Maulana Azad Medical College Pittsburgh, PA, United States
Rahul Karna, MD1, Sushil Kumar Sharma, MD2, Rishabh Kothari, MD2, Kunal Das, MD, DM2, Premashish Kar, MD, PhD, DM3 1Maulana Azad Medical College, Delhi, Delhi, India; 2Max Super Specialty Hospital, Ghaziabad, Uttar Pradesh, India; 3Max Super Specialty Hospital, Delhi, Delhi, India
Introduction: Hereditary spherocytosis (HS) is an inherited RBC disorder causing hemolytic anemia. It is a risk factor formation of pigment gall stones due to chronic hemolysis. We present a patient with HS with a total bilirubin of 79.2 mg/dl due to multiple simultaneous etiology.
Case Description/Methods: A 33-year-old male with a history of HS, gall stone disease presented with RUQ abdominal pain and clay-colored stools for a week. Examination revealed scleral icterus, hepatomegaly, and splenomegaly. Labs revealed Hb 12.6 gm/dl, WBC 19.2/µL, T.Bil. 79.2 mg/dl, D.Bil. 51.5 mg/dl, AST 21 U/L, ALT 53 U/L, ALP 133 IU/L and INR 1.2. Further labs revealed positive IgM anti-HAV while the rest of the viral hepatitis panel was negative. US revealed dilated common bile duct (CBD) and intrahepatic biliary radical dilation (IHBRD) with an echo density in the lower end of CBD, suggestive of choledocholithiasis and gross splenomegaly. We managed the patient with antibiotics for acute cholangitis followed by ERCP during which sphincterotomy followed by CBD stone removal, CBD, and pancreatic duct stenting was done. He had worsening abdominal pain, and a new WBC elevation three days later. CT scan showed thickly walled and edematous gall bladder with hyperdensity in the lumen representing sludge, pericholecystic fluid, and fat stranding. Repeat MRCP showed features suggestive of acute calculus cholecystitis with a calculus impacted in the cystic duct. He had refused cholecystectomy and splenectomy in the past. Subsequently, he underwent open cholecystectomy with CBD exploration, T tube insertion, and splenectomy. Pathology of gall bladder and spleen revealed xanthogranulomatous cholecystitis (XGC) and congestion respectively. Patient showed clinical and biochemical improvement after surgery and had no recurrent symptoms at 3 months follow up.
Discussion: Our patient with a history of HS and gall stone disease, presented with likely recent viral hepatitis, obstructive jaundice due to choledocholithiasis, and subsequently developed acute calculous cholecystitis. Such profound elevation in bilirubin is an unlikely presentation of HS in adults and should point towards other concomitant etiology. Splenectomy is recommended as the definitive treatment of HS during open cholecystectomy in these patients. Our patient might have developed XGC from pigment stones due to HS and hence, highlights the importance of splenectomy as definitive management of HS.
Rahul Karna indicated no relevant financial relationships.
Sushil Kumar Sharma indicated no relevant financial relationships.
Rishabh Kothari indicated no relevant financial relationships.
Kunal Das indicated no relevant financial relationships.
Premashish Kar indicated no relevant financial relationships.
Rahul Karna, MD1, Sushil Kumar Sharma, MD2, Rishabh Kothari, MD2, Kunal Das, MD, DM2, Premashish Kar, MD, PhD, DM3. P1132 - Profound Hyperbilirubinemia in Hereditary Spherocytosis With Complicated Gall Stone Disease, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.