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1218 Part VII Hematologic Malignancies

TABLE Prognostic Factors in Early and Advanced-Stage Hodgkin Lymphoma
75.3
Prognostic Group EORTC GHSG NCCN
Early-favorable CS I-II without risk factors (supra-diaphragmatic) CS I-II without risk factors CS IA-IIA without risk factors
Early-unfavorable CS I-II with ≥1 risk factor (supra-diaphragmatic) CS I-IIA with ≥1 risk factor C/D but not A/B CS I-II with ≥1 risk factor
(Intermediate)
Advanced CS III-IV CS IIB with risk factors A/B CS III-IV
CS III/IV
Prognostic factors (A) Bulky mediastinal mass a (A) Bulky mediastinal mass a (A) Bulky mediastinal mass a
(B) Age ≥50 years (B) Extranodal disease (>1 lesion) (B) Bulk >10 cm
(C) Elevated ESR (>50 mm/h without B (C) Elevated ESR (>50 mm/h without B (C) Elevated ESR (>50 mm/h
b
symptoms; >30 mm/h with B symptoms ) symptoms; >30 mm/h with B symptoms ) without B symptoms)
b
(D) ≥4 nodal areas (out of 5 supra-diaphragmatic (D) ≥3 nodal areas (out of 11 GHSG areas) (D) B symptoms
EORTC areas) (E) ≥4 nodal areas (out of
17 Ann Arbor regions)
a Bulky mediastinal mass: ratio ≥0.035 of the maximum horizontal chest diameter (EORTC); ratio ≥1/3 of the maximum horizontal chest diameter (GHSG); ratio >1/3 of
the maximum horizontal chest diameter (NCCN).
b B symptoms: night-sweats, fever, weight loss (unexplained, >10% over 6 months).
CS, Clinical stage; EORTC, European Organization for Research and Treatment of Cancer; ESR, estimated sedimentation rate; GHSG, German Hodgkin Study Group;
NCCN, National Comprehensive Cancer Network.

TABLE International Prognostic Score (IPS) for Advanced TABLE Standard Treatment Approach According to Prognostic
75.4 Hodgkin Lymphoma 75.5 Group
No of Prognostic Factors % of patients 5-year FFP (%) 5-year OS (%) Early-favorable HL Combined modality therapy
• 2−4 cycles of chemotherapy followed by
0−1 (low-risk) 29 79 90
involved-field radiotherapy
2−3 (intermediate-risk) 52 64 80
Early-unfavorable HL Combined modality therapy
4−7 (high-risk) 19 47 59 (intermediate-stage) • 4−6 cycles of chemotherapy followed by
FFP, Freedom from progression; OS, overall survival. involved-field radiotherapy
Advanced HL Extensive chemotherapy
• 6−8 cycles of chemotherapy ±
consolidation with localized radiotherapy
3
representing an FFP of 40%. Three risk groups were established as
a result, allowing therapy to be chosen according to these specific
clinical characteristics, with the consensus being that higher risk
patients should receive more intensive therapy (Table 75.4).
TREATMENT OF EARLY-STAGE HODGKIN LYMPHOMA
Treatment According to Prognostic Group Early-Stage Nodular Lymphocyte-Predominant
Hodgkin Lymphoma
The stratification of patients with newly diagnosed HL into early-
favorable, early-unfavorable, or advanced prognostic groups, has Traditionally, patients with NLPHL present with localized, early-
allowed initial treatment of these individuals to be risk-adapted. The stage peripheral lymphadenopathy, and are less likely to present with
treatment approach for those with early-favorable disease typically B symptoms, bulky disease, or mediastinal involvement. The behavior
involves combined modality therapy, with two to four cycles of of this disease entity contrasts favorably to cHL, and its clinical
chemotherapy followed by IFRT. For early-unfavorable disease the behavior is more comparable to that of an indolent non-HL. A ret-
treatment approach is similar, but with four to six cycles of chemo- rospective matched-pair analysis from the GHSG of 394 patients
therapy usually being administered before RT. For advanced disease with NLPHL (compared with 7904 patients with cHL) confirmed
a more aggressive approach is adopted with six to eight cycles of these findings and validated the improved prognosis (tumor control
combination chemotherapy alone, followed by consolidative localized and OS) of this disease entity. In addition, the European Task Force
RT in selected cases (Table 75.5). on Lymphoma (ETFL) conducted an analysis comprising 219
The importance of clinical prognostic factors in predicting patients with histologically confirmed NLPHL. Both analyses revealed
outcome and directing treatment for individuals with newly diag- that B symptoms, bulky/extranodal disease, increased ESR and lactate
nosed HL is well established, and has undoubtedly contributed to dehydrogenase, and involvement of three or more nodal areas were
the huge advances observed in HL over the past two decades. less frequently found in NLPHL than in cHL.
However, despite high cure rates, up to 30% of patients will still For localized asymptomatic nonbulky NLPHL, ISRT alone is the
relapse. Furthermore, a significant proportion of those who are cured preferred treatment. For the rare patient with localized NLPHL who
of their disease will go on to develop serious complications of treat- presents with B symptoms or bulky disease, ISRT remains a consoli-
ment later on in life. Therefore the identification of additional, and dation treatment after initial rituximab and/or chemotherapy.
more specific, biologic markers is needed to better discriminate these
individuals according to their unique risk profiles, with the subse-
quent delivery of therapy that is personalized. Response-adapted Early-Stage Classic Hodgkin Lymphoma
tailoring of treatment with PET (discussed later) and gene expression
profiling of primary tumor tissue are two approaches currently being Recent strategies for the management of early-stage cHL have con-
investigated for this role. tinued to focus on optimizing treatment by minimizing the extent of