platelet disorders Flashcards
(31 cards)
what is glanzmann’s thrombocytopenia ?
autosomal recessive disorder associated with a mutation or deficiency in the GpIIb/IIIa receptors
what are the key diagnostic findings associated with glanzmann’s thrombasthenia ?
prolonged bleeding time
islated platelets on blood smear
absent platelet aggregation
what is the bernard soulier syndrome ?
autosomal recessive disorder , associated with a deficiency in the Gp1b receptor
what are the key findings associated with bernard soulier syndrome ?
large platelets
prolonged bleeding time
thrombocytopenia
what is the wiskott aldrich syndrome ?
immunodeficiency associated with the WAS gene necessary forT cell cytoskeleton
what is the triad associated with wiskott aldrich syndrome ?
immune dysfuction
decreased platelets
eczema
what is ITP?
idiopathic cytopenic purpura
decreased platelet survival
usually due to anti-GpIIb/IIIa antibodies
leads to consumption by splenic macrophages
what is the treatment for ITP ?
steroids
IVIg
splenectomy
what is TTP ?
thrombotic thrombocytopenic purpra
disorder of small vessel thrombus
happens due to decreased activity of vWF and ADAMST13
what is ITP percipitated by ?
viral infections, it is a type 2 HS reaction
what is the usual presentation associated with vwd ?
heavy menses
nosebleeds
excessive bleeding with tooth extraction
present both a clotting and a platelet problem
what is the presentation associated with TTP?
thrombocytopenia - petechia and bleeding
schistocytes
renal insufficiency
fever
neurological signs
what is seen on peripheral blood smear in TTP ?
schistocytes
what is MAHA ?
microangiopathic hemolytic anemia
a type of hemolytic anemia caused by shearing of RBCs
what are the features of hemolytic anemias ?
increased LDH levels
decreased haptoglobin levels
what is the differential diagnosis of schistocytes ?
TTP
HUS
DIC
MAHA
what are the lab tests seen in TTP ?
hemolytic anemia
low platelets
schistocytes
PT and PTT will be normal
what are the MAHA ?
wide term used to describe
TTP
HUS
DIC
what is HUS ?
hemolytic uremic syndrome - platelet rich thrombi that cause damage to the RBC and eventually give schistocytes
whatis the most common presentaion associated with HUS ?
no fever or CNS symptoms
TTP bas for the kidney only
usually precipitated by E coli in children - due to shiga like toxin
what is DIC ?
disseminated intravascular hemolysis
where there is widespread activation of clotting cascade
what are the conditions that can lead to DIC ?
1- obstetric emergencies , amniotic fluid contains tissue factor
2- sepsis - due to endotoxin
3- leukemia
4- rattlesnake bites
what are the lab tests associated with DIC ?
elevated Pt PTT and thrombin time
thrombocytopenia
evidence of MAHA
anemia
elevated d dimer
what is the management for DIC ?
FFP
transfusion
cryoprecipitate
