A 7 year old in your practice is just starting to get better from a tough bout with EBV. While palpating his spleen you notice a rash on his stomach and inner thighs that consits of multiple small nonblanching red to purple dots (ranging in size from the size of a pinhead to the size of a marker dot) You suspect what bleeding disorder? How do you treat?
ITP Watch and wait for spontaneous remission and limit physical activities or steroids pending the results of a CBC
What is the pt profile most likely to develop TTP?
A 32 y.o. female presents with fever, slight jaundice, HA, and mild confusion. On PE you note diffuse bruising to which she explains �I don�t know how that happened it just appeared�
You are looking at blood smears and note schistocytes, helmet cells and triangle forms with several reticulocytes and thrombocytopenia what does this patient have?
T or F coags and clotting factors are normal in TTP
What is the treatment for TTP?
Large volume plasmapheresis, splenectomy to prevent relapse
What is the most common inherited disorder of hemostasis?
Von Willibrand�s disease
Von Willibrands disease results from defective formation of what?
What is the treatment for von Willibrand�s disease?
DDAVP (synthetic analog of ADH which releases any endogenous stored VIII:C from tissues into circulation) or
Factor VIII concentrates
Pt complains of mucosal bleeding and gingival bleeding after a minor dental procedure
Von Willibrand�s disese or Hemophilia
What causes a prolonged aPTT but a normal PT?
Bleeding into joints
What is the inheritance pattern of hemophilia?
What are some clinical manifestations of hemophilia?
Hemarthrosis- fixed joints
Soft tissue hematomas
CNS and neck bleeding
Gum bleeding with dental procedures
What is the treatment for hemophila A
Infusion of factor VIII
What is the treatment for Hemophilia B?
Which has a prolonged PTT Hemophila A or B?
What two medications are not given to Hemophila patients
DDVAP and Asprin
A pt of Ashkenazi Jewish heritage presents with a markedly prolonged PTT, which they got checked out after having a post op bleeding complication
Factor XI deficiency (Hemophila C)
What do you treat XI with
Fresh frozen plasma since factor XI is not available
You put a 10 y.o. at your practice on antibiotics for strep infection. Their mom brings them in a week and a half later with a new rash of petechiae with diffuse ecchymosis The child has completed their antibiotic course 2 days ago and when asked about any recent changes she states that the 10 year old has been eating only ice cream and sherbert for the last week because their throat hurt. What happened and why is your patient bleeding? What lab test will be abnormal? How would you treat?
The antibiotics killed the intestinal bacteria that help produce Vit. K and the child has not been eating green leafy vegitables and soybeans that have vitamin K so they are vitamin K deficient.
They will have PT prolongation
Vitamin K supplement (phytonadione)
What clotting factors are Vitamin K dependant
II, VII, IX, X
You admit a pt with sepsis to the ICU. The next morning they are oozing blood from their IV and a place in their mouth where they bit their cheek you suspect
Explain the pathophysiology of DIC
local or systemic activation of coagulation which depletes th coagulation factors, fibrinogen and platelets
Name some disorders that can cause DIC
DIC with malignancy that manifests as thrombosis is called
What is HELLP syndrome?
Hemolysis, elevated liver enzymes, low platelets
Sever form of DIC in peripartum women
Renal dysfunction due to hemoglobinuria and pigment nephropathy
How do you treat DIC that is mild?
No treatment but eliminate the underlying cause
What can you do for severe DIC?
Hemodynamic support (fresh frozen plasma, platelets, cryoprecipitate and antithrombin II)
Heparin for DIC with thrombosis
Antithrombolytics ONLY for life threatening bleeding and failure of blood component therapy
Overproliferation of all 3 hematopoietic cell lines
Polycythemia vera is caused by a mutation where?
JAK2 signaling molecule
T or F Polycythemia vera is a condition where there is an overproduction of erythrocytes due to an increase in EPO
False it is independent of EPO
What is the Incicende of polycythemia vera?
60 y.o. male
66 y.o. M presents with HA, dizziness, tinnitus blurred vision and fatigue
What are some signs and symptoms of polycythemia vera?