0. 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
( )%
6.3
5.4
3.0
2.7
RETIC ABSOLUTE( )
0.172
0.146
DAY 1
DAY 2
DAY 3
DAY 4 AM PM
DAY 5
Total Bilirubin ( ) mg/dl 9
9.7
12.7
8.8
6.3
4.8
Direct Bilirubin ( ) 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 cm 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.
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