Paroxysmal Cold Hemoglobinuria

Paroxysmal cold hemoglobinuria (PCH) is one of the most common causes of autoimmune hemolytic anemia in young children which produces severe anemia and hemoglobinuria due to massive intravascular hemolysis following exposure to cold temperature. Its diagnosis is challenging, and for effective management, it is important to differentiate it from other conditions with close differential diagnosis such as cold agglutinin disease (CAD), paroxysmal nocturnal hemoglobinuria (PNH), hemolytic uremic syndrome (HUS), drug-induced hemolytic anemia, etc. In this chapter, a clinical case scenario is presented followed by step-by-step evidence-based approach to utilize specialized and advanced tests to unravel final diagnosis of PCH. A comprehensive description of pathophysiology of disease, handling and processing of blood samples for suspected case of PCH and differentiation between hemoglobinuria, myoglobinuria, and hematuria are discussed in question–answer format. To gain expertise in this topic and enhance your diagnostic skills for effective planning of management, read this chapter on paroxysmal cold hemoglobinuria.