Nodal Marginal Zone Lymphoma is a highly rare form of Non-Hodgkin lymphoma, usually evolving a benign course. These indolent lymphomas, if untreated, can undergo transformation to clinically aggressive tumors. Here, we describe an interesting case of transforming marginal zone lymphoma presenting with worsening ascites in a patient with known alcoholic cirrhosis, causing a diagnostic and therapeutic challenge.
A 72-year-old gentleman with past medical history of hepatic cirrhosis, presented with worsening dyspnea, increasing abdominal ascites and discomfort. The patient had therapeutic paracentesis performed the day before the admission. Physical exam on arrival was consistent with abdominal distention with fluid thrill, and bilateral painless cervical lymphadenopathy. Initial labs at admission included leukocytosis with white blood cell (WBC) count of count of 16.9K/mm3 (Neutrophils 53%; Lymphocytes 20%). He was started on antibiotic therapy in consideration of spontaneous bacterial peritonitis (SBP) and diagnostic and therapeutic ascitic tap was performed. The ascitic fluid analysis was negative for SBP, and serum albumin ascetic (SAAG) gradient was 1.8. The WBC count, however, failed to improve and worsened to 20.9k/mm3 with worsening lymphocytosis. Blood peripheral smear revealed atypical lymphocytes. Flow Cytometry of both the blood and peritoneal fluid was concerning for malignant lymphoma. A computed tomography (CT) of the chest, abdomen, and pelvis revealed extensive cervical and iliac lymphadenopathy. Fine needle aspiration biopsy (FNAB) of the cervical lymph node of the neck was done, reporting marginal zone lymphoma. Further molecular markers revealed the diagnosis of marginal zone B-cell lymphoma (nodal). Fluorescence in-situ hybridization (FISH) studies were significant for a high-grade transformation of lymphoma. The patient required a frequent ascitic tap, resulting in placement of a pleur-X catheter for paracentesis. The disease process, prognosis, and therapeutic options with anticipated adverse effects were explained to the patient. He elected to proceed with supportive care and hospice.
Nodal Marginal Zone lymphoma (NMZL) is extremely rare and presents with painless adenopathy. It is a diagnosis of exclusion due to the absence of any positive immunologic and histologic markers.4 There are no treatment guidelines for NMZL, however, guideline directed therapy similar to follicular lymphoma may be appropriate.
Carr et al present a case of splenic marginal zone lymphoma that was masquerading as cirrhotic hypersplenism.1In our case the prior history of alcoholic cirrhosis masked and delayed the lymphoma diagnosis. The ascites was initially attributed to the hepatic cirrhosis however, worsening ascites lead to further workup, and yielding the diagnosis. To our best of knowledge, there are no prior reports of ascites secondary to NMZL in literature. Physicians should consider the diagnosis in resistant ascites in a patient with lymphadenopathy. It is imperative to adopt a multidisciplinary approach involving histopathologist, oncologist, and clinicians for best possible outcomes.