Cold agglutinin hemolytic anemia (cAHA) is a rare autoimmune disorder characterized by the production of cold agglutinins. We present a case of secondary cAHA in a 23-year-old female with severe anemia and unexplained hemolysis. The patient exhibited findings indicative of hemolysis and a positive direct antiglobulin test (DAT) with complement alone. Additional investigations revealed incidental lung infiltrates, negative serology for infections and autoimmune diseases, and a low cold agglutinin titer. The patient showed a favorable response to doxycycline and supportive therapy, including multiple packed red blood cell transfusions. At the two-week follow-up, the patient had a stable hemoglobin level with no evidence of ongoing hemolysis. This case highlights the importance of considering secondary cAHA in patients with cold symptoms or unexplained hemolysis. Primary cAHA patients may require more aggressive treatment, including rituximab and sutilumab.
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http://dx.doi.org/10.7759/cureus.39734 | DOI Listing |
Hematol Rep
January 2025
Children's Haematology and Cancer Centre, Mount Elizabeth Hospital, Singapore 228510, Singapore.
Background: Peripheral erythrophagocytosis appears to be a unique sign of acquired immune-mediated hemolytic anemia. It is said to be rare but its prevalence among patients with autoimmune hemolytic anemia has not been studied.
Methods: In this retrospective study from July 2014 to June 2024, the clinical and laboratory features, treatment and outcomes of children diagnosed with autoimmune hemolytic anemia were described.
JA Clin Rep
January 2025
Department of Anesthesiology and Pain Medicine, Gifu University Hospital, 1-1 Yanagido, Gifu, 501-1194, Japan.
Background: Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia that induces blood coagulation and hemolysis upon exposure to cold temperatures. Strict temperature control is essential to mitigate these effects, especially during surgical procedures where hypothermia is possible.
Case Presentation: A 57-year-old male, 165 cm and 72 kg, diagnosed with CAD, underwent cerebral vascular anastomosis.
Med Int (Lond)
December 2024
Department of Hematology, Dokuz Eylul University Hospital, Izmir 35000, Turkey.
Cold agglutinin syndrome is a form of acquired hemolytic anemia that typically arises from underlying conditions, such as infections, autoimmune disorders or lymphoid malignancies. The majority of patients remain asymptomatic and are diagnosed with anemia through routine complete blood count (CBC) testing. The present study describes the case of a male patient in his 50s who sought a second opinion at the authors' clinic due to newly detected anemia.
View Article and Find Full Text PDFCureus
December 2024
Department of Internal Medicine, Uwajima City Hospital, Uwajima, JPN.
We report a case of coexisting cold agglutinin and cryoglobulin in a patient with severe anemia following COVID-19 infection, in whom direct antiglobulin testing revealed C3d positivity and immunoglobulin G negativity. There was no evident hemolytic anemia, thrombosis, or clinically significant IgM monoclonal gammopathy. The anemia improved with folic acid supplementation alone accompanied by a decrease of the cold agglutination titer, and the direct antiglobulin test became negative.
View Article and Find Full Text PDFFront Med (Lausanne)
December 2024
Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria.
Introduction: Although rituximab is approved for several autoimmune diseases, no formal dose finding studies have been conducted. The amount of CD20+ cells differs significantly between autoimmune diseases and B-cell malignancies. Hence, dose requirements of anti-CD20 therapies may differ accordingly.
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