Hodgkin Disease

It is very simple & basic presentation on HD.

1. Hodgkin Disease Dr. (Major) Jahangir Alam MBBS, DCH, FCPS Classified child specialist CMH Dhaka, Bangladesh.

2. Hodgkin lymphoma (HL) is characterized by progressive enlargement of the lymph nodes. It is considered unicentric in origin and has a predictable pattern of spread by extension to contiguous nodes. Definition

3. Etiology is unknown. worldwide incidence of HL is approximately 2-4 new cases/100,000 population/yr HL accounts for approximately 5% of cancers in persons 14 yr of age or younger; it accounts for approximately 15% of cancers in adolescents (15-19 yr of age) ETIOLOGY AND EPIDEMIOLOGY Ref: Nelson textbook of Pediatrics 20 th ed. paediatric hematology- philip Lanzkowsky 5 th ed

4. EBV (mixed cellularity subtype) Family history of HL Low socioeconomic status Risk factors

5. Reed- sternberg (RS) cell is the hallmark of Hodgkin lymphoma. It is a large cell (15-45 µm) with multiple or multilobulated nuclei. It is neoplastic clone cell originating from B lymphocyte in lymphnode germinal centers. It can’t synthesize immunoglobulin due to dysregulation of nuclear of nuclear factor kappa B ( NFĸB ). Pathology

6. Characteristic bilobed nucleus with large nucleoli giving the classicial owl eye appearance.

7. Classification

8. Lymphadenopathy ( 90% cases) Usually painless Cervical LN/ supraclavicular LN are involved in 60-80% Discrete, elastic/rubbery, nontender Spreads mostly by contiguity from one chain to another Clinical presentation

9. Mediastinal adenopathy (60%): 20% of patient have bulky mediastinal disease. Persistent nonproductive cough Superior vena caval symptoms Enlargement of neck vessels Hoarseness of voice Dyspnoea Dysphagia

10. Splenomegaly Systemic symptoms: Pel-Ebstein fever Weight loss >10% in 6 months Drenching night sweats Mild itching may be present in 15-25% of cases but it is not considered as B symptoms B symptoms

11. Other less common manifestations are Pulmonary manifestation (17%) Neurological manifestation (late presentation) Bone disease(2%) Bone marrow infiltration(5%) Liver disease (2%) Renal manifestation

12. Haematological manifestation: Anemia Neutropenia (50%) Lymphocytopenia -Due to hypersplenism or BM infiltration Eosinophilia (50%) – due to IL-5 production In advance stage DAT test frequently positive with hemolysis Immune thrombocytopenia may be present in 1-2% cases

13. Staging of HD Note : Can be further subclassified as A catagories - Asypmtomatic B catagories - presence of B symptoms

14. CBC : Normocytic normochromic anemia Neutrophilia in 50% cases Eosinophilia in 50% cases Lymphocytopenia ESR: raiesd S. ferritin : raised CXR : both PA & Lateral view Mediastinal lymphadenopathy Lymphnode biopsy : P resence of RS cell with diffuse infiltration of lymphocyte,histiocyte and many eosinophil & plasma cell Laboratory diagnosis

15. CXR showing mediastinal mass

16. For staging : CT scan of neck, chest, abdomen, pelvis Positron emission tomography (PET) scan Technitium-99 bone scintography For classification : Immunohistochemistry

17. Liver function test Renal function test S. electrolyte S. uric acid S. inorganic PO4 S. calcium DAT Other investigations

18. In general Combined Chemotherapy Low dose involved field radiation Intensity of chemotherapy & volume of radiation depends on Presence of B symptoms Initial disease staging Presence of bulky disease Treatment Considered Standard therapy

19. Chemotherapy regimens commonly used for children & adolescents

20. Chemotherapy regimen Corresponding agents ADVD Doxorubicin ( Adriamycin ), bleomycin , vinblastine , dacarbazine ABVD- Rituxan Doxorubicin ( Adriamycin ), bleomycin , vinblastine , dacarbazine , rituximab COPP Cyclophosphamide , vincristine ( Oncovin ), prednisone, procarbazine OPPA ± COPP (females Vincristine ( Oncovin ), prednisone, procarbazine , doxorubicin ( Adriamycin ), OEPA ± COPP (males) Vincristine ( Oncovin ), etoposide , prednisone, doxorubicin ( Adriamycin ), BEACOPP (advanced stage) Bleomycin , etoposide , doxorubicin ( Adriamycin ), cyclophosphamide , vincristine ( Oncovin ), prednisone, procarbazine

21. Most relapse occurs in 1 st 3year after diagnosis, but relapse after 10 year have been reported. Treatment of relapse Relapse Nature of relapse treatment Relapse with favorable at diagnosis Chemotherapy + LD-IFRT Relapse with high risk disease Chemotherapy + Autologous HSCT Relapse with in 12 month of diagnosis Chemotherapy + Autologous HSCT + radiotherapy

22. With the use of current therapeutic regimens, With dose intense chemotherapy OS has approached to 100% Prognosis Disease stage event-free survival (EFS) Overall survival (OS) Early-stage disease + favorable prognostic factors 85-90% >95% Advanced-stage disease 80-85% 90%

23. Advanced stage of disease (Stage IIB, IIIB, or IV) The presence of B symptoms The presence of bulky disease Extranodal extension (liver) Male sex Elevated erythrocyte sedimentation rate White blood cell count 11,500/mm3 or higher Hemoglobin less than 11.0 g/dl Histology : classical HL Initially not respond to chemotherapy Poor prognostic factors

24. Secondary malignancy Cardiac toxicity Pulmonary dysfunction Thyroid dysfunction Gonadal dysfunction & infertility Growth retardation Psychosocial problem Long term Complication

25. During therapy Physical exam (LN, Liver, spleen) Lab: CBC, ESR, LFT Imaging : CT scan, PET Organ toxicity monitoring: cardiac function test, Pulmonary function test Disease monitoring after treatment: by CXR, CT scan Long term sequelae monitoring: life long Follow up evaluation

26. Conclusion

27. Thank you