The rare phenomenon of concurrent cold agglutinin disease (CAD) and myelodysplastic syndromes (MDS), along with neutrophilic erythrophagocytosis, has been reported in a 56-year-old male patient admitted to the emergency department (ED) at Kowsar Hospital, Kurdistan University of Medical Sciences in Sanandaj, Iran. The case study was published in Clinical Case Reports.
CAD is a type of autoimmune hemolytic anemia that results from autoantibodies attacking red blood cells (RBCs) in cold temperatures. In MDS, which are clonal hematologic malignancies, blood cell production is disrupted, with immature blood cells in the bone marrow not reaching maturity and therefore not becoming healthy blood cells.
MDS are often associated with autoimmune diseases and inflammatory responses by the immune system. In neutrophilic erythrophagocytosis—an uncommon occurrence reported among patients with MDS—RBCs are engulfed by neutrophils.
The current case presentation describes a man who presented to the ED with severe anemia and a low level of consciousness. The patient, who had a history of anemia, thrombocytopenia, and RBC agglutination, had recently been diagnosed with MDS with multilineage dysplasia (MDS-MLD) and 3% blast cells based on bone marrow examination. He had not yet received any medication to treat the MDS-MLD.
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The patient’s peripheral blood smear examination revealed hyposegmentation in neutrophils, high nucleated RBC count, mild RBC agglutination, and erythrophagocytosis in approximately 2% of the neutrophils. Following the elimination of blood group discrepancy due to the presence of cold agglutinins and the successful performance of crossmatching with preheated serum, the patient received a transfusion of 2 units of packed RBCs.
The patient’s condition worsened rapidly following the transfusions, and he died 10 hours later due to cardiac arrest.
Several aspects of this case warrant mention. The first is the coexistence of CAD and MDS, which is unusual. Second, the observation of erythrophagocytosis by neutrophils in the peripheral blood smear is a rare morphologic event. Further, the observation of a large number of phagocytized RBC precursor nuclei purportedly has not been observed in the peripheral blood smears of such individuals.
The authors concluded, “We hope this case [aids] researchers with better understanding of MDS and CAD
Amelirad A, Modaresi P, Soltani H. Neutrophilic erythrophagocytosis in myelodysplastic syndrome and cold agglutinin disease co-occurrence. Clin Case Rep. 2023;11(1):e6828. doi:10.1002/ccr3.6828