Chapter 121 Acute Hemolytic Disorders
INTRODUCTION
Hemolysis is the destruction of RBCs. All RBC are destroyed eventually, but pathologic hemolysis occurs when the rate of destruction is increased and the life span of RBCs is thus shortened. HA results when regeneration of RBCs from precursor cells is inadequate to replenish the destroyed cells. HA is caused by several immunologically and nonimmunologically mediated mechanisms (Box 121-1). It is crucial for the veterinarian to distinguish among these causes of hemolysis to provide appropriate therapy.
Box 121-1 Causes of Hemolysis
Evaluation of the Patient with Hemolysis
Regardless of the cause of hemolysis, certain therapeutic principles apply. Heme pigments released by hemolysis can cause nephrotoxicity in humans. Although acute renal failure resulting from hemolysis is not reported in small animals, adequate renal perfusion should be maintained to minimize this risk. Supportive care for animals with severe or rapid-onset anemia includes provision of either transfused RBCs or purified hemoglobin solution. The need for transfusion should be based on not merely an RBC count, hemoglobin measurement, or PCV but on clinical assessment (e.g., mental state, activity level, heart and respiratory rates, pulse quality). A packed RBC transfusion provides oxygen carrying capacity with less volume than whole blood and is often preferred in animals with hemolysis (see Chapter 66, Transfusion Medicine). Transfusion in cats must follow blood typing of both donor and recipient; blood typing of recipient dogs is not required for first-time transfusions or if only DEA 1.1-negative blood is transfused. Although transfusion of cross-matched blood is ideal, first transfusions in dogs and transfusion of blood-type compatible cats are usually successful without cross-matching.2 Transfused RBCs may be susceptible to the same cause of hemolysis as native RBCs. Purified hemoglobin (Oxyglobin) solutions do not require cross-matching or blood typing, although the future availability of these products is unknown.
FRAGMENTATION HEMOLYSIS
Fragmentation of RBCs results from numerous processes, but is most commonly the result of shearing of the RBC membrane in the small vessels (microangiopathic hemolysis) or from altered rheologic forces. Because shearing typically occurs inside the vascular space, hemoglobinemia and hemoglobinuria are common findings. The observation of schistocytosis on a peripheral blood smear provides supportive evidence of fragmentation; keratocytes and acanthocytes are also frequently identified.1 When fragmentation is suspected, diagnostic testing is aimed at identification of the underlying pathology. In a dog with a prominent heart murmur this might include an echocardiographic examination to rule out caudal caval syndrome, but the presence of marked splenomegaly would prompt ultrasonographic examination to rule out splenic torsion or neoplasia. Assays of coagulation are usually indicated because disseminated intravascular coagulation (DIC) can be a cause of fragmentation hemolysis, and because several of the conditions that lead to fragmentation hemolysis can precipitate DIC.1 Fragmentation hemolysis is a mechanical process, and management must be aimed at correction of the underlying disease (see Chapter 117, Hypercoagulable States). Supportive care includes provision of adequate oxygen carrying capacity (e.g., RBC transfusion, Oxyglobin) and prevention of complications of hemolysis (e.g., nephrotoxicosis, DIC).