presented 11/05/2016 at the Omni Hotel in Houston, TX, representing Internal Medicine department at TTUHSC-Amarillo

1. Marginal zone lymphoma initially presenting as
NASH-induced cirrhosis
Debdeep Banerjee A.B., ASM Islam M.D.
Department of Internal Medicine, Texas Tech University Health Sciences Center, Amarillo
Case Presentation
Marginal zone lymphoma (MZL) is an uncommon and typically
indolent subtype of B-cell lymphomas. The prevalence of the
marginal zone subtype is estimated to be roughly 5-17% of all
Non-Hodgkins Lymphomas. Although the median survival usually
spans >10 years, patients may be asymptomatic for long
durations before acutely deteriorating.
The spleen is one of the most common origins of MZL, with a
characteristic tumor marker profile of CD19/20/22 (+). This
alongside a bone marrow biopsy are considered diagnostic.
Extralymphatic sites of spread, such as the liver, may indicate
advanced disease.
If caught prior to acute symptomatic deterioration, splenectomy
and induction of chemotherapy with the anti-CD20 rituximab have
been deemed appropriate treatment.1-2
Introduction
This case demonstrates the importance of extranodal involvement in
marginal zone Non-Hodgkins Lymphoma. Its presentation can initially
confound diagnosis and lead workup specific for the affected organ
systems. Important highlights of this case include pancytopenia
secondary to splenic sequestration and cirrhosis stigmata secondary
to decompensated status of lymphoma. Relatively uncommon, MZL
usually has an indolent course. However, bone marrow biopsy in this
patient became significant for malignancy within only 8 months.
Perhaps most remarkable is the difficult clinical appearance of MZL,
such that diagnosis is often significantly delayed. MZL has been
further classified into 3 entities, based on the location from which it
originally derives from – including extranodal MZL, nodal MZL, and
splenic MZL.3 Increasingly, the liver has been recognized as a
distinctly local lymphoid organ. Hepatic cells are constantly exposed
to antigens and hematogenous introduction, via splanchnic
circulation, to gut microbes.4 Thus, the spread of lymphoma to
lymphoid organs, such as the liver, may present as clinically specific
for the target organ. Furthermore, the absence of regional or
peripheral lymphadenopathy may additionally confound diagnosis.
In addition, a faulty quick diagnosis of NASH-induced cirrhosis, in the
absence of alternative etiologies of cirrhosis, may steer the clinician in
an expensive and altogether unnecessary path. Ultrasound or
magnetic resonance imaging (MRI) are effective tools to gauge the
degree of hepatic steatosis, if hepatic biopsy is unable to be
performed secondary to the risk of major bleeding with
thrombocytopenia.5
Discussion
Prompt recognition of MZL can help avoid unnecessary
workup for confounding presentations in affected organs
and initiate treatment to help halt condition
deterioration.
Conclusions
References
1. Joshi M., Sheikh H., Abbi K., et. al. (2012). Marginal zone lymphoma: old,
new, targeted, and epigenetic therapies. Therapeutic Advances in
Hematology, 3(5), 275–290.
2. McDermott RS., Maher MM., Dunleavy K., et. al. (2002) Unusual
presentations of lymphoma. Journal of Clinical Oncology, 20(7), 1943-1946.
3. Zinzani PL. (2012) The many faces of marginal zone lymphoma. ASH
Education Book, 2012(1), 426-432.
4. Crispe IN. (2009) The liver as a lymphoid organ. Annual Review of
Immunology 2009(27), 147-163.
5. Lee SS., Park SH. (2014). Radiologic evaluation of nonalcoholic fatty liver
disease. World Journal of Gastroenterology, 20(23), 7392–7402.
A 63-year-old female with past medical history of hyperlipidemia,
diabetes, hypertension, and pancytopenia presented to her first
emergency room (ER) visit with worsening right upper quadrant
abdominal pain. Computed tomography (CT) scan at the time revealed
hepatosplenomegaly and stigmata concerning for cirrhosis, including
portal hypertension, esophageal varices, and intrahepatic biliary dilatation.
Coagulation studies and hepatitis panel were normal; however, complete
blood count revealed pancytopenia. History and physical exam were not
significant for bleeding episodes.
Gastroenterology was consulted, and her cirrhosis was believed to be
secondary to nonalcoholic fatty liver disease. Beta-blockers were initiated
for treatment of her portal hypertension. Percutaneous liver biopsy was
unable to be performed due to thrombocytopenia of <60K/ml. After pain
improvement, she was discharged and scheduled for bone marrow biopsy,
which demonstrated hypercellular marrow with no signs of malignancy.
8 months after initial discharge, the patient returned to the emergency
room with left upper quadrant abdominal distension with pain. Computed
tomography demonstrated significantly worsened splenomegaly with
consistent hepatomegaly from prior admission. Transjugular liver biopsy
was performed revealing cirrhosis with interface hepatitis and B-cell
marker infiltrates. A second bone marrow biopsy with flow cytometry was
performed and confirmed marginal zone Non-Hodgkins Lymphoma (NHL).
Treatment with rituximab, uric acid reducers, and neutropenic precautions
were implemented. The patient was discharged and portacath placement
was performed as an outpatient. Treatment was well-tolerated until 9 days
after discharge. The patient returned to the ER with altered mental status,
severe shock secondary to multiorgan failure and subsequently passed
away.
Figure 1. Axial CT on initial presentation revealing intrahepatic biliary dilation, portal hypertension,
and significant splenomegaly. Subsequent bone marrow biopsy revealed no malignancy.
Figure 2. Axial CT 8 months following initial admission revealing massive hepatosplenomegaly.
Subsequent liver and bone marrow biopsies confirmed marginal zone NHL.