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NYU Medical Grand Rounds Clinical Vignette Maryann Kwa, MD PGY-3 March 20, 2012 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Chief Complaint The patient is a 21-year-old male presenting with pain in the extremities and fatigue for three months. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS History of Present Illness • 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Additional History • Family History: • Mother, alive at age 50, with sickle cell anemia •Allergies: • No Known Drug Allergies •Medications: • None UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS 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 UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Laboratory Findings • LDH: 502 (110-225 U/L) • Haptoglobin: 45 (30-200 mg/dL) • Reticulocyte %: 5.1 (0.5-1.5) • Iron: 50 (42-146 ug/dL) • TIBC: 320 (250-450 ug/dL) • Ferritin: 650 (22-322 ng/mL) UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Other Studies • Chest X-ray: normal • Urinalysis: normal UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Working or Differential Diagnosis • The patient was admitted to the medicine service for further work-up of anemia. • Differential diagnoses: • Sickle Cell Anemia • Leukemia UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Hospital Course – 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. UNITED STATES DEPARTMENT OF VETERANS AFFAIRS Final Diagnosis • Sickle Cell Anemia (HbS) with Vaso-oclusive Crisis UNITED STATES DEPARTMENT OF VETERANS AFFAIRS