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Published byEzra Burns Modified over 9 years ago
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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 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
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Clinical Presentation Nontender lymph nodes enlargement ( localised ) –neck and supraclavicular area60-80% –mediastinal adenopathy50% –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
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Diagnosis of Hodgkin Disease is based on microscopic examination of lymph node or other involved tissue it requires identification of diagnostic Reed- Sternberg cells
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Pathologic Classification WHO Classical Hodgkin disease –lymphocyte rich (LR) –nodular sclerosis 1 and 2 (NS) –mixed cellularity(MC) –lymphocyte depletion (LD) Hodgkin lymphoma with lymphocyte predominance (LP)
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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 III 1 : with splenic hilar,celiac,portal nodes III 2 : 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
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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)
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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
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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)
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Treatment of Hodgkin Disesae (2) Radiation therapy80-90% RC –mantle field –paraaortic field –pelvic field dose: 35-40 Gy/T Combination chemotherapy –ABVD80% RC –BEACOPP90% RC
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Treatment of Hodgkin Disesae (3) Salvage therapy- resistance, relapse: Second-line noncross-resistant regimens CR 30-40% DFS10-25% –DHAP –CEP –EVAP High dose chemotherapy with autologous stem cell transplantation
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