Pediatric Hodgkin Lymphoma Clinical Presentation
- Author: Pedro A de Alarcon, MD; Chief Editor: Robert J Arceci, MD, PhD more...
History
Most patients with Hodgkin lymphoma present with persistent painless adenopathy, 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.
Patients with Hodgkin lymphoma may present with symptoms that are associated with advanced disease and adverse prognosis. The Ann Arbor staging system recognizes the following 3 symptoms, known as B symptoms, as having prognostic significance (see Staging):
- Unexplained fever with temperatures above 38°C for 3 consecutive days
- Unexplained weight loss of 10% or more in the previous 6 months
- Drenching night sweats
Patients may have other symptoms that relate to the cytokines produced by Hodgkin-Reed-Sternberg (HRS) cells or the supporting environment within the affected lymph nodes, such as pruritus, urticaria, and fatigue.
Several immune-mediated paraneoplastic syndromes, such as immune thrombocytopenic purpura, autoimmune hemolytic anemia, and nephritic syndrome can be associated with Hodgkin lymphoma. These paraneoplastic syndromes can present before, after, or at the time of presentation of Hodgkin lymphoma.
Physical Examination
Physical examination is important in the evaluation of patients with Hodgkin lymphoma 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.
Staging
After a tissue diagnosis is made, the disease is staged by using imaging studies, evaluating the bone marrow evaluation, and assessing for B symptoms.
The most widely used staging system is the Ann Arbor staging system, as follows:
- Stage I - Single lymph node region or single extranodal site
- Stage II - Two or more lymph node regions on the same side of the diaphragm
- Stage III - Lymph node regions on both sides of the diaphragm
- Stage IV - Diffuse or disseminated involvement of one or more extralymphatic organs (liver, bone marrow, lung) or tissues with or without associated lymph node involvement (The spleen is considered a nodal site.)
A or B designations are also used. B includes the presence of at least one of the following symptoms:
- Drenching night sweats
- Unexplained fevers with temperature more than 38°C for 3 consecutive days
- More than 10% loss of body weight in the past 6 months
The A designation involves the absence of symptoms described above. The E designation is extension or contiguous involvement of extranodal sites by large mediastinal masses that are not considered metastatic or stage IV.
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