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Cold agglutinin disease, a subtype of cold-type autoimmune hemolytic anemia, is referred to as cold agglutinin syndrome (CAS) when it develops secondary to other conditions. Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by the peripheral destruction of platelets. While both CAS and ITP can occur in patients with rheumatoid arthritis (RA), their concurrent manifestation in a single patient has not been reported, leaving the optimal treatment strategy for such a complex case unclear.

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Article Synopsis
  • A paraneoplastic syndrome involves symptoms caused by cancer that aren't directly due to the tumor's presence, commonly affecting older patients with solid tumors and blood cancers.
  • Autoimmune hemolytic anemia is a notable paraneoplastic syndrome linked to various malignancies, particularly in lymphoproliferative disorders and certain solid tumors.
  • The text discusses a specific case of cold-type autoimmune hemolytic anemia that manifests as a paraneoplastic symptom of a thyroid cancer.
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Diagnosis and management of Evans syndrome in adults: first consensus recommendations.

Lancet Haematol

August 2024

Hematology Unit, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Evans syndrome is a rare disease marked by a severe clinical course, high relapse rate, infectious and thrombotic complications, and sometimes fatal outcome. Management is highly heterogeneous. There are several case reports but few large retrospective studies and no prospective or randomised trials.

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