Internal Medicine

0521779407-19 CUNY1086/Karliner 0 521 77940 7 June 4, 2007 21:21

1346 Sickle Cell Syndromes

tests

Basic Blood Tests

■Sickle trait: normal CBC, normal LFTs

■SS: Hematocrit in 20’s, reticulocytes in 20’s, elevated MCV, elevated

WBC, elevated platelets, many sickle forms, Howell-Jolly bodies,

increased indirect bilirubin, low haptoglobin, mild increase in LFTs

■SC disease: Hematocrit in low 30’s, reticulocytes in 5–10 range, rare

sickle forms, slightly low MCV, rare mild increase in indirect bilirubin

■SS/thalassemia: Hematocrit in 20–30’s, reticulocytes in 5–20’s,

decreased MCV,±Howell-Jolly bodies,±increased indirect biliru-

bin

Basic Urine Tests

■All sickle syndromes (including sickle trait) – isosthenuria

■In SS, proteinuria can be early sign of sickle glomerulopathy and

should be monitored yearly

■Hematuria a fairly common problem in SC disease, can occur in

sickle trait, but specific non-sickle related causes must be ruled out

Recommended Tests in SS :

■Extended panel RBC typing (to assure appropriate choice in case of

transfusion)

■Yearly ophthalmologic exam to detect early retinopathy

■In children, transcranial Doppler to detect those at high risk for stroke

■Yearly baseline PFTs, ABGs, and ECG to compare with studies at time

of acute event

Periodic echocardiogram to identify adults with pulmonary hyper-

tension

differential diagnosis

■Hemoglobin electrophoresis and CBC can distinguish sickle trait, SS,

SC disease, and S/thalassemia.

management

Routine Health Maintenance

■Anticipatory testing as above

■Pneumococcal and influenza vaccines

■Prophylactic folate

Specific Complications

■High incidence of pain crises and/or acute chest syndrome use

hydroxyurea prophylaxis