Heme cases Mar 24_teaching aid

Heme cases Mar 24_teaching aid - Feb 24, 2011 SOM 214...

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Feb 24, 2011 SOM 214
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Hemolytic anemias Classifications: Intrinsic vs. Extrinsic = mechanism Hereditary vs. Acquired = chronicity, genetics Intravascular vs. Extravascular = pathophysiology Evaluation: Step 1: It is hemolysis? Reticulocyte production index high, bilirubin increased, LDH increased Step 2: What kind? History, family history, race/ethnicity, morphology, chronicity, supporting tests Step 3: What kind/how severe and what problems can occur? Intravascular vs. extravascular Serum free hemoglobin, urine heme, urine hemosiderin
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Case 8 21 year old African-American male with sickle cell anemia admitted because of right anterior chest pain x 12 hours Past History: The diagnosis of sickle cell anemia was made at age 18 months when the patient was admitted to the Pediatric Service because of painful swelling of both hands. The patient had multiple subsequent admissions from age 5 to 14 because of painful crises; treatment during each of these included parenteral fluids and analgesics. A palpable spleen was noted until age 10, not mentioned since. Hemoglobin at admissions and during outpatient follow-ups ranged between 7-10, retics 8-14%, total bilirubin 2.5-5 (mostly indirect). Transfused only once, during an admission at age 6 for a painful crisis associated with a pulmonary infiltrate. Since age 14 he has had less frequent, milder crises managed at home with oral fluids and analgesics.
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Present Illness: One day before admission low grade fever and malaise, with mild aching pain in back, left leg and right arm. He took aspirin and felt somewhat better. The night before admission he developed a cough, right anterior pleuritic chest pain and fever to 101 o F. No chills. The cough was productive of small amounts of white sputum. Just before coming to the hospital he noted blood streaking in his sputum. Physical Examination: T 102 o F P 110 BP 110/70 R 24 A well developed thin young black male, tachypneic at rest, splinting his right chest, and appearing acutely ill. Sclerae icteric. Fundi: moderate tortuosity of retinal veins; no hemorrhages or exudates. ENT: N. Lungs: clear to percussion; to and fro friction rub heard at right base; no rales, breath sounds normal. Heart: not enlarged; regular sinus rhythm; III/IV midsystolic murmur heard over the precordium without radiation. Abdomen: soft, without mass or tenderness. Liver and spleen not felt. Extremities: no edema; two well healed 3x3cm scars over left anterior tibia; joints normal. No calf tenderness. Neurological exam normal.
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Laboratory data: Hb 7.8 RBC 2.45 Hct 23 MCV 94 WBC 18,000 with segs 78, bands 11, lymphs 10, monos 1 and 1 nucleated RBC/100 WBC. Platelets 474,000, retics 10.3%, sed rate 2 mm.
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Heme cases Mar 24_teaching aid - Feb 24, 2011 SOM 214...

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