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REFERENCES
• Ghai O, Paul V, Bagga A. Essential Pediatrics. 7th ed. CBS
Publisher & Distributers; 2008.
• Lymphoma in Children [Internet]. Stjude.org. 2021 [cited 16 May 2021]. Available
from:
https://www.stjude.org/disease/lymphoma.html#:~:text=Childhood%20lymphoma%2
0is%20rare.,under%20the%20age%20of%2014.
• Lymphoma Action | Lymphoma in children [Internet]. Lymphoma Action. 2021 [cited
16 May 2021]. Available from: https://lymphoma-action.org.uk/types-lymphoma-
lymphoma-children-and-young-people/lymphoma-children.
• https://doi.org/10.1093/ije/24.1.27
2/13/2023 46](https://image.slidesharecdn.com/5-230213164549-418bb1cb/85/5-lymphomas-pptx-46-320.jpg)
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5. lymphomas.pptx
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- 2. z General Objective Atthe end of the session, participants will be able to explain about lymphoma. 2/13/2023 2
- 3. z Specific objectives Atthe end of the session, participants will be able to: - Define lymphoma - Explain the epidemiology of lymphomas - Classify lymphoma - List the causes of lymphoma 2/13/2023 3
- 4. z Specific objectives - Enlistthe clinical manifestations of lymphoma - State the diagnostic criteria lymphoma - Explain the management of lymphoma - State the prognosis of lymphoma 2/13/2023 4
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- 8. z Lymphoma Lymphoma isany cancer that starts in the lymphatic system. It can develop when a type of white blood cell called a lymphocyte starts to grow in an abnormal, uncontrolled way.
- 9. z Epidemiology - 3rd mostcommon cancer in children - Incidence is 15 per million children
- 10.
- 11. z Hodgkin’s Lymphoma Hodgkin’sdisease (HD) is a malignant disorder of lymphoreticular system; a lymphoreticular neoplasm primarily of B cell lineage involving lymph nodes.
- 12. z Hodgkin disease: Malignant processof lymphoreticular system 6% of childhood cancer 5% of cancer in < 14 yr 15% in person 15-19 yr Rare < 10 yr
- 13. z Epidemiology: Bimodal incidence Second peakafter 50 y Sex: Male > Female Associated with specific HLA antigen Infectious agents Human herpes virus 6 Cytomegalovirus Epstein – Barr virus Immunodeficiency
- 14. z Clinical Manifestations Lymphadenopathy cervical/ supraclavicular Painless, non tender, firm and rubbery Hepatosplenomegaly Cough, dyspnea, hypoxia Pleural or pericardial effusion Heptocellular dysfunction Bone marrow infiltration (Anemia, neutropenia, thrombocytopenia)
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- 17. Mediastinal Mass inHodgkin Disease
- 18. z 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
- 19. z DIAGNOSIS Excisional Biopsy Light Microscopy Immunocytochemistry MolecularStudies Chest X – Ray Mediastinal Mass CT Scan Chest Abdomen Pelvis Blood & ESR LFT’s Bone Marrow Aspiration Serum Copper & Ferritin Bone Scan PET
- 20. z Reed –Sternberg cellHallmark of disease.
- 21. z Ann Arbor StagingClassification for Hodgkin Disease ▪ Stage I Involvement of a single lymph node (1) or of a single extra lymphatic site or organ(1f) • 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 (IIf)
- 22. z STAGING StageIII Involvement of lymph 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.
- 23. z Staging The absenceor 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.
- 24. z TREATMENT Treatment depends on: Stage of 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.
- 25. z 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)
- 26. z P00R PROGNOSTIC FACTORS Tumor Bulk Advancedstage at diagnosis Presence of B symptoms
- 27. z LONG TERM COMPLICATIONS Secondarymalignancy Acute Myelogenous Leukemia Non Hodgkin lymphoma Carcinomas of breast , lungs & thyroid Short stature Hypothyroidism Sterility Dental caries Sub clinical pulmonary dysfunction Ischemic heart disease
- 28. z PROGNOSIS Early Stage Disease 5year survival ….95% Advanced Stage Disease 5 year survival ….90% Relapses common within first 3 years from diagnosis Relapses treated with Autologous Stem Cell Transplantation
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- 30. z EPIDEMIOLOGY 60% ofall lymphomas in children 8-10% of all malignancies in children between 5- 19 yrs of age Secondary causes of NHL include; Inherited / acquired immune deficiencies Viruses HIV EBV Genetic Syndromes Ataxia Telangiectasia Bloom syndrome
- 31. z CLINICAL MANIFESTATIONS Lymphadenopathy Superior venacava syndrome Dyspnea Abdominal Mass Intestinal obstruction / intussusception Ascites Nasal Stuffiness Earache Tonsil enlargement Localised bone involvement Acute paraplegia secondary to CNS / spinal cord compression Tumor Lysis Syndrome
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- 34. z Staging system forchildhood Non- Hodgkin lymphoma Stage I II Description A single tumor (extranodal) or single anatomic area (nodal) with the exclusion of mediastinum or abdomen A single tumor (extranodal) with regional node involvement two or more nodes areas on the same side of diaphragm Two single (extranodal) tumors with or without the regional node involvement on same side of diaphragm
- 35. z Stage II Aprimary gastrointestinal tract tumor usually in the ileocecal area, with or without involvement of associated mesenteric nodes, which may must be grossly ( > 90%) resected
- 36. z Stage III Two singletumors (extranodal) on opposite side of the diaphragm Two more nodal areas above and below the diaphragm Any primary intarthoracic tumor (mediastinal, pleural, or thymic) Any extensive primary intra – abdominal disease IV Any of the above, with initial involvement of central nervous system or bone marrow t time of diagnosis
- 37. z DIFFERENTIAL DIAGNOSIS Hodgkin Disease Leukemia Germ CellTumor Wilms Tumor Neuroblastoma Reactive lymphadenitis
- 38. z LABORATORY FINDINGS Tissue biopsyfor; Flow cytometry Karyotyping Complete Blood Count Serum Electrolytes, Calcium , Phosphorus , Uric acid LFT’s & RFT’s Bone Marrow Aspiration & Biopsy CSF Examination Chest X Ray CT Scan Head & Neck Chest Abdomen & Pelvis PET Scan & Bone Scan
- 39. z TREATMENT Systemic Chemotherapy Intrathecal chemotherapy Radiotherapyindicated in ; CNS Disease SVC Syndrome Paraplegia
- 40. z Chemotherapy Regimens ▪ COPAD (Cyclophosphamide, Vincristinr , Prednisolone , Doxorubicin) ▪ COMP (Cyclophosphamide , Vincristine , Methotrexate , 6 Mercaptopurine , Prednisolone)
- 41. z Duration of Treatment Burkitt Lymphoma & Diffuse Large B Cell Lymphoma ………. 6 weeks to 6 months Lymphoblastic Lymphoma …..24 months
- 42. z Supportive Treatment G-CSF prophylaxisfor fever & neutropenia Antibiotic prophylaxis Blood & platelet transfusions Allopurinol Parenteral nutrition
- 43. z COMPLICATIONS Infections Mucositis Pancytopenia Electrolyte imbalance Poor nutrition Growthretardation Cardiac Toxicity Gonadal Toxicity with Infertility Secondary malignancies
- 44. z PROGNOSIS ▪ Localized disease 90– 100% survival ▪ Advanced Disease 60-95% survival
- 45.
- 46. z REFERENCES • Ghai O,Paul V, Bagga A. Essential Pediatrics. 7th ed. CBS Publisher & Distributers; 2008. • Lymphoma in Children [Internet]. Stjude.org. 2021 [cited 16 May 2021]. Available from: https://www.stjude.org/disease/lymphoma.html#:~:text=Childhood%20lymphoma%2 0is%20rare.,under%20the%20age%20of%2014. • Lymphoma Action | Lymphoma in children [Internet]. Lymphoma Action. 2021 [cited 16 May 2021]. Available from: https://lymphoma-action.org.uk/types-lymphoma- lymphoma-children-and-young-people/lymphoma-children. • https://doi.org/10.1093/ije/24.1.27 2/13/2023 46
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Editor's Notes
- #10 Two broad categories Hodgkin disease Non- Hodgkin disease
- #12 and the lymphatic system has unique molecular, histologic, immune phenotypic and clinical features. The lymphoreticular system consists of the spleen, lymphnodes, lymphatic vessels, thymus, and bone marrow
- #14 Bimodal: having 2 mode 4: 1 for 3-7 yr 3: 1 for 7-9 yr 1-3: 1 for > 10 yr Human leukocyte Antigen
- #15 Disease below diaphragm is rare (only3%)
- #19 Drenching: soak Anergy: absence of the normal immune response to a particular antigen or allergen.
- #20 A positron emission tomography (PET) scan is an imaging test that allows your doctor to check for diseases in your body. The scan uses a special dye containing radioactive tracers. These tracers are either swallowed, inhaled, or injected into a vein in your arm depending on what part of the body is being examined fluorodeoxyglucose (FDG)-positron emission tomography (PET). The role of this procedure is to detect metabolically active .
- #21 Large (15-45 m) multiple / multilobulated nuclei. Colonal in origin. Arises from germinal center B cells.
- #25 Hilar lymphadenopathy is a common radiological finding associated with fungal infections, mycobacterial infections, and sarcoidosis. However, it is rarely seen in viral pneumonia.
- #29 Sometimes, despite the best care and significant progress made in treatment, cancer comes back. When this happens it is called a recurrence or relapse.
- #31 Ataxia Telangiectasia : Ataxia telangiectasia (A-T) is an autosomal recessive disorder primarily characterized by cerebellar degeneration, telangiectasia, immunodeficiency, cancer susceptibility and radiation sensitivity. A-T is often referred to as a genome instability or DNA damage response syndrome Bloom syndrome (BSyn) is a rare genetic disorder characterized by short stature; a sun-sensitive, red rash that occurs primarily over the nose and cheeks; mild immune deficiency with increased susceptibility to infections; insulin resistance that resembles type 2 diabetes; and most importantly, a markedly increased
- #40 Superior vena cava syndrome (SVCS) happens when the superior vena cava is partially blocked or compressed. Swelling of your face, neck, upper body, and arms Trouble breathing or shortness of breath Coughing