platelet disorders Flashcards
vascular disorders
senile purpura
hereditary hemorrhagic telangectasia
Elhers-Danlos syndrome
Senile purpura
older adults
degeneration of supportive collagen
normal testing results
no treatment
Hereditary Hemorrhagic Telangectasia
AD inherited
lesion of dilated capillaries with abnormal connective tissue
bleeding from lesions
normal testing results
supportive treatment
Ehlers-Danlos syndrome
“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
reasons for quantitative decrease in platelets
decreased production
ineffective production
increased destruction
abnormal distribution
Idiopathic thrombocytopenic purpura
increase destruction of plts in spleen
Ab to plts
acute: kids, rapid
chronic: adults, insidious
treat w/ steroids, splenectomy (chronic)
hypoplasia of BM= decreased plt production
Fanconi’s anemia
Medications
infection
radiation
aplastic anemia
myeophthisic anemia
ineffective production of platelets due to what disease states
May-Hegglin anomaly
Wiskott-Aldrich syndrome
alcohol related
DIC
non-immune consumption of plts
thrombic thrombocytopenic purpura
idiopathic
non immune
associated w/ pregnancy, oral contraceptives, SLE
microthrombi of plts
CNS damage and symptoms
hemolytic uremic syndrome
drug induced plt Ab
increased BUN coating plts
increased BT
ab. aggregation w/ collagen
ab. 2* aggregation w/ ADP & epinephrine
other reasons for decrease in platelets
splenomegaly (increased sequestration)
massive transfusion (diluting out plts)
heart bypass (mechanical damage)
quantitative increase in platelets
reactive thrombocytosis
thrombocythemia
reactive thrombocytosis
2* to another condition
plt # < 1mil.
transient
BT & plt fx normal
bleeding/thrombosis infrequent
thrombocythemia
malignant proliferation of megakaryocytes
plt # >1mil.
associated w/ MPD
BT and plt fx abnormal
bleeding/ thrombosis infrequent
qualitative platelet disorders
Bernard Soulier syndrome
Glanzman’s Thromboasthenia
Storage Pool disease
Thromboxane A2 deficiency
Gray Platelet syndrome
Uremia
Bernard Soulier syndrome
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
Glanzman’s Thrombasthenia
AR inherited
deficiency of thrombasthenin (contractile prot.)
dec. GPIIb-IIIa
BT- ab.
CR- absent
plt aggreg. ab. to all except ristocein
treatment: plt transfusion
Storage Pool disease
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
Thromboxane A2 deficiency
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
Gray Platelet syndrome
deficiency of alpha granules
mild bleeding
normal BT, PC, CR, plt aggreg.
plts appear agranular on PBS
no therapy
Uremia
mucous membrane bleeding
BT- ab.
plt aggreg. dec. w/ collagen, dec. 2* aggreg w/ ADP/ epinephrine
treatment: treat underlying cause of uremia
