Researchers reported a very rare case of cold autoimmune hemolytic anemia (AIHA) with myelodysplastic syndromes (MDS), as published in the Indian Journal of Pathology and Microbiology.

“An increased awareness of secondary AIHA associated with MDS may help in prompt action that can be taken for earlier recognition of autoimmune phenomena potentially, leading to more positive outcomes for patients,” Deepti Mutreja and the coauthors of the study wrote.

The case is that of a male patient aged 76 years who presented to the Armed Forces Medical College Pune in Maharashtra, India with bilateral pedal edema, progressive dyspnea, and a fever. He had a history of hypertension and type 2 diabetes.

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He had also been diagnosed with megaloblastic anemia for which he was being treated with vitamin B12 with no response. Blood tests revealed that he had red cell agglutination and thrombocytopenia.

Thermal agglutination test showed that he had cold agglutinin disease (CAD), and bone marrow evaluation showed that he had adequate megakaryocytes. Cytochemical analyses revealed deletion 5q31 in 32% of his cells and trisomy 8 in 58% of his cells.

“This report is being discussed in view of its rarity of presentation of cold AIHA with MDS,” the researchers wrote and added that more research is needed to investigate the occurrence of autoimmune phenomena in MDS.

Read more about CAD overview

MDS is the name given to a group of diseases characterized by clonal hematopoietic stem cell neoplasms due to bone marrow failure. This leads to ineffective hematopoiesis, dyspoiesis, and cytopenias and can progress to acute leukemia.

AIHA is a group of disorders characterized by the immune system mistakenly attacking the red blood cells as foreign. There are 2 types of AIHA. In CAD, the immune system produces so-called cold agglutinins against red blood cells at cold temperatures while in warm antibody hemolytic anemia, the autoantibodies attack and destroy the red blood cells at normal body temperature.


Mutreja D, Paliwal G, Mangal V, Biswas AK. Cold autoimmune hemolytic anemia with myelodysplastic syndrome: not just an “Open and Shut case”. Indian J Pathol Microbiol. 2022;65:195-7. doi:10.4103/IJPM.IJPM_981_20