Sickle Cell Disease and Acute Chest
Sickle Cell Disease and Acute Chest
Sickle Cell Disease and Acute Chest
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MKSAP<br />
This patient has Sβ+ + thalassemia, thalassemia,<br />
characterized by a hemoglobin<br />
S level that is greater than 60%, with an elevated hemoglobin A2<br />
level <strong>and</strong> mild microcytosis. microcytosis.<br />
These patients have painful crises,<br />
but to a lesser extent than patients homozygous for hemoglobin<br />
S. Patients with Sβ+ + thalassemia<br />
have target cells <strong>and</strong> few or no<br />
sickled<br />
erythrocytes on the peripheral blood smear. Patients with<br />
sickle cell trait (AS), who are often confused with patients with with<br />
Sβ+ + thalassemia, thalassemia,<br />
have a hemoglobin S level that is less than 50%<br />
<strong>and</strong> a hemoglobin A level that is correspondingly greater than<br />
50%. Patients with the sickle cell trait also have normal-sized<br />
normal sized<br />
erythrocytes. Patients with hemoglobin SC disease have painful<br />
crises in addition to an approximately 50:50 ratio of hemoglobin<br />
S to hemoglobin C on electrophoresis.
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