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Acidified glycerol lysis test
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Direct Coombs test
Fluorescent spot test
Hemoglobin electrophoresis
Serum iron level
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This patient with jaundice, normocytic hemolytic anemia, as well as a raised MCHC and RDW most likely has hereditary spherocytosis. The most specific test for confirming this diagnosis is an acidified glycerol test.Hereditary spherocytosis is an autosomal dominant disease that is caused by a mutation in red blood cell membrane proteins. The most common mutations involve spectrin, ankyrin, and band 3. Defective membrane proteins result in the loss of the outer membrane of erythrocytes, which leads to decreased erythrocyte surface area and the formation of spherocytes. Spherocytes are trapped and destroyed in the spleen, which results in splenomegaly. Laboratory studies are consistent with normocytic hemolytic anemia and commonly include an increased MCHC (due to decreased RBC volume), increased RDW (due to anisocytosis), and signs of extravascular hemolysis (increased LDH and indirect hyperbilirubinemia). The acidified glycerol lysis test or osmotic fragility test may be used for screening patients for hereditary spherocytosis, and the eosin-5-maleimide binding test can confirm the diagnosis. Treatment depends on the severity of the disease and can involve red blood cell transfusions, folic acid supplementation, or splenectomy.Manciu et al. review the diagnosis and treatment of hereditary spherocytosis. They discuss how total splenectomy remains the standard treatment in moderate and severe cases of hereditary spherocytosis. They recommend performing additional research on the efficacy and long-term outcomes of patients who receive partial splenectomy.Figure/Illustration A is a clinical photograph demonstrating scleral icterus (red circles). This finding is consistent with a hemolytic anemia leading to the production of bilirubin.Incorrect Answers:Answer 2: Direct Coombs test is used for the diagnosis of autoimmune hemolytic anemia (AIHA), which can present similarly with jaundice, splenomegaly, and an elevated MCHC; however, it primarily occurs in older individuals (> 40 years) and is associated with malignancy, drugs, or recent infection.Answer 3: Fluorescent spot test is used to diagnose glucose-6-phosphate dehydrogenase deficiency (G6PD deficiency), which can also present with jaundice and hemolytic anemia after medication use; however, it would not likely be precipitated by oseltamivir. The increased MCHC and RDW are more consistent with hereditary spherocytosis.Answer 4: Hemoglobin electrophoresis is used to diagnose hemoglobinopathies such as sickle cell disease or thalassemias. Hemoglobinopathies may also present with hemolytic anemia; however, these diseases do not typically have an increased RDW and MCHC.Answer 5: Serum iron level is useful in the diagnosis of iron deficiency anemia, which presents with a microcytic anemia and low iron levels.Bullet Summary: Hereditary spherocytosis is due to an autosomal dominant mutation in red blood cell membrane proteins and can be diagnosed with an acidified acid glycerol test.
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