Bleeding & Thrombosis II Flashcards
Thrombotic Thrombocytopenic Purpura Classic Symptoms
Thrombocytopenia
Anemia
Fever
Neurological Problems
Renal abnormalities
TTP Clinical Manifestations
What patient populations?
Patients may present with what two types of conditions?
May c/o what? (presumably d/t what?)
Signs of CNS dz such as what can be seen?
Two major hallmarks of TTP are what?
Unlike most patients with typical thrombocytopenia, who tend to bleed excessively patients with TTP have what?
Instead they are markedly predisposed to what?
May be associated with what?
Young, adult, female, black and overweight
mucocutaneous bleeding or a thrombotic event (Phlebitis, MI or stroke)
abdominal pain (intestinal ischemia)
somnolance & coma
macroangiopathic hemolytic anemia caused by RBC fragmentation and thrombocytopenia
few hemorrhagic complications
thrombosis
autoimmune d/o
TTP Labs to get?
BMP
CBC (anemia and thrombocytopenia)
Peripheral Smear
Reticulocyte count (elevated)
LDH (elevated)
Indirect Bilirubin (elevated)
haptoglobin (decreased)
coags (normal)
fibrinogen
d-dimer (elevated)
direct antiglobulin test (negative)
ADAMS13activity
TTP check a Plasmic Score:
Platelet count?
Hemolysis (recticulocyte count? haptoglobin? indirect bilirubin?)
cancer?
organ or stem cell transplant?
MCV?
INR?
Cr?
< 30,000
>2.5%; undetectable; >2 mg/dL
no active cancer
no organ or stem cell transplant
<90 fL
< 1
<2 mg/dL
Plasmic Scores and what they indicate?
0-4?
5?
6-7?
risk of TTP?
low risk
intermediate risk
high risk
Treatment of TTP includes?
PLEX with FFP transfusions is treatment of choice
Glucocorticoids
Caplacizumab
Rituximab (off label use)
Follow LDH, Hgb, Plt
PLEX with FFP transfusions for TTP
Has what effects?
Monitoring should include?
When can PLEX be dc?
removes pathogenic antibodies and repletes deficiency of ADAMTS13
CBC, LDH, retic count, and Cr
After hemolysis has been corrected for a few days
TTP Glucocorticoid and doses?
Methylprednisolone 1g/dx3 days (high risk pts)
Prednisone 1mg/kg/d (standard risk)
Clinical Manifestations of Hemolytic Uremic Syndrome?
Abdominal pain
N/V
diarrhea
ecchymosis
AKI
Labs to get for Hemolytic Uremic Syndrome?
BMP
CBC (anemia and thrombocytopenia)
Peripheral Smear (schistocytes)
LDH (elevated)
Indirect Bilirubin (elevated)
Haptoglobin (decreased)
Coags (normal)
Fibrinogen
D-dimer (elevated)
direct antiglobulin test (negative)
UA (hematuria and proteinuria)
complement H autoantibody levels
stool culture
Hemolytic Uremic Syndrome Treatment:
Supportive care including what?
Plasmapheresis may be beneficial in what type caused by dysregulation of complement?
Eculizumab or Ravulizumab (anti-C5, antibody that inhibits the terminal step in the complement cascade) for what forms?
if indicated use what?
dialysis
atypical forms
atypical forms
HD
von Willebrand Disease Clinical Manifestations include?
Mucocutaneous bleeding symptoms
easy bruising
prolonged or excessive bleeding from minor cuts or other injuries
nosebleeds or other mucosal bleeding
GI hemorrhage
Heavy Menstrual Bleeding
Increased risk for bleeding after surgery or invasive procedures
Sx can range from mild or infrequent bleeding to severe-life threatening bleeding
Diagnostic Tests for von Willebrand disease
VWF antigen and VWF activity/antigen ratio
VWF activity: ristocetin cofactor activity (VWF;Rco), levels < 30 are diagnostic for VWD
Factor VIII coagulant activity (decreased)
CBC, Coags (prolonged PTT)
VWD Treatment
c/s who?
Prevention of what?
give what to help increase plasma levels of VWF and augments aggregation?
Other treatments include? (3)
Hematology
bleeding episodes
desmopressin 0.3mcg/kg (DDAVP)
Recombinant VWF, VWF concentrate
Cryoprecipitate
OCP
Hemophilia’s Clinical Manifestations
Spontaneous bleeding
IM hematomas
compartment syndrome
excessive bleeding with trauma or surgery
hematuria
GI bleeding
Hemophilia’s can present with Hemarthrosis, what does this look like on PE?
the joint is swollen, hot and tender to palpation, with erythema of the overlying skin.
Join mobility is compromised by pain and stiffness, and the joint is usually maintained in a flexed position
Hemophilia’s can present with what problem in newborns and adults?
ICH
Diagnosis of Hemophilia’s includes
Hx?
Prolonged what with normal what? other studies to get?
c/w Hemophilia A?
c/w Hemophilia B?
Family or personal bleeding hx
Prolonged PTT w/ normal PT, mixing study, coagulation factor specific assays
Deficiency in Factor VIII
Deficiency in Factor IX
Treatment of Hemophilia
c/s who?
Prior to surgery give what for Hemophilia A? give what for Hemophilia B?
Mild to moderate Hemophilia A patients can receive what to increase plasma levels of Factor VIII and VWF?
Antifibrinolytic agents to give?
Hematology
Factor VIII concentrate; Factor XI concentrate
DDAVP
TXA or aminocaproic acid
