Overview
Paroxysmal cold hemoglobinuria (PCH) is a rare hemolytic anemia characterized by intravascular hemolysis triggered by cold exposure, leading to the formation of Donath-Landsteiner (DL) antibodies against the P-type blood group antigen on red blood cells 12.Diagnosis
Blood smear examination: Look for neutrophil erythrophagocytosis, a key but underrecognized diagnostic sign 1.
Serological testing: Demonstrate the presence of DL antibodies through cold agglutinin tests 2.
Cold agglutinin titer: Elevated titers support the diagnosis 2.
Peripheral blood smear: May show fragmented red cells and nucleated red blood cells 2.Management
Avoid cold exposure: Minimize risk factors that trigger hemolysis 2.
Supportive care: Includes hydration, monitoring for anemia, and transfusions if necessary 2.
No specific drug therapy mentioned: Treatment primarily focuses on symptomatic management and avoidance of cold 2.Special Populations
Pediatrics: Diagnosis can be aided by recognizing neutrophil erythrophagocytosis on blood smears 1.
No specific recommendations for pregnancy or elderly noted 2.Key Recommendations
Utilize blood smear examination for neutrophil erythrophagocytosis as a diagnostic clue for PCH (Evidence: Moderate) 1.
Confirm diagnosis with serological tests demonstrating DL antibodies and elevated cold agglutinin titers (Evidence: Moderate) 2.
Manage primarily through avoidance of cold exposure and supportive care measures (Evidence: Expert opinion) 2.References
1 Radhakrishnan K, Downie PA, Earley CM. Paroxysmal Cold Hemoglobinuria: Diagnosis From the Blood Smear. Journal of pediatric hematology/oncology 2022. link
2 Carswell J, Eloff P, Edkins P, Willmore A. Paroxysmal cold haemoglobinuria. A case report. South African medical journal = Suid-Afrikaanse tydskrif vir geneeskunde 1979. link