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Leukemias & Lymphomas
Keck Hematology
102
Medical
Graduate
02/15/2012

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Term
T vs. B ALL
Definition
T cell is worse than B cell ALL
Term
ALL Tx timeline
Definition
Induction-intensification 1 month, CNS prophylaxis, maintenance for 2-3 yrs
Term
AML Tx timeline
Definition
Induction- 1 mo, consolidation (post remission Tx) up to 6 mo
Term
Myeloperoxidase stain
Definition
helps distinguish acute myeloid leukemia
Term
CBF
Definition
core binding factor; normally activates txn; when fused to repressor complex, prevents difftiation of cells
Term
NPM1
Definition
nucleophosmin; shuttle ptn btwn nucleus and cytoplasm; common genetic event in AML, esp w/ age, usually in exon 12; favorable prognosis.
Term
MLL rearrangement
Definition
seen in infantile leukemia and 2' leukemia related to topo II inhibitors (etoposide); usually FABM4 or M5 phenotype, intermediate prognosis
Term
FLT3
Definition
trans-membrane TK receptor; may cause AML when duplicated such that it is no longer inhibitable
Term
arsenic trioxide
Definition
As2O3, used against APL, as a monotherapy its complete remission rate is 85%. With ATRA, it's even better.
Term
genetic locus associated w/ diabetes
Definition
DR3/DR4
Term
genetic locus associated w/ ankylosing spondylitis
Definition
B27
Term
CATCH-22
Definition
DiGeorge's syndrome: cardiac, abnormal faces, thymus, cleft palate, hypocalcemia, chromosome 22 microdeletions
Term
nitroblue tetrazolium test
Definition
test the ability of leukocytes to do a respiratory burst; negative in CGD
Term
when is neutropenia a problem
Definition
if absolute neutrophil count<300 due to marrow insufficiency
Term
leukemia associated with DIC
Definition
acute promyelocytic leukemia
Term
histology of CML
Definition
you'll see a variety of lineages and phases of difftiation, not just blasts.
Term
roth's spots
Definition
retinal spots seen in CGL/CML with leukocytosis and anemia
Term
Tx for chronic phase CML
Definition
delay blastic crises and comfort measures w/ chemo, IFNa, gleevec, HSC transplant
Term
most common childhood leukemia
Definition
pre-B ALL (AKA common leukemia)
Term
3 most common lymphomas
Definition
hodgkins, DLBCL, Follicular B Cell Lymphoma (FL)
Term
test to differentiate between lymphomas
Definition
flow cytometry; checks cell surface markers
Term
lymphoma/leukemia causing viruses
Definition
EBV-> Burkitt lymphoma, some Hodgkin? HTLV-1 -> adult T cell neoplasm
Term
Burkitt lymphoma genetic abnormality
Definition
8;14 translocation; EBV and malaria cause a translocation of myc from chr. 8 to chr 14 where it is overexpressed
Term
FCC lymphoma genetic abnormality
Definition
14;18 translocation upregulates Bcl-2; this doesn't usually accelerate growth, but it prevents cell death making for a slow-growing initial cancer
Term
schilling test
Definition
uses radiolabeled cobalamine to see if it comes out in urine or not. If not excreted, then there is an absorption (intrinsic factor, etc.) problem
Term
lymphadenopathy unlikely malignant IF:
Definition
lasts less than 2 weeks or over 1 year without growth
Term
follicles in malignant process indicates
Definition
B cells- T cell neoplasms DONT make follicles
Term
FISH and CHIPS
Definition
expensive, last-resort molecular tests for hard to identify leukemias/lymphomas that identify chromosomal abnormalities or protein expression abnormalities
Term
small cell lymphoma
Definition
slower growing, only a small percentage are dividing and susceptible to chemo; harder to cure
Term
large cell lymphoma
Definition
faster growing, more aggressive, high dividing percentage; easier to cure w/ chemo
Term
diffuse vs follicular B cell lymphomas
Definition
diffuse has a slightly worse prognosis; less difftiated
Term
6 major classes of lymphoma
Definition
precursor lymphoid, Mature B, Mature T or NK, Hodgkin, Immunodeficiency-associated, histiocytic/dendritic cell
Term
non-hodgkin lymphoma
Definition
mostly pts over 50, poor response to Tx, most of the lymph node is taken over by cancerous clonal cells
Term
Reed-Sternberg Giant Cells
Definition
hallmark of Hodgkins lymphoma; binucleate giant cells, produce and respond to all sorts of cytokines; mutated B cells that gather other reactive cells around them
Term
nodular sclerosis
Definition
fibrosis of lymph nodes caused by reed-sternberg cells releasing FGF
Term
surface markers of classical Reed Sternberg cells
Definition
CD30, CD15
Term
surface markers of lymphocyte predominant reed-sternberg cells
Definition
CD20, other B cell markers, not CD15 or 30
Term
CD19 presence on B cells
Definition
found on all stages of B cells except plasma
Term
CD20 presence on B cells
Definition
found on mature B cells, but not plasma cells
Term
tdt present in which cancers
Definition
terminal deoxytransferase only pre B and pre T cells (Blasts-acute cancers)
Term
tyrosine kinase inhibitors
Definition
imatinib or dasatinib are great for treating bcr-abl (philadelphia chromosome) ALL
Term
mediastinal mass is typical presentation
Definition
lymphoblastic lymphoma (LBL)
Term
lymphoma gold standard Dx
Definition
biopsy of whole lymph node
Term
bcl-1 associated cancer
Definition
mantle cell lymphoma
Term
bcl-2 associated cancer
Definition
follicular cell lymphoma
Term
c-Myc
Definition
Burkitt's lymphoma
Term
staging of Hodgkins lymphoma
Definition
I: single node. II: 2+ lymph nodes on same side of diaphragm. III: nodes on both sides of diaphragm. IV: diffuse outside of nodes too (liver, marrow, skin)
Term
owl-eye cell
Definition
reed-sternberg cell in hodgkins dz
Term
effect of cross linking cd20
Definition
downregulates Bcl-2; reduces resistance to apoptosis
Term
CLL
Definition
chronic lymphocytic leukemia; most common adult leukemia; mostly elderly males, often asymptomatic
Term
mycosis fungoides
Definition
T cell lymphoma that looks like a skin fungus
Term
LGL
Definition
indolent T cell lymphoma of CD8 cells usually treated just with immune suppression
Term
mantle cell lymphoma
Definition
aggressive B cell lymphoma; largely defined by 11;14 translocation and overexpression of cyclin D1, highly responsive to initial chemo but relapses are common. intermediate size cells, diffuse pattern w/ spleen, marrow, blood, GI. CD23+, mostly men
Term
surface markers of NK cell lymphoma
Definition
CD3 and CD56
Term
surface markers of anaplastic aggressive T cell lymphoma
Definition
CD3, CD30
Term
Sx of macroglobulinemia of waldenstrom
Definition
bleeding, CNS, CHF, sausaging of retinal veins; hyperviscosity
Term
casts in urine
Definition
indicate xs light chain production, think multiple myeloma
Term
Sx of multiple myeloma
Definition
CRAB: elevated Ca2+, Renal dysfn, Anemia, and Bone lesions
Term
to confirm Dx of multiple myeloma
Definition
serum protein electrophoresis (SPEP) shows M protein
Term
lymphoplasmacytic cells
Definition
dense, lymphocyte-like nucleus with the cytoplasm of a plasma cell; hallmark of macroglobulinemia of waldenstrom
Term
CD20+, CD3-, aggressive lymphoma
Definition
Burkitt's lymphoma has these characteristics
Term
Bence Jones Protein
Definition
Ig light chains in urine
Term
coexpression of CD4 and CD8 in periphery may indicate
Definition
T cell lymphoma
Term
On flow cytometry, Hodkins lymphoma resembles
Definition
a reactive proliferation (e.g. response to an infection)
Term
when CD8 cells outnumber CD4 cells, this may indicate
Definition
a loss of CD4+ cells, possibly HIV/AIDS
Term
Mantle Cell Lymphoma
Definition
t(11;14) translocation
- Pre-germinal
- Mantle cell
- Cyclin D1
- CD5
- Poor prognosis
Term
starry sky with oil red O
Definition
Burkitt's lymphoma
Term
Burkitt's lymphoma buzzwords
Definition
- “Starry sky,” Oil Red O
- Painful jaw (endemic)
- Swollen abdomen (non-endemic)
- EBV
- Overexpress c-myc
- t(8;14)
- children
Term
Follicular center cell lymphoma buzzwords
Definition
- Follicular pattern
- Butt cells/small cleaved
- Bcl-2 ->failure of apoptosis
- Splenomegaly
- CD20 (Ritxuimab/radiation)
- Disease of accumulation; indolent
- Huge lymphadenopathy
- T(14;18)
Term
Diffuse Large B cell Buzzwords
Definition
- Old
- Hashimoto’s/autoimmune disease
- Decreased lymphocytes
- CD 20
- Diffuse pattern despite germ ctr
- Bcl 6
- Open cytoplasm, large B cells
Term
MALT Lymphoma buzzwords
Definition
- H. pylori
- Hashimoto’s Thyroiditis
- t(11;18)
- Increase API
- Antibiotics
- CD25+
Term
Multiple myeloma buzzwords
Definition
#NAME?
Term
4 ways EPO works
Definition
increased progenitor division, less apoptosis, faster maturation in marrow of RBC precursors, early release of RBCs from marrow
Term
Leukemoid rxn
Definition
looks like leukemia on blood smear, including blasts and bands, but it's a benign response to an infection or other stressor
Term
prognostic indicators for follicular lymphoma
Definition
LASH: LDH elevation, Age> 60, Stage 3/4, Hb <12g/dl (anemia). 0-1 is low risk, 2 is intermediate, 3-4 is poor
Term
B symptoms
Definition
caused by IL-6, IL-1, others; Fever, night sweats, weight loss. worse prognosis, adds the "B" to stage
Term
DDx for mediastinal mass
Definition
4Ts: Thyroid, teratoma, Thymoma, Terrible lymphomas (T cell lymphoma, B cell lymphoma, Hodgkins)
Term
MOPP regimen
Definition
mechlorethamine, oncovin (vincristine), procarbazine and prednizone; great for Hodgkins
Term
ABVD
Definition
adriamycin, bleomycin, vinblastine, and dacarbazine; useful for Hodgkins dz to avoid mechlorethamine. good for LOW RISK pts
Term
CD68+ macrophages
Definition
indicate poor prognosis for Hodgkins dz
Term
S and E in Hodgkins staging
Definition
S: splenic involvement. E: extranodal involvement
Term
intermediate risk hodgkins Tx
Definition
standard BEACOPP
Term
high risk Hodgkins Tx
Definition
escalated BEACOPP
Term
Pel-Ebstein fever
Definition
recurrent high fevers every 3-4 days; indicates Hodgkins
Term
CD138
Definition
distinctive marker exclusive to plasma cells
Term
paraprotein
Definition
AKA M-protein; Ig product of multiple myeloma
Term
MGUS
Definition
monoclonal gammopathy of unknown significance; tends to precede amyloidosis, multiple myeloma, etc. usually in the range of 1g/dL or so.
Term
waldenstrom's macroglubulinemia
Definition
lots of IgM is produced by a low grade lymphoma-> increased viscosity of blood-> anemia, fatigue, neurologic Sx, bleeding, CHF.
Term
solitary osseous plasmacytoma
Definition
a single bone lesion caused by sheets of plasma cells, produces monoclonal Ab
Term
mechanism of bone damage in MM
Definition
myeloma cells bind stromal cells w/ vcams and integrins. RANKL is uncovered, and MM cell releases MIP1 and DKK1 activating osteoclasts
Term
B2M
Definition
beta 2 microglobulin; shed HLA light chain elevated by renal failure, important prognostic factor for MM,
Term
Dx of multiple myeloma
Definition
SPEP, Xrays, marrow aspirate/Bx, UA
Term
Acute leukemia
Definition
rapid onset (days, weeks) malignant clonal event and block to diffitiation= BLASTS. Must be treated immediately and aggressively
Term
Chronic Leukemia
Definition
Chronic onset (months years) w/ mature or difftiated cells
Term
Leucostasis
Definition
WBCs from leukemia occlude blood vessels, more characteristic of acute leukemia
Term
ALL
Definition
acute lymphoblastic leukemia; mostly in male children, curable in 70% of children, less so in adults
Term
AML
Definition
Acute myeloid leukemia; mostly in male adults, curable in a minority
Term
Definition of acute leukemia
Definition
presence of >20% blasts in bone marrow; confirmed w/ immunophenotyping, flow cytometry, molecular genetics, cytogenetics
Term
Auer rods
Definition
cytoplasmic inclusions laden w/ cytokines, indicative of AML
Term
APL
Definition
Acute promyelocytic leukemia w/ translocation 15;17 makes PML-RARa fusion gene that causes maturation arrest; lots of purple granules, low fibrinogen, granules initiate coag cascade-> DIC, >90% cure rate w/ ATRA
Term
ATRA
Definition
all trans retinoic acid, treats APL well, helps overcome block to difftiation
Term
T vs. B ALL
Definition
T cell is worse than B cell ALL
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