HEMA FL1: TABLES Flashcards by ASHLEY JARED DEFENSOR

Q

Quantitative Thrombocytopenia

May-Hegglin Anomaly
Montreal Platelet syndrome
Autosomal and X-linked thrombocytopenia
Fechtner Syndrome

A. Increased platelet destruction
B. Impaired or Decrease production (Congenital)
C. Impaired or Decrease production (Acquired)
D. Abnormal Distribution or Dilution

A

B. Impaired or Decrease production

Others:
- Bernard-Soulier syndrome
- Sebastian syndrome
- Epstein syndrome
- Fanconi anemia
- Wiskott-Aldrich syndrome
- Thrombocytopenia with absent radii
(TAR) syndrome

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Q

ITP
HIV
HDN
Kasabach Merritt Syndrome
Hypothermia

A. Increased platelet destruction (immune)
B. Increased platelet destruction (nonimmune)
C. Impaired or Decrease production (Congenital)
D. Impaired or Decrease production (Acquired)
E. Abnormal Distribution

A

A
B
B
1.E
E

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Q

Neonatal
PTP
TTP
DIC
HUS

A. Increased platelet destruction (immune)
B. Increased platelet destruction (nonimmune)
C. Impaired or Decrease production (Congenital)
D. Impaired or Decrease production (Acquired)
E. Abnormal Distribution

A

AABBB

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Q

Viral
Splenic sequestration
Epstein
Heparin
Massive transfusion, extracorpeal

A. Increased platelet destruction (immune)
B. Increased platelet destruction (nonimmune)
C. Impaired or Decrease production (Congenital)
D. Impaired or Decrease production (Acquired)
E. Abnormal Distribution

A

DECAE

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Q

Glanzmann-thrombasthenia or BSS

poor binding with VWF

A

Both

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Q

Glanzmann-thrombasthenia or BSS

poor binding with Fibrinogen, VWF, fibronectin, vitronectin

A

Glanz

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Q

Glanzmann-thrombasthenia or BSS

Defective Genes: ITGA2B and ITGB3 (chr. 17)
Defective Glycoprotein complex: Gp lIb/Illa

A

Glanx

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Q

Glanzmann-thrombasthenia or BSS

Defective Genes: GP 1ba (chr.17); GP 1bB (chr.22); GP IX and GP V (chr. 3)
Defective Glycoprotein complex: gplb/IX/V

A

BSS

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Q

Glanzmann-thrombasthenia or BSS

epistaxis,

A

Both

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Q

Glanzmann-thrombasthenia or BSS

Ecchymoses, gingival bleeding, epistaxis, hemarthroses, expanding hematoma

A

BSS

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Q

Glanzmann-thrombasthenia or BSS

Menorrhagia, gastrointestinal
bleeding, hematuria, epistaxis, gingival bleeding, bleeding after circumcision

A

Hemorrhagic manifestation

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Q

Glanzmann-thrombasthenia or BSS

platelet component, recombinant factor VIIa treatment

A

Both

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Q

Glanzmann-thrombasthenia or BSS

desmopressin treatement

A

BSS

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Q

Glanzmann-thrombasthenia or BSS

Normal platelet count

A

Glanz

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Q

Glanzmann-thrombasthenia or BSS

Increase platelet count

A

D

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Q

Glanzmann-thrombasthenia or BSS

Study These Flashcards

A

Q

Glanzmann-thrombasthenia or BSS

Enlarged giant platelet
Abnormal bleeding time
Abnormal CRT

identify each

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A

BSS
Both
Glanz

Q

Glanzmann-thrombasthenia or BSS

ADP EPI Collagen Abnormal
Ristocetin abnormal
Thrombin abnormal

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A

Glanzz
BSS
Both

Q

Disorders of platelet secretion

Which does not belong

HPS1 gene
X linked
Swiss cheese
Albinism

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A

X linked (auto recessive)
Hermansky-Pudlak

Q

Disorders of platelet secretion

Except

WASp gene
X linked
Albinism
Recurrent infection
Eczema

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A

Albinism (none)
Wiskott-Aldrich Syndrome

Q

Disorders of platelet secretion

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A

Q

Disorders of platelet secretion

Except:

Pancytopenia
Mild to progressive bleeding
Easy bruising
NBEAL2 gene

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A

Pancy (Chediak) – GRay platelet syndrome

Q

Disorders of platelet secretion

LYST gene
Auto recessive
Pancytopenia
ALbinism

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A

Chediak

Q

Disorders of platelet secretion

Except

Alpha defect
RBM8A
Auto recessive
Absence of albinism
1. Congenital absence of radial bones

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A

Alpha defect (dense granule) – TAR syndrome

# Disorders of platelet secretion * Bleeding symptoms, hemarthroses, hematuria, and large bruising * Autosomal dominant * uPA gene

Quebec platelet disorder

# Disorders of platelet secretion * NBEAL2 Gene * Auto recessive * Easy bruising and increased * risk of epistaxis * Mild to moderate bleeding

Gray platelet syndrome