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 ) 60-80% 50%

systemic symptoms (B symptoms)

fever night sweats unexplained weight loss (10% per 6 months)

30%

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 ReedSternberg 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 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

Staging evaluation for Hodgkins 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 Hodgkins 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
mantle field paraaortic field pelvic field dose: 35-40 Gy/T

80-90% RC

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