Cold agglutinin disease

Paul L. Swiecicki, Livia T. Hegerova, Morie Gertz

Research output: Contribution to journalReview article

103 Citations (Scopus)

Abstract

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 themedian 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.

Original languageEnglish (US)
Pages (from-to)1114-1121
Number of pages8
JournalBlood
Volume122
Issue number7
DOIs
StatePublished - Aug 15 2013

Fingerprint

Autoimmune Hemolytic Anemia
Drug therapy
cold agglutinins
Age of Onset
Drug Therapy
Survival
Therapeutics

ASJC Scopus subject areas

  • Biochemistry
  • Immunology
  • Hematology
  • Cell Biology

Cite this

Swiecicki, P. L., Hegerova, L. T., & Gertz, M. (2013). Cold agglutinin disease. Blood, 122(7), 1114-1121. https://doi.org/10.1182/blood-2013-02-474437

Cold agglutinin disease. / Swiecicki, Paul L.; Hegerova, Livia T.; Gertz, Morie.

In: Blood, Vol. 122, No. 7, 15.08.2013, p. 1114-1121.

Research output: Contribution to journalReview article

Swiecicki, PL, Hegerova, LT & Gertz, M 2013, 'Cold agglutinin disease', Blood, vol. 122, no. 7, pp. 1114-1121. https://doi.org/10.1182/blood-2013-02-474437
Swiecicki PL, Hegerova LT, Gertz M. Cold agglutinin disease. Blood. 2013 Aug 15;122(7):1114-1121. https://doi.org/10.1182/blood-2013-02-474437
Swiecicki, Paul L. ; Hegerova, Livia T. ; Gertz, Morie. / Cold agglutinin disease. In: Blood. 2013 ; Vol. 122, No. 7. pp. 1114-1121.
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