5. Subtypes

in treatment decision. Another study showed that the incidence of bone marrow involvement was 3.6% in 192 patients with stage I and II DLBCL [52]. Echocardiogram or multigated acquisition (MUGA) scan if required if anthracycline-based regimen is being considered. In selected cases discussion of fertility issues and sperm banking should be considered. Consider lumbar puncture if there is suspicion of CNS disease or if patient is at high risk of CNS involvement such in patients with testicular lymphoma, aggressive histology, human immunodeficiency virus (HIV) lymphoma, double-expressor lymphoma and in patients with 4–6 factors on the prognostic score which is discussed in the section on CNS prophylaxis. The role of various

Ann Arbor staging system was originally introduced for Hodgkin's lymphoma and was later adopted for NHL. Lister et al. classified patients with NHL into stages I (localized) to IV (extensive) disease [54]. Patients are classified into A or B depending on the absence or presence of B symptoms, respectively. B symptoms mainly include fevers, drenching night sweats or weight loss of 10% or more within 6 months of diagnosis. DLBCL is a noncontiguous disease while Hodgkin's lymphoma involves contiguous sites hence the Ann Arbor staging has limited utility in DLBCL. In 2014, the Lugano classification was proposed (Table 1) [55]. According to this classification, patients with stage I or II disease can be grouped and considered as having limited disease while patients with Ann Arbor stage III or IV disease can be grouped as advanced stage disease. The suffix A and B is only reserved for Hodgkin's lymphoma. The X for bulky disease is now replaced with the recording of the largest nodal diameter by CT scan. Limited evidence suggests that 6–10 cm should be considered as bulky disease in the rituximab era for DLBCL. National comprehensive cancer network (NCCN)

imaging modalities has been reviewed in detail elsewhere [53].

50 Hematology - Latest Research and Clinical Advances

Stage Involvement Extranodal (E) status

II as above with "bulky" disease Not applicable

IV Wide spread extralymphatic involvement Not applicable

II Two or more lymph nodal groups on the same side of the

III Lymph nodes on both sides of the diaphragm or lymph nodes above the diaphragm with splenic involvement

Table 1. Revised staging system for primary nodal lymphomas.

I Single lymph node or a group of adjacent nodes One extranodal lesion without nodal

Note: Extent of disease in DLBCL is determined by PET-CT or CECT if former is not available. Tonsils, Waldeyer's ring,

The decision to treat stage II bulky disease as limited or advanced disease is determined by the histology and IPI.

involvement

Not applicable

Stage I or II by nodal involvement with limited

contiguous extranodal extension

3.5. Staging

Limited

II bulky1

1

Advanced

diaphragm

and spleen are considered nodal tissue.

Several distinct subtypes of large B cell lymphoma need to be distinguished from DLBCL, NOS (see sec 2.6 above) [8].

