Waldenström Macroglobulinemia
Dongyou Liu in Handbook of Tumor Syndromes, 2020
IgM paraprotein directed to erythrocyte antigens can lead to autoimmune hemolytic anemia. While 10% of patients may have a positive Coombs test, only 3% will develop significant hemolysis [57]. Immune thrombocytopenic purpura and acquired von Willebrand disease are rare but well-described manifestations in WM [65]. Cryoglobulinemia, manifested by Raynaud phenomenon, acrocyanosis, peripheral neuropathy, vasculitis, or renal failure, is the result of IgM immune-complex precipitation [66]. Cold agglutinin disease, manifested by extravascular hemolytic anemia after cold exposure, is the result of IgM binding to RBC surface antigens [67]. Both manifestations have been described in approximately 10% of patients with WM [57].
Bronchiolitis obliterans organizing pneumonia induced by drugs or radiotherapy
Philippe Camus, Edward C Rosenow in Drug-induced and Iatrogenic Respiratory Disease, 2010
A 40-year-old man began taking phenytoin 300 mg daily after a craniotomy, and in 4 weeks developed fever, rash, unproductive cough, shortness of breath and bilateral crackles.51 The chest radiograph showed bilateral patchy infiltrates, and the open lung biopsy showed myxoid granulation tissue in the bronchioles extending to alveolar ducts and alveoli consistent with BOOP. The patient had also developed new-onset cold agglutinin disease. Corticosteroid therapy was given for 1 year. The chest radiograph, liver function and haematological abnormalities resolved.
Immunohematology
Gabriel Virella in Medical Immunology, 2019
Laboratory diagnosis. Testing for cold agglutinins is usually done by incubating a series of dilutions of the patient's serum (obtained by clotting and centrifuging the blood at 37°C immediately after drawing) with normal group O RBC at 4°C. Titers up to the hundreds of thousands can be observed in patients with cold agglutinin disease. Intermediate titers (below 1000) are when the cold agglutinins are associated with an infectious disease.
Transitioning Select Chemotherapeutics to the Outpatient Setting Improves Care and Reduces Costs
Published in Oncology Issues, 2021
Since 2015 when we transitioned certain rituximab administrations to the outpatient setting, we decreased our inpatient bed stays, reduced our inpatient chemotherapy costs, and increased the use of our own specialty pharmacy for patients receiving intravenous rituximab combination regimens, as well as an increased use of this model post-implementation for standard order sets. However, not every patient receiving rituximab can be treated in the outpatient setting. Accordingly, we have developed patient restrictions for rituximab in the outpatient setting, including:Immune thrombocytopenic purpura—dose-reduced rituximab, 100 mg9.Cold agglutinin disease.Post-transplant lymphoproliferative disease.Autoimmune hemolytic anemia.Prolonged chemotherapy inpatient stays requiring continued treatment.Infusion reaction or need for rituximab desensitization.
Healthcare resource utilization among commercially insured patients with cold agglutinin disease in the United States
Published in Journal of Medical Economics, 2020
Jun Su, Lauren C. Bylsma, Xiaohui Jiang, Jaime Morales Arias, Nisha Jain, Robert J. Nordyke
Cold agglutinin disease (CAD) is a rare chronic subtype of autoimmune hemolytic anemia with an estimated prevalence of 16 cases per 1 million individuals1. CAD is mediated by complement-fixing autoantibodies, which bind to the I antigen on the surface of red blood cells2. Termed cold agglutinins, these monoclonal immunoglobulin M antibodies are most pathogenic when their thermal amplitude (the highest temperature at which they bind to red blood cell antigens) overlaps with vascular temperatures at the lower limit of normal3. When bound to red blood cells, cold agglutinins activate complement component 1 (C1 complex) and trigger the classical complement pathway, resulting in extravascular hemolysis and, to a lesser extent, intravascular hemolysis2,4,5. CAD typically affects middle-aged and elderly individuals and is slightly more frequent among females than males4. Clinical manifestations include anemia and debilitating fatigue as well as cold-induced circulatory symptoms such as Raynaud’s phenomenon and acrocyanosis4,6. CAD may be primary, in which it is considered a clonal lymphoproliferative disorder, or it may be secondary to an underlying disease (recently termed cold agglutinin syndrome in the literature), such as aggressive lymphoma, the Epstein–Barr virus infection, or Mycoplasma pneumoniae infection5,6.
Complement-directed therapy for cold agglutinin disease: sutimlimab
Published in Expert Review of Hematology, 2023
Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia (AIHA) with autoantibodies produced by a distinct low-grade clonal B-cell lymphoid proliferation in the absence of underlying disease [1–4]. Lymphoproliferative disorders are a group of heterogeneous diseases characterized by unregulated and excessive production of lymphocytes (i.e. B-cells, T-cells, or natural killer cells), which may be caused by iatrogenic or acquired genetic mutations [5]. CAD falls within the category of small B-cell lymphoid neoplasms, which is the preferred terminology for low-grade B-cell lymphomas as they may not be indolent. However, CAD does not fulfill the criteria of a B-cell lymphoma and is instead described as a B-cell lymphoid proliferation. CAD is distinct from lymphoplasmacytic lymphoma and unlike lymphoplasmacytic lymphoma or marginal zone lymphoma it does not transform into large cell lymphoma (Table 1) [1,2,6,7]. CAD is distinguishable from cold agglutinin syndrome (CAS), which is characterized by the presence of cold agglutinin antibodies secondary to underlying conditions such as malignancy or infection [8]. Primary CAD accounts for about 15–25% of AIHAs and has a prevalence of 16/1,000,000 in Europe [3,9–11].
Related Knowledge Centers
- Agglutination
- Autoimmune Hemolytic Anemia
- Fatigue
- Hemolytic Anemia
- Immunoglobulin M
- Lysis
- Autoimmune Disease
- Red Blood Cell
- Rare Disease
- Cold Sensitive Antibodies