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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.
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 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
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 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)
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. AsymptomaticB. 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 therapy 80-90% RC– mantle field
– paraaortic field
– pelvic field
dose: 35-40 Gy/T
• Combination chemotherapy– ABVD 80% RC
– BEACOPP 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