Heme/Onc Flashcards

1
Q

typical features of multiple myeloma (think CRAB)

A

hyperCalcemia (from osteoclast release by osteoclast activating factor IL-1, IL-6)
Renal insufficiency (Ig light chains in urine - Bence Jones protein)
Anemia, primary Amyloidosis (beta2-microglobulin)
Bone lytic lesions (punched-out) + Back pain

also, M-spike
increased suscept to infections
Rouleaux formation (RBCs stacked like poker chips)

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2
Q

eosinophils contain

A

histaminase
arylsulfatase

limits rxn following mast cell degran

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3
Q

basophils contain

A

heparin
histamine
leukotrienes (LTD4)

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4
Q

mast cells can bind what?

A

bind Fc portion of IgE onto membrane –> when IgE cross-links upon antigen binding –> degranulation

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5
Q

fibrin mesh formed by what bonds

A

covalent

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6
Q

proteins C + S inactivate what factors

A

Va

VIIIa

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7
Q

warfarin inhibits what enzyme

A

epoxide reductase

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8
Q

antithrombin inhibits activated forms of factors:

A
II
VII
IX
X
XI
XII
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9
Q

vit K deficiency increases what lab values

A

PT and PTT!!

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10
Q

factor w/ shortest 1/2 life

A

VII

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11
Q

what are located inside endoth cells

A

tPA
thromboplastin (tissue factor)
vWF
PGI2

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12
Q

acanthocytes (spur cell) seen w/

A

liver disease
abetalipoproteinemia

IRREG spikes

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13
Q

basophilic stipping is what? seen w/?

A

aggregates of rRNA

Thalessemias
Anemia of chronic dz
Lead Poisoning
Alcoholism

“ACTors are based in LA”

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14
Q

bite cells seen w/

A

G6PD def

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15
Q

elliptocyte (aka pencil cell) seen w/

A

hereditary elliptocytosis

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16
Q

macro-ovalocytes seen w/

A

megaloblastic anemia

marrow failure

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17
Q

Ringed sideroblasts

A

**iron granules surround/encircle nucleus

sideroblastic anemia
lead poisoning
drugs, genetic conditions, myelodysplastic synd

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18
Q

schistocyte seen w/

A

DIC
TTP/HUS
traumatic hemolysis (metal heart valve prosthesis)
micro/macroangiopathic anemia

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19
Q

echinocyte (burr cell) seen w/

A
uremia
renal failure
pyruvate kinase deficiency
microangiopathic hemolytic anemia
mechanical trauma (prosthetic valves)

REG spikes

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20
Q

spherocytes seen w/

A
hereditary spherocytosis
autoimmune hemolysis (warm agglut type)
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21
Q

teardrop cells seen w/

A

myelofibrosis

bone marrow infiltration

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22
Q

target cells seen w/

A

HbC disease
Asplenia
Liver disease
Thalessemia

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23
Q

Heinz bodies are what? seen in?

A

oxidation of Hb sulfhydryl groups –> denatured Hb precipitation + damage RBC membrane –> form bite cells (when it goes thru spleen)

seen in G6PD def

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24
Q

Howell-Jolly bodies are what? seen in?

A

basophilic nuclear remnants in RBCs

seen w/ asplenia or w/ mothball digestion (naphthalene)

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25
disting b/w Howell-Jolly bodies vs. Heinz bodies?
only ONE HJ body per cell; MANY Heinz bodies per cell
26
cause of alpha thal
deletions in alpha-globin genes | 4 alpha alleles on chrom 16
27
cause of beta thal
point mutations in splice sites + promoter seq | 2 beta genes on chrom 11
28
primary Hb seen w/ Beta-thal minor
HbA2 (alpha 2, delta 2)
29
primary Hb seen w/ Beta-thal major
HbF
30
lead inhibits what enzymes
ALA-D (buildup of ALA) | ferrochelatase (buildup of protoporphyrin)
31
lead poisoning sympt
Lead lines on gingivae or metaphyses of long bones Encephalopathy and Erythrocyte Basophilic stippling Abd pain and sideroblastic Anemia Drops - wrist/foot drop
32
sideroblastic anemia etiology + tx
X-linked defect in ALA-S gene acquired: alcohol, lead, INH (causes B6 def) tx = B6
33
what does hepcidin do in anemia of chronic dz
limits Fe transfer from macrophages to erythroid precursors by binding ferroportin on intestinal mucosal cells (inhib Fe transport) + suppresses EPO prod
34
folate vs B12 def (non-symptoms)
folate: - green vegetables/fruit - absorbed in JEJUNUM - normal methylmalonic acid levels B12: - animal products! (strict vegans often affected) - absorbed in ILEUM - increased methylmalonic acid levels
35
tx for orotic aciduria
uridine monophosphate
36
nonmegaloblastic macrocytic anemia causes =
liver dz alcoholism reticulocytosis drugs (5-FU, AZT, hydroxyurea)
37
signs of intravascular hemolysis
``` decreased haptoglobin increased LDH Hb in urine hemoglobinemia hemosiderinemia ```
38
signs of extravascular hemolysis
increased LDH increased unconj bilirubin --> jaundice! splenomegaly increased risk of gallstones
39
causes of aplastic anemia
radiation drugs (benzene, chloramphenicol, alkylating agents, antimetabolites) virus (parvovirus B19, EBV, HCV, HIV) - esp in sickle cell pts Fanconi's anemia (DNA repair defect) Idiopathic (primary stem cell defect - ex. telomerase def) autoimmune
40
ass w/ pure red cell aplasia
thymomas parvovirus B19 lymphocytic leukemias
41
PNH lab findings/tx?
CD55/59 negative on RBCs!! | tx = eculizumab
42
PNH triad?
hemolytic anemia pancytopenia venous thrombosis
43
"crew cut" on skull x-ray due to marrow hyperplasia seen in:
sickle cell anemia | beta-thal major
44
complic in sickle cell anemia
- aplastic crisis (parvo B19) - **autosplenectomy (shrunken fibrotic spleen due to multiple infarcts) - increased suscept to encapsulated organisms; Howell-Jolly bodies seen - splenic sequestration crisis - wedge-shaped infarcts + decreased H&H - Salmonella osteomyelitis - painful crises (dactylitis, acute chest synd, avascular necrosis usually of femoral head) - renal papillary necrosis (due to low O2 in papilla) + microhematuria
45
what can cause both warm or cold type AIHA?
EBV, CLL
46
causes of warm agglutinin AIHA
SLE CLL alpha-methyldopa infections/viruses
47
causes of cold agglutinin AIHA
CLL Mycoplasma pneumo infectious mononucleosis
48
microangiopathic anemia ass w/
DIC TTP-HUS SLE malig HTN
49
macroangiopathic anemia ass w/
prosthetic valves | aortic stenosis
50
infections that destroy RBCs
malaria | Babesia
51
transferrin levels are a PRIMARY increase in what conditions
preg + OCP use
52
acute intermittent porphyria sympt + tx
``` 5P's: Painful abd Port-wine colored urine Polyneuropathy (peripheral) Psych disturbances Precip by drugs ``` tx = glucose + heme (inhibs ALA-S)
53
acute intermittent porphyria enzyme affected + substrate accum =
enzyme = porphobilinogen deaminase (aka uroporphyrinogen-1-synthase) accum = - porphobilinogen - ALA - uroporphyrin (in urine)
54
porphyria cutanea tarda enzyme affected + substrate accum =
enzyme = uroporphyrinogen decarboxylase accum = uroporphyrin (tea-colored urine!!!!!!)
55
porphyria cutanea tarda sympt + ass w/?
***blistering cutaneous photosensitivity! (from ROS damage) hypertrichosis (excessive hair growth) facial pigmentation ass w/ HCV, alcoholism, increased LFTs
56
ALA-S needs what cofactor
B6
57
Bernard-Soulier synd
decreased GpIb --> decreased platelet-vWF adhesion | see: mild TCP w/ ENLARGED platelets
58
Glanzmann's thrombasthenia
decreased GpIIb/IIIa --> defect in platelet-platelet aggreg | Lab = NO platelet clumping
59
ITP
anti-GpIIb/IIIa Ab (IgG) increased megakaryocytes usually wks after viral infection or immunization
60
TTP
deficiency of ADAMTS13 (vWF metalloprotease) --> large vWF multimers (the NON-active form) --> increased platelet aggreg --> thrombosis labs: schistocytes!, increased LDH
61
sympt of TTP
``` pentad: Neuro sympt Fever TCP Hemolytic anemia Kidney damage ``` "Nasty Fever Torched His Kidneys"
62
blood transfusion risks =
infection transmission (low) transfusion rxns Fe overload hypocalcemia (due to citrate = Ca + Mg chelator) hyperkalemia (RBC lysis in old blood units)
63
cryoprecipitate contains
``` fibrinogen factor VIII factor XIII vWF fibronectin ```
64
Reed-Sternberg cells = + for what markers
CD30+ CD15+ both B-cell origin
65
marginal zone lymphoma ass w/?
chronic inflamm conditions like: Hashimoto's thyroiditis Sjogren's dz H.pylori gastritis
66
Waldenstrom's macroglobulinemia
IgM M-spike NO lytic bone lesions visual/neuro deficits + bleeding
67
Hairy cell leukemia unique features/tx
absent LAD!! dry tap w/ bone marrow aspiration TRAP + tx = cladribine (adenosine deaminase inhibitor) or adenosine analog
68
common presentation of M3 AML
DIC
69
characteristic increase in what cell in CML
basophils
70
RF for CML =
ionizing radiation | benzene
71
Langerhans cell histiocytosis features?
lytic bone lesions + skin rash in child do not efficiently stim T-lymphocytes via APC cells express S-100 (neural crest origin) + CD1a Birbeck granules!!
72
HIT caused by:
Ab-heparin-PF4 complex --> activate platelets --> thrombosis, TCP
73
cilostazol, dipyridamole MOA
PDE3 inhib --> increases cAMP in platelets --> inhib aggreg also, arterial vasodilators
74
tx for CLL
fludrabine
75
prevent cardiotox induced by doxorubicin
dexrazoxane (iron chelating agent)