Table 1.

Initial assessment of MZLs

MZL subtype and anatomical siteStaging
All MZLs Familial and individual history
Physical examination with PS and B symptoms
Lab: complete blood and differential counts, lactate dehydrogenase, beta-2-microglobulin, renal and liver function, protein electrophoresis, serum and urine immunofixation
Viral serologies: complete HBV markers, HIV and HCV serologies (if HCV positive, HCV-RNA and genotype; cryoglobulins and cryocrit)
Direct Coombs' test (if clinically indicated, especially in SMZL)
Bone marrow biopsy in SMZL and NMZL (recommended in EMZL, particularly nongastric, if cytopenias are present or only local treatment is planned)
CT of the neck, chest, abdomen, and pelvis
MRI in specific situations
PET/CT when only local treatment is planned and to identify biopsy site if clinical or laboratory data suggest histologic transformation 
NMZL Flow cytometry (bone marrow and peripheral blood) 
SMZL Flow cytometry (bone marrow and peripheral blood) 
Stomach Esophagogastroduodenoscopy
Endoscopic ultrasonography (optional)
Helicobacter pylori status by IHC (mandatory) (if IHC negative fecal antigen or breath test and serology studies)
FISH or PCR assay for the t(11;18) translocation (optional) 
Small intestine Campylobacter jejuni status (PCR, IHC, or ISH) 
Colon Colonoscopy
Esophagogastroduodenoscopy 
Salivary glands MRI of head and neck
ENT examination and ultrasonography
Esophagogastroduodenoscopy
anti-SSA/Ro and anti-SSB/La antibodies 
Ocular adnexa Head and neck and orbit imaging
Ophthalmologic examination
Chlamydophila psittaci status (PCR) in the tumor biopsy, conjunctival swab, and PBMCs (optional, geographic variability)
anti-SSA/Ro and anti-SSB/La antibodies (lacrimal gland) 
Lung Bronchoscopy and bronchoalveolar lavage
Esophagogastroduodenoscopy (optional) 
Breast Mammography
Breast ultrasonography
MRI (or CT scan) of breast 
Thyroid Thyroid ultrasonography
Thyroid function tests 
Central nervous system (dura mater) MRI of brain 
Skin Borrelia burgdorferi status (PCR) in the tumor biopsy (optional) 
MZL subtype and anatomical siteStaging
All MZLs Familial and individual history
Physical examination with PS and B symptoms
Lab: complete blood and differential counts, lactate dehydrogenase, beta-2-microglobulin, renal and liver function, protein electrophoresis, serum and urine immunofixation
Viral serologies: complete HBV markers, HIV and HCV serologies (if HCV positive, HCV-RNA and genotype; cryoglobulins and cryocrit)
Direct Coombs' test (if clinically indicated, especially in SMZL)
Bone marrow biopsy in SMZL and NMZL (recommended in EMZL, particularly nongastric, if cytopenias are present or only local treatment is planned)
CT of the neck, chest, abdomen, and pelvis
MRI in specific situations
PET/CT when only local treatment is planned and to identify biopsy site if clinical or laboratory data suggest histologic transformation 
NMZL Flow cytometry (bone marrow and peripheral blood) 
SMZL Flow cytometry (bone marrow and peripheral blood) 
Stomach Esophagogastroduodenoscopy
Endoscopic ultrasonography (optional)
Helicobacter pylori status by IHC (mandatory) (if IHC negative fecal antigen or breath test and serology studies)
FISH or PCR assay for the t(11;18) translocation (optional) 
Small intestine Campylobacter jejuni status (PCR, IHC, or ISH) 
Colon Colonoscopy
Esophagogastroduodenoscopy 
Salivary glands MRI of head and neck
ENT examination and ultrasonography
Esophagogastroduodenoscopy
anti-SSA/Ro and anti-SSB/La antibodies 
Ocular adnexa Head and neck and orbit imaging
Ophthalmologic examination
Chlamydophila psittaci status (PCR) in the tumor biopsy, conjunctival swab, and PBMCs (optional, geographic variability)
anti-SSA/Ro and anti-SSB/La antibodies (lacrimal gland) 
Lung Bronchoscopy and bronchoalveolar lavage
Esophagogastroduodenoscopy (optional) 
Breast Mammography
Breast ultrasonography
MRI (or CT scan) of breast 
Thyroid Thyroid ultrasonography
Thyroid function tests 
Central nervous system (dura mater) MRI of brain 
Skin Borrelia burgdorferi status (PCR) in the tumor biopsy (optional) 

ENT, ear, nose, and throat; FISH, fluorescence in situ hybridization; IHC, immunohistochemistry; PBMC, peripheral blood mononuclear cells; PS, performance status; SSA, Sjogren's syndrome.

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