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Similar to Paithankar Adwait 7610 m2a Hodgkins Lymphoma hemat.pptx
Paithankar Adwait 7610 m2a Hodgkins Lymphoma hemat.pptx
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- 2. Hodgkin disease Definition: Aneoplastic transformation of lymphocytes particularly in lymph nodes. Characterized by: 1) the presence of Reed-Sternberg cells on histology 2) spreading in an orderly fashion to contagious lymph nodes ( Forexample, Hodgkin lymphoma that starts in thecervical lymph nodes may spread first to the supraclavicular nodes then to the axillary nodes ) ⚫ 6% of childhood cancer ⚫ 5% of cancer in < 14 yr ⚫ 15% in person 15-19 yr ⚫ Rare < 10 yr
- 3. Red-Sternberg cells Largecells (>45um in diameter) with classically binucleate or bilobed central nucleus each with a large acidophilic central nucleoli surrounded byaclear halo. “owl’seyeappearance”
- 4. Epidemiology of Hodgkin’slymphoma ⚫ Hodgkin disease has bimodal agedistribution--onepeak in the 20s and 60s. ⚫ Early peak middle to late 20s ⚫ Second peak after50 yr ⚫ Sex Male : Female 4: 1 for3-7 yr 3: 1 for7-9 yr 1-3: 1 for > 10 yr ⚫ 100 folds risk forunaffected monozygotictwin of affected twin ⚫ Associated with specific HLA antigen ⚫ Infectiousagents Human herpesvirus 6 CMV Epstein – Barrvirus ⚫ Immunodeficiency
- 5. Etiology/Risk Factors Doctors seldomknow why one person develops Hodgkin lymphomaand anotherdoes not. But research shows thatcertain risk factors increase the chance thata person will develop thisdisease. Having one or more risk factors does not mean that a person will develop Hodgkin lymphoma. Most people who have risk factors neverdevelopcancer.
- 6. Risk Factors 1) Certainviruses: Epstein-Barr virus (EBV) Human immunodeficiency virus (HIV) 2) Weakened immunesystem: inherited condition certaindrugs used afteran organ transplant 3) Age: Hodgkin lymphoma is mostcommonamong teensand adults aged 15 to 35 years and adults aged 55 years and older. 4) Family history: Family members, especially brothers and sisters, of a person with Hodgkin lymphoma orother lymphomas may havean increased chance of developing thisdisease.
- 7. Lymphocyte Predominant 10-15% ofpatients More common in male Younger patients Localized disease Has best prognosis Mixed cellularity 30% of patients < 10 yr of age Advanced disease Extranodal extension Lymphocyte depletion Rare in children Common with HIV Has worst prognosis Nodular sclerosis Most common 40% of younger patients 70% of adolescents Classification Rye Classification System
- 8. REAL Classification ( RevisedEuropean – American Classification of Lymphoid Neoplasms ) ⚫Nodular lymphocyte predominance ⚫Classical Hodgkin lymphoma ⚫Lymphocyte rich ⚫Mix cellularity ⚫Nodularsclerosis ⚫Lymphocyte depletion ⚫ Anaplastic large cell lymphoma Hodgkin like
- 9. Enlarged, painless,rubbery, non- erythematous, nontender lymph nodes are the hallmarkof thedisease. May become painful afterdrinking alcohol Hepatosplenomegaly Cough, dyspnea, hypoxia Pleural orpericardial effusion Heptocellulardysfunction B.M infiltration(Anemia, neutropenia, thrombocytopenia) 25% have ''B'' symptoms Although pruritus is common in thedisease it is not one of the ‘’B’’ symptoms. Cervical, supraclavicularand axillary lymphadenopathyare the most common initial signsof thedisease. Disease belowdiaphragm is rare (only3%) Clinical presentation
- 10. Systemic Symptoms (Bsymptoms) ⚫Important in staging ⚫Unexplained fever > 390C ⚫ Weight loss > 10% in 3m ⚫ Drenching night sweats Immune System abnormalities ⚫Anergy to delayed-hypersensitivity skin test ⚫Abnormal cellular immune response ⚫ Decreased CD4:CD8 ratio ⚫ Reduce natural killer cell cytotoxicity
- 11. Extralymphaticsites may beinvolved such as: # Spleen # Liver # Bone marrow # Lung # CNS Extralymphatic involvement is morecommon with non-hodgkin lymphoma. Emergencypresentation: Infections SVC obstruction ( facial edema, increased JVP and Dyspnea)
- 12. Thedoctorconsiders the followingtodetermine the stageof Hodgkin lymphoma: The numberof lymph nodes affected. Whether these lymph nodes areon one or both sides of thediaphragm. Whether thedisease has spread to the bone marrow, spleen, liver, or lung. Each stage is divided into A or B symptomsaccording to the presence of systemic symptoms. Staging of Hodgkin’s Lymphoma
- 13. Ann Arbor StagingClassification for Hodgkin Disease StageI Involvementof a single lymph node (1) orof a singleextra lymphatic siteororgan(1f) StageII Involvementof twoor more lymph node regionson the same side of thediaphragm(II) or localised involvementof an extra lymphaticsite ororganand oneor more lymph node regionson the same side of the diaphragm (IIf)
- 14. Stage III Involvement oflymph node regions on both sides of the diaphragm (III) which may be accompanied by the involvement of spleen (IIIS) or by localized involvement of an extra lymphatic site or organ ( IIIf) or both ( IIIsf) Stage IV Diffuse or disseminated involvement of one or more extra lymphatic organs or tissues with or without associated lymph node involvement. The absence or presence of fever > 38C for three consecutive days , drenching night sweats , or unexplained loss of > 10% body weight in the 6 months preceding admission are to be denoted in all cases by the suffice letters A & B respectively.
- 16. DIAGNOSIS Anexcisional lymphnode biopsy is theessential first step in diagnosis. A biopsy is theonlysureway todiagnose Hodgkin lymphoma. ⚫ Excisional Biopsy ⚫ Light Microscopy ⚫ Immunocytochemistry ⚫ Molecular Studies ⚫ ChestX – Ray ⚫ Mediastinal Mass ⚫ CT Scan ⚫ Chest ⚫ Abdomen ⚫ Pelvis ⚫ Blood CP & ESR ⚫ LFT’s ⚫ BoneMarrow Aspiration ⚫ Serum Copper& Ferritin ⚫ Bone Scan ⚫ Gallium 67 Scan / FDG/PET
- 17. TREATMENT ⚫Treatmentdepends on : ⚫Stageof thedisease ⚫ Ageatdiagnosis ⚫ Presence / absenceof B symptoms ⚫ Presenceof hilar lymphadenopathy ⚫ Presenceof bulky nodal disease ⚫CurrentTreatment Regimen ⚫ Combined chemotherapywith orwithout low dose involved field radiation therapy
- 18. Chemotherapy Regimens MOPP (Mechlorethamine, Vincristine , Procarbazine , Prednisolone) COPP (Cyclophosphamide , Vincristine , Procarbazine , Prednisolone) ABVD (Adriamycin , Bleomycin , Vinblastine , Dacarbazine) BEACOPP ( Foradvanced stage disease ) (Bleomycin , Etoposide , Doxorubicin , Cyclophosphamide , Vincristine , Procarbazine , Prednisolone) TREATMENT
- 19. Therapy isentirelybased on thestage. Localized disease ( stage IA and IIA ) is managed predominantlywith radiation. All patientswithevidenceof ‘’B’’ symptomsaswell as stage III and IV are managed withchemotherapy. The most effective combination chemotherapeutic regimen for Hodgkin lymphoma is ABVD ( adriamycin, bleomycin, vinblastinand dacarbazine). ABVD is superior to MOP (meclorethamine, vincristin(oncovin) , prednisoloneand procarbazine) becauseABVD has fewersideeffects suchas: 1) Permanentsterility 2) Secondarycancerformation 3) Aplasticanemia 4) Peripheral neuropathy TREATMENT
- 20. International Prognostic Index The International Prognostic Index (IPI) was first developed to help doctors determine the prognosis for people with fast-growing lymphomas. The indexdepends on 5 factors: 1) The patient’sage 2) Thestageof the lymphoma 3) Whetheror not the lymphoma is in organsoutside the lymph system 4) Performance status (PS) – howwell a person can complete normal dailyactivities 5) The blood (serum) level of (LDH)
- 22. LONG TERM COMPLICATIONS ⚫Secondarymalignancy ⚫ Acute Myelogenous Leukemia ⚫ Non Hodgkin lymphoma ⚫ Carcinomasof breast , lungs & thyroid ⚫Shortstature ⚫Hypothyroidism ⚫Sterility ⚫Dental caries ⚫Subclinical pulmonary dysfunction ⚫Ischemic heartdisease
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