Reviewer: S. Randhawa, M.D.
A 29-year-old African American male (AAM) with a known history of sickle cell disease and multiple blood transfusions was admitted through the ER with symptoms consistent with his prior sickle cell crises. He complained of pain in his knees, hips, and back that had been "off and on" for the last couple of months. It became markedly worse this afternoon and began to involve his chest and his arms. He denies fevers, chills, rigors, or night sweats.
Past medical history (PMH)
A 29-year-old African American male (AAM) with a known history of sickle cell disease and multiple blood transfusions was admitted through the ER with symptoms consistent with his prior sickle cell crises. He complained of pain in his knees, hips, and back that had been "off and on" for the last couple of months. It became markedly worse this afternoon and began to involve his chest and his arms. He denies fevers, chills, rigors, or night sweats.
Past medical history (PMH)
Sickle cell disease, aseptic necrosis of bone (hips and back).
Medications
Hydroxyurea, gabapentin, methadone, deferasirox, folic acid, multivitamin (MVT).
Family medical history (FMH)
Family medical history (FMH)
Mother, father and brother with sickle cell trait.
Physical examination
Physical examination
Stable vital signs (VSS).
Eyes: anicteric.
ENT: Oropharynx clear, no plaques or exudates.
Respiratory: clear to auscultation bilaterally, no respiratory distress, no rales, no rhonchi, no wheezing.
Cardiovascular: no murmurs, no rubs, no gallops, regular rate and rhythm.
Gastrointestinal: soft, nontender, nondistended.
Genitourinary: no CVA tenderness
Extremities: no c/c/e.
What is the most likely diagnosis?
Eyes: anicteric.
ENT: Oropharynx clear, no plaques or exudates.
Respiratory: clear to auscultation bilaterally, no respiratory distress, no rales, no rhonchi, no wheezing.
Cardiovascular: no murmurs, no rubs, no gallops, regular rate and rhythm.
Gastrointestinal: soft, nontender, nondistended.
Genitourinary: no CVA tenderness
Extremities: no c/c/e.
What is the most likely diagnosis?
Sickle cell crisis.
What diagnostic tests would you suggest?
What diagnostic tests would you suggest?
CBC
Reticulocyte count
CMP
CXR
UA
What happened?
Reticulocyte count
CMP
CXR
UA
What happened?
CBC showed anemia with evidence of iron overload from previous ferritin measurements.
Figure 1. CBC in sickle cell anemia (click to enlarge the image).
Figure 2. Increase in ferritin level secondary to blood transfusions in a 29-year-old patient with sickle cell disease (click to enlarge the image).
Sickle cell crisis resolved with pain medications, NS at 100 cc/hr and O2 via NC. The patient received 2 U PRBC. CXR was negative for acute chest syndrome and UA was negative for infection.
Deferasirox (Exjade), an oral iron chelator, was continued.
Final diagnosis
Figure 1. CBC in sickle cell anemia (click to enlarge the image).
Figure 2. Increase in ferritin level secondary to blood transfusions in a 29-year-old patient with sickle cell disease (click to enlarge the image).
Sickle cell crisis resolved with pain medications, NS at 100 cc/hr and O2 via NC. The patient received 2 U PRBC. CXR was negative for acute chest syndrome and UA was negative for infection.
Deferasirox (Exjade), an oral iron chelator, was continued.
Final diagnosis
Chronic iron overload due to blood transfusions in sickle cell disease.
Summary
Summary
There is no specific hemoglobin number which triggers blood transfusion. Iron overload is a significant problem for patients with sickle cell disease who commonly have a ferritin level of 3,000. Many patients are able to tolerate a hemoglobin level in 6-7 mg/dL range without adverse symptoms.
Related reading
Related reading
Sickle cell drug hydroxyurea underused, experts agree. Reuters, 02/2008.
Sickle Cell Anemia Centenary: the article "Peculiar elongated and sickle-shaped red blood corpuscles" published in 1910 http://goo.gl/SAg54
Published: 09/13/2007
Updated: 02/01/2010
Published: 09/13/2007
Updated: 02/01/2010
extremities : no c/c/e
ReplyDeletewut does c/c/e mean?
no c/c/e =
ReplyDeleteno cyanosis/clubbing/edema