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79 Cards in this Set

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t (8; 21)
good prognosis for AML-M2
t (15;17)
diagnostic and favorable for AML-M3
Treatment is all-trans retinoic acid
AML-M3
Leukemia that often has DIC with it
AML-M3
What do Auer rods indicate?
AML
Which leukemia has a favorable prognosis with the absence of chromo 16?
AML-M4-E0
Buzzword: leukemic hiatus
AML
most common chronic leukemia in the western world
CLL
deletion of chromo 13 is favorable
CLL
trisomy 12 is a worse prognosis
CLL
CD markers for CLL
19, 23, 5
Richeters syndrome and life expectancy
large cell lymphoma with life expectancy less than 1 yr
chronic b cell leukemia with lymphadenopathy? and without?
with: CLL w/o: Hairy B Cell
treatment for hairy b cell
chemo with purine analogs
cytochemical stain for hairy b cell
trap
bone marrow feature of hairy b cell
dry tap
most remarkable feature of CBC for Hairy B Cell
pancytopenia
what are the four myeloproliferative disorders
CML, idiopathic myelofibrosis, essential thrombocythemia, polycythemia vera
CBC for CML
leukocytosis and thrombocytosis
What is the pathophys difference between the Ph chromo in ALL and CML?
Ph in AML prevents maturation and is 190 kD while Ph in CML allows maturation and is 210 kD
treatment for CML
gleevac
What is gleevac used to treat?
CML
What do smudge cells indicate?
fragile lymphocytes which can be seen in ALL or CLL
Treatment of CLL
Rituximab
Mechanism of Rituximab
Ab that binds CD20
What is the first type of MDS?
Refractory anemia
which category of diseases is commonly associated with cytogenetic or molecular abnormalities
MPS and MDS
what percent of siderblasts are required for RA to be called RARS
>15% in the marrow
median survival of RARS
6 years
median survival of RAEB 1? 2?
18 mo/10 months
what's special about -5q syndrome and survival
it has thrombocytosis which is unusual because all of the other MDS's cause -penias; survival is very long
(General Feature) In MPS, what does the bone marrow look like and what is the pathophys behind it?
fibrosis due to cytokines from megakaryocytes
what is the JAK2 mutation helpful in diagnosing
PV (97%), ET (50-70%), CIMF (35-50%)
most consistent physical finding in PV
splenomegaly 70%; see hepatomegaly 40%
LAP levels in PV
increased
WBC and PLT in PV
increased
prognosis of PV
6-18 mo if untx. or >10 yrs if tx
only lab value decreased on CBC of CIMF
platelets
DDx for dry tap
hairy cell and CIMF
How do you make the dx of essential thrombocythemia?
It's a dx of exclusion
Presenting Sx of essential thrombocythemia
hemorrhage and thrombosis; hemorrhage because the platelets are bad platelets
How common is it for ET to progress to marrow fibrosis relative to the other MPS's?
very uncommon
-4q12
deltion activates a tyrosine kinase that is diagnositc for Chronic Eosinophilic Leukemia; this deletion is not present in Hyper Eosinophilic Syndrome though
Tx for CEL
Gleevac bc the 4q12 deletion activates a tyr kinase and gleevac can block it
gene product of 4q12 deletion
FIP1L1-PDGFRA gene product that creates a tyr kinase
What are the 4 plasma cell dyscrasias discussed?
MM, Plasmacytoma, Osteosclerotic Myeloma (POEMS), Waldenstrom's
What distinguishes Multiple Myeloma from Plasmacytoma?
MM has mutliple lesions while a plasmacytoma is a solitary lesion
What are Bence Jones proteins?
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
What is the MC lymphoid malignancy in African Americans in the US?
MM
What is the second most common lyphoid malignancy in the White US?
MM
Who is often the pt. with MM?
elderly person
What is the median survival of a pt with MM?
3-5 yrs
Unique clinical finding in MM?
amyloidosis
Presenting sx of pt with MM?
recurrent infections
What determines the prognosis in MM?
results from cytogenetics/FISH; karyotype isn't useful however
Dx Criteria for MM
one minor and one major OR three minor
Major criteria for MM
>30% plasma cells in marrow, plasmacytoma on tissue biopsy, M spike above a certain amt
Minor criteria for MM
lytic lesions, 10%<x<30% plasma cells in marrow, M spike less than the set amount, reduced normal Ig's
survival of MM
3 year
tx possibilities for MM
BM transplant (MM takes over the transplant w/i 10 yrs hwvr), not currently curable, chemo, corticosteroids, alkylating agents
What is lymphoplasmacytoid lymphoma?
AKA Waldenstroms macroglobulinemia
3 Distinguishing features of Waldenstrom's with MM
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
Dx of Waldenstrom's
Electrophoresis and get M spike of IgM with Kappa chain
what does POEMS stand for?
polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, skin changes
For the plasma cell dyscrasias, which one can cause anemia?
MM
Which cell line is increased in POEMS/Osteosclerotic Myeloma?
platelets
What is meant by paraprotein?
an abnormal protein in the urine or blood, most often associated with mutiple myeloma; this is the M spike
Which plasma cell dyscrasias can be diagnosed with the M protein elctrophoresed?
MM and Waldenstroms
prognosis of POEMS
better than nomal plasma cell myeloma
Which plasma cell dyscrasias typically have the paraprotein present?
MM, Waldenstrom, and POEMS(very low levels)
prognosis of plasmacytoma
can be cured with radiation
In which extraosseous location can a plasmacytoma develop most commonly?
oropharynx, nasopharynx, sinuses, larynx
What does AML-Eo stand for?
AML with abnormal eosinophils
What histochemically can be used to distinguish ALL from AML?
ALL will be myeloperoxidase neg whiel AML will be positive
What does a positive stain for bcl-1 indicate?
mantle cell lymphoma
What does a t (11:14) indicate and what is the protein produced?
mantle cell lymphoma and bcl-1
What does a t (14;18) indicate and which protein is expressed?
follicular cell lymphoma and bcl-2
What are the common cytogenetic disturbances in Hodgkins?
There are none. Cytogenetic abnormalities are uncommon.
What disease has Ig rearrangement abnormalities that can be picked up 98% of the time by PCR?
Hodgkins