Hodgkin's Disease

Epidemiology

• bimodal distribution: Highest rates in adolescents and young adults, second peak in middle age > 50 y/o., rare in children under 5 yo.
• Incidence: 5% of cancers in kids <15 year old
• M>F under 10 yo; M=F in adolescents
• Predispositions: EBV infection (also predisp to NHL), genetic, pre-existing immunodeficiency

Pathology & Histology

• Reed-Sternberg cell (multinucleated giant cells); their normal counterparts have not been definitively identified, they may originate from activated B or T-lymphocytes or from an antigen presenting cell
• Lymphocyte predominant, nodular sclerosing (most common subtype in children), mixed cellularity, and lymphocyte depleted

Presentation

• Tumor: Orderly, contiguous spread, Rarely extranodal
• Most common presentation: Painless, firm cervical or supraclavicular adenopathy
• B symptoms: Fever, weight loss, night sweats
• Anterior mediastinal mass (66%)
• Effusions
• Bone marrow infiltration -> cytopenias
• HSM (rare)
• Nephrotic syndrome
• Metastasis: Liver, spleen, bone, BM, brain

Diagnosis:

• CXR to evaluate mediastinum
• Imaging – chest/abdomen/pelvis
• Excisional biopsy
• Tests necessary for staging: CBC, ESR, ferritin, serum copper, LFTs, CXR, chest and abdominal CT with contrast, gallium scan, BM biopsy

Treatment:

• exquisitely responsive to radiation therapy
• Chemotherapy: Favorable: COPP-ABV, Unfavorable: ABVD + Radiation
  • MOPP: mechlorethamine, oncovin (vincristine), procarbazine, prednisone
  • COPP: like MOPP, except cyclophosphamide substituted
  • COMP: like COPP but MTX sub for procarbazine
  • ABVD: adriamycin (doxorubicin), bleomycin, vincristine, dacarbazine
  • DBVE: doxorubicin, bleomycin, vincristine, etopside
  • DBVE+PC: + prednisone, cyclophosphamide
  • Each cycle consists of 21 to 28 days dependent on toxicity

Prognosis:

• Excelllent for stage I & II : 90% cure rate
• Good to fair for Stage III & IV and recurrent: 60-70% cure

CHLA Board Review 2005
Schwartz. The 5-minute pediatric consult, 3rd edition.