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79 Cards in this Set
- Front
- Back
t (8; 21)
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good prognosis for AML-M2
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t (15;17)
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diagnostic and favorable for AML-M3
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Treatment is all-trans retinoic acid
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AML-M3
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Leukemia that often has DIC with it
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AML-M3
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What do Auer rods indicate?
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AML
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Which leukemia has a favorable prognosis with the absence of chromo 16?
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AML-M4-E0
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Buzzword: leukemic hiatus
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AML
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most common chronic leukemia in the western world
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CLL
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deletion of chromo 13 is favorable
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CLL
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trisomy 12 is a worse prognosis
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CLL
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CD markers for CLL
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19, 23, 5
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Richeters syndrome and life expectancy
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large cell lymphoma with life expectancy less than 1 yr
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chronic b cell leukemia with lymphadenopathy? and without?
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with: CLL w/o: Hairy B Cell
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treatment for hairy b cell
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chemo with purine analogs
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cytochemical stain for hairy b cell
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trap
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bone marrow feature of hairy b cell
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dry tap
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most remarkable feature of CBC for Hairy B Cell
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pancytopenia
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what are the four myeloproliferative disorders
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CML, idiopathic myelofibrosis, essential thrombocythemia, polycythemia vera
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CBC for CML
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leukocytosis and thrombocytosis
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What is the pathophys difference between the Ph chromo in ALL and CML?
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Ph in AML prevents maturation and is 190 kD while Ph in CML allows maturation and is 210 kD
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treatment for CML
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gleevac
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What is gleevac used to treat?
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CML
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What do smudge cells indicate?
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fragile lymphocytes which can be seen in ALL or CLL
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Treatment of CLL
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Rituximab
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Mechanism of Rituximab
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Ab that binds CD20
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What is the first type of MDS?
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Refractory anemia
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which category of diseases is commonly associated with cytogenetic or molecular abnormalities
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MPS and MDS
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what percent of siderblasts are required for RA to be called RARS
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>15% in the marrow
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median survival of RARS
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6 years
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median survival of RAEB 1? 2?
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18 mo/10 months
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what's special about -5q syndrome and survival
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it has thrombocytosis which is unusual because all of the other MDS's cause -penias; survival is very long
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(General Feature) In MPS, what does the bone marrow look like and what is the pathophys behind it?
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fibrosis due to cytokines from megakaryocytes
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what is the JAK2 mutation helpful in diagnosing
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PV (97%), ET (50-70%), CIMF (35-50%)
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most consistent physical finding in PV
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splenomegaly 70%; see hepatomegaly 40%
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LAP levels in PV
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increased
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WBC and PLT in PV
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increased
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prognosis of PV
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6-18 mo if untx. or >10 yrs if tx
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only lab value decreased on CBC of CIMF
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platelets
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DDx for dry tap
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hairy cell and CIMF
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How do you make the dx of essential thrombocythemia?
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It's a dx of exclusion
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Presenting Sx of essential thrombocythemia
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hemorrhage and thrombosis; hemorrhage because the platelets are bad platelets
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How common is it for ET to progress to marrow fibrosis relative to the other MPS's?
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very uncommon
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-4q12
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deltion activates a tyrosine kinase that is diagnositc for Chronic Eosinophilic Leukemia; this deletion is not present in Hyper Eosinophilic Syndrome though
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Tx for CEL
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Gleevac bc the 4q12 deletion activates a tyr kinase and gleevac can block it
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gene product of 4q12 deletion
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FIP1L1-PDGFRA gene product that creates a tyr kinase
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What are the 4 plasma cell dyscrasias discussed?
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MM, Plasmacytoma, Osteosclerotic Myeloma (POEMS), Waldenstrom's
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What distinguishes Multiple Myeloma from Plasmacytoma?
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MM has mutliple lesions while a plasmacytoma is a solitary lesion
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What are Bence Jones proteins?
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light chains that are secreted into the urine; note: it is very uncommon to see light chains in the plasma because they are readily filtered
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What is the MC lymphoid malignancy in African Americans in the US?
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MM
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What is the second most common lyphoid malignancy in the White US?
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MM
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Who is often the pt. with MM?
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elderly person
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What is the median survival of a pt with MM?
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3-5 yrs
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Unique clinical finding in MM?
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amyloidosis
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Presenting sx of pt with MM?
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recurrent infections
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What determines the prognosis in MM?
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results from cytogenetics/FISH; karyotype isn't useful however
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Dx Criteria for MM
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one minor and one major OR three minor
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Major criteria for MM
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>30% plasma cells in marrow, plasmacytoma on tissue biopsy, M spike above a certain amt
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Minor criteria for MM
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lytic lesions, 10%<x<30% plasma cells in marrow, M spike less than the set amount, reduced normal Ig's
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survival of MM
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3 year
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tx possibilities for MM
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BM transplant (MM takes over the transplant w/i 10 yrs hwvr), not currently curable, chemo, corticosteroids, alkylating agents
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What is lymphoplasmacytoid lymphoma?
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AKA Waldenstroms macroglobulinemia
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3 Distinguishing features of Waldenstrom's with MM
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Wal: (1) IgM excess (2) enlarges nodes and spleen (also catches BM) (3) causes hyperviscosity due to IgM being larger than IgG; MM is mostly IgG with no extramedullary involvement
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Dx of Waldenstrom's
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Electrophoresis and get M spike of IgM with Kappa chain
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what does POEMS stand for?
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polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes
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For the plasma cell dyscrasias, which one can cause anemia?
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MM
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Which cell line is increased in POEMS/Osteosclerotic Myeloma?
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platelets
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What is meant by paraprotein?
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an abnormal protein in the urine or blood, most often associated with mutiple myeloma; this is the M spike
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Which plasma cell dyscrasias can be diagnosed with the M protein elctrophoresed?
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MM and Waldenstroms
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prognosis of POEMS
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better than nomal plasma cell myeloma
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Which plasma cell dyscrasias typically have the paraprotein present?
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MM, Waldenstrom, and POEMS(very low levels)
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prognosis of plasmacytoma
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can be cured with radiation
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In which extraosseous location can a plasmacytoma develop most commonly?
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oropharynx, nasopharynx, sinuses, larynx
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What does AML-Eo stand for?
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AML with abnormal eosinophils
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What histochemically can be used to distinguish ALL from AML?
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ALL will be myeloperoxidase neg whiel AML will be positive
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What does a positive stain for bcl-1 indicate?
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mantle cell lymphoma
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What does a t (11:14) indicate and what is the protein produced?
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mantle cell lymphoma and bcl-1
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What does a t (14;18) indicate and which protein is expressed?
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follicular cell lymphoma and bcl-2
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What are the common cytogenetic disturbances in Hodgkins?
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There are none. Cytogenetic abnormalities are uncommon.
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What disease has Ig rearrangement abnormalities that can be picked up 98% of the time by PCR?
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Hodgkins
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