Cold autoimmune haemolytic anaemia secondary to Epstein Barr virus infection presenting with peripheral gangrene; case report

Karunarathne, Suneth; Weerasinghe, Sajitha; Govindapala, Dumitha; Fernando, Harshini; Jayaratne, B

doi:10.1186/1477-9560-10-4

Cited by 15 publications

(13 citation statements)

References 8 publications

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“…Polyclonal antibodies, typically seen in the postinfectious setting, are selfresolving, although treatment of the underlying infection may hasten resolution. Postinfectious cold agglutinins are seen with viral and bacterial pathogens, including mycoplasma, 19 Epstein-Barr virus, [20][21][22] and legionella. Case reports of cold agglutinin hemolysis induced by varicella, [23][24][25] Citrobacter, 26 and influenza 27,28 have been described.…”

Section: History and Mechanismmentioning

confidence: 99%

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Cold agglutinin disease

Swiecicki

Hegerova

Gertz

2013

Blood

265

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%

“…[38][39][40][41][42][43][44][45] In addition to hemolysis, clinical manifestations include coldinduced circulatory symptoms; livedo reticularis, Raynaud disease, acrocyanosis and, rarely, cutaneous necrosis. 20,[46][47][48][49] Berentsen et al 5 reported cold-induced symptoms in more than 90% of patients. Splenomegaly is not prominent in CAD.…”

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confidence: 99%

Cold agglutinin disease

Swiecicki

Hegerova

Gertz

2013

Blood

265

298

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“…[3][4][5] Several reports of EBV infections associated with cold agglutinins have been described; [6][7][8][9][10][11] all were associated with IgM. Anti-I and anti-i are more common cold-agglutinin specific antibodies directed against poly-N-acetyl-lactosamine type 2 sequences.…”

mentioning

confidence: 99%

Fatal Autoimmune Hemolytic Anemia Due to Immunoglobulin G Autoantibody Exacerbated by Epstein-Barr Virus

Fadeyi

Simmons

Jones

et al. 2015

Lab Med

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Most cases of autoimmune hemolytic anemia (AIHA) are caused by the production of an autoantibody that targets determinants on red blood cells (RBCs). This autoantibody can be immunoglobulin (Ig) G, IgM, or IgA. Some autoantibodies react optimally at 0° to 4°C (ie, cold agglutinin) and usually are clinically insignificant. High-titer cold agglutinins are associated with IgM autoantibody and complement fixation induced by infectious agents, including the Epstein-Barr virus (EBV). This case report describes a 31-year-old man who had jaundice, a hemoglobin of 6.0 gdL, and was diagnosed with a hemolytic crisis of AIHA. He received a total of 11 RBC transfusions during a 15-hour period without sustained response and later died. The direct antiglobulin test results for this patient were positive, whereas the cold-agglutinin-testing results were negative. We detected EBV DNA in blood via polymerase chain reaction (PCR). We report a rare case of AIHA associated with an IgG autoantibody and exacerbated by EBV infection, causing a fatal hemolytic anemia.

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“…The haemolysis is due to complement fixation in the intravascular compartment, and the majority of the complement coated cells are destroyed extravascularly by the reticulo-endothelial system. 2 Cold agglutinin disease was first reported in animals in 1903 by Landsteiner, and first human case reported in 1918 by Clough. 3 It is a rare disease, most often affecting females in the seventh decade of life, with an incidence of one case per million people per year.…”

Section: Discussionmentioning

confidence: 99%