LABORATORY DATA(LFT TRENDS) Acute Massive Hepatic Enlargement in Sickle Cell Disease Minhaj Musa MD, Kyaw Zin Thein MD, Bisrat Haile MD, Harish Patel MD,FCCP , Rajat Mukherji MD,FCCP Kingsbrook Jewish Medical Center, Department of Internal Medicine • Brooklyn, New York OBJECTIVE Enlargement of the liver is a common condition in clinical practice and has multiple etiologies. Acute massive increase in liver size within a matter of 48hrs is an unusual event. LABORATORY DATA LABORATORY DATA(LFT TRENDS) DAY 1 DAY 2 DAY 3 AM PM DAY 4 DAY 5 WBC COUNT 36.1* 45.9* 24.5 14 12.3 8.2 7.9 RBC COUNT 2.74 2.12 2.79 2.55 2.69 3.30 2.98 HEMOGLOBIN 6.8* 8.6 8 8.4 9.4 8.8 HEMATOCRIT 25 19* 22 24 28 MCV 89.6 91.4 88.7 86.9 89.1 84.1 84.8 RDW 17 16.9 16.3 16.7 15.3 15.6 PLATELET COUNT 503 376 375 351 196 193 RETIC PERCENT (0.5-2.0)% 6.3 5.4 3.0 2.7 RETIC ABSOLUTE(0.02-0.11) 0.172 0.146 DAY 1 DAY 2 DAY 3 DAY 4 AM PM DAY 5 Total Bilirubin (0.2-1.0) mg/dl 9 9.7 12.7 8.8 6.3 4.8 Direct Bilirubin (0.0-0.2) mg/dl 1.3 2.4 3.9 3.2 2.2 1.8 Alkaline Phosphatase (32-92) U/L 75 77 84 74 59 Alanine Transferase (10-40) U/L 89 63 47 37 29 25 AST (SGOT) (10-42) U/L 91 60 42 31 27 LD (LDH) Lactate Dehydrogenase (91-180) U/L 371 279 210 CASE REPORT We report a case of a 25y/o female patient with sickle cell disease who presented with right upper quadrant abdominal pain, fever and leukocytosis of 36,000mm3. A urine analysis suggested UTI and she was treated with antibiotics along with intravenous fluids. On the 2nd hospital day, the hemoglobin dropped to 6.8g/dl from 8.2g/dl on admission. The leukocytosis increased to 45,900mm3 and the total bilirubin from a baseline of 9.0mg/dl on admission, rose to 12.7mg/dl two days later. A CT scan of the abdomen on admission showed a liver of normal size with mild intrahepatic biliary ductal dilatation. Two days later with persistent right upper quadrant discomfort, worsening leukocytosis and the rapid drop in hemoglobin, the patient underwent a repeat CT scan followed by MRI of the abdomen. In two days, the liver size had drastically increased from 17.5cm to 20.5cm in vertical length along the mid clavicular line. An infiltrative or cobble stone type pattern had appeared in the liver parenchyma. There was no intrahepatic or common duct dilatation. The hepatic and portal veins were patent. On subsequent vascular duplex study of the inferior vena cava (IVC), the hepatic and portal veins showed no evidence of thrombosis. Because of the patient’s deteriorating status, exchange transfusion was performed, following which, the liver function tests improved and liver size clinically diminished. RADIOLOGY REPORTS DISCUSSION This intriguing case illustrates the rare syndrome of hepatic sequestration in sickle cell disease. Our patient had an acute increase in liver size which was not due to hepatic or portal vein thrombosis; nor was it due to cholestasis. Sickle cell disease may result in sequestration of red blood cells in the spleen or lungs. On occasion, the liver is the site of RBC sequestration. In addition to pain and a falling hematocrit, liver size increases rapidly. This sequence of events in the liver may be missed unless liver size is regularly measured. When hepatic sequestration occurs, prognosis is grave and exchange transfusion should be considered as a potentially life saving strategy. Our patient with sickle cell disease had a dramatic picture of an acutely enlarging liver, which with appropriate treatment rapidly returned to normal size. REFERENCES Banerjee S, Owen C, Chopra S. Sickle cell hepatopathy. Hepatology 2001; 33:1021. Berry PA, Cross TJ, Thein SL, et al. Hepatic dysfunction in sickle cell disease: a new system of classification based on global assessment. Clin Gastroenterol Hepatol 2007; 5:1469. Ebert EC, Nagar M, Hagspiel KD. Gastrointestinal and hepatic complications of sickle cell disease. Clin Gastroenterol Hepatol 2010; 8:483. Hatton CS, Bunch C, Weatherall DJ. Hepatic sequestration in sickle cell anaemia. Br Med J (Clin Res Ed) 1985; 290:744. Hernández P, Dorticós E, Espinosa E, et al. Clinical features of hepatic sequestration in sickle cell anaemia. Haematologia (Budap) 1989; 22:169. Lee ES, Chu PC. Reverse sequestration in a case of sickle crisis. Postgrad Med J 1996; 72:487. http://healthfavo.com/wp-content/uploads/2014/04/sickle-cell-disease,-definition,-pathophysiology,-diagnosis-and-treatment.jpg http://epomedicine.com/wp-content/uploads/2014/05/sickle-cell-anemia-pathogenesis.jpg CT ABDOMEN & PELVIS WITH CONTRAST ON DAY 1 PATHOGENESIS CT ABDOMEN & PELVIS WITH CONTRAST ON DAY 3 MRI ABDOMEN WITH & WITHOUT CONTRAST ON DAY 4