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