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Paroxysmal Nocturnal Hemoglobinuria

Nocturnal 2

Step 1: View clinicals

A 24 year old woman presents with jaundice and abdominal pain for 1 day. The pain is mild and diffuse, and aching in nature. She has also experienced intermittent episodes of dark colored urine for 7 days. On the 2nd such day, she was evaluated by her primary care practitioner, who found no abnormal clinical findings. At that time, a complete blood cell count, urinalysis, renal profile and renal ultrasound were all normal. There is no history of renal stones, NSAID abuse, weight loss, night sweats, fever, malena, hemoptysis or pale stools. Her menstrual cycles are regular, and 30 days in length. The last period was 2 week ago; a urine pregnancy test is found to be negative. Her medical, surgical and drug histories are unremarkable. There is no family history of bleeding disorders. She neither smokes nor consumes alcohol.

Step 2: Order all relevant investigations

Repeat Complete Blood Count

RBC: 1.6x10^6/mm3 (3.9x10^6-5.0x10^6) Hb: 5 g/dL (11-18) Hct: 17.2% MCV: 75 fl (75-95) RDW: 28 (11.6-14.6) Reticulocyte count: 10.9% (0.5-2.3) WBC: 3,600/mm3 (3,500-10,500) Plt: 189,000/mm3 (150,000-450,000) The peripheral smear reveals hypochromic red blood cells, polychromatophilia and normoblasts. The other cell lines appear normal.

Repeat Urinalysis

Color: Dark Microscopy of centrifuged sediment: no erythrocytes Dipstick test: positive for heme

Liver Profile

Total serum bilirubin: 7.5 mg/dL (0.1-1.2) Indirect bilirubin: 5.5 mg/dL (0.1-1.0) AST: 213 U/L Serum LDH: 1500 U/L (259-613) Serum haptoglobin: <10 mg /dL (45-165) ALT, ALP, INR, S. Albumin: within normal parameters

Direct Antiglobulin Test

The Direct Antiglobulin Test (DAT) is negative.

Step 3: Select appropriate management

Blood Transfusion
Folic Acid
Iron Therapy

Score: ★★☆