Hodgkin's lymphoma

Hodgkin's lymphoma

1. HODGKIN’S LYMPHOMA Medrockets.com

2. Hodgkin disease Definition : A neoplastic 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 ( For example, Hodgkin lymphoma that starts in the cervical 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. Large cells ( >45um in diameter) with classically binucleate or bilobed central nucleus each with a large acidophilic central nucleoli surrounded by a clear halo. “ owl’s eye appearance ” Red-Sternberg cells Medrockets.com

4. Epidemiology of Hodgkin’s lymphoma Hodgkin disease has bimodal age distribution--one peak in the 20s and 60s . Early peak middle to late 20s Second peak after 50 yr Sex Male : Female 4: 1 for 3-7 yr 3: 1 for 7-9 yr 1-3: 1 for > 10 yr 100 folds risk for unaffected monozygotic twin of affected twin Associated with specific HLA antigen Infectious agents Human herpes virus 6 CMV Epstein – Barr virus Immunodeficiency Medrockets.com

5. Etiology /Risk Factors Doctors seldom know why one person develops Hodgkin lymphoma and another does not. But research shows that certain risk factors increase the chance that a person will develop this disease. Having one or more risk factors does not mean that a person will develop Hodgkin lymphoma. Most people who have risk factors never develop cancer. Medrockets.com

6. Risk Factors 1) Certain viruses : Epstein-Barr virus (EBV) Human immunodeficiency viru s (HIV) 2) Weakened immune system: inherited condition certain drugs used after an organ transplant 3) Age : Hodgkin lymphoma is most common among teens and 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 or other lymphomas may have an increased chance of developing this disease. Medrockets.com

7. Lymphocyte Predominant 10-15% of patients 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 Medrockets.com

8. REAL Classification ( Revised European – American Classification of Lymphoid Neoplasms ) Nodular lymphocyte predominance Classical Hodgkin lymphoma Lymphocyte rich Mix cellularity Nodular sclerosis Lymphocyte depletion Anaplastic large cell lymphoma Hodgkin like Medrockets.com

9. Enlarged, painless, rubbery, non- erythematous, nontender lymph nodes are the hallmark of the disease. May become painful after drinking alcohol Hepatosplenomegaly Cough, dyspnea, hypoxia Pleural or pericardial effusion Heptocellular dysfunction B.M infiltration(Anemia , neutropenia, thrombocytopenia ) 25% have ''B'' symptoms Although pruritus is common in the disease it is not one of the ‘’B’’ symptoms. Cervical, supraclavicular and axillary lymphadenopathy are the most common initial signs of the disease. Disease below diaphragm is rare (only3%) Clinical presentation Medrockets.com

10. Systemic Symptoms (B symptoms) Important in staging Unexplained fever > 39 C 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 Medrockets.com

11. Extralymphatic sites may be involved such as: # Spleen # Liver # Bone marrow # Lung # CNS Extralymphatic involvement is more common with non- hodgkin lymphoma. Emergency presentation: Infections SVC obstruction ( facial edema , increased JVP and Dyspnea ) Medrockets.com

12. The doctor considers the following to determine the stage of Hodgkin lymphoma : The number of lymph nodes affected. Whether these lymph nodes are on one or both sides of the diaphragm. Whether the disease has spread to the bone marrow, spleen, liver, or lung. Each stage is divided into A or B symptoms according to the presence of systemic symptoms. Staging of Hodgkin’s Lymphoma Medrockets.com

13. Ann Arbor Staging Classification for Hodgkin Disease Stage I Involvement of a single lymph node (1) or of a single extra lymphatic site or organ(1 f ) Stage II Involvement of two or more lymph node regions on the same side of the diaphragm(II) or localised involvement of an extra lymphatic site or organ and one or more lymph node regions on the same side of the diaphragm ( II f ) Medrockets.com

14. Stage III Involvement of lymph node regions on both sides of the diaphragm (III) which may be accompanied by the involvement of spleen (III S ) or by localized involvement of an extra lymphatic site or organ ( III f ) or both ( III sf ) 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. Medrockets.com

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16. DIAGNOSIS An excisional lymph node biopsy is the essential first step in diagnosis. A biopsy is the only sure way to diagnose Hodgkin lymphoma . Excisional Biopsy Light Microscopy Immunocytochemistry Molecular Studies Chest X – Ray Mediastinal Mass CT Scan Chest Abdomen Pelvis Blood CP & ESR LFT’s Bone Marrow Aspiration Serum Copper & Ferritin Bone Scan Gallium 67 Scan / FDG/PET Medrockets.com

17. TREATMENT Treatment depends on : Stage of the disease Age at diagnosis Presence / absence of B symptoms Presence of hilar lymphadenopathy Presence of bulky nodal disease Current Treatment Regimen Combined chemotherapy with or without low dose involved field radiation therapy Medrockets.com

18. Chemotherapy Regimens MOPP ( Mechlorethamine , Vincristine , Procarbazine , Prednisolone) COPP (Cyclophosphamide , Vincristine , Procarbazine , Prednisolone) ABVD (Adriamycin , Bleomycin , Vinblastine , Dacarbazine ) BEACOPP ( For advanced stage disease ) ( Bleomycin , Etoposide , Doxorubicin , Cyclophosphamide , Vincristine , Procarbazine , Prednisolone) TREATMENT Medrockets.com

19. Therapy is entirely based on the stage. Localized disease ( stage IA and IIA ) is managed predominantly with radiation. All patients with evidence of ‘’B’’ symptoms as well as stage III and IV are managed with chemotherapy. The most effective combination chemotherapeutic regimen for Hodgkin lymphoma is ABVD ( adriamycin , bleomycin , vinblastin and dacarbazine ). ABVD is superior to MOP ( meclorethamine , vincristin ( oncovin ) , prednisolone and procarbazine ) because ABVD has fewer side effects such as: 1) Permanent sterility 2) Secondary cancer formation 3) Aplastic anemia 4) Peripheral neuropathy TREATMENT Medrockets.com

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 index depends on 5 factors: 1) The patient’s age 2) The stage of the lymphoma 3) Whether or not the lymphoma is in organs outside the lymph system 4) Performance status (PS) – how well a person can complete normal daily activities 5) The blood (serum) level of (LDH) Medrockets.com

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22. LONG TERM COMPLICATIONS Secondary malignancy Acute Myelogenous Leukemia Non Hodgkin lymphoma Carcinomas of breast , lungs & thyroid Short stature Hypothyroidism Sterility Dental caries Sub clinical pulmonary dysfunction Ischemic heart disease Medrockets.com