1、霍奇金淋巴瘤指南解读程淑琴（WHO）HL 分为两种类型 淋巴细胞为主型霍奇金淋巴瘤（LPHL）经典的霍奇金淋巴瘤（CHL）CHL 分为4 种亚型：结节硬化型CHL（NSCHL）混合细胞型CHL（MCCHL）淋巴细胞消减型CHL（LDCHL）富淋巴细胞型CHL（LRCHL）。LPHL 占HL 病例的5%，CHL 占95%。病 理CHL 的特点是在炎性背景下存在R-S 细胞，LPHL 缺乏R-S 细胞，但存在肿瘤性淋巴细胞，有时称为爆米花细胞LPHL 表现为结节性或弥漫性形式结节性亚型：表现为大量B 淋巴细胞中出现肿瘤性淋巴细胞弥漫性亚型：背景细胞主要为T 细胞。免疫组化CHL：R-S 细胞大多表
2、达CD15和CD30，而CD3 和CD45 常常阴性 40%以下的病人可检测到CD20 推荐CD3CD15CD20、CD30 和CD45LPHL：CD45+和CD20+，不表达CD15，极少表达CD30 指南推荐CD3、CD15CD20CD21、CD30 和CD57 染色经典的霍奇金淋巴瘤在初始诊断和检查后，病人分为下列几组：I-II 期 III-IV 期I-II 期的病人，根据是否存在不利因素，进一步分为下列亚组：IA-IIA 期（有利）I-II 期（不利伴巨块型疾病）I-II 期（不利伴非巨块型疾病）Staging and PrognosisEach stageis subdivided
3、into A and B categories.“A”indicates that no systemic symptoms are present and“B”is assigned to patients with unexplained weight loss of 10%of their body weight,unexplained fevers,ordrenching night sweats.Patients with HL are usually classified into 3groups:early-stage favorable(stage I-II with no u
4、nfavorable factors);early-stage unfavorable(stage I-II with any of the unfavorable factors such as largemediastinal adenopathy;23 nodal sites of disease;B symptoms;extranodal involvement;or significantly elevated erythrocyte sedimentation rate ESR 50)advanced-stage disease(stageIII-IV).FOLLOW-UP AFT
5、ER COMPLETION OF TREATMENT AND MONITORING FOR LATE EFFECTSCR should be documented including reversion of PET to negative within 3 months following completion of therapy.It is recommended that the patient be provided with a treatment summary at the completion of his/her therapy,including details of r
6、adiation therapy,organsat risk,and cumulative anthracycline dosage given.Follow-up with an oncologist is recommended,especially during the first 5 years after treatment to detect recurrence,and then annually due to the risk oflate complications including second cancers and cardiovascular disease.kk,
7、ll Late relapse or transformation to large cell lymphoma may occur in NLPHL.The frequency and types of tests may vary depending on clinical circumstances:age and stage at diagnosis,social habits,treatment modality,etc.There arefew data to support specific recommendations;these represent the range of
8、 practice at NCCN Member Institutions.Follow-up After Completion of Treatment up to 5 YearsInterim H&P:Every 36 mo for 12 y,then every 612 mo until year 3,then annually Annual influenza vaccine Laboratory studies:CBC,platelets,ESR(if elevated at time of initial diagnosis),chemistry profile asclinica
9、lly indicatedThyroid-stimulating hormone(TSH)at least annually if RT to neck.Acceptable to obtain a CT scan at 6,12,and 24 mo following completion oftherapy,or as clinically indicated.PET/CT only if last PET was Deauville 4-5,toconfirm complete response.Counseling:Reproduction,health habits,psychoso
10、cial,cardiovascular,breast self-exam,skin cancer risk,end-oftreatmentdiscussion.Surveillance PET should not be done routinely due to risk forfalse positives.Management decisions should not be based onPET scan alone;clinical or pathologic correlation is neededFollow-up and Monitoring After 5 YearskkI
11、nterim H&P:AnnuallyAnnual blood pressure,aggressive management of cardiovascular risk factorsPneumococcal,meningococcal,and H-flu revaccination after 57 y,ifpatient treated with splenic RT or previous splenectomy(according to CDCrecommendations)Annual influenza vaccine Cardiovascular symptoms may em
12、erge at a young age.Consider stress test/echocardiogram at 10-y intervals after treatment iscompleted.Consider carotid ultrasound at 10-y intervals if neck irradiation.Laboratory studies:CBC,platelets,chemistry profile annuallyTSH at least annually if RT to neckBiannual lipidsAnnual fasting glucoseF
13、ollow-up and Monitoring After 5 YearskkConsider low-dose chest CT for patients at increased risk for lungcancer.mm Annual breast screening:Initiate 810 y post-therapy,or at age 40,whichever comes first,if chest or axillary radiation.The NCCN HodgkinLymphoma Guidelines Panel recommends breast MRI in
14、additionto mammography for women who received irradiation to the chestbetween ages 1030 y,which is consistent with the American CancerSociety(ACS)Guidelines.Consider referral to a breast specialist.Colonoscopy every 10 years for patients age 50,if high risk begin atage 40,which is consistent with AC
15、S Guidelines.Counseling:Reproduction,health habits,psychosocial,cardiovascular,breast self-exam,and skin cancer risk.Treatment summary and consideration of transfer to PCP.Consider a referral to a survivorship clinic.PRINCIPLES OF SYSTEMIC THERAPY(1 of 2)Classical Hodgkin Lymphoma The most common va
16、riants of chemotherapy used at NCCN Member Institutions include ABVD and Stanford V.Routine use of growth factors is not recommended.Leukopenia is not a factor for delay of treatment or reduction of dose intensity(except for escalated BEACOPP).Regimens ABVD(doxorubicin,bleomycin,vinblastine,and daca
17、rbazine)ISRTStanford V(doxorubicin,vinblastine,mechlorethamine,etoposide,vincristine,bleomycin,and prednisone)*Escalated BEACOPP(bleomycin,etoposide,doxorubicin,cyclophosphamide,vincristine,procarbazine,and prednisone)Escalated BEACOPP followed by ABVD with ISRTRegimens Nodular Lymphocyte-Predominan
18、t Hodgkin Lymphoma*The most common chemotherapies used at NCCN Member Institutions for NLPHL are listed below.ABVD(doxorubicin,bleomycin,vinblastine,dacarbazine)rituximabCHOP(cyclophosphamide,doxorubicin,vincristine,prednisone)rituximabCVP(cyclophosphamide,vinblastine,prednisolone)rituximabRituximab
19、Involved-site Radiation Therapy(ISRT)Dose:Combined Modality TherapyNon-bulky disease(stage I-II):20*30 Gy(if treated with ABVD),30 Gy(if treated with Stanford V)Non-bulky disease(stage IB-IIB):30 GyBulky disease sites(all stages):3036 GyPET scan Deauville 3-4 following chemotherapy:3045 Gy ISRT Alon
20、e(uncommon,except for NLPHL):Involved regions:3036 Gy(the dose of 30 Gy is mainly used for NLPHL)Uninvolved regions:2530 Gy PRINCIPLES OF SYSTEMIC THERAPY FOR RELAPSED OR REFRACTORY DISEASE(1 OF 2)Regimens(listed in alphabetical order Brentuximab vedotin(only for CHL)1 C-MOPP(cyclophosphamide,vincri
21、stine,procarbazine,prednisone)(category 2B)DHAP(dexamethasone,cisplatin,high-dose cytarabine)2,3 ESHAP(etoposide,methylprednisolone,high-dose cytarabine and cisplatin)4,5,6 GCD(gemcitabine,carboplatin,dexamethasone)7,8 GVD(gemcitabine,vinorelbine,liposomal doxorubicin)9 ICE(ifosfamide,carboplatin,et
22、oposide)10,11 IGEV(ifosfamide,gemcitabine,vinorelbine)12 MINE(etoposide,ifosfamide,mesna,mitoxantrone)13 Mini-BEAM(carmustine,cytarabine,etoposide,melphalan)14,15PRINCIPLES OF SYSTEMIC THERAPY FOR RELAPSED OR REFRACTORY DISEASE(1 OF 2)Regimens(listed in alphabetical orderAdditional Therapy Options*(only for CHL)(listed in alphabetical order):Bendamustine16 Everolimus17 Lenalidomide18 Nivolumab19,20 Pembrolizumab21
