Extranodal Marginal Zone B Cell Lymphoma
Grading / Staging / Report
- While staging is linked to prognosis, it does not generally determine therapy
- Therapy generally determined by clinical signs and symptoms
- Pathologic staging is usually limited to bone marrow biopsy and biopsies of other sites to confirm clinical evidence of involvement
Ann Arbor Staging System
- Stage I
- I if involvement of a single lymph node region
- IE if involvement of a single extralymphatic organ or site
- Stage II
- II if two or more lymph node regions on same side of diaphragm
- IIE if localized involvement of an extralymphatic organ or site and one or more lymph node regions on the same side of the diaphragm
- Stage III
- III if Involvement of lymph node regions on both sides of the diphragm
- IIIS if spleen involved
- IIIE if extralymphatic site involved
- Stage IV
- Diffuse or disseminated involvement of one or more extralymphatic organs or tissues, with or without associated lymph node involvement
- Systemic Symptoms in 6 months preceding admission
- Fever, night sweats, 10% weight loss
- A = absent
- B = present
- Extranodal sites are also designated
- M+ = marrow
- L+ = lung
- H+ = liver
- P+ = pleura
- O+ = bone
- D+ = skin and subcutaneous tissue
- Although originally designed for Hodgkin lymphoma, the Ann Arbor System is also used for non-Hodgkin lymphomas.
The pathology report should contain the following information:
- Diagnosis in the World Health Organization (WHO) classification
- Equivalent diagnosis in other classifications used by relevant clinicians
- Results of supplementary studies if performed
- Relationship to other specimens from the same patient
- Information relevant to staging if available
- Report should address relevant associations such as Helicobacter, Hashimoto disease etc.
- If present, comment on transformation
