Published online Feb 27, 2018. doi: 10.4254/wjh.v10.i2.347
Peer-review started: November 1, 2017
First decision: December 1, 2017
Revised: December 20, 2017
Accepted: January 23, 2018
Article in press: January 24, 2018
Published online: February 27, 2018
A 37-year-old male with a past medical history of Epstein-Barr Virus infection reported having jaundice, right upper quadrant pain, and decreased appetite and weight loss of 10 lbs over the past two months.
Abdominal magnetic resonance imaging showed mild intrahepatic ductal dilatation, peripheral areas of arterial enhancement in the liver felt to be related to vascular shunting, periportal edema, a cut off in the course of biliary tree at the bifurcation, simple liver cyst, and enlarged left retroperitoneal nodes.
Cirrhosis, hepatocellular carcinoma, cholangiocarcinoma.
Laboratory was significant for total bilirubin of 5.7 mg/dL, alkaline phosphatase of 1005 U/L, albumin of 3.2 g/dL, and AST/ALT of 257/239 U/L.
Upper endoscopy showed gastropathy in the gastric fundus and body. Endoscopic ultrasound was unremarkable.
A liver biopsy showed several large dense clusters of atypical T-lymphocytes, which appeared to be centered in portal areas. The atypical lymphocytes were medium to large in size and were CD2+, CD3+, CD5+, CD7-, CD4+, CD8-, CD56-, CD57-, CD30+, by immunohistochemistry. The proliferation index was approximately 70% by labeling for ki67/mib1. Labeling for CD68 was seen in Kupffer cells, and in a few scattered histiocytes only. There were rare, scattered, unremarkable small B-lymphocytes (CD20+, CD79a+). Stains for CD138, kappa, lambda, were noncontributory. The above histological profile was consistent with hepatic peripheral T-cell lymphoma (H-PTCL).
The patient was transferred to a tertiary center for chemotherapy (CHOP) treatment.
H-PTCL has a poor prognosis due to life threatening complications and tumor progression. Clinical studies reports that CHOP therapy can provide up to 60% complete remission, and a 30%-50% five-year survival rate.
H-PTCL is one of the rarest forms of non-Hodgkin lymphoma. It constitutes approximately 0.0016% of all extranodal lymphomas.
In the setting of worsening symptoms and abnormal liver enzymes of unknown etiology, clinicians should consider performing a differential liver biopsy. Clinicians should also be aware of the risk factors for H-PTCL.