1. The Adult Survivor of Hodgkin Disease: Falling Through the Cracks
From the Publishers of
Consult Guys
The Adult Survivor of Hodgkin Disease: Falling Through the Cracks
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3. Yesterday I saw a 63-year-old man as a new patient
Yesterday I saw a 63-year-old man as a new patient. He moved to our town 1 month ago and had an employment physical at his company, and his blood pressure was mildly elevated. He was told to see an internist and saw me.
Guys:
I need your advice.
He has a history of Hodgkin lymphoma diagnosed at age 21 (42 years ago). He recalls that he presented with painless adenopathy. He remembers that he had staging splenectomy and that he underwent mantle and paraaortic radiation therapy. He doesn’t remember much more but does remember being told that he was cured. He followed up with an oncologist for 5 years but ended follow-up after that time. He did not remember much else but says that he was cured. I obtained components of his prior medical record and the best that I could determine the history is as follows:
Age 21: Painless adenopathy. Staging splenectomy. Stage I HL
Rx: Mantle and paraaortic XRT (?dose)
6 months later: Relapse. Stage 4 HL
Rx: Chemo: MOPP
5 years after initial rx: HL remains in remission, hypothyroid, infertile, hyperlipidemia,
Cure
thyroid supplement, no lipid-lowering therapy
No follow-up in the past 37 years.
Exam:
BP 160/92, HR 80
No adenopathy
Splenectomy site healed
Labs: Hgb 13.5, Plts 400K, WBC 8.0 (normal diff)
TSH (3.0)
So while he was sent to me for evaluation of hypertension there is much more to his story.
Cholesterol 326, TG 148, HDL 42, LDL 154
Can you advise me as to what I should – or should not– be doing for this man who was treated and apparently cured of Hodgkin lymphoma 42 years ago and last seen in medical follow-up 37 years ago.
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4. Staging Before 1960s: Incurable 1960s Staging Early stage
Stage I: single lymph node region or extralymphatic organ
Stage II: two or more lymph node regions same side of diaphragm
Advanced stage:
Stage III: involved node regions both sides of diaphragm
Stage IV: disseminated additional extralymphatic involvement

5. Approach to Treatment 1960s, 1970s Staging laparotomy and splenectomy
Early stage
Extended-field high-dose XRT
Advanced stage
Chemotherapy
Alkylating agents MOPP
ABVD (doxorubicin + bleomycin + vinblastine + dacarbazine)

6. Approach to Treatment 1980s Staging: imaging studies
Early stage: extended-field XRT
Advanced stage: chemotherapy (ABVD)

7. Approach to Treatment 1990s Staging: imaging studies
Chemotherapy (ABVD) + limited-field lower-dose XRT

8. Approach to Treatment 2000s Staging: imaging studies Early stage
Chemotherapy (ABVD)
Further limiting of XRT field and dose
Advanced stage
Combination chemotherapy
Limited radiation therapy in some
Remission: >80-85%

9. Cumulative incidence of cause-specific mortality of long-term HL survivors.
Republished with permission of American Society of Hematology, from Current survivorship recommendations for patients with Hodgkin lymphoma: focus on late effects, Andres Ng, 124, 23, 2014; permission conveyed through Copyright Clearance Center, Inc. ©2014 by American Society of Hematology

10. The Risk After Cure
Second malignancy
10- to 15-year latency
Solid tumors
Radiation therapy
Younger age at Hodgkin disease treatment
Smoking

11. Second Tumor Breast cancer in women Women treated age <30
Mantle radiation
Latency to breast cancer years

12. Schaapveld M, Aleman BM, van Eggermond AM, Janus CP, Krol AD, van der Maazen RW, et al.
Second Cancer Risk Up to 40 Years after Treatment for Hodgkin's Lymphoma. N Engl J Med. 2015;373:
[PMID: ] doi: /NEJMoa

13. The Risk After Cure: Other Cancers
Lung cancer
Directly related to radiation dose
Linked to alkylating agents
Tobacco
Gastrointestinal cancer
Esophagus, stomach, pancreas, colon
Related to radiation dose
Chemotherapy

14. The Risk After Cure: Cardiovascular Disease
Mantle field radiation therapy
Anthracycline (ABVD)
Up to 6X incidence:
CAD
Less likely to have typical angina
Valvular heart disease
Cardiomyopathy
Pericardial constriction
Hypertension, tobacco, hyperlipidemia, physical inactivity further increase risk

15. The Risks After Cure Hypothyroidism Neck XRT Infertility Pelvic XRT
Alkylating agents
Aggressive salvage therapy
Standard ABVD does not affect fertility
Neck muscle weakness
Mantle XRT
>15-20 years after XRT
Dropped head syndrome

16. Surveillance HLS Treated by Mediastinal XRT
Yearly history and physical
CAD
Valvular heart disease
Cardiomyopathy
CHF
Conduction disease
Carotid disease
CV risk factors and treat
If screening suggests CV dx, imaging studies as indicated
Lipid panel every 1-2 years

17. Surveillance HLS Treated by Mediastinal XRT
Asymptomatic
Transthoracic echocardiogram
Initial: 5 years after exposure in high-risk patients
High risk: mediastinal XRT >15 Gy, one or more cardiac risk factors
Initial: 10 years after exposure in others
Follow-up: every 5 years
Stress test for CAD detection
5-10 years after exposure in high-risk patients
Lancellotti P, Nkomo VT, Badano LP, Bergler-Klein J, Bogaert J, Davin L, et al; European Society of Cardiology Working Groups on Nuclear Cardiology and Cardiac Computed Tomography and Cardiovascular Magnetic Resonance. Expert consensus for multi-modality imaging evaluation of cardiovascular complications of radiotherapy in adults: a report from the European Association of Cardiovascular Imaging and the American Society of Echocardiography. Eur Heart J Cardiovasc Imaging. 2013;14: [PMID: ] doi: /ehjci/jet123

18. Surveillance HLS Treated by Mediastinal XRT
Breast cancer screening in women
National Comprehensive Cancer Network
Annual mammogram 8-10 years post Rx or at age 40, whichever comes first
Add breast MRI to mammogram if XRT between ages 10-30
Late Effects of Childhood Cancer Guideline
Annual mammogram, breast MRI, or both for women who had received chest XRT >20 Gy starting at age 25 or 8 years after treatment, whichever comes later
Lung cancer screening in high-risk patients (tobacco use)
Low-dose CT
Frequency?

19. Surveillance Thyroid function Neck XRT: annual TSH
Annual skin cancer exam of radiation field
Abdominal-pelvic XRT (>30 Gy)
Colonoscopy beginning age 35 or at 10 years post XRT (whichever comes first) and repeated every 5 years

20. Can You Lower the Risk? Splenectomy or splenic XRT
Immunization (pneumococcal, meningococcal, influenza)
Cardiac risk factor reduction-Rx
Physical activity
>2-2.5 hours of cycling or walking per week

21. Copyright © 2017

22. Our Patient 63-year-old man
42 years following Hodgkin lymphoma treatment
Splenectomy
Chest and paraaortic XRT
Chemotherapy
Hypothyroid
Hyperlipidemia
Hypertension

23. Our Patient Treat cardiac risk factors Hypertension Hyperlipidemia
Treat hypothyroidism
Cardiac surveillance
Echocardiogram
Stress test
Cancer screening
Skin exam
Colonoscopy
Immunization (pneumococcal, meningococcal, influenza)
Exercise plan

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