Hodgkin Disease Definition neoplastic disorder with development of

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

Clinical Presentation • Nontender lymph nodes enlargement ( localised ) – neck and supraclavicular area – mediastinal adenopathy – other ( abdominal, extranodal disease ) • systemic symptoms (B symptoms) 60 -80% 50% 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 nodular sclerosis 1 and 2 mixed cellularity lymphocyte depletion (LR) (NS) (MC) (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, > 10 cm 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 therapy 80 -90% RC – mantle field – paraaortic field – pelvic field dose: 35 -40 Gy/T • Combination chemotherapy – ABVD – BEACOPP 80% RC 90% RC

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