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Hodgkin Disease

Hodgkin Disease. Definition: neoplastic disorder with development of specific infiltrate containing pathologic Reed-Sternberg cells. It usually arises in lymph nodes and spreads to contiguous groups. Extranodal presentation are rare. Disease is associated with defective cellular immunity.

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Hodgkin Disease

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  1. Hodgkin Disease • Definition: neoplastic disorder with development of specific infiltrate containing pathologic Reed-Sternberg cells. It usually arises in lymph nodes and spreads to contiguous groups. Extranodal presentation are rare. Disease is associated with defective cellular immunity.

  2. Hodgkin Disease • Incidence: - 2-4 cases per 100000 population / year - bimodal age distribution : 15-35 years and above 50 years - male predominance M:F = 1,7:1

  3. Clinical Presentation • Nontender lymph nodes enlargement ( localised ) • neck and supraclavicular area 60-80% • mediastinal adenopathy 50% • other ( abdominal, extranodal disease ) • systemic symptoms (B symptoms) 30% • fever • night sweats • unexplained weight loss (10% per 6 months) • other symptoms • fatigue, weakness, pruritus • cough , chest pain, shortness of breath, vena cava syndrome • abdominal pain, bowel disturbances, ascites • bone pain

  4. Diagnosis of Hodgkin Disease • is based on microscopic examination of lymph node or other involved tissue • it requires identification of diagnostic Reed-Sternberg cells

  5. Pathologic ClassificationWHO • Classical Hodgkin disease • lymphocyte rich (LR) • nodular sclerosis 1 and 2 (NS) • mixed cellularity (MC) • lymphocyte depletion (LD) • Hodgkin lymphoma with lymphocyte predominance (LP)

  6. Staging Classification Ann Arbor modified by Cotswolds • Stage I: involvement of single lymph node region or lymphoid structure • Stage II: involvement of two or more lymph node regions on same side of diaphragm • Stage III: involvement of lymph node regions or structures on both sides of diaphragm III1: with splenic hilar,celiac,portal nodes III2: with para-aortic,iliac,mesenteric nodes • Stage IV: involvement of extranodal site(s) A. Asymptomatic B. Symptomatic (B symptoms) X. Bulky disease ( > 1/3 widening of mediastinum, > 10cm max.dimension of nodal mass) E. Involvement of a single, localised, extranodal site

  7. Staging evaluation for Hodgkin’s Disease (1) • Essential • pathologic documentation by hemopathologist • physical examination • documentation of B symptoms • laboratory evaluation • complete blood count, ESR • liver function tests • renal function tests • lactate dehydrogenase • chest radiograph • ultrasonography • CT scan of chest, abdomen and pelvis • bone marrow aspiration / biopsy (bilateral)

  8. Staging evaluation for Hodgkin’s Disease (2) • Essential under certain circumstances • liver biopsy • gallium scan • technetium bone scan • bone radiographs • MRI • bipedal lymphangiogram • staging laparotomy • Useful but not essential tests • cell-surface marker phenotypic analysis • gene rearrangement analysis

  9. Treatment of Hodgkin Disesae (1) With appropriate treatment about 85% of patients with Hodgkin disease are curable • I A,B: radiation therapy • II A : combination chemotherapy + radiotherapy • IIB IIIA,B IVA,B : combination chemotherapy (+/- radiotherapy)

  10. Treatment of Hodgkin Disesae (2) • Radiation therapy 80-90% RC • mantle field • paraaortic field • pelvic field dose: 35-40 Gy/T • Combination chemotherapy • ABVD 80% RC • BEACOPP 90% RC

  11. Treatment of Hodgkin Disesae (3) Salvage therapy- resistance, relapse: • Second-line noncross-resistant regimens CR 30-40% DFS 10-25% • DHAP • CEP • EVAP • High dose chemotherapy with autologous stem cell transplantation

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