Heme Lecture 4 Practice

Which pathway does PTT look at?

Intrinsic pathway

What factors are involved in the common pathway?

I, II, V, X

What is another name for factor I?

Fibrinogen

What is another name for factor II?

Prothrombin

What is another name for factor III?

Tissue factor

Which factors are vitamin K dependent?

II, VII, IX, X

If factor VII is low, what happens to the PT time?

Prolonged

A pt with INR of 4 is more likely to bleed or clot?

Bleed (less likely to clot)

Partial thromboplastin time (PTT) looks at which intrinsic factors?

XII, XI, IX, VIII

Aside from DIC, what are the other 2 causes of an increase in PTT?

Heparin and hemophilia

What is the test that is helpful as a screening test for platelet disorders?

Platelet function assay (PFA)

What is the gold standard test for platelet function that measures plt response to a panel of agonists?

Platelet aggregometry

Which test is used to determine if there's active clotting?

D-Dimer

Between Fresh frozen plasma and cryocipitate, which one contains all clotting factors and can be used for acute bleeding and/or reversal of anticoagulant effects?

Fresh frozen plasma (FFP)

What is the purpose of platelet transfusions?

prevent hemorrhage

What is the term for low platelet count (<150k)?

Thrombocytopenia

What is the precursor for platelets?

Megakaryocyte (MGK)

Kevin comes in for routine bloodwork. You see on his CBC that plt count is low and report indicates clumping. What should you do next?

Repeat CBC

When the bone marrow is unable to maintain balance/replenish turnover, what can this lead to?

marrow crowding or supression

When there is an increase consumption of platelets, like in the spleen, this would eventually lead to decrease plt circulation. How would this manifest in a physical examination?

Splenomegaly

Which cutaneous bleeding is 1-2 mm also called "pinpoint hemorrhage"?

Petechiae

If platelet count is <10,000, what would this mean in terms of spontaneous bleeding and post traumatic bleeding?

Frequent spontaneous bleeding and will always have post traumatic bleed

Which value in terms of platelet count is equivalent to MCV in RBC?

MPV (mean plt volume)

Which two lab values would you use to look for signs of hemolytic anemia?

Haptoglobin and LDH

Which lab value would you look for evidence of fibrinolysis?

D-dimer

What is the most common cause of thrombocytopenia in pregnancy?

Gestational thrombocytopenia

True or false: in gestational thrombocytopenia, all other cell lines are decreased/affected as well?

False! Only plts are decreased

True or false: you should expect resolution after delivery in gestational thrombocytopenia?

True, but keep in mind it can be delayed up to 6 wks

Aside from gestational thrombocytopenia, what other condition/syndrome can occur during pregnancy?

HELLP syndrome

What condition is considered to be a severe form of preeclampsia (severe HTN and proteinuria)?

HELLP syndrome

How should you treat a pregnant pt with HELLP syndrome (2 things)?

provide supportive care of mother

active management to deliver

What are ALL the causes of DIC?

STOP Making New Thrombi

Sepsis

Trauma

Obstetric complications

Pancreatitis

Malignancy

Nephrotic syndrome

Transfusion

In which type of DIC would labs be normal, production of plt is greater than or equal to consumption of plts and coagulation factors?

Chronic DIC (compensated)

In which disease would have a simultaneous manifestation of bleeding and clotting?

DIC

Characteristics of this disease are: bleeding at multiple sites, and widespread bleeding (purpura fulminans). What is the disease?

DIC

If DIC is malignnacy related, what syndrome can this lead to?

Trousseau syndrome

Interpret this lab:

Smear: schistocytes

Platelets: dec

D-Dimer: inc

PT/INR: Inc

PTT: Inc

DIC

If you suspect DIC but the D-Dimer is negative, what can you rule out?

DIC

The pathology of this disease is that there is antibody production against your own platelet antigens leading to increase platelet destruction. What disease/disorder is this?

Immune thrombocytopenia purpura (ITP)

What is the most common cause of thrombocytopenia?

Immune thrombocytopenia purpura (ITP)

Which type of ITP is most common in adults?

Chronic

Which type of ITP typically spontaneously resolve?

Acute

What test can be used to confirm ITP?

None- There are no tests sensitive or specific enough to confirm this diagnosis

What other conditions should you consider testing for in ITP work up?

HIV and Hep C in adults

What is the mainstay of initial therapy for ITP?

Steroids and IV immunoglobulin (IVIG)

How would you treat ITP for splenectomy resistant pts?

Immunotherapy -honestly idk what she means by this if anyone can clarify that would be amazing thank u sm !

Which condition is characterized by a deficiency/mutation in the vWF-cleaving protease ADAMTS-13?

Thrombotic thrombocytopenia purpura (TTP)

How would PT/PTT look in TTP?

normal

What is the classic pentad of TTP? (Hint: Nasty Fever Ruined My Tubes)

Neurologic deficits

Fever

Renal failure

Microangiopathic hemolytic anemia

Thrombocytopenia

Nicki, 36 year old female comes in complaining of feeling confused, weak all over, and feverish. You take her oral temperature - 101.2 F. You run some bloodwork and find evidence of hemolysis, low plt, BUN inc, GFR dec. What is your most likely diagnosis?

TTP

How would you treat a pt with familial TTP?

replace cleaving protease activity with plasma transfusion

How would you treat acquired TTP?

Plasmapheresis

What is contraindicated in TTP because of the risk of thrombosis?

Platelet transfusion

What is the most common trigger for Hemolytic uremic syndrome (HUS)?

infection

Timmy, 7 year old male, comes in to the ER with mother stating that he has been having diarrhea for the past 2 days. When taking a SAMPLE history you find:

S/S- dysentery

Allergy- NKDA

Meds: none

Past med hx: none

Last oral intake: hamburger from the streets 2 days ago

Event: the boy was playing in the streets

Bloodwork shows: hemolytic anemia, uremia, thrombocytopenia. Oral temp is 102.6, and he is now experiencing neurologic symptoms.

What do you most likely suspect?

HUS

Timmy, 7 year old male, comes in to the ER with mother stating that he has been having diarrhea for the past 2 days. When taking a SAMPLE history you find:

S/S- dysentery

Allergy- NKDA

Meds: none

Past med hx: none

Last oral intake: hamburger from the streets 2 days ago

Event: the boy was playing in the streets

Bloodwork shows: hemolytic anemia, uremia, thrombocytopenia. Oral temp is 102.6, and he is now experiencing neurologic symptoms. You suspect he may have HUS caused by the shigella toxin from E.coli in the burger he got from the streets. You provide supportive care, balance fluids/electrolytes. What should you NOT do for Timmy?

you should NOT start him on antibiotics

What complex do antibodies attack in HIT (heparin induced thrombocytopenia) ?

Heparin-PF4 complex

What is a classic physical examination finding in pts with HIT?

necrotic skin lesions at heparin injection sites

What two tests can you use to diagnose HIT?

immunoassay (test for PF4-heparin ab)

Functional assay (serotonin release assay)

Of the two tests for HIT, which one detects platelet activation after exposure to serum antibody in the presence of heparin (slower but more accurate test)?

Functional assay (serotonin release assay)

Is it safe to still continue heparin after pt is showing signs of HT?

NO! IT'S NOT SAFE, DISCONTINUE AND LABEL THEM ALLERGIC!

What is the most common inherited coagulopathy?

Von Willebrand's disease (VWD)

What is the most common type of VWD?

autosomal dominant

Selena, 32 year old female, comes in complaining of menorrhagia, bruising easily, and bleeding gums. You order some blood work, results are as follows:

Bleeding time: Inc

PTT: Inc

Ristocetin test: abnormal

What is your most likely diagnosis?

VWD

Selena, 32 year old female, comes in complaining of menorrhagia, bruising easily, and bleeding gums. You order some blood work, results are as follows:

Bleeding time: Inc

PTT: Inc

Ristocetin test: abnormal

You suspect VWD. What can you start her on that would possibly also help with her menorrhagia?

Estrogen based birth control

There are 2 types of hemophilias. Which type is a deficiency in factor IX?

Hemophilia type B

Which type of hemophilia is most common and also known as the classic type?

Type A (factor VIII)

Marshall, 8 year old male, comes in after tripping and falling to the ground a few hours ago. Neurologic exam is normal, but you notice hemarthrosis around his left patella. Blood work shows increased PTT and factor counts. What is your most likely diagnosis?

Hemophilia

What treatment would you use for a pt with hemophilia type A?

DDAV

What factors are affected if there is a vitamin K deficiency?

II, VII, IX, X