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Sunday 21 February 2016

APPROACH TO HEMOLYTIC ANEMIA

General approach to patient with hemolytic anemia

●Suspecting hemolysis – Suspecting the presence of hemolysis is not difficult in the classic patient, who may have many of the following findings: rapid onset of pallor, anemia, and jaundice, decrease in hemoglobin or hematocrit more rapidly than can be explained by decreased red blood cell production, history of pigmented (bilirubin) gallstones, splenomegaly, presence of circulating spherocytic or fragmented red cells on the peripheral blood smear, and/or a positive direct antiglobulin test (Coombs test).


●Causes of hemolysis – The different causes for hemolysis are reviewed in the table (as in photos). A thorough history and physical examination is important, as many of these causes include drugs and toxins or systemic disorders (eg, infection, autoimmune disease, malignancy).

●Diagnosing hemolysis – The combination of an increased serum lactate dehydrogenase (LDH) and a reduced haptoglobin is 90 percent specific for diagnosing hemolysis, while the combination of a normal serum LDH and a serum haptoglobin >25mg/dL is 92 percent sensitive for ruling out the presence of hemolysis.

•Extravascular hemolysis – Abnormalities on the peripheral blood smear suggesting extravascular hemolysis include: spherocytes, microspherocytes, elliptocytes, "bite" or blister cells, schistocytes, acanthocytes, and teardrop red cells.

•Intravascular hemolysis – Abnormalities suggesting that the hemolysis is intravascular include the presence of free hemoglobin in plasma or urine, a urine sediment positive for iron (hemosiderinuria), and, in rare cases, the presence of circulating red cell "ghosts".

●Treatment – There is no generic treatment for hemolytic anemia. Appropriate management of a patient with hemolytic anemia requires that the underlying cause be determined, and may include red cell transfusion if the anemia is severe. Treatment of the different causes of hemolytic anemia (eg, autoimmune hemolytic anemia, hereditary spherocytosis, thrombotic thrombocytopenic purpura, paroxysmal nocturnal hemoglobinemia) is dependent on specific cause, will be discussed later