HEMA LAB - Diseases

Question 1

Inherited disorder of platelet GpIb/IX/V

Answer 1

Bernard-Soulier Syndrome

Question 2

caused by absence or deficiency of membrane GP IIb/IIIa complex.

Answer 2

Glanzmann’s Thrombasthenia

Question 3

autosomal recessive disorder that is due to genetic Human Prostate-specific gene 1 (HPG-1)

Answer 3

Hermansky-Pudlak Syndrome

Question 4

Also known as Giant platelet disorder

Answer 4

Bernard-Soulier Syndrome

Question 5

shows severe eczema, recurrent infections, immune defects, thrombocytopenia, and small platelets.

Answer 5

Wiskott-Aldrich Syndrome

Question 6

decrease platelet P-Selectin (CD62P)

Answer 6

Storage pool deficiency

Question 7

Defect in surface glycoprotein sialophorin (CD43, gp115, leukosialin)

Answer 7

Wiskott-Aldrich syndrome

Question 8

Factor V deficiency

Answer 8

Von Willebrand disease

Question 9

What is the common complication of Uremia?

Answer 9

Bleeding

Question 10

Result in bruising but are NOT associated with any specific disease

Answer 10

Primary Purpura

Question 11

It is a clinical manifestation of RBC extravasation into mucosa or skin, results from rheumatologic, infectious, dermatologic, traumatic, and hematologic disorders.

Answer 11

Purpura

Question 12

occurs due to skin fragility

Answer 12

Simple purpura

Question 13

Due to sudden increase in capillary pressure

Answer 13

Mechanical purpura

Question 14

Seen in older individuals, or in individuals undergoing corticosteroid therapy

Answer 14

Senile purpura

Question 15

seen in individuals with emotional problems,
often after severe trauma or extensive surgery, which may be hypersensitive to RBC membrane components or DNA hypersensitivity

Answer 15

Psychogenic purpura

Question 16

development of cayenne pepper petechiae on a background of hyperpigmented brown or orange oval patches often seen in tibial regions bilaterally as a chronic eruption; common in diabetic patient.

Answer 16

Schamberg’s purpura

Question 17

• distinctive purpuric eruption in association with
  various constitutional and localized symptoms
• result of an autoimmune process or allergic
  vasculitis

Answer 17

Allergic purpura

Question 18

Due to excessive destruction or loss; defficient production in bone marrow

Answer 18

Thrombocytopenia

Question 19

Characterized by various degrees of thrombocytopenia

Answer 19

May-Hegglin Anomaly

Question 20

No release reaction of ADP, ATP, 5-HT, and calcium

Answer 20

Storage pool disease

Question 21

autosomal recessive that is dense granules deficiency; and also has large lysosomal granules in WBC.

Answer 21

Chediak-Higashi

Question 22

Heridetary, probably autosomal that is alpha granules deficiency.

Answer 22

Gray platelet syndrome

Question 23

Decreased platelet fibrinogen, PF4, beta-thromboglobulin, platelet derived growth factor

Answer 23

Gray platelet syndrome

Question 24

Poor PF3 activity; SPT shortened

Answer 24

Thrombocytopathia