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.

Hodgkin Disease Incidence: cases per population / year - bimodal age distribution : years and above 50 years - male predominance M:F = 1,7:1

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

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

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)

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

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)

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

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)

Treatment of Hodgkin Disesae (2) Radiation therapy80-90% RC –mantle field –paraaortic field –pelvic field dose: Gy/T Combination chemotherapy –ABVD80% RC –BEACOPP90% RC

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