Guideline sources The following summarized guidelines for the evaluation and management of marginal zone lymphoma (MZL) are prepared by our editorial team based on guidelines from the European Society of Medical Oncology (ESMO 2020). 1 Calculator Calculator Calculator Eastern Cooperative Oncology G Karnofsky performance status s Modified A Guidelines 1. Screening and diagnosis Diagnostic criteria: use the current 2017 WHO classification for the diagnosis of MZL. B Show 3 more https://web.pathway.md/diseases/rec6ZhhQB9RhMDTR0 1/4 6/30/23, 1:00 AM Marginal zone lymphoma Pathway 2. Classification and risk stratification Prognosis: use clinical and biological prognostic systems (HPLL, MALT-IPI) in clinical routine to estimate clinical behavior. B 3. Diagnostic investigations Initial evaluation: Elicit history, perform a physical examination, and obtain the following laboratory tests in the initial staging of all MZL subtypes: CBC and differential LFTs renal function tests protein electrophoresis LDH -2 microglobulin serum and urine immunofixation serology for HBV, HCV, human immunodeficiency virus, and cryoglobulins and cryocrit if HCV- positive. Imaging for staging: Obtain complete chest and abdominal CT (or MRI) and imaging of the orbits and salivary glands in patients with MZL. B Consider obtaining positron emission tomography when clinical and/or laboratory findings suggest transformation to high-grade histology and to guide lymph node biopsy. C 4. Diagnostic procedures Biopsy and histopathology: perform adequate tumor biopsy for diagnosis of MZL. B Show 2 more 5. Medical management Management of nodal MZL: follow the principles of therapy for follicular lymphoma. B Show 2 more Management of splenic MZL: monitor asymptomatic patients with splenic MZL being managed with the watch-and-wait approach by performing a physical examination and obtaining imaging as clinically required, blood counts, and biochemistry every 6 months. E Show 7 more Management of extranodal MZL: initiate H. pylori eradication therapy in all patients with gastric MZL, irrespective of stage. B https://web.pathway.md/diseases/rec6ZhhQB9RhMDTR0 2/4 6/30/23, 1:00 AM Marginal zone lymphoma Pathway Show 8 more 6. Follow-up and surveillance Follow-up: monitor asymptomatic patients with disseminated MZL (splenic MZL and nodal MZL) being managed with the watch-and-wait approach by performing a physical examination and obtaining imaging as clinically required, blood counts, and biochemistry every 6 months. E Show 3 more Clinical findings Patient demographics Symptoms Elderly Abdominal discomfort Abdominal pain Past medical history Diarrhea Epigastric pain Acquired vWD Fatigue Antiphospholipid syndrome Fever Autoimmune hemolytic anemia LUQ pain GERD Loss of appetite Gastrointestinal bleeding Malabsorption H. pylori infection Night sweats HBV infection Shortness of breath HCV infection Watery eyes Human immunodeficiency virus infection Weight loss Immune thrombocytopenia Abdominal exam SLE Sj gren's disease Splenomegaly Lymphatic exam Lab findings Lymphadenopathy serum LDH serum transaminases Hematological findings serum -2 microglobulin Anemia Imaging findings Cold agglutinins blood platelet count Pulmonary consolidation Pulmonary nodules References https://web.pathway.md/diseases/rec6ZhhQB9RhMDTR0 3/4 6/30/23, 1:00 AM Marginal zone lymphoma Pathway 1. E Zucca, L Arcaini, C Buske et al. Marginal zone lymphomas: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2020 Jan;31 1 17 29. Open https://web.pathway.md/diseases/rec6ZhhQB9RhMDTR0 4/4