BLEEDING DISORDERS CAUSED BY VASCULR DEFECTS

Question 1

This disease is a sex-linked disorder characterized by hyperextensive joints and hyperplastic skin

Answer 1

Ehlers-Danlos Syndrome

Question 2

What causes Ehlers-Danlos syndrome?

Answer 2

A defect in the peptidase enzyme that converts procollagen to collagen

Question 3

What are the laboratory findings of the Ehlers-Danlos Syndrome?

Answer 3

Normal coagulation test and normal platelet function studies

Question 4

This is an x-linked/ autosomal recessive whereas it is characterized by yellow papules on the skin and eye problems

Answer 4

Pseudoxanthoma elasticum

Question 5

This disorder is also characterized by calcified and structurally abnormal elastic fibers

Answer 5

Pseudoxanthoma elasticum

Question 6

PXE is aka?

Answer 6

Gronblad-Strandberg syndrome

Question 7

What is the importance of Vitamin C?

Answer 7

For the formation of the intact structure of the vascular basement

Question 8

This disease is characterized by gingival bleeding or gum bleeding as well as hemorrhage into subcutaneous tissues and muscles

Answer 8

Vitamin C deficiency (Scurvy)

Question 9

What are the other characteristics of Vitamin C deficiency (Scurvy)?

Answer 9

Large hemorrhagic areas may develop just below the eyes

Splinter-like hemorrhage may also appear in the fingernail beds

Question 10

What is the laboratory findings of Scurvy?

Answer 10

CFT is usually positive

Question 11

This is related to elderly being exposed to the sun that causes skin damage or the purple macules on the skin?

Answer 11

Senile purpura

Question 12

What are the laboratory findings of Senile purpura?

Answer 12

CFT= Positive
Bleeding time = normal or slightly prolonged

Question 13

This is an autosomal dominant trait characterized by thin-walled, focally disorganized and dilated blood vessels with a discontinuous endothelium appearing as a red pinpoint lesions

Answer 13

Hereditary hemorrhagic Telangiectasia (Rendu-Osler-Weber Syndrome)

Question 14

This is characterized by recurrent nosebleed?

Answer 14

Hereditary Hemorrhagic Telangiectasia (Rendu-Osler-Weber-Syndrome)

Question 15

What are the laboratory findings of Hereditary Hemorrhagic Telangiectasia?

Answer 15

BT, Platelet function test and capillary fragility and coagulation tests are NORMAL

Question 16

This occurs in infants and is associated with tumors composed of many vessels

Answer 16

Congenital Hemangiomata (Kasabach-Meritt Syndrome)

Question 17

In this hereditary alteration of vessel wall structure, fibrin clots forms, platelet consumption and red cell destruction secondary to vascular obstruction occur at the site of tumor

Answer 17

Congenital-Hemangiomata (Kasabach-meritt syndrome)

Question 18

What causes Diabetes mellitus?

Answer 18

Deposition of glycosylated proteins

Question 19

This is the deposition of glycosylated CHONs leading to thickening of capillary basement membrane affecting often the capillaries of the renal glomeruli and retina

Answer 19

Diabetes Mellitus

Question 20

This is the excessive amyloid deposit in small vessels

Answer 20

Amyloidosis

Question 21

What are the two types of Autoimmune Vascular Purpura?

Answer 21

Drug Induced Purpura
Allergic Purpura

Question 22

What are the common drugs that induce purpura?

Answer 22

Sulfonamides, iodides, procaine, penicillin, aspirin, sedatives, coumarine

Question 23

This type of purpura is associated with abdominal pain secondary to GIT hemorrhaging

Answer 23

Henoch’s purpura

Question 24

This purpura is associated with joint, especially in the knees, ankles and wrists

Answer 24

Schonlein’s Purpura

Question 25

This is associated with bacterial, viral, Rickettsial and protozoal infections

Answer 25

Infectious purpura