Heme/Onc Flashcards

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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
Q

disting b/w Howell-Jolly bodies vs. Heinz bodies?

A

only ONE HJ body per cell; MANY Heinz bodies per cell

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26
Q

cause of alpha thal

A

deletions in alpha-globin genes

4 alpha alleles on chrom 16

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27
Q

cause of beta thal

A

point mutations in splice sites + promoter seq

2 beta genes on chrom 11

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28
Q

primary Hb seen w/ Beta-thal minor

A

HbA2 (alpha 2, delta 2)

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29
Q

primary Hb seen w/ Beta-thal major

A

HbF

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30
Q

lead inhibits what enzymes

A

ALA-D (buildup of ALA)

ferrochelatase (buildup of protoporphyrin)

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31
Q

lead poisoning sympt

A

Lead lines on gingivae or metaphyses of long bones
Encephalopathy and Erythrocyte Basophilic stippling
Abd pain and sideroblastic Anemia
Drops - wrist/foot drop

32
Q

sideroblastic anemia etiology + tx

A

X-linked defect in ALA-S gene
acquired: alcohol, lead, INH (causes B6 def)
tx = B6

33
Q

what does hepcidin do in anemia of chronic dz

A

limits Fe transfer from macrophages to erythroid precursors by binding ferroportin on intestinal mucosal cells (inhib Fe transport) + suppresses EPO prod

34
Q

folate vs B12 def (non-symptoms)

A

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
Q

tx for orotic aciduria

A

uridine monophosphate

36
Q

nonmegaloblastic macrocytic anemia causes =

A

liver dz
alcoholism
reticulocytosis
drugs (5-FU, AZT, hydroxyurea)

37
Q

signs of intravascular hemolysis

A
decreased haptoglobin
increased LDH
Hb in urine
hemoglobinemia
hemosiderinemia
38
Q

signs of extravascular hemolysis

A

increased LDH
increased unconj bilirubin –> jaundice!
splenomegaly
increased risk of gallstones

39
Q

causes of aplastic anemia

A

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
Q

ass w/ pure red cell aplasia

A

thymomas
parvovirus B19
lymphocytic leukemias

41
Q

PNH lab findings/tx?

A

CD55/59 negative on RBCs!!

tx = eculizumab

42
Q

PNH triad?

A

hemolytic anemia
pancytopenia
venous thrombosis

43
Q

“crew cut” on skull x-ray due to marrow hyperplasia seen in:

A

sickle cell anemia

beta-thal major

44
Q

complic in sickle cell anemia

A
  • 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
Q

what can cause both warm or cold type AIHA?

A

EBV, CLL

46
Q

causes of warm agglutinin AIHA

A

SLE
CLL
alpha-methyldopa
infections/viruses

47
Q

causes of cold agglutinin AIHA

A

CLL
Mycoplasma pneumo
infectious mononucleosis

48
Q

microangiopathic anemia ass w/

A

DIC
TTP-HUS
SLE
malig HTN

49
Q

macroangiopathic anemia ass w/

A

prosthetic valves

aortic stenosis

50
Q

infections that destroy RBCs

A

malaria

Babesia

51
Q

transferrin levels are a PRIMARY increase in what conditions

A

preg + OCP use

52
Q

acute intermittent porphyria sympt + tx

A
5P's:
Painful abd
Port-wine colored urine
Polyneuropathy (peripheral)
Psych disturbances
Precip by drugs

tx = glucose + heme (inhibs ALA-S)

53
Q

acute intermittent porphyria enzyme affected + substrate accum =

A

enzyme = porphobilinogen deaminase (aka uroporphyrinogen-1-synthase)

accum =

  • porphobilinogen
  • ALA
  • uroporphyrin (in urine)
54
Q

porphyria cutanea tarda enzyme affected + substrate accum =

A

enzyme = uroporphyrinogen decarboxylase

accum = uroporphyrin (tea-colored urine!!!!!!)

55
Q

porphyria cutanea tarda sympt + ass w/?

A

***blistering cutaneous photosensitivity! (from ROS damage)
hypertrichosis (excessive hair growth)
facial pigmentation

ass w/ HCV, alcoholism, increased LFTs

56
Q

ALA-S needs what cofactor

A

B6

57
Q

Bernard-Soulier synd

A

decreased GpIb –> decreased platelet-vWF adhesion

see: mild TCP w/ ENLARGED platelets

58
Q

Glanzmann’s thrombasthenia

A

decreased GpIIb/IIIa –> defect in platelet-platelet aggreg

Lab = NO platelet clumping

59
Q

ITP

A

anti-GpIIb/IIIa Ab (IgG)
increased megakaryocytes
usually wks after viral infection or immunization

60
Q

TTP

A

deficiency of ADAMTS13 (vWF metalloprotease) –> large vWF multimers (the NON-active form) –> increased platelet aggreg –> thrombosis
labs: schistocytes!, increased LDH

61
Q

sympt of TTP

A
pentad:
Neuro sympt
Fever
TCP
Hemolytic anemia
Kidney damage

“Nasty Fever Torched His Kidneys”

62
Q

blood transfusion risks =

A

infection transmission (low)
transfusion rxns
Fe overload
hypocalcemia (due to citrate = Ca + Mg chelator)
hyperkalemia (RBC lysis in old blood units)

63
Q

cryoprecipitate contains

A
fibrinogen
factor VIII
factor XIII
vWF
fibronectin
64
Q

Reed-Sternberg cells = + for what markers

A

CD30+
CD15+
both B-cell origin

65
Q

marginal zone lymphoma ass w/?

A

chronic inflamm conditions like:
Hashimoto’s thyroiditis
Sjogren’s dz
H.pylori gastritis

66
Q

Waldenstrom’s macroglobulinemia

A

IgM M-spike
NO lytic bone lesions
visual/neuro deficits + bleeding

67
Q

Hairy cell leukemia unique features/tx

A

absent LAD!!
dry tap w/ bone marrow aspiration
TRAP +

tx = cladribine (adenosine deaminase inhibitor) or adenosine analog

68
Q

common presentation of M3 AML

A

DIC

69
Q

characteristic increase in what cell in CML

A

basophils

70
Q

RF for CML =

A

ionizing radiation

benzene

71
Q

Langerhans cell histiocytosis features?

A

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
Q

HIT caused by:

A

Ab-heparin-PF4 complex –> activate platelets –> thrombosis, TCP

73
Q

cilostazol, dipyridamole MOA

A

PDE3 inhib –> increases cAMP in platelets –> inhib aggreg

also, arterial vasodilators

74
Q

tx for CLL

A

fludrabine

75
Q

prevent cardiotox induced by doxorubicin

A

dexrazoxane (iron chelating agent)