NHL – clinical

question

what is the most common subtype of NHL? the next after that?
answer

diffuse large B cell lymphoma, followed by follicular lymphoma
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how are follicular lymphomas categorized in terms of grade?
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follicular lymphoma 1,2 are low grade while 3 is high grade and is aggressive
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who does lymphoma affect most commonly?
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it makes up 5% of total malignancies, (4-5th most common CA), incidence per year seems to be increasing, it has a higher rate in whites, and of them, men are most affected. there is some family association. people who are immunosuppressed are at an increased risk for lymphoma (organ transplant), and usually get very aggressive NHL.
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is there a correlation between anti-TNF alpha tx and lymphomas?
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yes, people treated for rheumatologic diseases with anti-inflammatory/anti-TNF antibodies – there is starting to be an increase among this group
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what lymph nodes are particularly important to examine when performing a physical?
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*peripheral, cervical, axillary, groin
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other than checking lymph nodes in the physical, what is another universal must for diagnosing lymphomas?
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complete blood count/blood smear
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how does flow cytometery fit into lymphoma dx?
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this will be standardized soon, it is sensitive to antibody markers, and allows the pathologist to recognize the immunophentotypic signature and be able to distinguish the form of lymphoma
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what is significant to look for in evaluating liver function in lymphoma pts?
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LDH (measures tissue breakdown)
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what tests are standard for lymphoma (NHL/HL)?
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CAT scans, bone marrow bx, pet scans
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what are the lab tests ordered for NHL?
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CBC, chemistries, Ca/PO4/creatinine/LDH/B2M, SPEP(**some subtypes of lymphomas present with elevation of various Igs, IgM = higher viscosity), cryoglobins, and DAT. (serum protein electrophoresis = SPEP)
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what diseases are there increased risks of NHL with?
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sjogren’s, RA, SLE, h. pylori (MALToma)
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how does NHL present clinically?
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lymphadenopathy (most common), bone marrow involvement (usually low grade), extranodal involvement (often in skin), fever/night sweats/weight loss (B symptoms), asymptomatic, and ureter/bowel obstruction (doesn’t take a high sized tumor to do this, but in the abdomen big tumors can cause hydronephrosis)
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what is absolutely necessary for a good lymphoma dx?
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a good path specimen (usually unilateral bone marrow bx), flow cytometry, and cytogenetics <= for every pt!
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what is needed for good staging of NHL? how many stages are there for NHL?
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CT scans, PET scans. (gallium scan NOT USED ANYMORE). staging for NHL is the same as HL -> 1-4
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what are some characteristics of indolent NHLs?
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these are generally disseminated at dx (lymph nodes, spleen, bone marrow, peripheral blood), small lymphocytes (usually B cell lineage), relatively long median survival, and they are sensitive to chemotherapy/radiation (but not with permanent effect and each successive tx has less effect)
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what is someone with lymphadenopathy/splenomegaly positive until proven otherwise?
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lymphoma/leukemia
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what is follicular lymphoma?
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the “flagship” of lymphomas. it is indolent, peaks in 5-6th decade, seen equally in both sexes, affects more whites and is caused by t(14:18) associated with BCL2 gene <- know this
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what is the FLIPI?
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the follicular lymphoma international prognostic index which is used to rate pts according to level of prognostic factors – the less they have the better their
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what are the prognostic factors for the FLIPI?
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nodal site >4 (bilaterals close together counts as 1), elevated LDH, age >60, stage 3 or 4, and hgb <120 g/dL (LANSH)
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why are most pts dx with follicular lymphoma not treated initially?
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there is no evidence that treatment at the time of dx improves the outcome. if chemotherapy is not curative (and it isnt w/follicular lymphoma), then it will just make them sicker/give them side effects
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when is treatment given to pts with follicular lymphoma?
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when pts are symptomatic; doubling time increase, B symptoms:weight loss, night sweats, or intrabdominal disease that is impinging/causing problems w/normal body function, or cosmetic reasons
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what is rituximab?
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an anti-CD20 monoclonal antibody that everyone gets. it doesn’t cause hair loss/nausea/vomiting/drop in blood counts – it simply hones in on the CD20 and destroys lymphomas
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what is rituximab usually paired with?
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CVP: cyclophosphamide, vinicristine and prednisone = very effective anti-lymphoma tx
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what is bortezomib?
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usually used in tx of multiple myelomas, this is being considered for NHL
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what is bendamustin?
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a newer agent, unknown M/A and is very good for lymphomas
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what is radioimmunotherapy?
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zevalin and bexxar are both antibodies that target CD20 and deliever radioactive moeities to lymphomas (can put disease in remission for ~18 mos)
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what is the most common variant of NHL? is it aggressive? is there a genetic component? what is the median pt age affected by this?
answer

diffuse large B cell lymphoma which composes 40% of all B cell lymphomas and is aggressive. *c-myc is mutates in up to 15% of cases. most pts are in mid 60s
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what is the standard tx for DLBCL? what if the disease is recurrent?
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R-CHOP; combination chemotherapy that includes rituximab (R). bone marrow transplants are indicated for recurring iterations
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what is the IPI? what does it consist of?
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international prognostic index for aggressive lymphomas (including DLBCL) consisting of age, performance status, LDH, extranodal sites and stage (APLES). 4-5 of these = poor prognosis
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what is burkitt’s lymphoma?
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a very aggressive NHL derived from post germinal center B cells. it features rapid proliferation, spontaneous cell death, a starry sky appearance and a translocation of c-myc t(8:14)
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where is the endemic form of burkitt’s lymphoma?
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africa (there is also a sporadic one seen in other parts of the world)
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are immunosuppressed pts at more of a risk for burkitt’s lymphoma?
answer

yes
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can burkitt’s lymphoma be cured?
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yes, through high/prolonged doses of chemotherapy. the disease invariably invades the CNS, so pts need intrathecal cheom and stem cell transplants. pts will be come sterile and there is no time for sperm banking
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what are advantages for radioimmunotherapy in treatment of NHL?
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lymphoma cells are inherently sensitive to radiotherapy and even if tumors are bulky/poorly vascularlized, tumor cells distant from bound antibody can still be affected by ionizing radiation
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what is a disadvantage of radiotimmunotherapy?
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prolonged myelosuppression
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what are the 2 radioimmunosuppressive agents? what isotopes do they use?
answer

zevalin (atrium 90) and bexxar (radioactive iodine)
question

how is a typical radioimmunotherapy tx given?
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a little retuximab is given to clear most circulating blood cells. a small amount of zevlin (ibritumomab) is given to get image recognition that radiation will go to the right place. a few days later the radio labeled antibodies are given
question

even though T cell lymphomas are rarer, why are they still very important?
answer

they can be very aggressive malignancies (with the exception of mycosis fungiodies, sezary, primary cutaneous CD30+, and T cell large granular lymphocytic
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what is the most common type of T cell lymphoma?
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the peripheral T cell lymphoma (not otherwise specified)
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what does it mean if someone is ALK+?
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they have a very good prognosis if they get anaplastic large cell lymphoma
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has there been an increase in T cell lympoma since the 1990s?
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yes, likely due to better pathology
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how do T cell lymphoma pts (except for ALK+ w/anaplastic LCL) compare to intermediate B cell lymphoma pts (DLBCL) pronosis-wise?
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most T cell lymphoma pts will do worse than those w/DLBCLs
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what is the distinction between cutaneous and peripheral T cell lymphomas?
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cutaneous T cell lymphomas are usually indolent and only require local tx by a dermatologist unless it transforms or if it is sezary syndrome
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what level of disease do T cell lymphoma pts usually present at?
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advanced stage, usually with systemic symptoms (disseminated disease, B symptoms, bone marrow +, and skin lesions)
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can IPI be used for T cell lymphomas?
answer

yes
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what are markers for T cell lymphomas?
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CD 2,3,5,8. (CD 15/30 are used b/c those are activated T/B cells)
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what treatments are used commonly for PTCL?
answer

CHOP or hyper-CVAD

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