P1873 - A Rare Case of Liver Failure From Primary Hepatic Peripheral T Cell Lymphoma

Agnieszka M. Maniak, MD, Jeremy Van, DO, Mary M. Biglin, MD, Sheila Eswaran, MD, MS
Rush University Medical Center, Chicago, IL

Introduction: Peripheral T cell lymphoma (PTCL) is a rare condition, typically involving the lymph nodes, spleen and bone marrow. Primary hepatic PTCL not otherwise specified (NOS) is even more uncommon. It is diagnostically challenging given the wide spectrum of presentations and low incidence. We report an atypical case of liver failure from primary hepatic PTCL-NOS.

Case Description/Methods: A 65-year-old female with well controlled autoimmune hepatitis (AIH) cirrhosis on tacrolimus presented with a neutropenic fever. On presentation, she was febrile to 101.4°F but physical exam was unremarkable. Labs showed platelets of 37 K/uL, ANC 0.78 K/uL, LDH 676 U/L, total bilirubin 1.6 mg/dL, ALP 95 U/L, AST 44 U/L, ALT 11U/L and INR 1.3. Prednisone 40mg daily was started for possible AIH flare after LFT’s rose. Ferritin was >40,000 ng/mL, triglyceride 448 mg/dL and fibrinogen 113 mg/dL. MRI abdomen with and without IV contrast showed cirrhotic morphology but no focal hepatic lesions (Figure 1a). Bone marrow biopsy showed no evidence of HLH or primary lymphoma. The patient developed shock and altered mental status requiring intubation and vasopressor support. LFT’s rose again; ALT 355 U/L, AST 2427 U/L, ALP 316 U/L, total bilirubin 11.1 mg/dL and INR 2. LDH was 5274 U/L, Ammonia 296 ug/dL, and platelets 26 K/uL. Liver biopsy demonstrated PTCL-NOS (Figure 1b). Her clinical status quickly declined, comfort care measures were implemented and she passed away.

Discussion: Only ~12% of hepatic lymphomas have a T cell lineage with 9% of those PTCL-NOS. Our patient had a negative bone marrow biopsy and no mediastinal or abdominopelvic lymphadenopathy on imaging, which further supported a hepatic origin. Primary hepatic PTCL-NOS can present with vague symptoms and focal, diffuse or no hepatic lesions on imaging. On liver biopsy, there are four possible infiltration patterns including sinusoidal predominant, portal predominant with sinusoidal infiltrates, portal and lobular infiltrates with lymphoid aggregates or mass forming infiltrates. Our patient had underlying cirrhosis and significantly elevated LDH which are markers for a poor prognosis. It is imperative to keep hepatic PTCL on the differential and quickly pursue liver biopsy when a patient presents with B-symptoms and elevated liver enzymes with progressive liver dysfunction in order to expedite treatment given its aggressive course.

Li P, Zhang D, Zhou J, et al. Hepatic involvement by T-cell neoplasms: a clinicopathologic study of 40 cases. 2020 Dec. 1-12.


Figure: a) MRI abdomen with and without IV contrast (T2 Coronal Haste view) demonstrating cirrhotic morphology and no focal hepatic lesions
b) Liver biopsy demonstrating portal and lobular infiltrates (black arrow) with lymphoid aggregates


Disclosures:
Agnieszka Maniak indicated no relevant financial relationships.
Jeremy Van indicated no relevant financial relationships.
Mary Biglin indicated no relevant financial relationships.
Sheila Eswaran indicated no relevant financial relationships.


Agnieszka M. Maniak, MD, Jeremy Van, DO, Mary M. Biglin, MD, Sheila Eswaran, MD, MS. P1873 - A Rare Case of Liver Failure From Primary Hepatic Peripheral T Cell Lymphoma, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.