Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

eltrombopag response rate and time to effect

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

eltrombopag dosing

A

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

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

WAS clinical features

A

eczema/atopy
FTT
immunodeficiency
microthrombocytopenia
malignancy risk

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

WAS HSCT indications

A

<5yo with MSD

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

neutrophil giant inclusions

A

Chediak Higashi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Chediak Higashi features and etiol

A

oculocutaneous albanism
giant lysosomal granules
neutropenia

plt storage pool deficiency

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

SCN DDx

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

SCN BM finding

A

promyelocyte maturational arrest

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

SCN and Kostmann mutations

A

ELANE (AD)
HAX1 (AR)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

SCN tx

A

GCSF
2nd line: HSCT

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

SCN malignancy risk

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Bernard Soulier pathophys, plt agg

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

ITP tx

A

observation
CS
IVIG

acute: TXA, ENT, Niastase, plt transfusion

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

PlaNet2 study

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

NAIT antibodies

A

HPA-1
Asian: HPA-4 and -5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

neutrophil production

A

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

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

neutropenia grading

A

mild 1-1.5
mod 0.5-1
sev <0.5

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
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
drug induced neutropenia
atb: cephalosporin, penicillin, sulfa anti-thyroid: carbimazole, methimazole anticonvulsant: carbamazepine, VPA, clozapine
26
alloimmune neonatal neutropenia pathophys, timeline, features
maternal IgG against fetal neutrophils present at birth, resolves week-6mo delayed cord separation, skin infection
27
Primary autoimmune neutropenia pathophys, epi, mgt
acquired anti-neutrophil Ab <3yo sometimes associated monocytosis usually self-limited
28
Cyclic neutropenia features
21d cycles reciprocal monocytosis fever, pharyngitis, aphthous ulcers, peridontitis
29
Schwachman Diamond Syndrome features and pathophys
neutropenia, macrocytic anemia exocrine pancreas dysfxn Skeletal anomalies SBDS mutations, aut rec ribosomopathy
30
reticular dysgenesis define and tx
T-B-NK- SCID with agranulocytosis HSCT
31
WHIM define and tx
Warts hypogamm infection myelokathexis: BM sequestration of mature neutrophils CXCR4 GOF mutation supportive, GCSF, IVIG
32
leukocytosis DDx
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
Eosinophilia definition and ix
>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
Hypereosinophilic Syndrome definition, etiol, tx
eosinophilia + end organ damage 1o: clonal expansion 2o: polyclonal expansion, cytokine driven CS 2nd line: HU, interferon alpha, CSA
35
lymphopenia DDx
SCID viral suppression sepsis drugs (chemo) trauma, hemorrhage malig
36
acute infectious lymphocytosis - etiol, mgt
enterovirus, pertussis self-resolving
37
Thrombocytopenia DDx
Production: infiltrative, BMFS, TAR, CAMT, Bernard-Soulier, Gray Plt, infectious, drugs, toxins, malnutrition Destruction: ITP, NAIT, drugs, consumption, thrombosis, vascular anomaly Sequestration
38
Inherited macrothrombocytopenia DDx
May-Hegglin anomaly Bernard Soulier Gray Plt Paris-Trousseau DiGeorge X linked macrothrombocytopenia (GATA-1 mutation)
39
ITP tx efficacy
CS - response 72h - 70-80% response rate IVIG - response 24-48h - 90% response rate
40
IVIG S/E
neutropenia allergic reaction - aseptic meningitis bleeding h/a hemolysis
41
ITP BMA indications
chronic other CBC anomalies systemic symptoms
42
HIT type I vs II
I: direct heparin effect on plt, 2d, mild, self-limited I: autoAb against heparin:plt factor 4 complex; 5d; more severe
43
Kasabach-Merritt heme features
consumptive coagulopathy - hypofibrinogenemia - severe thrombocytopenia - prolonged INR/PTT - elevated D-dimer
44
TTP pentad
MAHA thrombocytopenia fever renal dysfunction neuro changes
45
oculocutaneous albanism heme DDx
Chediak Higashi - immunodeficiency, HLH - giant lysosomal granules Hermansky Pudlack - plt dysfxn - plt EM absense of plt dense granules
46
Gray Plat syndrome pathophys and genetics
macrothrombocytopenia absent alpha granules NBEAL2 mutations
47
TAR pathophys, presentation, tx
RBM8A mutation thrombocytopenia bilataral absence of radii (normal thumbs) supportive - most transfusion independent >1y HSCT if ongoing cytopenias (rare)
48
CAMT pathophys, presentation, tx
c-MPL mutation (thrombopoietin receptor) isolated thrombocytopenia in infancy with absent BM megakaryocytes supportive HSCT prior to severe pancytopenia
49
RUSAT
radioulnar synostosis with amegakaryocytic thrombocytopenia HOXA11, MECOM mutations
50
alloimmune vs autoimmune neutropenia of infancy
alloimmune; maternal Ab; sick (delayed cord separation, omphalitis, infection, fever); lasts w-mo autoimmune: acquired Ab; well; self-limited 2-4w
51
Cu deficiency heme consequence
neutropenia smear: myeloid lineage vacuolization
52
Plt function disorders - which have normal plt vs thrombocytopenia
Macrothrombocytopenia: Bernard Soulier and Gray Plt Normal plt: Glanzmann and Hermansky Pudlack
53
alpha vs dense granules
alpha: large proteins - VWF - fibrinogen - PF4 - growth factors - Factor V - P-selectin dense: small molecules - ADP/ATP - serotonin - Ca
54
Plt dense granule conditions
Hermansky Pudlack Chediak-Higashi
55
Plt alpha granule conditions
grey plt syndrome ARC syndrome Quebec plt disorder Paris-Trousseau
56
gray play syndrome plt agg
absent second wave to epinephrine

H/O RC (31 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions