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H/O RC > WBC, plt > Flashcards

WBC, plt Flashcards

1
Q

Chronic ITP definition, epi, RF

A

plt <100 12+mo from dx
1/3 of ITP
RF: autoimm, teenager, F, DAT+, plt>20 at presentation

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2
Q

PETIT trial ITP (outcome, a/e)

A

eltrombopag for chronic ITP
phase 2
62% vs 32% had plt >50 at 6w
A/E: diarrhea, ALT/AST elevation

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3
Q

eltrombopag response rate and time to effect

A

60-80% response rate
most by 2w
only effective while drug is being admitted

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4
Q

eltrombopag dosing

A

50 mg daily, titrate to plt>50 max 75mg

East Asian and 1-5yo start at 50% dosing

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5
Q

WAS clinical features

A

eczema/atopy
FTT
immunodeficiency
microthrombocytopenia
malignancy risk

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6
Q

WAS HSCT indications

A

<5yo with MSD

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7
Q

neutrophil giant inclusions

A

Chediak Higashi

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8
Q

Chediak Higashi features and etiol

A

oculocutaneous albanism
giant lysosomal granules
neutropenia

plt storage pool deficiency

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9
Q

SCN DDx

A

Cyclic neutropenia
primary immune disorders
drug
nutritional (folate, B12, copper)
infectious suppression

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10
Q

SCN BM finding

A

promyelocyte maturational arrest

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11
Q

SCN and Kostmann mutations

A

ELANE (AD)
HAX1 (AR)

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12
Q

SCN tx

A

GCSF
2nd line: HSCT

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13
Q

SCN malignancy risk

A

MDS/AML
cumulative incidence 22% - 2%/y

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14
Q

Bernard Soulier pathophys, plt agg

A

GpIb-IX-V = CD42
plt-VWF adhesion defect
macrothrombocytopenia
agg: absent response to ristocetin only

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15
Q

Glanzmann’s Thrombasthenia pathophys, plt agg

A

GpIIb-IIIa = CD41
plt-VWF/fibrinogen aggregation defect
normal plt
agg: absent response to all except ristocetin

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16
Q

ITP tx

A

observation
CS
IVIG

acute: TXA, ENT, Niastase, plt transfusion

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17
Q

PlaNet2 study

A

<34w transfusion treshold <50 had increased death or major bleeding compared to <25

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18
Q

NAIT antibodies

A

HPA-1
Asian: HPA-4 and -5

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19
Q

NAIT tx

A

self limited, resolution in 1-4w as maternal Ab disappear

HUS
plt >30 or if bleeding
- maternal washed > HPA-1 neg > pooled
IVIG

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20
Q

fxn of
- neutrophil
- CD4 T
- CD8 T
- B
- monocyte
- NK
- eosinophil

A
  • neutrophil: degranulation, NETosis, phagocytosis
  • CD4 T: helper; class switching
  • CD8 T: cytotoxic
  • B: Ab production
  • monocyte: Ag presentation
  • NK: innate immunity
  • eosinophil: allergy, Ag presentation
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21
Q

neutrophil production

A

proliferation 1w (myeloblast - promyelocyte, myelocyte)
maturation 1w (metamyelocyte - band - neutrophil)
intravascular 1d
tissues 1d

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22
Q

neutropenia grading

A

mild 1-1.5
mod 0.5-1
sev <0.5

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23
Q

neutrophil fxn steps

A

rolling/adherence
Chemotaxis (lipids, GMCSF, GCSF, IL-8)
Diapedesis (crossing endothelium)
Phagocytosis (CD11b, 18)
Degranulation

24
Q

Neutropenia DDx

A

acquired
- post infectiou
- drug
- nutritional: B12, folate, Cu
- autoimmune: chronic benign neutropenia, Evans, ALPS, SLE
- hypersplenism

Congenital
- primary immune disorders
- SDS
- Chediak Higashi,
- SCN
- Cyclic

25
Q

drug induced neutropenia

A

atb: cephalosporin, penicillin, sulfa
anti-thyroid: carbimazole, methimazole
anticonvulsant: carbamazepine, VPA, clozapine

26
Q

alloimmune neonatal neutropenia pathophys, timeline, features

A

maternal IgG against fetal neutrophils

present at birth, resolves week-6mo

delayed cord separation, skin infection

27
Q

Primary autoimmune neutropenia pathophys, epi, mgt

A

acquired anti-neutrophil Ab
<3yo
sometimes associated monocytosis
usually self-limited

28
Q

Cyclic neutropenia features

A

21d cycles
reciprocal monocytosis
fever, pharyngitis, aphthous ulcers, peridontitis

29
Q

Schwachman Diamond Syndrome features and pathophys

A

neutropenia, macrocytic anemia
exocrine pancreas dysfxn
Skeletal anomalies

SBDS mutations, aut rec
ribosomopathy

30
Q

reticular dysgenesis define and tx

A

T-B-NK- SCID with agranulocytosis
HSCT

31
Q

WHIM define and tx

A

Warts
hypogamm
infection
myelokathexis: BM sequestration of mature neutrophils

CXCR4 GOF mutation

supportive, GCSF, IVIG

32
Q

leukocytosis DDx

A

1o neutrophilia: CSF3R mutation (GCSF receptor)
infection
malignancy
Drugs: CS (demargination)
autoimm/infl: KD, JIA
preg
smoking
T21
physiologic: altitude, vigorous exercise, heat stroke, sx, trauma

33
Q

Eosinophilia definition and ix

A

> 500 cells/uL over 1 mo period and/or tissue confirmation

Mild 500-1500
Mod 1500-5000
Sev >5000

CBCD, smear
strongyloides and toxocara serology
B12
tryptase
Ig, T/B cell subsets
BMA/B

34
Q

Hypereosinophilic Syndrome definition, etiol, tx

A

eosinophilia + end organ damage

1o: clonal expansion
2o: polyclonal expansion, cytokine driven

CS
2nd line: HU, interferon alpha, CSA

35
Q

lymphopenia DDx

A

SCID
viral suppression
sepsis
drugs (chemo)
trauma, hemorrhage
malig

36
Q

acute infectious lymphocytosis - etiol, mgt

A

enterovirus, pertussis
self-resolving

37
Q

Thrombocytopenia DDx

A

Production: infiltrative, BMFS, TAR, CAMT, Bernard-Soulier, Gray Plt, infectious, drugs, toxins, malnutrition

Destruction: ITP, NAIT, drugs, consumption, thrombosis, vascular anomaly

Sequestration

38
Q

Inherited macrothrombocytopenia DDx

A

May-Hegglin anomaly
Bernard Soulier
Gray Plt
Paris-Trousseau
DiGeorge
X linked macrothrombocytopenia (GATA-1 mutation)

39
Q

ITP tx efficacy

A

CS
- response 72h
- 70-80% response rate

IVIG
- response 24-48h
- 90% response rate

40
Q

IVIG S/E

A

neutropenia
allergic reaction - aseptic meningitis
bleeding
h/a
hemolysis

41
Q

ITP BMA indications

A

chronic
other CBC anomalies
systemic symptoms

42
Q

HIT type I vs II

A

I: direct heparin effect on plt, 2d, mild, self-limited

I: autoAb against heparin:plt factor 4 complex; 5d; more severe

43
Q

Kasabach-Merritt heme features

A

consumptive coagulopathy
- hypofibrinogenemia
- severe thrombocytopenia
- prolonged INR/PTT
- elevated D-dimer

44
Q

TTP pentad

A

MAHA
thrombocytopenia
fever
renal dysfunction
neuro changes

45
Q

oculocutaneous albanism heme DDx

A

Chediak Higashi
- immunodeficiency, HLH
- giant lysosomal granules

Hermansky Pudlack
- plt dysfxn
- plt EM absense of plt dense granules

46
Q

Gray Plat syndrome pathophys and genetics

A

macrothrombocytopenia
absent alpha granules
NBEAL2 mutations

47
Q

TAR pathophys, presentation, tx

A

RBM8A mutation

thrombocytopenia
bilataral absence of radii (normal thumbs)

supportive
- most transfusion independent >1y
HSCT if ongoing cytopenias (rare)

48
Q

CAMT pathophys, presentation, tx

A

c-MPL mutation (thrombopoietin receptor)

isolated thrombocytopenia in infancy with absent BM megakaryocytes

supportive
HSCT prior to severe pancytopenia

49
Q

RUSAT

A

radioulnar synostosis with amegakaryocytic thrombocytopenia

HOXA11, MECOM mutations

50
Q

alloimmune vs autoimmune neutropenia of infancy

A

alloimmune; maternal Ab; sick (delayed cord separation, omphalitis, infection, fever); lasts w-mo

autoimmune: acquired Ab; well; self-limited 2-4w

51
Q

Cu deficiency heme consequence

A

neutropenia
smear: myeloid lineage vacuolization

52
Q

Plt function disorders - which have normal plt vs thrombocytopenia

A

Macrothrombocytopenia: Bernard Soulier and Gray Plt

Normal plt: Glanzmann and Hermansky Pudlack

53
Q

alpha vs dense granules

A

alpha: large proteins
- VWF
- fibrinogen
- PF4
- growth factors
- Factor V
- P-selectin

dense: small molecules
- ADP/ATP
- serotonin
- Ca

54
Q

Plt dense granule conditions

A

Hermansky Pudlack
Chediak-Higashi

55
Q

Plt alpha granule conditions

A

grey plt syndrome
ARC syndrome
Quebec plt disorder
Paris-Trousseau

56
Q

gray play syndrome plt agg

A

absent second wave to epinephrine

H/O RC (31 decks)

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