The patient is a 30-year-old female, who presented to the emergency room with profound anemia and renal failure. She had a known genetic background of Hemoglobin S/C but no prior anemia. Her complaints were of fairly recent onset, no longer than 4 weeks, mainly anorexia, fatigue and dyspnea on minimal efforts, epigastric pain and nausea.
She was started on dialysis and given steroids.
Pregnancy with a natural term delivery in the past.
Episode of stridor leading to ENT evaluation and a diagnosis of vocal cord edema.
Laboratory and other data:
Her presenting labs:
Serum creatinine 12 mg/dl, BUN 60 mg/dl, bicarbonate 15 mEq/l, Albumin 3 g/dl
Current ANCA 1:320, perinuclear and MPO positive, 50 units.
Hb 3.6 g/dl and on peripheral smear many target cells rare sickled cells
Complement normal, ESR > 140, Hgb S=46% Hgb C=46% HgbA= 5%,
ANA negative, Hepatitis serologies negative, erythropoietin significantly elevated
Hematuria and proteinuria on urine sediment.
Renal US: Normal size and good cortical thickness.
Chest film: mild CHF.
Research on old records weak positive pANCA 5 years ago
Short term follow up:
She was started on rituximab with little hope to reverse renal damage but to control the systemic disease for which she has no symptoms at this time.
One month into therapy remains on dialysis and has severe arterial hypertension. Considering a change to CCPD due to poor vascular access.