Introduction: Lymphoma is the most common blood cancer. The two main forms of lymphoma are Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL) Marginal zone lymphomas (MZLs) are a group of indolent (slow- growing) B-cell NHLs, which account for approximately eight percent of all NHL cases. The average age at diagnosis is 60 years. NHL is slightly more common in men. There are 3 types of marginal zone lymphomas: extranodal of mucosa-associated lymphoid tissue,the splenic MZL, and the nodal MZL. Despite being the third most common indolent non-Hodgkin’s lymphoma (iNHL), marginal zone lymphoma (MZL) remains largely understudied, and given its underlying disease heterogeneity, it is challenging to define a single treatment approach for these patients. Many cases have been reported with ischemic stroke associated with different types of NHL but Marginal zone lymphoma has not been reported so far.

Case report: Patient is 64-year-old male with known history of pulmonary embolism in 2019 as well as chronic DVT in left lower extremity, atrial fibrillation, decompensated liver cirrhosis, chronic thrombocytopenia who presented to our emergency room with right-sided weakness and facial droop. During this admission he had an MRI of the brain showed Left MCA ischemic stroke, echocardiogram ruled out cardiac embolus and showed left ventricular ejection fraction of 25 to 30% with moderate to severe right ventricular dysfunction. Patient had persistent leukocytosis and thrombocytopenia of 91 and trending down to 64. Patient had an abdominal ultrasound which showed cirrhosis with splenomegaly and very small amount of ascites with gallbladder wall thickening and multiple gallstones. EGD showed normal esophagus, portal hypertension gastropathy, nonbleeding erosive gastropathy and no stigmata of recent bleeding. Patient denies any B symptoms, does not have any lymph nodes on physical examination. Heme oncology was consulted for his persistent leukocytosis and thrombocytopenia. He had absolute lymphocyte count of 12,000 patient and peripheral smear reviewed by pathology showed some atypical reactive appearing lymphocytes along with smudge cells, anemia, thrombocytopenia, leukocytosis with left shift. Flow cytometry showed CD20 plus B cells co-express lambda light chain and partial CD11c and negative for CD5 and CD10, comprising 88% of lymphocytes and approximately 76% of all analyzed white blood cells. It showed diagnostic considerations which include marginal zone lymphoma and lymphoplasmacytic lymphoma. Patient has been discharged for an outpatient follow-up with heme-oncology for further work-up.

Conclusion: Our case attempts to further highlight the importance of unusual initial presentation of lymphoma in a patient who presented with acute stroke and did not have any B symptoms on presentation and how a detailed workup for mild leukocytosis and thrombocytopenia and not assuming it to be secondary to liver cirrhosis helped establish the diagnosis.