Hereditary spherocytosis vs Autoimmune hemolytic anemia
How to tell Hereditary spherocytosis and Autoimmune hemolytic anemia apart when a board question makes them look alike.
Spherocytes plus negative Coombs and family history points hereditary; spherocytes plus positive Coombs points autoimmune.
Side by side
Hereditary spherocytosis causes inherited membrane instability with chronic extravascular hemolysis, jaundice, splenomegaly, pigmented gallstones, high MCHC, and family history.
Autoimmune hemolytic anemia causes antibody-mediated RBC destruction, often with positive direct Coombs test and warm IgG or cold IgM complement patterns.
Spectrin, ankyrin, band 3, or protein 4.2 defects reduce membrane stability, creating rigid spherocytes that are removed by the spleen.
Autoantibodies or drug-induced antibodies bind RBCs, causing splenic clearance or complement-mediated hemolysis.
Spherocytes, high MCHC, negative Coombs test, increased osmotic fragility, and increased EMA binding abnormality support hereditary spherocytosis.
Positive direct antiglobulin test supports autoimmune hemolytic anemia; warm disease is usually IgG and cold agglutinin disease is often IgM/complement.
The trap
Both can show spherocytes and extravascular hemolysis, so the direct Coombs test is the fastest separator.
Watch it explained
The repair move
When spherocytes appear, ask whether they are inherited membrane spherocytes or antibody-coated RBCs.
Try a free question
CoreA teenager has chronic jaundice, splenomegaly, pigmented gallstones, family history of anemia, high MCHC, spherocytes, and a negative direct Coombs test. Which diagnosis is most likely?
Show answer and explanation
Answer: A. Hereditary spherocytosis. Coombs-negative spherocytes with high MCHC and family history point to hereditary spherocytosis.
Why the other choices are tempting
- B. Warm autoimmune hemolytic anemia usually has a positive direct Coombs test.
- C. Cold agglutinin disease is immune mediated and often complement positive.
- D. G6PD deficiency causes oxidant-triggered Heinz bodies and bite cells.
- E. Beta thalassemia trait causes microcytosis and target cells rather than high-MCHC spherocytes.
Flashcards
Tap a card to flip it.
What test separates hereditary spherocytosis from AIHA?
The direct Coombs test separates them: negative in hereditary spherocytosis and positive in autoimmune hemolytic anemia.
Why is MCHC often high in hereditary spherocytosis?
Membrane loss makes RBCs denser and more spherical, increasing MCHC.
Quick answers
What is the fastest way to tell Hereditary spherocytosis from Autoimmune hemolytic anemia?
Spherocytes plus negative Coombs and family history points hereditary; spherocytes plus positive Coombs points autoimmune.
What is the key clue for Hereditary spherocytosis?
Hereditary spherocytosis causes inherited membrane instability with chronic extravascular hemolysis, jaundice, splenomegaly, pigmented gallstones, high MCHC, and family history.
What is the key clue for Autoimmune hemolytic anemia?
Autoimmune hemolytic anemia causes antibody-mediated RBC destruction, often with positive direct Coombs test and warm IgG or cold IgM complement patterns.
What is the common trap with Hereditary spherocytosis vs Autoimmune hemolytic anemia?
Both can show spherocytes and extravascular hemolysis, so the direct Coombs test is the fastest separator.