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17 Cards in this Set
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so we know how acute leukemias are divided - how are the chronic leukemias divied up and which should we know about? What's the basic concept of the error?
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In chronic blood cancers, it's NOT a problem with differentiation as in acute - here, it's a more general problem with DISORGANIZED PROLIFERATION - so find ADULT and BLAST cells, not just blasts.
when talking chronic, have to divide first into MYELOID and LYMPHOID. Our myeloid diseases have already been covered (CML, P. vera, ET, myelofibrosis). Lymphoid is divided into B and T cells: B cell = CLL/SLL (chronic lymphocytic leukemia, small lymphocitic lymphoma) Hairy Cell Myeloma. If it's T cell: ATCL (adult t cell leukemia) LGL (large granular lymphocitic leukemia) MF/SS (mycoses fungiodies/sezary syndrome) |
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talk about CLL/SLL - who gets it? characteristics on smear? treatment?
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most common leukemia in adults - NEVER IN CHILDREN. ALL = kids, CLL = adults.
SMUDGE CELLS B cell chronic leukemia. chronic lymphocitic leukemia: there are circulating cells, if some are in the lymph node, it's also SLL (small lymphocitic lymphoma, so the naming is arbitrary). LONG clinical history - usually discovered by mistake, can live many years. Some have B symptoms - night sweats, weight loss, fevers). may wait/watch, cured only with BMT, hard to do in old people. possible "richter transformation" into bad cancer. rare. because you're dealing with B cells, it's possible to get autoantibody hemolytic anemia. |
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what's a B symptom?
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night sweats, fevers, weight loss.
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Hairy Cell Lekemia?
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Also B cell origin.
TRAP test White dudes, >60 years "DRY TAP" |
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What's a lymphoma of plasma cells?
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plasma cells are terminally differentiated B cells that make antibodies - so think MULTIPLE MYELOMA.
key words: "M" Spike, hypercalcemia, lytic bone lesions, fried eggs, really high GAMMA GLOBULIN = "M" spike. *NOT CURABLE* MGUS = no big deal. |
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T - cell leukemias - talk about ATCL - what do cells look like, disease course
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adult T cell leukemia, caused by HTLV-1. Flower cells present, AGGRESSIVE disease
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Mycoises fungiodies/sezary syndrome?
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this is proliferating CD4 cells - tends to involve the skin = MF. it's sezary if it involves SKIN and BLOOD.
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LGL?
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large granular lymphocitic leukemia - this is CD8 cells, maybe NK cells.
NO big deal. |
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Generally, how is lymphoma divided?
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Hodgkin's from non-hodgkins (HD vs NHL) is determined by the presence of Reed Sternberg cells.
NHL is divided into B and T cells. Note that B is further divided into "large" and "small" cell groups |
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Hodgkin's lymphoma - who gets it, can it be cured, disease course, symptoms, how is its progress tracked,
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Reed Sternberg cells are how you know it's hodgkin's - these are dual cells with owls eye nuclei.
BIMODAL incidence - peaks in late teens and then again at age 60. Can have "b" symptoms - night sweats, weight loss, fevers. Swollen lymph node (Rubbery). It's very curable in early stages - know COMBO therapy (MOPP). Ann Arbor staging: I = one lymph node II = >1 lymph node, same side of diaphragm. III = both sides of diaphragm IV = extranodal invasion (excluding spleen) |
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what are the subsets of hodgkins we should know about?
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definitely know NODULAR SCLEROSIS - need to identify the slide of it. see FIBROTIC tissue "fibrous bands" dividing the follicle inside the LN into sections. Organized.
also lymphocyte depletion, lymphocite rich, nodular lymphocide predomiant HD (NLPHD). |
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how do we evaluate the prognosis of non hodgkins' lymphoma? what diseases fall into these groups?
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because bone marrow is already involved, generally, it's automatically stage 4.
we therefore use GRADING - this is more predictive. Kahn likes to divide the diseases up into Low, Medium, and High grades. Low = CLL/follicular Mid = Diffuse large cell High = Burkitt's NOTE - low grade is incurable, but can last decades and may not be treated. -Medium grade is curable, uses CHOP -High grade - burkitt's - emergent, complicated regiments. |
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with non-hodgkins, small B cell lymphomas, what are the diseses and key words we should know about?
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CLL/SLL - low grade, incurable.
Follicular - low grade, 14;18 translocation!. this ups BCL-2 Mantle - 11:14 translocation, up cyclin D Marginal zone - 11:18 translocation, mucosa Lymphoplasmocitid - this is plasma cell issue, so get high IgM = macroglobulinamia = HYPERviscosity syndromes (CNS, bleeding, visual problems) |
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onto the B cell, large lymphomas - what are our key words?
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Diffuse Large B Cell Lymphoma - immediately associate this with BCL-6 mutation, use of CHOP, potentially curable. "intermediate" grade. These can be in the body cavity (HHV8) or part of post-transplant lymphoproliferative disease.
Burkitt's - "high grade", 8:14 translocation. STARRY SKY, due to histeocytes eating up dead cells. "VACOULED CELLS" associated with EBV. Remember C-myc brought in front of the IgH promoter. |
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NHL branch that is T cell based - what are our diseases and what should we associate with them?
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ALCL - anaplastic Large Cell Lymphoma. T 2:5, mut in ALK. probably not on test.
Extranodal Nk/T cell lymphoma - fatal midline granulomas Peripheral T cell lymphoma - wastebasket for everything else. |
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What should we know about CD-marker staining in terms of hodgkins/non hodgkin's? Don't forget to talk about Reed Sternberg cells -
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All lymphocytes should stain positive for Cd45.
Note that Reed Sternberg cells stain negative for CD45, but ARE POSITIVE FOR CD15 and CD30 T cells are the early stain markers - 3, 4, 8. B cells are later: cd 20, etc. |
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what is a syndrome specifically associated with burkitt's?
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tumor lysis syndrome.
see the products you'd expect if cells were rapidly growing and then dying. high CPK high urate high potassium high vitamin K LOW calcium! |
