1. ABNORMAL WBC’S

2. A 19 yr old man consults a physician about a 2 cm neck Mass. The patient has no systemic symptoms at that time. The mass is resected and proves to be a lymph node showing replacement of normal follicles by sheets of a mixed population of cells including histocytes, lymphocytes, monocytes, plasma cells and eosinophils, scattered large binucleated cells are also seen. These cells are positive for CD15 and CD30 as immunohistochemical staining. Which of the following is the most likely diagnosis? a) leukoctopenia b) acute myeloid leukemia c) hodgkin’s disease d) hairy cell leukemia e) burkitt lymphoma

3. Epidemiology of Hodgkin’s 5 % of all ped.ca. Incidence by age is bimodal – In industrialized countries, peak- late 20’s and after 50’s – In developing countries, early peak is before adolescence Epidemiologic studies demonstrate 3 distinct forms: – Childhood form (<14 years) – Young adult form (15-34) – Older form (55-74)

4. Epidemiology Rarely diagnosed in kids <10 years – In kids F – In adolescence, M=F More common in patients with congenital and acquired immune system abnormalities – Ataxia telangiectasia – AIDS

5. Who is at higher risk Clustering of cases in families & concordance in primary relatives – ?genetic predisposition – ?common exposures to causal agent Higher concordance in monozygotic twins

6. What causes it? Environmental factors: – Occupation in the wood industries Studies have suggested several infectious agents: – EBV ( ebstein barr virus) – Human Herpes Virus 6 – CMV ( cytomegalovirus) High EBV titers and the presence of EBV genomes in Reed-Sternberg cells

7. Reed sternberg cell

8. Clinical Presentation Most common presentation in children is asymptomatic cervical lymphadenopathy – Painless, firm,not inflammatory Extension from one lymph node group to another 2/3 of patients have mediastinal adenopathy at presentation – Cough or SOB if significant compression Infrequently presents as axillary or inguinal adenopathy Spleenomegally Fever, wt loss, night sweat Bone leasions and fractures

9. Extranodal Metastasis Hodgkin’s spreads through the lymphatic system Most frequent sites of extranodal involvement in decreasing order of frequency – bone marrow, bone,liver, lung, pericardium or pleura Paraneoplastic syndromes – More likely seen in relapsing patients with widespread disease – Hematologic, skin, nervous system, kidneys

10. Diagnostic Workup Tissue is needed for definitive histologic diagnosis – Sample the node that is most accessible PE with careful attention and measurement of lymph nodes Labs – CBC with diff – ESR – LFT,Renal function – Alkaline phosphatase; ferritin,copper elevated – (Immune response decreased, – Cytokines Il 1,6,TNF- B symptoms, Il 2 elevated)

11. Diagnostic & Staging Workup Cervical area US/CT/MR Thoracic imaging – Chest Xray, CT scan of chest (ant/middle mediastinum) best visualization of lung parenchyma, pleura Abdominal imaging – US/CT/MRI – Lymphangiogram Most reliable method of detecting retroperitoneal lymph nodes Rarely done in children

12. Diagnostic & Staging Workup Gallium Scan/ PET scan – Search the body for other involvement Staging laparotomy – Not used routinely any more – Previously done routinely as part of staging Bone marrow biopsy – Recommended for stage IIB or higher – Bone marrow involvement at presentation is rare Bone scan – Recommended for kids with bone pain, elevated alk phos, or extranodal disease

13. Treatment chemotherapy radiation extended field radiation involeved field radiation Max. efficacy min. toxicity

14. DRUGDOSE ( mg/M 2 ) ROUTE ABVD Adriamycin ( doxorubicin ) Bleomycin Vinblastin Dacarbazine REPT EVRY 28 DAYS 25 10 6 375 IV MOPP Mechlorethamine Vincristine Procarbazine Prednisone REPT EVRY 21 DAYS 6 1.4 100 40 IV ORAL

15. EARLY STAGE Favourable prognosis Unfavourable prognosis Ext. field radiation or 2-4 cycles of ABVD+ involved field radiation 4-6 cycles of ABVD+ involved field radiation ADVANCED STAGE: relapsed after radiation 6-8 cycles of MOPP/ABV+ involved field radiation+ radiation 6-8 cycles of chemo…

16. A 19 yr old man consults a physician about a 2 cm neck Mass. The patient has no systemic symptoms at that time. The mass is resected and proves to be a lymph node showing replacement of normal follicles by sheets of a mixed population of cells including histocytes, lymphocytes, monocytes, plasma cells and eosinophils, scattered large binucleated cells are also seen. These cells are positive for CD15 and CD30 as immunohistochemical staining. Which of the following is the most likely diagnosis? a) leukoctopenia b) acute myeloid leukemia c) hodgkin’s disease d) hairy cell leukemia e) burkitt lymphoma Reed sternberg cell