NYU Medical Grand Rounds Clinical Vignette Maryann Kwa, MD PGY-3 March 20, 2012 U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

The patient is a 21-year-old male presenting with pain in the extremities and fatigue for three months. Chief Complaint U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

The patient was in his usual state of health until one year prior to admission when he started experiencing intermittent episodes of pain in his extremities. The pain was severe, sharp and crampy, involving the arms and legs, lasting several hours to days at a time and were self-limited. Accompanied by fatigue. No clearly defined precipitating factors. Severity, frequency and duration of these painful episodes gradually worsened over the last three months, including a recent episode that had lasted for 4 days without improvement at which time he presents to the ER. History of Present Illness U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Additional History Past Medical History: None Past Surgical History: None Social History: Denied tobacco, alcohol or drug use Immigrated from Africa to the United States six months ago U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Additional History Family History: Mother, alive at age 50, with sickle cell anemia Allergies: No Known Drug Allergies Medications: None U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Physical Examination General: Young male who appeared fatigued and in mild acute distress Vital Signs: T: 98.7 BP: 117/65 HR: 90 RR: 18 and O2 sat: 95% room air HEENT: scleral icterus, pale mucous membranes Cardiovascular: II/VI systolic murmur heard over the precordium Abdomen: palpable spleen tip Extremity: trace lower extremity edema bilaterally The remainder of the physical exam was normal U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Laboratory Findings CBC: WBC 10.0 Hg 8.0 (MCV 87) Hct 24.5 Platelets 350 Differential: neutrophils 75%, lymphocytes 10%, monocytes 7%, basophils 7%, eosinophils 1% Basic Metabolic panel: within normal limits Hepatic panel: total bilirubin 2.7, direct bilirubin 0.7 Remainder of hepatic was within normal limits U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Laboratory Findings LDH: 502 ( U/L) Haptoglobin: 45 ( mg/dL) Reticulocyte %: 5.1 ( ) Iron: 50 ( ug/dL) TIBC: 320 ( ug/dL) Ferritin: 650 ( ng/mL) U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Other Studies Chest X-ray: normal Urinalysis: normal U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

The patient was admitted to the medicine service for further work-up of anemia. Differential diagnoses: Sickle Cell Anemia Leukemia Working or Differential Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

–Analysis of a peripheral smear revealed sickled erythrocytes. –The patient’s symptoms improved with IV hydration, opioids for pain and 1 unit packed red blood cells. Folic acid was also initiated. –Hemoglobin electrophoresis confirmed HbS –Symptom resolution by hospital day 3. –He was initiated on hydroxyurea and received a pneumococcus vaccine prior to discharge. Hospital Course U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS

Sickle Cell Anemia (HbS) with Vaso-oclusive Crisis Final Diagnosis U NITED S TATES D EPARTMENT OF V ETERANS A FFAIRS