Cold agglutinin disease in fibrolamellar hepatocellular carcinoma: a rare association with a rare cancer variant

Abstract

Cold agglutinin disease (CAD) is a rare autoimmune hemolytic anemia. Although it can occur secondary to lymphoproliferative disorders and autoimmune or infectious diseases, CAD is rarely reported as secondary to solid tumors. We report a case of a woman aged 18 years diagnosed with a well-differentiated hepatocellular carcinoma of the fibrolamellar subtype, who was shown to have CAD also. Her general condition, including CAD, improved after targeted therapy with sorafenib for the hepatocellular carcinoma and only conservative measures for the CAD that consisted of avoidance of cold. In summary, although it is an extremely rare association and less common than lymphoproliferative disorders, CAD can be associated with solid tumors.

Cold autoimmune hemolytic anemia: a rare association with triple-positive breast cancer

“…Cold AIHA is a rare condition caused by an increase in the level of cold-reactive antibodies. Mostly, it has been reported in patients with lymphoproliferative disorders including Non-Hodgkin lymphoma or Chronic lymphocytic leukemia (CLL) [5,6]. Autoimmune disorders including rheumatoid arthritis, systemic lupus erythematosus (SLE), scleroderma, ulcerative colitis, and Crohn's disease are also a significant cause of AIHA [7,8].…”

Pembrolizumab-associated autoimmune haemolytic anaemia

“…It is possible that in our case, AIHA was secondary to the malignancy rather than the immunotherapy, pembrolizumab. However, clinically this did not appear to be likely, as there had been no significant change in his burden of malignancy and case reports of AIHA and solid tumour malignancy appear to be correlated with tumour burden (either initially presenting with AIHA, which then improves/resolves with the treatment of the new underlying malignancy or progression of metastatic malignant disease with subsequent development of AIHA) 15 18–21. Furthermore, our patient had complete resolution of AIHA with steroid treatment and withdrawal of immunotherapy with no relapse on subsequent disease progression.…”

A Novel Approach for Treatment of Cold Agglutinin Syndrome-Related Severe Hemolysis

“…Presentation with acute, severe intravascular hemolysis is rare [1,3]. Cold agglutinin syndrome has been linked most often to underlying lymphoproliferative disorders [4]; however, rare cases of paraneoplastic cold agglutinin syndrome associated with solid tumors have been reported [5][6][7]. In the absence of any other findings, we hypothesize that the cold agglutinin syndrome may be a paraneoplastic manifestation of active Figure 1.…”