Hodgkin Lymphoma
Hodgkin Lymphoma: Excerpt from The 5-Minute Pediatric Consult
Leslie S. Kersun, MD
Hodgkin Lymphoma - BASICS
Hodgkin Lymphoma - description
Malignant enlargement of lymph nodes characterized by a pleomorphic cellular infiltrate with multinucleated giant cells (Reed-Sternberg cells)
Hodgkin Lymphoma - epidemiology
Hodgkin Lymphoma - incidence
- Male > Female
- Incidence shows bimodal age distribution:
- Early peak, before adolescence in developing countries, mid to late 20s in US
- Second peak, late adulthood >50 years of age
- Childhood cases rare before 5 years of age
- Most common in whites >15 years of age
Hodgkin Lymphoma - risk factors
Risk groups:
- Low risk: IA-IIA, without bulk disease
- Intermediate risk:
- IA-IIA with bulk disease (defined by nodal aggregate >6 cm or mediastinal mass > 1/3 the thoracic diameter)
- IAE, IIAE: IB-IIB
- IIIA, IVA
- High risk: IIIB, IVB
- Prognostic factors:
- Disease stage
- Presence of B symptoms
- Bulk disease or mediastinal mass
- Laboratory abnormalities including hemoglobin (Hb) <11 g/dL, WBC >13.5 cc/mm3, elevated ESR
- Timing of response to treatment
Hodgkin Lymphoma - genetics
Familial clustering suggests both genetic and environmental factors in pathogenesis:
- 3–7-fold increased risk of disease among siblings in families where twins are concordant
- Reports of parent–child pairs
Hodgkin Lymphoma - pathophysiology
Reed-Sternberg cells are the malignant cells of Hodgkin lymphoma. They are monoclonal and derived from germinal center B cells.
- Rye classification divides disease into 4 histological categories:
- Lymphocyte predominant
- Mixed cellularity
- Lymphocyte depleted
- Nodular sclerosis—most common subtype in children (~50% of cases)
Hodgkin Lymphoma - etiology
- Exact cause unknown
- Infections with Epstein-Barr virus may play role in transmission of disease.
Hodgkin Lymphoma - DIAGNOSIS
Hodgkin Lymphoma - signs & symptoms
Hodgkin Lymphoma - history
- Stage B symptoms occur in 20–30% and include 1 of the following:
- Unexplained fever >38°C for ≥3 days
- Unexplained weight loss >10% of body weight in previous 6 months
- Drenching night sweats
- Stage A disease signifies absence of B symptoms or asymptomatic.
- Other systemic symptoms can include fatigue, anorexia, pruritus, chest pain, orthopnea
- History relative to possible immunodeficiency, Epstein-Barr virus or HIV infection should be recorded.
Hodgkin Lymphoma - physical exam
Painless lymphadenopathy most common:
- Nodes usually firmer, rubbery in texture, and less mobile than inflammatory nodes. Cervical chain involved in 80% of patients
- Mediastinal mass in 2/3 of patients that may cause nonproductive cough or difficulty breathing
- Hepatosplenomegaly and bone tenderness in advanced stages
- If bone marrow involvement, can see pallor, bruising, or petechiae
- Rare cases present with autoimmune hemolytic anemia (AIHA) or idiopathic thrombocytopenic purpura (ITP) and can have resulting jaundice, petechiae, or bleeding as a result.
Hodgkin Lymphoma - tests
Hodgkin Lymphoma - lab
- CBC, ESR
- Liver and renal function studies
- Baseline thyroid function (preradiotherapy)
- Baseline electrocardiogram, echocardiogram
- Baseline pulmonary function tests (preradiotherapy and/or bleomycin)
- Special tests
- Bone marrow biopsy in selected cases
Hodgkin Lymphoma - imaging
- Chest radiograph (posterior-anterior and lateral) for mediastinal mass
- CT scan (neck, chest, abdomen, pelvis) to rule out disseminated disease
- Bone scan if presenting with bone pain
- Other: Gallium scan standard, but PET scan is standard in adults, being studied in children and used more frequently than Gallium in some centers.
Hodgkin Lymphoma - diag proced-surgery
Lymph node biopsy for definitive diagnosis Ann Arbor Staging System:
- I: Involvement of a single lymph node region (I) or of a single extralymphatic organ or site (IE) by direct extension
- II: Involvement of 2 or more lymph node regions on the same side of the diaphragm (II) or localized involvement of an extralymphatic organ or site and 1 or more lymph node regions on the same side of the diaphragm (IIE)
- III: Involvement of lymph node regions on both sides of the diaphragm (III), which may be accompanied by involvement of the spleen (IIIS) or by localized involvement of an extralymphatic organ or site (IIIE) or both (IIIES)
- IV: Diffuse or disseminated involvement of 1 or more extralymphatic organs or tissues with or without associated lymph node involvement
- Staging further subclassified A or B according to absence or presence of symptoms (listed above), respectively.
Hodgkin Lymphoma - differencial diagnosis
- Infection is most common cause for acute lymphadenopathy:
- Bacterial (Staphylococcus aureus, hemolytic streptococcus, tuberculosis, atypical mycobacterium)
- Other (Epstein-Barr virus, cytomegalovirus, cat scratch disease, toxoplasmosis, HIV, histoplasmosis)
- Malignancy more common with chronic adenopathy:
- Non-Hodgkin lymphoma
- Neuroblastoma
- Leukemia
- Rhabdomyosarcoma
- Mediastinal masses divided anatomically:
- Anterior: Lymphoid and thyroid tumors, bronchogenic cysts, aneurysms, lipomas
- Middle: Lymphoid tumors, angiomas, pericardial cysts, teratomas, esophageal lesions, hernias
- Posterior: Neurogenic tumors, cysts, thoracic meningocele, sarcomas
Hodgkin Lymphoma - TREATMENT
Hodgkin Lymphoma - special therapy
Hodgkin Lymphoma - radiotherapy
Exquisitely responsive to radiotherapy: The doses below are used only in conjunction with chemotherapy. Higher doses are necessary if using radiation as primary therapy, which is quite uncommon in the pediatric setting.
Hodgkin Lymphoma - medication
Chemotherapy:
- Multiple agents allow different mechanisms of action (to circumvent resistance) and nonoverlapping toxicities so that full doses can be given. The most common combinations used in initial therapy include:
- COPP: Cyclophosphamide, vincristine (oncovin), procarbazine, prednisone. Often used in combination with ABV below.
- ABV: Doxorubicin (adriamycin), bleomycin, vinblastine
- ABVE: Doxorubicin + bleomycin + vincristine + etoposide
- ABVE-PC: ABVE + prednisone and cyclophosphamide
- VAMP: Vinblastine, adriamycin, methotrexate, prednisone
- BEACOPP: Bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, prednisone
Hodgkin Lymphoma - second line
For patients with lymphocyte predominant histology: Excision alone has been used for patients with low stage disease.
Hodgkin Lymphoma - FOLLOW UP
Hodgkin Lymphoma - prognosis
With current therapy including chemotherapy and/or radiation, 5-year disease-free survival:
- Low risk disease: >90%
- Advanced disease: 60–95%
Hodgkin Lymphoma - complications
Acute toxicity of treatment:
- Radiation: Include erythema, nausea, fatigue, possibly myelosuppression
- Chemotherapy: The general side effects include:
- Hair loss
- GI toxicity including nausea, vomiting, diarrhea, and mouth sores
- Myelosuppression (most common dose-limiting toxicity)
- Transfusions may be required.
- All patients take prophylaxis for pneumocystis.
Hodgkin Lymphoma - patient monitoring
Office visits every 3 months for exam, CBC, ESR:
- CT scan of involved areas every 3 months for 1st 2 years, then every 6 months for 3 years
- Relapse of disease usually occurs within 1st 3 years. Some may relapse as late as 10 years after initial diagnosis.
- Special studies as needed for toxicity-related complications. For example:
- Yearly thyroid function tests if history of irradiation
- Regular self-breast examination for females treated with chest radiation
- Mammograms beginning by the age of 25 or 10 years post-chest radiation (whichever is later in females)
- Periodic EKGs, ECGs, and Holter monitors if treated with radiation and/or anthracyclines
- Periodic pulmonary function tests if treated with radiation and/or bleomycin
- Late effects secondary to chemotherapy and/or radiation
- Pulmonary: Pneumonitis, pulmonary fibrosis, decreased pulmonary function, pneumothorax
- Cardiac/Vascular: Cardiomyopathy resulting in congestive heart failure, pericarditis, valvular damage, coronary heart disease, arrhythmias, myocardial infarction, and stroke
- Gonadal dysfunction: Ovarian damage can be avoided by performing temporary oophoropexy prior to involved field radiotherapy (IFRT). Azoospermia secondary to alkylating agents is almost always permanent in postpubertal boys. Sperm banking recommended for boys with development of Tanner 3 or higher.
- Thyroid: Hypothyroidism, hyperthyroidism, thyroid nodules, thyroid cancer
- Growth/Musculoskeletal: Growth retardation more common in past when prepubertal patients received high doses of radiotherapy
- Secondary malignant neoplasms: A major concern in selecting therapy:
- Breast cancer most common solid tumor
- Other secondary neoplasms: Thyroid and skin carcinomas, bone, colorectal, gastric, leukemia
Hodgkin Lymphoma - bibliography
- Bhatia S, Yasui Y, Robinson LL, et al. Late Effects Study Group. High risk of subsequent neoplasms continues with extended follow-up of childhood Hodgkin’s disease: Report from the Late Effects Study Group. J Clin Oncol. 2003;21:4386–4394.
- Smith RS, Chen Q, Hudson MM, et al. Prognostic factors for children with Hodgkin’s disease treated with combined-modality therapy. J Clin Oncol. 2003;21:2026–2033.
Weinstein HJ, Hudson MM, Link MP, eds. Pediatric Lymphomas. Berlin: Springer-Verlag; 2007.
Hodgkin Lymphoma - CODES
Hodgkin Lymphoma - icd9
- 201.9 Hodgkin’s disease, unspecified
Hodgkin Lymphoma - FAQ
- Q: Is my child at risk for other cancers?
- A: Yes. Although the incidence is low, children with Hodgkin disease are primarily at risk for cancers resulting from their treatment. Breast cancer is the most common solid tumor and can occur decades after therapy. Therefore, long-term follow-up is essential.
- Q: Will my child be infertile following treatment?
- A: It depends on the therapy received. Certain chemotherapy agents are associated with a higher risk of infertility (alkylating agents), and boys are more sensitive than girls. Radiation to the gonads is also associated with infertility.
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Book Source Details
- Book Title: The 5-Minute Pediatric Consult
- Author(s): M. William Schwartz MD; et al.
- Year of Publication: 2008
- Copyright Details: The 5-Minute Pediatric Consult, Copyright © 2008 Lippincott Williams & Wilkins.
More About Hodgkin's Disease
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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More About This Book:
Title: The 5-Minute Pediatric Consult
Authors: M. William Schwartz MD; et al.
Publisher: Lippincott Williams & Wilkins
Copyright: 2008
ISBN: 0-7817-7577-9
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