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FRONTIERS IN MEDICAL CASE REPORTS - Volume 1; Issue 1, (Jan-Jun,2020), Cancer Case Reports

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A Rare Case of T- cell Lymphoma Found in the Liver

Author: Neethi Dasu, Kailash Lal, Lucy Joo, Donald McMahon

Category: Cancer Case Reports

Abstract:

Peripheral T-cell lymphomas, a group of non-Hodgkin lymphomas, are rare and aggressive neoplasms which usually portend a poor prognosis. In this case report, we present a 65-year-old male patient with rapidly progressing peripheral T-cell lymphoma found in his liver and spleen. He initially presented with elevated liver enzymes with initial tests failing to reveal a clear etiology. A liver biopsy revealed malignant infiltration with a peripheral T-Cell lymphoma. This case is important for practitioners to keep in mind rarer causes of hepatic dysfunction, as swift and careful evaluations to establish accurate diagnoses and initiate treatment is critical to improving outcomes.

Keywords: T- Cell Lymphoma, Liver Biopsy, Neoplasms

DOI URL: https://dx.doi.org/10.47733/GJCCR.2020.1101

Full Text:

Introduction

Elevation in liver enzymes can be due to a variety of reasons including toxins, drugs, autoimmune process, sepsis, malignant infiltration, alcoholic hepatitis, and viral hepatitis. In this case study, our patient presented with an obstructive pattern of liver test abnormalities. Workup to exclude other causes of liver injury was performed and eventually a liver biopsy was needed. The biopsy revealed a malignant infiltration of peripheral T-cell lymphoma.

Case Report

A 65-year-old male presented with decreased responsiveness, fever and hypotension. His past medical history included cerebrovascular accident at age 21, cerebral aneurysm, seizure disorder, and malignant tumor of the larynx status post chronic tracheostomy. On physical exam, he appeared diaphoretic and difficult to arouse on initial presentation. His abdomen was soft, non-tender, with normal bowel sounds. Superficial excoriations were observed on his sacrum. He was found to have severe sepsis with septic shock. His total bilirubin was 2.2, direct bilirubin of 1.5, alkaline phosphatase 630, ALT 41, AST 66, and a GGTP of 616. A CT scan with oral and IV contrast of his chest, abdomen and pelvis demonstrated bibasilar consolidations and patchy nodules involving the left upper lobe and lower lobes, rectum distended with stool and perirectal edema. An abdominal ultrasound revealed the liver was of normal size and echotexture, with no evidence of cholecystitis.

The patient was admitted to the Intensive Care Unit and started on broad spectrum antibiotics for pneumonia and bacteriuria. An acute hepatitis panel was negative and a repeat abdominal ultrasound showed a borderline diffusely thickened gallbladder wall measuring 3.0 mm - an increase from the 1.5 mm measurement found in the prior study. Questionable common bile duct/common hepatic duct measured 5.3 mm.

The patient admitted to consuming 1-2 drinks of alcohol per day, but his Maddrey's discriminant function was less than 32. The patient's LFTs continued to trend upward in an obstructive pattern with no obvious etiology. A MRCP study was deemed necessary; however, metallic clips in the patient's brain prevented the study from being performed. An ERCP was normal. Hence, it was proposed that the patient's elevated LFTs results were likely secondary to drug-induced liver injury or portal vein thrombosis. However, a review of the patient's medications revealed none that could cause the obstructive pattern of LFTs. A right upper quadrant ultrasound with duplex demonstrated a patent portal vein with normal directional hepatopedal blood flow. The liver was steatotic with mild splenic enlargement noted. The gallbladder wall was mildly thickened and edematous, with trace pericholecystic fluid.

As the patient's LFTs continued to worsen, a liver biopsy was done. Chronic liver serologies showed a positive smooth muscle antibody, positive ANA, nucleolar ANA pattern, and negative IGG. EBV IgG was high. However, he did not meet criteria for Autoimmune Hepatitis.

Eventually, he was found to have MRSA bacteremia and required vasopressor support. A repeat CT scan revealed a hypodense lesion in the inferior right hepatic lobe and splenic enlargement with multiple masses; the overall size of the spleen and the masses increased compared to the initial CT. His bilirubin continued to rise but his AST/ALT levels remained stable. Since he was deemed a poor operative candidate, surgical intervention was not indicated. Eventually, he became diffusely jaundiced and was in hepatic failure. The family elected to have comfort measures initiated and the patient expired the next day.

Discussion

The pathology report of the patient's core biopsy revealed liver showing involvement by a CD30- positive T-cell lymphoma. Hematoxylin- and eosin-stained sections demonstrated periportal involvement by pleomorphic large hyperchromatic cells with prominent nucleoli associated with numerous mitotic figures (Fig. 1 and 2). Immunohistochemical stains revealed positivity with CD3 and CD30 (Fig. 3 and 4). This staining pattern is consistent with involvement of a CD30 peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS); however, an ALK-negative anaplastic T-cell lymphoma could not be excluded (Fig. 5).

Figures 1 and 2. Hematoxylin and Eosin stained sections demonstrate periportal involvement by pleomorphic large hyperchromatic cells with prominent nucleoli associated with numerous mitotic figures. Rare uninvolved portal tracts show granulomatous inflammation with evidence of bile duct destruction.

Figure 3. CD30- stain positive.

Figure 4. CD3- stain positive.

Figure 5. ALK - negative stain

Figure 6. Ki-67- demonstrates a proliferative index on the order of >95% on these cells.

Peripheral T-cell lymphomas are a group of aggressive neoplasms which are grouped under non-Hodgkin lymphomas. The most common subtype is PTCL- NOS, (Campo et al., 2011; Swerdlow et al., 2016). This disease occurs most commonly in males, with a mean age of diagnosis at 60 years. The highest incidence is found among African Americans (Adams et al., 2016; Weisenburger et al., 2011). Histologically, atypical lymphoid cells contain pleomorphic, irregular, vesicular, or hyperchromatic nuclei with prominent nucleoli and display a very high mitotic rate (Campo et al., 2011), which was noted on this patient's liver biopsy findings with a KI-67 stain demonstrating a proliferative index >95% (Fig. 6). The immunophenotype of PTCL-NOS usually demonstrates expression of one or more of the pan-T antigens (i.e. CD2, CD3, CD5, CD7) (Hastrup et al., 1989; Pinkus et al., 1990), as also seen on the liver biopsy.

Anaplastic T-cell lymphomas are neoplasms of mature T lymphocytes which are categorized into two subtypes: those that express anaplastic lymphoma kinase (ALK) fusion proteins versus those that are ALK negative. These Lymphomas are more common among Caucasians, African Americans, Pacific Islanders, and American Indians and have a bimodal age of incidence (Armitage et al., 1998; Medeiros et al., 1991; The Non-Hodgkin's Lymphoma Classification Project., 1997; Abramson et al., 2014). ALK-negative lymphomas generally portend a poorer overall survival rate (Swerdlow et al., 2016; Armitage et al., 1998). Anaplastic large cell lymphoma (ALCL) that is ALK-negative is a different entity from CD30+ PTCL-NOS, but can be difficult to differentiate (Swerdlow et al., 2016). One way to distinguish between these disease processes includes evaluation of immunophenotypic features.

Clinical manifestations of T-cell lymphomas include progressive lymphadenopathy, night sweats, weight loss and fevers (Schlegelberger et al., 1994; Ong et al., 1998; Hartmann et al., 2010). 66% of patients present with extranodal involvement (Schlegelberger et al., 1994; Ong et al., 1998; Hartmann et al., 2010). This patient presented with extranodal involvement as evidenced by the splenic lesions and biopsy-proven liver involvement. The patient's initial CT scan did not reveal any lesions on his liver or spleen, which suggests that this process was fast growing.

Treatment options for newly diagnosed T-cell lymphomas include induction combination with cyclophosphamide, doxorubicin, vincristine and prednisone, with addition of Etoposide if younger than 60 years of age. Other treatment modalities include radiation therapy and/or autologous hematopoietic cell transplantation. Survival rates for the majority of T-cell lymphomas are relatively low, with a current 5-year overall survival rate of 10-30% (Zhang et al., 2016). A number of novel treatment agents have been introduced in recent years, these therapies hold some promise for improving outcomes in patients with PTCL (Zhang et al., 2016).

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