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lymphoma [2020/02/22 11:11] (current)
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 +====== lymphoma ======
  
 +see also:
 +  *[[neoplasia]]
 +  *[[ebv]]
 +  *[[https://​www.eviq.org.au/​haematology-and-bmt/​lymphoma|NSW Cancer Institute eviQ cancer therapy EBM website]] ​
 +
 +=====introduction=====
 +  *lymphomas are the 6th most common type of cancer excluding non-melanoma skin cancers with 1 in 39 people developing a NH lymphoma and 1 in 414 people developing Hodgkin'​s lymphoma by age 85yrs
 +  *incidence is rising in Australia (doubled in past 20 yrs)(([[https://​www.lymphoma.org.au/​page/​19/​types-of-lymphoma]]))
 +  *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
 +  *first identified by Thomas Hodgkin in the 1830's
 +  *defined by the presence of Reed-Sternberg cells (discovered in 1898 and 1902 by Reed and Sternberg)
 +  *it is thought to devlop from an abnormal response to [[EBV]] infection in those who are susceptible
 +  *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]] genome, esp. in:
 +    *patients with mixed cellularity tumours
 +    *young children
 +    *elderly
 +
 +====risk factors====
 +  *genetic:
 +    *twin with HL => 99x risk(([[http://​info.cancerresearchuk.org/​cancerstats/​types/​hodgkinslymphoma/​riskfactors/​|Cancer Research UK]]))
 +    *parent or sibling with HL or any blood or lymphatic cancer => 7x risk
 +  *infectious mononucleosis infection => 3x risk
 +    *EBV infection in people with homozygous CD70 protein gene defect (([[http://​www.news-medical.net/​news/​20161223/​Inherited-CD70-deficiency-increases-susceptibility-to-Epstein-Barr-virus-and-EBV-related-cancer.aspx]]))
 +  *immunosuppression:​
 +    *HIV/AIDS => 11x risk
 +    *organ transplantation
 +  *autoimmune diseases such as:
 +    *rheumatoid arthritis, systemic lupus erythematosus and sarcoidosis
 +  *patients with [[psoriasis]] have almost 2x risk (([[https://​www.news-medical.net/​news/​20200219/​Cancer-risk-in-psoriatic-patients.aspx]]))
 +  *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]]
 +    *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%
 +  ***classification**
 +    *before the discovery of B-cells and T-cells in the mid 1960s, Rappaport had developed a classification in 1956 which consisted of well-differentiated,​ poorly differentiated and histiocytic lymphomas
 +    *The Lukes-Collins classification was published in 1975 and attempted to relate cell morphology to immunologic function
 +    *The Working Formulation for classifying non-Hodgkin'​s lymphomas was published in 1982 and it quickly replaced the Rappaport and Lukes-Collins classification with the introduction of three prognostic groups: low, intermediate and high grade
 +    *the Revised European-American Classification of Lymphoid Neoplasms (REAL classification) was introduced in 1994 taking into account immunologic,​ genetic and clinical characteristics of the disorders and not solely histopathologic characteristics of the tumor cells. This was then adopted by WHO.
 +====risk factors====
 +  *[[EBV]] + malaria => endemic Burkitt'​s lymphoma in children
 +  *[[EBV]] + 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]] is associated with an increased risk of T-cell lymphoma specifically
 +  *patients with [[psoriasis]] have 50% increased risk - may be due to the higher risk of cutaneous T-cell lymphoma, which is markedly more common in people with severe psoriasis (([[https://​www.news-medical.net/​news/​20200219/​Cancer-risk-in-psoriatic-patients.aspx]]))
 +  *genetic:
 +    *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====
 +  ***B cell tumours**
 +    * Diffuse Large B Cell Lymphoma
 +    * **Follicular Lymphoma**
 +      * the most common sub-type of indolent NHL, comprising 20% to 30% of all NHL
 +      * typically affects middle-aged or older adult
 +      * a small percentage will transform into a more aggressive form of NHL, usually a DLBC
 +    * **Mantle Cell Lymphoma**
 +      *see [[lymphoma_mantlecell]]
 +      *2 types of MCL(([[https://​www.targetedonc.com/​publications/​evolving-paradigms/​2018/​mantle-cell-lymphoma/​diagnosis-staging-and-risk-in-mcl]])):​
 +        * classical MCL
 +          * usually composed of IGHV-unmutated B cells and SOX11 expression and involves lymph nodes and extranodal sites
 +        * leukaemic non-nodal MCL
 +          * generally composed of IGHV-mutated genes without SOX11 expression and involves the bone marrow, peripheral blood, and spleen; leukemic non-nodal MCL also typically has an indolent presentation
 +      * majority of people with MCL have stage IV disease at diagnosis
 +      *usually affects those over aged 50yrs (men have 2-7x risk) and accounts for 5-10% of NHL cases
 +      *increased risk of MCL is associated with certain autoimmune disorders, FH of hematopoietic malignancy, and Borrelia burgdorferi infection (Lyme disease)
 +    *Burkitt’s Lymphoma (BL) 
 +    *Chronic Lymphocytic Leukeamia (CLL)/Small Lymphocytic Lymphoma (SLL)
 +    *Marginal Zone Lymphoma (MZL)
 +    *Waldenstroms Macrogloulineamia (WM)
 +    *B Cell Acute Leukaemia/​Lymphoma (B-ALL)  ​
 +  ***T cell tumours**
 +    *Cutaneous T Cell Lymphoma (CTCL)
 +    *Anaplastic Large T Cell Lymphoma
 +    *Adult T Cell Lymphoma/​Leukaemia
 +    *Peripheral T Cell Lymphoma (PTCL) ​
 +    *T Cell Acute Leukaemia/​Lymphoma (T-ALL) ​
 +
 +
 +====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
 +
 +====grading of NHL====
 +  ***low grade, slow growing or indolent**
 +    * often can be watched conservatively with "​watchful waiting"​
 +    * premature Rx may cause unnecessary side effects, and worse, may make subsequent Rx more resistant and likely to fail
 +    * many follicular type NHLs fall into this category
 +  ***higher grade, faster growing**
 +    * usually need urgent Rx such as chemotherapy,​ steroids, monoclonal antibody targeted Rx, radiotherapy +/- stem cell transplant ​
lymphoma.txt · Last modified: 2020/02/22 11:11 (external edit)