A 57 y.o. woman presents with jaundice and a Hct of 16. Her peripheral smear is shown below.

What is wrong?

Our patient had spur cell hemolytic anemia. The other name for these speculated cells is acanthocytes. ( from the Greek word acanthi meaning thorn) This can occur in about 5% of patients end stage liver disease.  Liver dysfunction causes accumulation of abnormal apolipoprotein A-II-deficient lipoprotein in the plasma.  Red cells are loaded with cholesterol by this lipoprotein and acquire too much cholesterol in the outer layer of the membrane.  This causes the outer layer of the membrane to bulge out in spicules which are trapped in the spleen and destroyed.

While similar cells can be seen in other diseases like myxedema, and inherited disorders like abetalipoproteinemia the causes there are different with congenital membrane abnormalities being common in abetalipoproteinemia and lipid abnormalities in hypothyroidism.

echinocytes

Spur cells are not to be confused with echinocytes( from the Greek echinos meaning sea urchin) which may appear in uremia or  caused by excess  EDTA in an underfilled collection tube  or slow drying of the cells on a slide. The projections are smaller and more uniform in echinocytes.

JUST TO REVIEW HEMOLYTIC ANEMIA

A. CONGENITAL

Sickle cell

Thalassemia

G6PD deficiency

Hereditary spherocytosis

 abetalipoproteinemia

B. ACQUIRED

Infections- malaria, mycoplasma, mono, cmv, HIV, clostridia

Medications (sulfa, penicillin, antimalarial meds)

Autoimmune-SLE, lymphoproliferative disorders

Ulcerative colitis

Hypersplenism

Mechanical heart valves

Snake venoms

Liver disease

Microangiopathic hemolytic anemia- TTP

The only cure for spur cell hemolytic anemia is a liver transplant and our patient was being prepared for a transplant.

Doll DC, Doll NJ. Spur cell anemia. South Med J. 1982 Oct 75(10):1205-10.

Gerber B, Stussi G. Reversibility of spur cell anemia. Blood 2011. 118:4304 https: //doi.org/10.2282/blood-2010-11-321034.

L’Acqua C, Hod E. New perspectives on the thrombotic complications of haemolysisBr J Haematol 2015;168-175.