History
Most patients with Hodgkin lymphoma (HL) present with persistent painless adenopathy, usually cervical and/or mediastinal, unresponsive to antibiotic therapy. More than 70% of patients present with cervical lymphadenopathy. Patients with mediastinal adenopathy may present with respiratory symptoms such as shortness of breath, chest pain, or cough. These patients are at risk for respiratory failure, especially if they undergo sedation or anesthesia for diagnostic procedures. A large mediastinal mass may also cause superior vena cava syndrome. Approximately 25% of patients present with one or more systemic symptoms that are associated with advanced disease and an adverse prognosis.
The Ann Arbor staging system recognizes the following 3 symptoms, known as B symptoms, as having prognostic significance (see Staging):
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Unexplained fever with temperatures above 38°C for 3 consecutive days
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Unexplained weight loss of 10% or more in the previous 6 months
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Drenching night sweats
Patients may have other symptoms that relate to the cytokines produced by Hodgkin lymphoma Reed-Sternberg cells (RSCs) or the supporting environment within the affected lymph nodes, such as pruritus, urticaria, and fatigue.
The clinical manifestations of Hodgkin lymphoma result from the mass effect that is mostly due to the reactive tissue surrounding RSCs, as well as cytokine production by RSCs. Systemic symptoms have been attributed to the production of interleukin (IL)–6, whereas some of the histopathological characteristics, such as eosinophilia and collagen sclerosis, have been attributed to cytokine production, such as IL-4, IL-5 exotoxin, IL-6, IL-7, tumor necrosis factor (TNF), lymphotoxin, transforming growth factor β (TGF-β), and basic fibroblast growth factor.
Several immune-mediated paraneoplastic syndromes, such as immune thrombocytopenic purpura, autoimmune hemolytic anemia, and nephrotic syndrome, can be associated with Hodgkin lymphoma. These paraneoplastic syndromes can present before, after, or at the time of presentation of HL.
Physical Examination
Physical examination is important in the evaluation of patients with Hodgkin lymphoma (HL) because it allows the clinician to monitor the response to treatment. Careful evaluation of all lymph node stations, hepatosplenomegaly, and involvement of Waldeyer or tonsillar tissues should always be performed, and the findings should be documented.
Patients may have firm, nontender lymphadenopathy. This lymphadenopathy is cervical in 70-80% of patients and axillary in 25%. Other sites are supraclavicular, inguinal, and, less often, epitrochlear or popliteal. A mediastinal mass may cause superior vena cava obstruction, respiratory symptoms, or both. Splenomegaly, hepatomegaly, or both may be present.
Disease extension is predictable, is contagious, and can affect other organs and systems. Organs that are predominantly affected include the lungs, bone, bone marrow, liver parenchyma, and, rarely, the central nervous system.
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Mixed cellularity Hodgkin lymphoma showing both mononucleate and binucleate Reed-Sternberg cells in a background of inflammatory cells (hematoxylin and eosin, original magnification X200).
Tables
Risk Groups |
Children’s Oncology Group |
German Multicenter Studies |
Stanford/Dana Farber/St. Jude |
Low risk |
I A no bulk or E II A no bulk or E |
I A /B II A |
I A II A no bulk |
Intermediate risk |
I A with bulk or E, I B II A with bulk or E II B III A IV A E |
II B III A – E III B |
I A bulk I B II A with bulk IIB III IV |
High risk |
III B IV B |
II B – E III A / B - E IV A / B |
|
Regimen |
Involved-Field Radiation Dose |
Five-Year Survival Rate |
2-4 ABVD |
20-40 Gy |
90% |
2-4 ABVE |
21 Gy |
88.3% |
2 OEPA/OPPA |
20-35 Gy |
94% |
4 VAMP |
15-25.5 Gy |
93% |
4 COPP/ABV |
21 Gy |
100% |
3 MOPP/3 ABVD |
15-25.5 Gy |
100% |
2 MOPP/2 ABVD |
20-40 Gy |
87% |
Regimen |
Involved-Field Radiation Dose |
Five-Year Survival Rate |
4-8 ABVD |
|
80% |
3-5 ABVE-PC |
21 Gy |
84% |
OPPA/OEPA |
20-25 Gy |
91% |
COPP/ABV |
21 Gy |
72% |
4 MOPP/4 ABVD |
10-21 Gy |
78% |
6 VAMP/COP |
15-25 Gy |
68% |
BEACOPPesc |
21 Gy |
94% |
