Hereditary Spherocytosis

Inherited defect in the red cell membrane skeleton that renders the cells spheroidal, less deformable, and vulnerable to splenic sequestration and destruction.
mutations most frequently involve ankyrin, band 3, and spectrin
most commonly autosomal dominant
Loss of central pallor, hyperchromic cells
Anisocytosis, spherocytes
Features of anemia with extravascular hemolysis

Extravascular hemolysis takes place within the macrophages of the spleen and liver. Any reduction in red cell deformability leads to splenic sequestration, followed by phagocytosis.

not associated with hemoglobinemia and hemoglobinuria
often produces jaundice
can lead to the formation of gallstones ( pigment stones).
Haptoglobin levels are decreased
splenomegaly.

Splenectomy is helpful in these patients. Asplenic patients show Howell-Jolly bodies (nuclear remnants) in RBCs
Osmotic fragility is increased when placed in hypertonic salt solutions - characteristic that is helpful for diagnosis.

Links to:

Thalassemia	Sickle Cell Anemia
G-6-PD Deficiency	Immunohemolytic anemias
Hemolytic Anemias Introduction	Anemia

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