences may have a critical role in receptor recognition. Rarely, antibodies have been noted to target antigens outside the I/i system such as the Pr antigen.…”

Section: History and Mechanismmentioning

confidence: 99%

Cold agglutinin disease

Swiecicki

Hegerova

Gertz

2013

Blood

266

298

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Cold agglutinin disease is a rare and poorly understood disorder affecting 15% of patients with autoimmune hemolytic anemia. We reviewed the clinical and pathologic features, prognosis, and management in the literature and 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 to 83 years), whereas the median age at diagnosis was 72 years (range, 43 to 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.

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Section: History and Mechanismmentioning

confidence: 99%

Cold agglutinin disease

Swiecicki

Hegerova

Gertz

2013

Blood

266

298

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“…Smith, unpublished observations, 2008). The latter property indicates Abs may have specificity for carbohydrate antigens [31]. The fact that Abs work better in the cold indicates their pathogenecity may be unique to cardiac transplantation, where patients are subjected to moderate hypothermia during cardiopulmonary bypass, during the transplant procedure, and when the transplanted heart is subjected to cold storage.…”

Section: Complement-fixing Igm Non-hla Absmentioning

confidence: 99%

Clinical relevance of complement-fixing antibodies in cardiac transplantation

Rose

Smith

2009

Human Immunology

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“…Cold autoantibody AIHA with Pr specificity is uncommon with the majority of cases occurring due to autoantibodies formed against the I/i blood groups. The antigenic determinants of the Pr antigen have been identified as O‐glycosidically linked disialo‐tetra/monosialo‐trisaccharides and are found on almost all RBCs at the distal N terminus of glycophorins . Hence, another characteristic of this group, helpful in antibody identification, is sensitivity to enzyme treatment.…”

Section: Case Discussionmentioning

confidence: 99%