L E A R N I N G P O I N T S

MORNING REPORT 06/09

WHAT IS THE DIAGNOSIS?

CAROTID ARTERY STENOSIS

• Causes approximately 10 to 20% of strokes.• Occurs most frequently at its bifurcation.•  Atherosclerotic plaques cause symptoms most

often through distal embolism to branches of the retinal or cerebral arteries (MCA).• 50% patients with symptomatic carotid disease

report TIA symptoms preceding the stroke.

Grotta JC. Carotid stenosis. N Engl J Med 2013;369:1143-1150

TREATMENT

• In patients with symptomatic disease, carotid endarterectomy should be considered within 2 weeks if there is stenosis of more than 70% of the diameter of the ipsilateral carotid artery• Less benefit in patients with stenosis of 50 to 69% and in

asymptomatic patients, and there is no benefit in patients with stenosis of less than 50%.

• Carotid stenting is an alternative to carotid endarterectomy, particularly in patients at high surgical risk and in younger patients (<70 years of age).

Grotta JC. Carotid stenosis. N Engl J Med 2013;369:1143-1150

LEARNING POINTS

AUTOIMMUNE HEMOLYTIC ANEMIA

Lechner K, Jäger U (2010) How I treat autoimmune hemolytic anemias in adults. Blood 116(11):1831–8.

AUTOIMMUNE HEMOLYTIC ANEMIA

Warm Ab

IgGReacts against protein Ag on

the RBC surface

Cold Ab

90% IgMReacts against polysaccharide

Ag

Mixed Ab

Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013;122:1114–21.

COLD AGGLUTININ DISEASE

• 15% of patients with autoimmune hemolytic anemia. • Cold agglutinin IgM can be:• Monoclonal: underlying lymphoproliferative disorder• Polyclonal: post-infectious setting (mycoplasma, EBV,

legionella)

• The diagnosis is establish with hemolytic anemia, reticulocytosis, hyperbilirubinemia, ↑ LD, and + Coombs test for anti-C3 and classically negative anti-IgG.• After test findings suggest CAD, the antibody titer and thermal

activity should be determinedSwiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013;122:1114–21.

TREATMENT

• Nonpharmacologic measures are the cornerstone of management of CAD: avoiding cold exposure.

• Supportive transfusions may be used in patients with severe anemia. An in-line blood warmer should be considered to minimize cold agglutinin binding to transfused red cells.

• The most appropriate pharmacotherapy for CAD has remained an area of research. • 35% of patients treated with single-agent prednisone had a response, and most

responders needed further therapy. • Alkylating agent–containing regimens had a 44% response rate, but a similar low

proportion of patients achieved independence from further therapy compared with those being given corticosteroid-containing regimens.

• Response rate of 50% with purine analogs, with 40% of treated patients achieving independence from further therapy.

• Rituximab response rates were 83% in single-agent therapy and 79% in combination therapy with 51% patients achieving independence from further therapy.

Swiecicki PL, Hegerova LT, Gertz MA. Cold agglutinin disease. Blood 2013;122:1114–21.