JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma-10. Hodgkin lymphoma (HL)
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GUIDELINE
JSH practical guidelines for hematological malignancies, 2018: II. Lymphoma‑10. Hodgkin lymphoma (HL) Hirokazu Nagai1 Received: 18 November 2019 / Accepted: 18 November 2019 © Japanese Society of Hematology 2019
Overview Hodgkin lymphoma (HL) accounts for approximately 30% of all malignant lymphomas in Western countries, and approximately 5–10% in Japan. It has a bimodal incidence pattern across age groups with one peak in young adults (20s) and the other in middle-aged adults (50–60 years old). Many patients are initially asymptomatic or have painless superficial lymphadenopathy. Approximately 75% of cases are detected from cervical and supraclavicular lymphadenopathy. A mediastinal mass is detected in approximately 60% of patients with nodular sclerosis HL. Hematologic findings include leukocytosis, lymphopenia, eosinophilia, anemia, elevated alkaline phosphatase, elevated erythrocyte sedimentation rate, elevated CRP, and reduced cell-mediated immunity. Histopathologically, HL is a lymphoma characterized by proliferation of tumor cells such as Hodgkin/Reed–Sternberg (HRS) cells and lymphocyte predominant (LP) cells (“popcorn” cells). In 2017 WHO Classification, HL is classified into two subtypes: nodular lymphocyte-predominant Hodgkin lymphoma (NLPHL) and classical Hodgkin lymphoma (CHL) [1, 2]. HRS cells are characteristic of CHL, and LP cells of NLPHL. CHL is further classified into four additional subtypes: nodular sclerosis, lymphocyte-rich classical, mixed cellularity, and lymphocyte-depleted Hodgkin lymphomas.
HL is classified into stages I through IV using the Ann Arbor staging system. 1. Prognostic factors in limited-stage CHL Table 1 shows the prognostic factors in limited-stage (stages I and II) CHL. It should be noted that different research groups emphasize different prognostic factors [3–5]. 2. Prognostic factors in advanced-stage CHL The International Prognostic Score (IPS) is used as a prognostic model for advanced-stage CHL (stages III and IV) [6]. It is based on seven prognostic factors identified using time to progression (TTP) as an endpoint in an analysis of 4695 patients aged 15–65 years with advanced-stage CHL treated with a regimen such as the MOPP regimen [mechlorethamine (not approved in Japan), vincristine, procarbazine, and prednisolone] or ABVD regimen (doxorubicin, bleomycin, vinblastine, and dacarbazine). It is considered possible to predict TTP from the number of these factors a patient has. In this system, the projected five-year rate of freedom from progression of disease is 84% for a patient with no unfavorable prognostic factors, compared with a poor 42% for patients with 5 or more factors.
* Hirokazu Nagai [email protected] 1
Clinical Research Center, National Hospital Organization Nagoya Medical Center, 4‑1‑1 Sannomaru, Naka‑ku, Nagoya, Aichi 460‑0001, Japan
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H. Nagai Table 1 Prognostic factors in limited-stage CHL identified by various research groups Research group
GHSG
EORTC
NCIC/ECOG
NCCN2011
Favorable disease
Stage
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