HEMA LAB - Diseases Flashcards
Inherited disorder of platelet GpIb/IX/V
Bernard-Soulier Syndrome
caused by absence or deficiency of membrane GP IIb/IIIa complex.
Glanzmann’s Thrombasthenia
autosomal recessive disorder that is due to genetic Human Prostate-specific gene 1 (HPG-1)
Hermansky-Pudlak Syndrome
Also known as Giant platelet disorder
Bernard-Soulier Syndrome
shows severe eczema, recurrent infections, immune defects, thrombocytopenia, and small platelets.
Wiskott-Aldrich Syndrome
decrease platelet P-Selectin (CD62P)
Storage pool deficiency
Defect in surface glycoprotein sialophorin (CD43, gp115, leukosialin)
Wiskott-Aldrich syndrome
Factor V deficiency
Von Willebrand disease
What is the common complication of Uremia?
Bleeding
Result in bruising but are NOT associated with any specific disease
Primary Purpura
It is a clinical manifestation of RBC extravasation into mucosa or skin, results from rheumatologic, infectious, dermatologic, traumatic, and hematologic disorders.
Purpura
occurs due to skin fragility
Simple purpura
Due to sudden increase in capillary pressure
Mechanical purpura
Seen in older individuals, or in individuals undergoing corticosteroid therapy
Senile purpura
seen in individuals with emotional problems,
often after severe trauma or extensive surgery, which may be hypersensitive to RBC membrane components or DNA hypersensitivity
Psychogenic purpura
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.
Schamberg’s purpura
- distinctive purpuric eruption in association with
various constitutional and localized symptoms - result of an autoimmune process or allergic
vasculitis
Allergic purpura
Due to excessive destruction or loss; defficient production in bone marrow
Thrombocytopenia
Characterized by various degrees of thrombocytopenia
May-Hegglin Anomaly
No release reaction of ADP, ATP, 5-HT, and calcium
Storage pool disease
autosomal recessive that is dense granules deficiency; and also has large lysosomal granules in WBC.
Chediak-Higashi
Heridetary, probably autosomal that is alpha granules deficiency.
Gray platelet syndrome
Decreased platelet fibrinogen, PF4, beta-thromboglobulin, platelet derived growth factor
Gray platelet syndrome
Poor PF3 activity; SPT shortened
Thrombocytopathia
