A patient with idiopathic cold agglutinin disease (CAD) was successfully treated with steroids, according to a new case study published in Cureus

Haider et al wrote about a 71-year-old lady who presented with a generalized weakness for 3 days. She was referred to the emergency department by her primary care doctor due to low hemoglobin of 5.1 g/dL. She denied chest pain, shortness of pain, melena, hematuria, hemoptysis, or taking nonsteroidal anti-inflammatory medication, and she had an unremarkable colonoscopy a year prior to admission.

Upon examination, the patient appeared to be alert and comfortable. Chest and abdominal physical examinations were unremarkable. Laboratory findings demonstrated severe macrocytic hemolytic anemia with the following results: 

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  • Hemoglobin: 5.1 g/dL 
  • Mean corpuscular volume: 120 fL
  • Bilirubin: 3.3 mg/dL 
  • Reticulocyte: 18.7% 
  • Lactate dehydrogenase: 459 U/L 
  • Haptoglobin: <10 mg/dL 

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A direct Coombs test was performed, which returned positive for IgG-C3bC4d antibodies. The stool test was negative for occult blood. A cold agglutinin antibody test demonstrated elevated antibody titers of 1:610 at 4 °C.

Additional tests were performed to identify secondary CAD disease but the results returned negative. The patient was diagnosed with primary CAD and started on prednisolone 40 mg twice daily. She also received a transfusion of 2 units of packed red blood cells. Repeat hemoglobin showed an improvement to 8.0 g/dL.

Her clinical status improved and she was discharged. She continued to be seen in the clinic. Her prednisolone was eventually tapered down to 5 mg/day when her hemoglobin levels stabilized.

CAD is not usually responsive to steroid therapy, unlike warm autoimmune hemolytic anemia, the authors said. However, there have been cases in which CAD has been successfully treated with steroids, such as in this case study.


Haider A, Alavi F, Siddiqa A, et al. A case of idiopathic cold agglutinin hemolytic anemia successfully treated with steroids. Cureus. 2022;14(3):e23172. doi:10.7759/cureus.23172