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G6PD Deficiency Anemia & Autoimmune Hemolytic Anemia: USMLE Step 1 Pathology

Glucose6phosphate dehydrogenase (G6PD) deficiency anemia and autoimmune hemolytic anemia (AIHA) are two distinct hematological disorders frequently tested in USMLE Step 1 Pathology. G6PD deficiency is an Xlinked recessive disorder characterized by a deficiency of the enzyme G6PD, which protects red blood cells (RBCs) from oxidative damage. Exposure to oxidative stressors, such as certain medications (e.g., sulfonamides, antimalarials) or infections, can lead to hemolysis in individuals with G6PD deficiency. Clinical manifestations include episodic hemolytic anemia triggered by oxidative stressors. Autoimmune hemolytic anemia, on the other hand, is characterized by the production of autoantibodies directed against selfantigens on RBCs, leading to their destruction. This condition can be classified as warm AIHA, where autoantibodies bind to RBCs at body temperature, or cold AIHA, where autoantibodies bind at colder temperatures. Diagnosis involves laboratory tests such as direct antiglobulin test (DAT) and identifying underlying triggers for hemolysis. Treatment strategies for both conditions focus on managing underlying triggers, providing supportive care with blood transfusions and immunosuppressive therapy for AIHA, and avoiding oxidative stressors for G6PD deficiency. Understanding the pathophysiology, clinical features, and management of G6PD deficiency anemia and autoimmune hemolytic anemia is essential for success on the USMLE Step 1 exam.

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