Flashcards - HemeOnc
1
Q
ristocetin test
A
von Willebrand Disease - decreased platelet aggregation
2
Q
PALS (perarterial lymphoid sheath) contain?
A
T-cells
3
Q
Paroxysmal Nocturnal Hemoglobinuria
A
PIGA mutation –> unable to synthesize GPI anchor (CD55, CD59) that inactivate complement and prevent MACs - sucrose test can activate complement - treat with eculizumab
4
Q
Lupus anticoagulant
A
Prolonged PTT, not correctable with FFP
5
Q
Hereditary Spherocytosis defect
A
ankrin or spectrin
6
Q
TTP Autoantibody
A
ADAMTS13 (von Willebrand factor metalloprotease)
7
Q
tartrate-resistant acid phosphatase (TRAP)
A
Hairy Cell Leukemia
8
Q
EBV infection increases risk of
A
Hodgkin’s disease, nasopharyngeal cancer
9
Q
Plummer Vinson Triad
A
Iron deficiency anemia, dysphagia, esophageal webs
10
Q
Cold agglutinin
A
IgM - CLL, mycoplasma pneunomia, mononucleosis,
11
Q
Warm agglutinin
A
IgG - SLE, CLL, drugs (a-methyldopa)
12
Q
acute promyelocytic leukemia translocation
A
t(15:17)
13
Q
chronic myelogenous leukemia translocation
A
t(9:22)
14
Q
5-flourouracil
A
pyrimidine antimetabolite
15
Q
cyclophosphamide
A
alkylating agent
16
Q
cytarabine
A
pyrimidine analog
17
Q
methotrexate
A
folate antimetabolite
18
Q
6-mercaptopurine
A
purine nucleotide synthesis inhibitor
19
Q
Waldenstrom Macroglobulinemia
A
B-cell lymphoma with monoclonal IgM - visual/neuro defects - treat with plasmapheresis
20
Q
Glanzmann’s thrombastehnia
A
decreased glycoproteins (Gps) IIb/IIIa
21
Q
Lead poisoning
A
Blocks ALA dehydrogenase and ferrochelatase - increased gamma-aminovulinate dehydratase
22
Q
ADAMTS13
A
cleaves vWF - inhibition leads to TTP
23
Q
CA-125
A
ovarian cancer
24
Q
carcinoembryonic antigen/CEA
A
colorectal cancer
25
alpha-fetoprotein
hepatocellular carcinoma
26
CA 19-9
pancreatic cancer
27
GI mucosal layers
Serosa --> Musclaris (outer, myenteric/Auerbach, inner) --> Submucosa (Meissner, glands) --> Mucosa (musclaris mucosa, lamina propria, epithelium)
28
Inguinal layers
skin, scarpas, fascia, external oblique, internal oblique, transversus, fascia, extraperitoneal fat, peritoneum
29
Cremaster origin
Internal oblique
30
sheets/nests of uniform cells, oval/round stippled nuclei
carcinoid tumor
31
MIF secretion
Sertoli cells
32
theca cell hormones
stimulaton from LH - cholesterol --> progesterone + androgens
33
granulosa cell hormones
stimulation from FSH - androgen --> estrogen
34
congenital rubella/rubeola infection
deafness, cataracts, cardiac malformations
35
basophilic stippling of hypochromic microcytic RBCs
lead poisoning
36
CML
complete spectrum of granulocytic cells, <10% blasts, decreased Alk Phos
37
AML
increased number of myeloblasts >20%
38
CLL
increased mature lymphoid cells
39
Cladribine
purine analog for hairy cell leukemia
40
Vincristine
inhibits microtubule polymerization by binding to B-tubulin, prevents mitotic spindle (side effect of neurotoxicity)
41
etoposide
targets topoisomerase II -induces double stranded DNA breaks
42
clopidigrel
inhibits ADP mediated platelet aggregation
43
argatroban, dabigatran
direct thrombin inhibitor
44
enoxaparin
low-weight molecular heparin - ATIII activator
45
apixaban, rivaroxaban
Factor Xa inhibitor
46
GP IIb/IIIa
platelet aggregation protein - attaches to fibrinogen - inhibited by Abciximab - deficient in Glanzmann thrombasthenia, autoantibodies in ITP
47
acute porphyria attack treatment
inhibit ALA synthase (a-aminolevulinic acid) with hemin or glucose
48
bite cells
G6PD
49
burr cells, helmet cells
microangiopathic hemolysis, mechanical damage
50
RBCs that are basophilic on Wright-Giemsa
reticulocytes - still have residual ribosomal RNA
51
endothelin
vasocontricter
52
Leukemia in Down
ALL, acute megakaryoblastic leukemia
53
Sickle cell amino acid
Glutamic acid to Valine
54
chloramphenicol adverse effect
reversible dose-dependent anemia, leukopenia, thrombocytopenia
55
splenectomy infections
Group B strep, H. flu, S. pneumonieae, N. meningitidis, S. typhi
56
leukocyte rolling proteins
L-selectin on neutrophils, E/P-selectin on endothelial cells
57
tight adhesion/crawling proteins
CD18 beta-2 integrins (Mac-1, LFA-1) to intercellular adhesion molecule-1 (ICAM-1)
58
transmigration protein
platelet endothelial cell adhesion molecule 1 (PECAM-1)
59
Weibel-Palade bodies
contain vWF and P-selectin
60
polycythemia vera
mutations in JAK2 - constitutive kinase activation
61
acute lymphoblastic leukemia
usually B-cell (70%), but T-cell can present as mediastinal mass
62
"waxing waning" lymphadenopathy
follicular lymphoma (14:18)
63
signet ring carcinoma
gastric adenocarcinoma - cells that do not form glands
64
intestinal type adenocarcinoma
stomach cancer resembling colon cancer - nodular polypoid - glands/columnar/cuboidal
65
non-rhythmic conjugate eye movements in children
neuroblastoma
66
Charcot-Leyden crystals
sign of eosinophilic infiltration - allergies
67
Langerhans cell
dendritic cell - phagocytic antigen presenting cell - MHC II
68
Protein C action
activated protein C + protein S cleaves and inactivates Va and VIIIa
69
acanthocyte
"spur cell" - liver disease, abetalipoproteinemia
70
dacrocyte
tear drop shaped - bone marrow infiltration (myelofibrosis)
71
Howell Jolly bodies
basophilic nuclear remnant normally removed by spleen - can be seen in asplenic pts
72
alpha-thalasssemia
4-allele deletion = Hb Barts, hydrops fetalis/death
3-allele deletion = HbH, B4 chains
1-2 allele deletion = less clinically severe anemia
73
orotic aciduria
megaloblastic anemia - inability to convert orotic acid to UMP (Uridine monophosphate synthetase) - treat with uridine monophosphate to bypass enzyme
74
HbC defect
glutamic acid to lysine mutation in B-globin
75
Bernard-Soulier syndrome
Defect in platelet to vWF adhesion - decreased GpIb - no agglutination on ristocetin assay
76
acute intermittent porphyria enzyme
porphobilinogen deanimase - accumulates porphobilinogen, delta-ALA, coporphobilinogen - painful abdomen, portwine urine, polyneuropathy, psych, precipitated by drugs/EtOH, starvation
77
porphyria cutanea tarda enzyme
uroporphyrinogen decarboxylate - accumulates uroporphyrin - most common porphyria
78
increased bleeding time
platelet disorder
79
prothrombin gene mutation
hypercoagulability - 3' untranslated region mutation - increased production of prothrombin
80
reed-sternberg cells CDs
CD15 and CD30 B-cell origin
81
CD5+ lymphoma
mantle cell t(11:14)
82
adult t-cell lymphoma
caused by HTLV - cutaneous lesions, lytic bone lesions, hypercalcemia
83
mycosis fungoides/Sezary syndrome
skin patches/plaques (cutaneous T-cell lymphoma), microabscesses - atypical CD4+ cells with "cerebriform" nuclei
84
fried egg/clock faced chromatin on histo, rouleaux formations
multiple myeloma
85
monoclonal gammopathy of undertermined significance (MGUS)
asymptomatic - 1-2% progress to MM
86
TdT+,
ALL - associated with downs
87
TRAP+
hairy cell leukemia - treat with cladribine, pentostatin
88
smudge cells
CLL - CD20+, CD5+ B-cells - progresses slowly
89
Auer rods
AML - M3 subtype is t(15:17)
90
tennis rackets
langerhans cell histiocytosis - S100, CD1a
91
LWMH
enoxparin, dalteparin - 2-4x longer halflife
92
cilostazol, dipyridamole
phosphodiesterase III inhibitor - increases cAMP in platelets, inhibiting aggregation
93
prothrombin factor
Factor II - converts fibrinogen to fibrin
94
tPA
converts plasminogen to plasmin --> cleaves fibrinogen/fibrin, destroys clotting factors, blocks platelet aggregation
95
radical prostatectomy DIC
increased urokinase --> decreased a2-antiplasmin --> degrades fibrinogen with no D-Dimer
96
sideroblastic anemia
defect in protoporphyrin synthesis - sideroblast rins - from ALAS mutation, EtOH, lead, B6 def
97
acute monocytic leukemia
AML subtype - monoblasts - lacks MPO - infiltrates gums
98
avute megakaryoblastic leukemia
AML subtype - lacks MPO - associated with Downs
99
B-ALL
most common - TdT+ - CD10,19,20 - prophylactic chemotherapy to scrotum and brain - t(12:21) is good, t(9:22) is bad
100
T-ALL
mediastinal mass in teenager (lymphoma) - TdT+ CD2-8, no CD10
101
Letterar-Siwe disease
histiocytosis - rash/cystic skeletal defects <2yo, rapidly fatal
102
Hand-Schuller-Christian disease
histiocytosis - scalp rash, lytic skin defects, DI, exopthalmos in child >3yo
103
Hodgkin lymphoma prognosis
lymphocyte rich = good prognosis, depleted = elderly, HIV, worst prognosis
104
marginal zone lymphoma
expands into marginal zone - from chronic inflammatory state or MALToma
