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2016/06/05 08:48 1/4 lymphoma lymphoma see also: neoplasia EBV / glandular fever / infectious mononucleosis introduction 85% of malignant lymphomas are non-Hodgkins lymphomas (NHL) median age at diagnosis is in 6th decade with exception of Burkitt lymphoma and lymphoblastic lymphoma which occur in younger patients Hodgkin's lymphoma accounts for 12% of cases of lymphoma most patients are young adults (20-35yrs old) or in the 70's. 5 year survival with Rx: stage I/II 90% stage III 84% stage IV 65% ~50% contain EBV / glandular fever / infectious mononucleosis genome, esp. in: patients with mixed cellularity tumours young children elderly risk factors genetic: twin with HL ⇒ 99x risk1) parent or sibling with HL or any blood or lymphatic cancer ⇒ 7x risk infectious mononucleosis infection ⇒ 3x risk immunosuppression: HIV/AIDS ⇒ 11x risk organ transplantation autoimmune diseases such as: rheumatoid arthritis, systemic lupus erythematosus and sarcoidosis childhood affluence (presumably via delay in acquiring EBV, or less childhood infections resulting in altered immunity) PH Non-Hodgkin's lymphoma ⇒ 4-16x risk some alcohol consumption appears to reduce risk as does being breastfed clinical features painless and slowly progressive, rubbery lymphadenopathy OzEMedicine - Wiki for Australian Emergency Medicine Doctors - http://www.ozemedicine.com/wiki/ Last update: 2014/09/09 23:20 lymphoma http://www.ozemedicine.com/wiki/doku.php?id=lymphoma 80% are above the diaphragm, 60-80% involve neck, <20% axillary, <20% groin <10% have pain at nodal sites precipitated by drinking alcohol if massive mediastinal nodes, may develop superior vena cava syndrome extranodal involvement splenomegaly hepatosplenomegaly 40% have B symptoms T > 38°C, night sweats weight loss >10% from baseline within 6 months fatigue/weakness 35% have intermittent fever some have classic Pel-Ebstein fever high fever for 1-2 wks followed by an afebrile period of 1-2 wks pruritus chest pain, cough &/or SOB paraneoplastic syndromes rarely: bone/back pain haemoptysis non-Hodgkin's lymphoma 85% of NHLs are of B-cell origin 15% are derived from T/NK cells the remainder arise from macrophages 5 year survival is just over 60% risk factors EBV / glandular fever / infectious mononucleosis + malaria ⇒ endemic Burkitt's lymphoma in children EBV / glandular fever / infectious mononucleosis + immunosuppression ⇒ post-transplant lymphoma 13% of heart, and 33% of heart/lung recipients have been reported to develop NHL The latest meta-analysis showed an 8-fold increased risk of NHL in transplant recipients ⇒ HIV-related lymphoma HIV is 80x risk with 3-5% developing NHL and HIV accounts for 6% of NHL cases in developed countries EBV is present in 2/3rds of AIDS-related NHL strong association between Helicobacter pylori and lymphomas of mucosa-associated lymphoid tissue (MALT) in the stomach 4% of NHL cases in developed countries are due to infection with H.pylori Hepatitis C virus infection ⇒ 2-2.5x risk Human T-Cell Lymphoma Virus 1 causes a small number of cases of NHL in developed countries auto-immune conditions haemolytic anaemia, systemic lupus erythematosus and Sjogren syndrome coeliac disease is associated with an increased risk of T-cell lymphoma specifically genetic: http://www.ozemedicine.com/wiki/ Printed on 2016/06/05 08:48 2016/06/05 08:48 3/4 lymphoma FH NHL ⇒ 2x risk FH coeliac disease in a sibling ⇒ increased risk carcinogens: A meta-analysis of case-control studies reported a 35% increase in risk for people with occupational exposure to pesticides, increasing to 65% for more than 10 years' exposure Occupational exposure to benzene has been linked to a 22% increase in NHL risk in a meta-analysis obesity: a 40% increased risk of diffuse large B-cell NHL for people with a BMI of 30 or higher. sub-types of NHL Anaplastic Large Cell Lymphoma Follicular Lymphoma B Cell Lymphoma Diffuse Large Cell Lymphoma Diffuse Mixed Lymphoma Lymphoblastic Lymphoma Mantle Cell Lymphoma has the worst prognosis of the B-cell lymphomas characterised by over-expression of cyclin D1 and the dysregulation of the cell cycle Mycosis Fungoides Primary CNS Lymphoma Cutaneous T-Cell Lymphoma clinical features painless and slowly progressive lymphadenopathy cytopenias with advanced stages of low grade tumours, or early stages of high grade tumours: extranodal involvement esp. GI/GU tracts (including Waldeyer ring), skin, bone marrow, sinuses, thyroid, CNS splenomegaly hepatosplenomegaly testicular mass skin lesions (mycosis fungoides) B symptoms T > 38°C, night sweats weight loss >10% from baseline within 6 months fatigue/weakness 1) Cancer Research UK OzEMedicine - Wiki for Australian Emergency Medicine Doctors - http://www.ozemedicine.com/wiki/ Last update: 2014/09/09 23:20 lymphoma http://www.ozemedicine.com/wiki/doku.php?id=lymphoma From: http://www.ozemedicine.com/wiki/ - OzEMedicine - Wiki for Australian Emergency Medicine Doctors Permanent link: http://www.ozemedicine.com/wiki/doku.php?id=lymphoma Last update: 2014/09/09 23:20 http://www.ozemedicine.com/wiki/ Printed on 2016/06/05 08:48