Rapid Review 5 Flashcards
virus a/w ATLL
HTLV-1
virus a/w primary effusion lymphoma
HHV8
what are the 2 markers that are different btwn Sezary syndrome and ATLL?
CD25 and CD26: negative in Sezary
what 4 markers are the same between Sezary and ATLL?
CD3+
CD7-
CD4+
CD8-
what’s the chemical name for A antigen
N-acetyl-D-galactosamine
what places A antigen on the RBC?
alpha-3-N-acetyl-galactosaminyltransferase
what’s the chemical name for B antigen?
D-galactose
what places B antigen on the RBC?
alpha-3-D-galactosyltransferase
what’s the chemical name for O antigen?
aka H substance
L-fucose
what’s H substance?
O antigen
what places O antigen on the RBC?
alpha-2-fucosyltransferase
what are the 2 things that all of the ABO blood group antigens have in common, structurally?
fuc-alpha-1,2
gal beta 1,3 GlcNAc
if you see “popcorn” cells, think of THIS
nodular lymphocyte-predominant Hodgkin’s lymphoma
what type of cells “ring” the popcorn cells?
PD1+
CD279+
T-cells!!!!!!!!!
what is the biggest difference between RS and popcorn cells?
RS cells: 15+/30+
popcorn cells: 15-/30-
all Burkitt lymphomas have translocations involving chromosome 8. what gene is involved on chrom 8?
c-MYC
what are the 2 new markers that CLL is positive for? on flow
CD200
LEF1
what is the confusing mimic for hairy cell in a peripheral smear?
circulating villous lymphocytes in splenic MZL (usu not entirely circumferential)
how does hairy cell look in the bone marrow?
interstitial lymphoid aggs or diffuse involvement
LOTS OF FIBROSIS
what 2 IHC/flow markers are positive in Hairy cell as a result of BRAF V600E mut?
TRAP and CD25
caribbean lymphoma
ATLL
ATLL is a/w what virus?
HTLV1
virus a/w extranodal NK/T cell lymphoma
EBV
what’s this
hallmark cell
ALCL
what is the neoplastic cell in AITL?
T follicular helper cells
what happens to B cells in AITL?
TFH cells cause differentiation/activation of B cells
B cells are usu EBV+
what are the SENSITIVE IHCs for AITL?
PD-1 & ICOS
what are the SPECIFIC IHCs for AITL?
CXCL13 > CD10
what are CD8+ T cells doing in AITL?
they have an “exhausted phenotype”
POS for:
- CD8
- CD45RO
- CD27
- PD-1
- TIGIT
- ICOS
- TIM3
90% of AITL cases have this broad abnormality in molecular testing
karyotypic abnormalities
tri 3/5/19
gain of X
driver mutation identified in ~70% of AITL cases
RHOA p.Gly17Val
AITL has 4 genes that are most commonly mutated. what are they?
- RHOA (driver in ~70%)
- TET2
- DNMT3
- IDH2-R172
MC mutation in ALCL & what chromosome is the gene on?
ALK
chromosome 2
2 characteristic findings for follicular dendritic cell sarcoma
1 morpho & 1 IHC
morpho: whorled spindle cells
IHC: CD35+ (and CD21+)
3 mutations that you can see in hemophagocytic syndrome
PRF1
UNC13D
STX11
(PRooF of your UNCle on the STyX)
what is the super specific lab finding in HLH?
soluble CD25 > 2400 U/mL
what are endothelial cells positive for in bacillary angiomatosis?
ulex europaeus
which is also the lectin for H antigen
what’s this
ring granuloma
Q fever - Bartonella quintana/henselae
what’s the disease & the 2 specific findings in this pic?
lupus/SLE
hematoxylin bodies + karyorrhectic debris/coagulative necrosis
which one has eos?
kikuchi or kimura
kimura
which one has plasmacytoid dendritic cells?
kikuchi or kimura
kikuchi
which 4 genetic alterations can you use to diagnose AML even if the blast count is less than 20%?
- t(8;21) RUNX1/RUNX1T1
- t(16;16) CBFB/MYH11
- inv(16) CBFB/MYH11
- t(15;17) PML/RARA
what IHC can you use to clue you into a KIT mutation?
CD56
25% of patients with t(8;21) RUNX1-RUNX1T1 have a mutation in THIS gene
KIT (it makes it a worse prognosis)
which heme malignancy is a/w abnormal eos with basophilic granules?
AML with inv(16) or t(16;16) CBFB-MYH11
what 2 extra mutations confer a worse prognosis in AML with t(16;16) or inv(16)?
KIT mutations
FLT3-ITD
which heme malignancy do you treat with ATRA?
AML with t(15;17) PML-RARA
there are 2 things that will confer resistance to ATRA. what are they?
variant fusions involving RARA and either:
ZBTB1 (11q23)
or
STAT5 (17q11)
AML with mutated NPM1 usually has a mutation where?
exon 12
:(
concurrent mutation in THIS negates any good prognosis that an NPM1 mutated AML might have
FLT3-ITD
what is the latent period between therapy & the development of therapy-related AML/MDS/MDS-MPN if you are treated with an alkylating agent?
5-7 years
what are the therapies a/w therapy-related myeloid neoplasms?
radiation/alkylating agents
topoisomerase II inhibitors
mutations in THIS GENE are pathognonomic for down-syndrome related myeloid proliferations
GATA1
what IHC stain is a surrogate marker for FLT3 mutation?
CD117
chronic neutrophilic leukemia gene
CSF3R
what umbrella does chronic neutrophilic leukemia go under?
(chronic) myeloproliferative neoplasms
what are the 4 phases of polycythemia vera?
- prodrome (pre-polycythemic)
- proliferative/polycythemic phase
- spent phase (post-PV myelofibrosis)
- secondary AML (blasting off)
you can get a JAK2 V617F mutation in prefibrotic PMF (primary myelofibrosis), but what other 3 genetic categories are there?
CALR exon 9 mut
MPL W515K/L
triple negative
what are the lab (WBC) criteria for CNL diagnosis?
leukocytosis > 25 x 109/L
>80% of those are neutrophils
<10% are precursors
genetic mutation a/w systemic mastocytosis
KIT D816V
what are the 4 MDS/MPN diseases?
aCML
CMML
JMML
MDS/MPN unclassifiable
which AMLs may have eosinophilia?
inv(16) / t(16;16)
and
t(8;21)
this is a disease entity: “Myeloid and lymphoid neoplasms with eosinophilia and rearrangements of…”
what are the rearrangement genes?
PDGFRA
PDGFRB
FGFR1
PCM1-JAK2
that is as far as I will be remembering that
CMML
2 most common cytogenetic abnormalities
+8
-7
CMML
4 most common gene mutations
MC: ASXL1
TET2
SRSF2
SETBP1
percentage cutoffs for CMML 0, 1, and 2
0: <2% blasts in blood, <5% blasts in BM
1: 2-4% in blood, 5-9% in BM
2: 5-19% in blood, 10-19% in BM
atypical CML does NOT have this genetic alteration, but instead has mutations in these genes
NO BCR-ABL1!!!
mut in SETBP1 & ETNK1
MDS/MPN with ring sideroblasts and thrombocytosis
MUTATION
SF3B1
but you still need 15% ring sideroblasts
what are the mutations you could potentially have in JMML?
mut PTPN11
mut KRAS
mut NRAS
clinical Dx of NF1 or NF1 mut
CBL (germline or LOH)
monosomy 7
T/F: dysplasia of one or more hematopoietic lineages is common in aplastic anemia
FALSE
if you’re gonna get a dys-, it’s gonna be dyserythropoiesis
but you do NOT get dysgranulopoiesis or dysmegakaryopoiesis
“standard risk” genetics in plasma cell myeloma
t(11;14)
t(6;14)
[14 & std risk: 11 & 6]
hyperdiploidy
B2 microglobulin < 3.5 mg/L
HIGH RISK genetics in plasma cell myeloma
del(17p)
t(4;14)
t(14;16)
[14 & high risk: 4 & 16]
+1q21
hypodiploidy
del13q
B2 microglobulin >5.5 mg/L
congenital TTP
eponym
inheritance
gene & chromosome
Upshaw-Shulman
autosomal recessive
ADAMTS13 gene on 9q34
what role does calcium play in clotting?
provides a positive charge for negative Gla domains on vitamin K-dependent factors
what are the ingredients for PT testing?
thromboplastin (tissue factor + phospholipid), usu with a heparin neutralizer
calcium
plt-poor plasma
what are the ingredients for aPTT testing?
partial thromboplastin (phospholipid)
negatively charged surface
plt-poor plasma
~incubate~
add Ca
NO HEPARIN NEUTRALIZER/SENSITIVE TO LUPUS ANTICOAG
how do you calculate residual factor activity?
factor activity at that dilution
divided by
factor activity in a negative control
MoA: heparin
antithrombin enhancer
unfractionated: inhibits factor Xa & thrombin equally
LMWH: inhibits predominantly factor Xa
what assay is (hypothetically) used to measure the effecct of unfractionated heparin, but it’s too expensive to be practical?
anti-Xa assay
how does the anti-Xa assay work?
chromogenic
factor Xa is a protease > it cleaves a reagent and forms a color > if you have an inhibitor present (ie unfractionated heparin) then you get less color
what gets used in actual practice to measure heparin activity?
thrombin time
PTT or aPTT
which bug has colonies resembling “grains of sand”?
(small pinpoint convex colonies)
brucella (gram negative coccobacillus)
three biochemical properties of brucella
- rapid hydrolysis of urea (contains urease)
- reaction in 1-4 hours on Christians agar (wtf is this)
- positive cytochrome oxidase reaction (spot test)
which bacteria does the hockey puck thing?
moraxella catarrhalis
which bacteria does this, and what is the phenomenon called?
clostridium perfringens
double hemolysis: narrow zone of complete/B hemolysis d/t theta toxin + surrounding zone of incomplete/alpha hemolysis d/t alpha toxin
who has more vWF: babies or adults?
babies
which HLA is LEAST important to match in a transplant?
HLA DP and HLA DQ
which HLA is the MOST important to match in a transplant?
HLA A, B, C, and DRB1 (specifically)
universal PLASMA donor
AB
universal PLASMA recipient
O
universal RBC donor
O
universal RBC recipient
AB
preferred specimen for highest yield culture positivity in MUCOR
sliced (not ground) tissue
plasma for PT
how long is it stable at room temp?
24h
plasma for aPTT
how long is it stable at room temp?
4h
plasma for PT
and
plasma for aPTT
what temp do you freeze them at?
how long are they stable at that temp?
freeze @ -70C
stable for up to 1 year for coag testing
if you’re going to test plasma for FVIII or vWF, what temp can you store it at and for how long?
room temp: up to 4 h
frozen at -20 or -70C if testing will be delayed (idk for how long)
do NOT PUT THIS ON ICE
reversal agent for factor Xa inhibitors
andexanet alpha
reversal agent for dabigatran
idarucizumab
warfarin cytochrome genotype/phenotype with the lowest enzyme activity and therefore the one that requires the lowest doses?
CYP2C9 *2*2
VKORC1 AA (warfarin sensitive)
what 2 testing derangements will you see with defects in cytoskeletal proteins in RBCs?
decreased levels of AE1 (band 3)
decreased staining/decreased fluorescence intensity with eosin-maleimide (EMA) by flow
mutation in LPL that confers more symptomatic/active disease and drug resistance?
CXCR4
(not MYD88)
what is the atypical cell you see in peripheral blood smears of active EBV infxn?
it’s a reactive T-cell
(not a fucking monocyte)
which mycobacteria (TB complex) is pyrazinamide resistant?
bovis (BCG)
platelet plug: what are the 2 things that interact?
vWF on endothelium
+
GPIb on plts
(as opposed to aggregation, which is GPIIb/IIIa + fibrinogen)
mutation in hepatosplenic T-cell lymphoma
isochromosome 7q
mutation in T-cell large granular lymphocytic leukemia
TCR rearrangement
STAT3 mut in 1/3
chromogenic anti-Xa test
what does “positive” look like?
what does “negative” look like?
“positive” i.e. pt is on a Xa inhibitor: there is less Xa around in pt sample, so less rxn occurs, so less color change
“negative” i.e. pt is NOT on a Xa inhibitor, so MORE Xa is around, so MORE rxn occurs, so stronger color
what can you look for in meconium if you are worried about cocaine exposure in the baby?
M-hydroxybenzoylecgonine
what’s the platelet count for plt-poor plasma?
less than 10 x 109/L
one gram pos and one gram neg that are intrinsically resistant to colistin
gram pos: Enterococcus faecium
gram neg: Burkholderia cepacia
which benzo doesn’t show up on a UDS?
lorazepam
mutation in chronic neutrophilic leukemia
CSF3R
warfarin mechanism
reduce the enzymatic activity of vitamin K epoxide reductase
heparin mechanism of action
indirect inhibitors of FXa and FII (thrombin)
“indirect thrombin inhibitor”
it binds to antithrombin and makes it do what antithrombin does
BNP vs NT-proBNP
name 3 differences
- NT-proBNP is more stable
- NT-proBNP has a longer half-life
- BNP is susceptible to degradation by neprilysin
what do you use liquid Amies media for?
wound/throat specimen for bacterial culture
what do you use Cary-Blair media for?
stool specimens for bacterial culture
what is universal transport media good for transporting?
a sample for viral culture
it contains vanc and colistin to inhibit bacteria
which bacteria has no peptidoglycan and therefore doesn’t stain with Gram stain?
Mycoplasma
broadly, defect in
dyskeratosis congenita
telomere maintenance defect
broadly, defect in
Chediak-Higashi
lysosomal trafficking defect
broadly, defect in
severe congenital neutropenia
primary granule formation defect
broadly, defect in
Schwachman Diamond
ribosome biogenesis defect
broadly, defect in
Fanconi anemia
DNA repair pathway defect
which bacteria has “bull’s eye” colonies?
Yersinia (enterocolitica)
what does CIN stand for (agar)
cefsulodin-irgasan-novobiocin
what are the 3 bacteria that can have elevated minimum inhibitory concentrations (MIC) to imipenem, but are NOT considered carbapenem-resistant enterobacteriaceae?
Morganella
Proteus
Providencia
classic pentad of TTP
- thrombocytopenia
- microangiopathic hemolytic anemia (MAHA)
- fever
- renal failure
- altered mental status
what are the minimum diagnostic criteria for TTP?
microangiopathic hemolytic anemia + thrombocytopenia without a better explanation
how do you measure bilirubin in serum?
van den Bergh or “diazo” rxn
bilirubin + diazotized sulfanilic acid = azobilirubin
mutation in factor V leiden
Arg 506 Glu
you normally have an Arg, it substitutes a Glutamine
AML with monocytic differentiation + eos
inv(16)
AML with bilobed “apple core” nuclei
t(15;17) PML-RARA
“apple core” = APL (acute promyelocytic leukemia)
high urine pyridinium collagen cross-links (deoxypyridinoline, Dyd)
Paget disease
hepatosplenic T-cell lymphoma genetic mutation
isochromosome 7q
gamma/delta T-cells on flow should make you think of WHAT?
hepatosplenic T-cell lymphoma
T-prolymphocytic leukemia genetics
inv(14)(q11q32)
extranodal NK/T cell lymphoma genetics
del(6)(q21q25)
NK cells are NEGATIVE for this on flow but POSITIVE for this on IHC
CD3
the thing that is shaped like a banana in plasmodium falciparum is what part of the life cycle?
gametocyte
how does plasmodium falciparum cause end organ damage?
schizonts get sequestered in peripheral tissue capillary beds > sludging
secretin stimulation testing
what are you actually measuring?
bicarb secretion (from panc into duodenum)
AABB Standard 5.12 (31st edition) states that ABO group confirmation is required for units of red cells, whole blood and granulocytes, but you only have to do Rh type confirmation for WHAT units?
Rh NEGATIVE
(not positive!)
beta globin gene is on what chromosome?
11
alpha globin gene is on what chromosome?
16
what Rh type requires confirmation to r/o weak D?
Rh negative
what test do you use to monitor unfractionated & low molecular weight heparin therapeutic levels?
anti-Xa assay
unfractionated: after 6h
LMWH: after 4h
testing algorithm for polycythemia vera
JAK2 V617F testing
then reflex to JAK2 exon 12 if negative
testing algorithm for MPN (PV vs ET vs PMF)
JAK2 V617F
reflex to CALR & MPL if negative
name 4 species intrinsically resistant to vancomycin
GPCs: Leuconostoc, Weisella, Pediococcus
GPRs: Clostridium innocuum
4 tests that require platelet poor plasma
- anti-Xa
- coagulation factor activities
- vWF
- lupus anticoagulant
light chain type a/w AL amyloidosis?
Lambda
light chain type a/w light chain deposition disease?
kappa
minimum platelets in an apheresis unit?
3 x 1011 plts/bag
minimum platelets in a whole blood-derived unit of plts?
5.5 x 1010 plts/bag
amyloidosis is a/w an acquired factor deficiency of what factor?
factor X
what cell does Ehrlichia infect?
monocytes
what cell does Anaplasma infect?
granulocytes
what cell does Babesia infect?
MATURE red cells (not reticulocytes)
what is special about a stomatocyte
it’s Rh NULL
lab values diagnostic of acute pancreatitis
serum amylase and/or lipase
> 3x upper limit of normal
if you can only pick one lab value to evaluate for acute pancreatitis, which should you pick?
lipase
it’s more specific and will stay elevated longer than amylase
autoimmune lymphoproliferative syndrome
genes affected
germline or somatic?
GERMLINE
heterozygous mutations in FAS, FASLG, CASP10
most common cause of NAITP (neonatal alloimmune thrombocytopenia) in white people
HPA-1a
prothrombin gene mutation G20210A specifics
guanine to adenine substitution at position 20210 of the 3’ untranslated region of the Factor II gene
only human-infecting intracellular helminth
Trichinella spiralis
dRVVT
the venom directly activates which coagulation factor?
factor X
which red cell antigen contains disulfide bonds and is therefore destroyed with DTT?
Kell!
you know how you can get factor XI deficiency in Ashkenazi Jewish patients? what’s another name for that
hemophilia C
in a karyotype, how many cells have to have the same abnormality for you to call it a clone?
3
what’s delta bilirubin?
bilirubin bound to albumin
how do you estimate the concentration of delta bilirubin?
it’s like measuring gap
total bili - [conj + unconj bili] = delta bili
candida species with intrinsic resistance to fluconazole
krusei
what can you measure instead of GFR if you’re looking for a sex-independent method?
iothalamate clearance
mast cell leukemia
mutation
C-KIT
mast cell leukemia
aberrant flow markers
CD2
CD25
normal mast cell markers
mast cell tryptase
CD117 BRIGHT
what antibiotics is pseudomonas NOT intrinsically resistant to?
amikacin
cipro
colistin
pip-tazo
what lab value should you monitor in a plasma exchange?
fibrinogen
which mycobacteria is fastidious so it only grows on chocolate agar?
mycobacterium haemophilum
which developmental stage of plasmodium should you NOT include on your parasitemia percentage?
gametocytes! ack!
