Successful treatment of cold agglutinin disease with anti-CD20 antibody                 (rituximab) in a patient with systemic lupus erythematosus

Kotani, Takuya; Takeuchi, Takumi; Kawasaki, Yuki; Hirano, Suzue; Tabushi, Yoko; Kagitani, Maki; Makino, Shigeki; Hanafusa, Toshiaki

doi:10.1177/0961203306070983

Cited by 20 publications

(11 citation statements)

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“…Though extremely rare, cold antibody AIHA in SLE has been reported 12 13. Binding of antibodies to RBCs activates the classical pathway of the complement system leading to the formation of membrane attack complex and intravascular haemolysis.…”

Section: Discussionmentioning

confidence: 99%

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Chaubey

Chhabra

2013

BMJ Case Reports

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Systemic lupus erythematosus (SLE) is a chronic relapsing autoimmune disease associated with several autoantibodies targeted to nuclear and cytoplasmic antigens. Serum antinuclear antibody (ANA) is considered an important diagnostic marker of SLE. However, 2–3% of patients with typical clinical picture of SLE may have persistently negative ANA tests. Autoimmune haemolytic anaemia (AIHA) in SLE is usually mediated by warm IgG anti-erythrocyte antibodies. Our report describes a female patient who presented with clinical manifestations of SLE including photosensitivity, joint pains and AIHA. Further workup revealed high cold IgM agglutinin titres. A comprehensive workup for infectious aetiologies was negative. Autoimmune studies revealed negative ANA, but positive anti-double-stranded DNA and antiphospholipid antibodies. Lymphoproliferative disorder was excluded by imaging studies. Initial treatment with steroids proved of little benefit; however, rituximab resulted in significant clinical improvement. To the best of our knowledge, this is perhaps the first report of ANA-negative SLE presenting with cold AIHA.

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Section: Discussionmentioning

confidence: 99%

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Chaubey

Chhabra

2013

BMJ Case Reports

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“…Postinfectious cold agglutinins most notably are associated with mycoplasma pneumonia and infectious mononucleosis. Cold agglutinin disease, however, has been described and associated with systemic lupus erythematosus (Kotani et al , 2006), Epstein–Barr virus infection (Dourakis et al , 2006) and pregnancy (Batalias et al , 2006). Low‐titre cold agglutinin disease has been associated with systemic sclerosis (Oshima et al , 2004) and after mycoplasma infection in a patient who already had chronic haemolysis from haemoglobin SC disease (Inaba et al , 2005).…”

Section: Pathogenesis Of Cold Agglutinin Diseasementioning

confidence: 99%

Management of cold haemolytic syndrome

Gertz

2007

Br J Haematol

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SummaryMost haemolytic disease is mediated by immunoglobulin G (IgG) antibodies and leads to red blood cell destruction outside of the circulatory system. However, rare syndromes, such as paroxysmal cold haemoglobinuria, show IgG antibodies causing intravascular destruction. Haemolysis may also occur because of immunoglobulin M antibodies. Historically, these antibodies have been termed 'cold agglutinins' because they cause agglutination of red blood cells at 3°C. Cold agglutinin haemolytic anaemia has been associated with a number of autoimmune and lymphoproliferative disorders, and its management differs substantially from warm antibodymediated haemolytic anaemia. This review of cold haemolytic syndromes describes new therapies and clinical strategies to determine a correct diagnosis.Keywords: cold agglutinin, IgM monoclonal protein, MGUS, paroxysmal cold haemoglobinuria, Waldenström macroglobulinaemia. Paroxysmal cold haemoglobinuriaParoxysmal cold haemoglobinuria was first recognised as a distinct clinical entity in 1872 (Crosby, 1952), and the haemolytic antibody was described by Donath and Landsteiner (1904). Landsteiner later received the 1930 Nobel Prize in Medicine for his discovery of the ABO blood groups (Win et al, 2005). The antibody first was observed as a cross-reacting antibody to an antigen on Treponema pallidum and frequently was detected in patients with secondary or tertiary syphilis. Obviously, this is now rare. Today, it is identified in children with postviral illness, much like immune thrombocytopenic purpura. Albeit severe, paroxysmal cold haemoglobinuria is not persistent and generally is not a chronic disorder.The test for paroxysmal cold haemoglobinuria [detection of the haemolytic (anti-P) antibody] is simple to perform. In its original description, 2 aliquots of the patient's red blood cells are collected. One is incubated for 60 min at 37°C, and the other is incubated for 30 min at 3°C and then 30 min at 37°C. The blood is centrifuged, and the plasma is inspected for evidence of haemoglobin. Although it was not understood at the time the test was developed, complement-mediated haemolysis in the control tube (with never-chilled blood) does not occur because anti-P antibodies do not bind the red blood cell surface and complement is never fixed. In the second tube, incubation at 3°C allows binding of the polyclonal anti-P antibody and complement fixation. Subsequent warming to 37°C allows complement activation (C4 through C9), red blood cell lysis and intravascular haemoglobin release.False-negative findings may occur with the Donath-Landsteiner test. If additional complement is not added to a blood specimen, lysis of cell membranes may not occur. Modern iterations of the Donath-Landsteiner test add complement to the specimens to guard against its depletion (a consequence of prior consumption by the antibody). The anti-P antibody also may be consumed by haemolysis. Further modifications of the assay include incubation of papain-treated group-O cells with the plasma. Papain digests the...

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“…After CAD is established, patients should be evaluated for infections, underlying malignancies, and autoimmune disease because more than 70% of cases may be attributable to these processes. 2,6,53 CAD has been associated with lupus 54,55 and rheumatoid arthritis. 56 The IgM antibodies resulting in classical CAD are monoclonal, and the differential diagnosis includes disorders such as IgM monoclonal gammopathy of undetermined significance (MGUS) and Waldenström macroglobulinemia.…”

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confidence: 99%

Cold agglutinin disease

Swiecicki

Hegerova

Gertz

2013

Blood

258

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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Successful treatment of cold agglutinin disease with anti-CD20 antibody (rituximab) in a patient with systemic lupus erythematosus

Cited by 20 publications

References 10 publications

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Cold agglutinin-induced haemolysis in association with antinuclear antibody-negative SLE

Management of cold haemolytic syndrome

Cold agglutinin disease

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