platelet disorders

Question 1

vascular disorders

Answer 1

senile purpura
hereditary hemorrhagic telangectasia
Elhers-Danlos syndrome

Question 2

Senile purpura

Answer 2

older adults
degeneration of supportive collagen
normal testing results
no treatment

Question 3

Hereditary Hemorrhagic Telangectasia

Answer 3

AD inherited
lesion of dilated capillaries with abnormal connective tissue
bleeding from lesions
normal testing results
supportive treatment

Question 4

Ehlers-Danlos syndrome

Answer 4

“rubber man”
AD inherited
decreased and abnormal synthesis of collagen
bleeding due to fragile vessels
hyperextendable skin, hypermobile joints
abnormal capillary fragility
possible ab. BT

Question 5

reasons for quantitative decrease in platelets

Answer 5

decreased production
ineffective production
increased destruction
abnormal distribution

Question 6

Idiopathic thrombocytopenic purpura

Answer 6

increase destruction of plts in spleen
Ab to plts
acute: kids, rapid
chronic: adults, insidious
treat w/ steroids, splenectomy (chronic)

Question 7

hypoplasia of BM= decreased plt production

Answer 7

Fanconi’s anemia
Medications
infection
radiation
aplastic anemia
myeophthisic anemia

Question 8

ineffective production of platelets due to what disease states

Answer 8

May-Hegglin anomaly
Wiskott-Aldrich syndrome
alcohol related

Question 9

DIC

Answer 9

non-immune consumption of plts

Question 10

thrombic thrombocytopenic purpura

Answer 10

idiopathic
non immune
associated w/ pregnancy, oral contraceptives, SLE
microthrombi of plts
CNS damage and symptoms

Question 11

hemolytic uremic syndrome

Answer 11

drug induced plt Ab
increased BUN coating plts
increased BT
ab. aggregation w/ collagen
ab. 2* aggregation w/ ADP & epinephrine

Question 12

other reasons for decrease in platelets

Answer 12

splenomegaly (increased sequestration)
massive transfusion (diluting out plts)
heart bypass (mechanical damage)

Question 13

quantitative increase in platelets

Answer 13

reactive thrombocytosis
thrombocythemia

Question 14

reactive thrombocytosis

Answer 14

2* to another condition
plt # < 1mil.
transient
BT & plt fx normal
bleeding/thrombosis infrequent

Question 15

thrombocythemia

Answer 15

malignant proliferation of megakaryocytes
plt # >1mil.
associated w/ MPD
BT and plt fx abnormal
bleeding/ thrombosis infrequent

Question 16

qualitative platelet disorders

Answer 16

Bernard Soulier syndrome
Glanzman’s Thromboasthenia
Storage Pool disease
Thromboxane A2 deficiency
Gray Platelet syndrome
Uremia

Question 17

Bernard Soulier syndrome

Answer 17

mucous membrane bleeding
BT abnormal
plt # norm/dec
plt aggreg. normal to all except ristocetin
cannot correct plt aggerg. w/ NP
treatment: anti fibrinolytic drugs

Question 18

Glanzman’s Thrombasthenia

Answer 18

AR inherited
deficiency of thrombasthenin (contractile prot.)
dec. GPIIb-IIIa
BT- ab.
CR- absent
plt aggreg. ab. to all except ristocein
treatment: plt transfusion

Question 19

Storage Pool disease

Answer 19

AD inherited
deficiency of dense granules (ADP)
dec. release of ADP
mucous membrance bleeding
BT- ab.
plt aggreg. no 2* aggreg. w/ ADP/ epinephrine; no aggreg. w/ collagen
treatment: plt transfusion

Question 20

Thromboxane A2 deficiency

Answer 20

deficiency of cyclo-oxygenase (aspirin ingestion)
mucous membrane bleeding
BT- ab
plt aggreg. no 2* aggreg. w/ ADP/epinephrine; no aggreg. w/ collagen
treatment: discontinue med., plt transfusion

Question 21

Gray Platelet syndrome

Answer 21

deficiency of alpha granules
mild bleeding
normal BT, PC, CR, plt aggreg.
plts appear agranular on PBS
no therapy

Question 22

Uremia

Answer 22

mucous membrane bleeding
BT- ab.
plt aggreg. dec. w/ collagen, dec. 2* aggreg w/ ADP/ epinephrine
treatment: treat underlying cause of uremia