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Heme/Onc > Lecture 6: Bleeding Disorders > Flashcards

Lecture 6: Bleeding Disorders Flashcards

1
Q

What is primary hemostasis?

A

Activation of platelets and formation of initial platelet plug in wall of injured vessels

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2
Q

What screening tests are included for primary hemostasis? (3)

A

(1) Platelet count
(2) Platelet function assay (PFA)
(3) Platelet aggregation studies- PT, PTT, thrombin time

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3
Q

What is secondary hemostasis?

A

Activation of plasma coagulation factors and formation of a fibrin clot

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4
Q

What screening tests are included in secondary hemostasis? (3)

A

(1) Prothrombin time (PT)
(2) Partial Thromboplastin Time (PTT)
(3) Thrombin Time (TT)

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5
Q

What is the difference between primary hemostasis and secondary hemostasis?

A

Primary hemostasis is a platelet-type disorder while secondary hemostasis is a clotting cascade problem

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6
Q

What will you see on physical exam for primary hemostasis? (5)

A

(1) Superficial bleeding
(2) Mucocutaneous bleeding
(3) Petechiae
(4) Immediate bleeding after trauma
(5) Bleeding controlled by direct pressure

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7
Q

Which hemostasis is not controlled by direct pressure?

A

Secondary hemostasis

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8
Q

What will you see on physical exam for secondary hemostasis? (4)

A

(1) Deep muscle bleeding: Hemarthrosis
(2) NOT petechiae
(3) Delayed bleeding after trauma
(4) Not controlled by direct pressure

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9
Q

When do you want to pursue further workup? (in relation to abnormal labs and bleeding history)

A

When you have abnormal labs AND a bleeding history!

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10
Q

When do you suspect a PLT disorder? (PLT=platelet) (4)

A

(1) Bleeding pattern consistent with quantitative or qualitative PLT dysfunction
- superficial bleeding
- mucocutaneous bleeding
- petechiae
- immediate bleeding after trauma/surgery
(2) Thrombocytopenia
(3) Abnormal # or size PLT on blood smear
(4) Abnormal PLT aggregation studies or PFA

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11
Q

What are 2 inherited PLT disorders? What are 2 major differences in diagnosing these disorders?

A

(1) Bernard-Soulier
- Thrombocytopenia and giant PLT size
- PLT aggregation studies NORMAL except to ristocetin
(2) Glanzmann thrombasthenia
- Normal PLT count and size
- PLT aggregation studies ABNORMAL except to ristocetin

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12
Q

What is the treatment of Inherited PLT disorders if bleeding?

A

PLT transfusion if bleeding

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13
Q

What is defective on PLT with Bernard-Soulier disorder? Glanzmann thrombasthenia?

A

Defective GpIb/IX complex (receptor to vWF); Defective GpIIB/IIIa (receptor for fibrinogen)

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14
Q

T/F Both Bernard-Soulier and Glanzmann thrombasthenia are autosomal dominant inheritance.

A

FALSE; autosomal RECESSIVE inheritance

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15
Q

What are 2 acquired PLT disorders?

A

(1) Drug induced qualitative platelet disorder

2) Immune Thrombocytopenic Purpura (ITP

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16
Q

What is seen with ITP (in regards to blood)?

A
  • Isolated thrombocytopenia

- Blood count is normal

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17
Q

What is the pathophysiology of ITP? (2)

A

(1) Immune mediated destruction

(2) Decreased PLT production

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18
Q

When is spontaneous remission of ITP seen?

A

In children; NOT in adults (uncommon)

19
Q

What is the point of differentiation between patients with ITP that have increased mortality rate?

A

The “Safe” vs. “Normal” PLT count; though you may have less PLT count, you will have NO excess mortality in patients with mild to moderate disease as they have a “safe” number of platelets

20
Q

How do you diagnose ITP?

A

Diagnosis of exclusion

  • Isolated thrombocytopenia
  • History, PE, CBC, and PBS do not suggest other etiologies
21
Q

When would you consider doing a bone marrow biopsy? (in regards to diagnosing ITP)

A

To rule out cancer in patients >60 y/o

22
Q

What is most common treatment for ITP?

A

DO NOTHING!

23
Q

What will happen to PTT, PT, TT in secondary hemostasis?

A

They will be prolonged

24
Q

What are 2 types of hemophilia? What are their deficiencies?

A

(1) Hemophilia A (classic hemophilia)- defect in Factor VIII

(2) Hemophilia B (Christmas disease)- defect in Factor IX

25
Q

Why does hemophilia effect primarily males?

A

Because it is an x-linked recessive disorder

- Genes encoding for factors VIII and IX are located on X-chromosome

26
Q

T/F Hemophilia can present as an acquired disorder.

A

True. Rarely, it can be antibody mediated as well

27
Q

Which type of hemophilia is more common? Why?

A

Hemophilia A (80-85%); because the FVIII gene is larger and thus more susceptible to mutations

28
Q

What is the variability (in levels) in severity of bleeding for hemophilia? (3)

A

(1) Factor VIII levels 5-25%- MILD; very few problems unless surgery or major trauma
(2) 1-5%- MODERATE; bleeding after minor trauma
(3) <1%- SEVERE; presents in childhood

29
Q

What are the various sites of bleeding in hemophilia? (4) What is absent?

A

(1) Intra-articular bleeding
(2) Intra-muscular hemorrhage
(3) Hematuria
(4) Life threatening hemorrhage
- GI bleeding
- Retroperitoneal
- Intracranial
- Retropharyngeal
~Absent: Petechiae and superficial ecchymoses~

30
Q

What are the most common sites of bleeding with hemophilia?

A

Hemarthrosis- joints

31
Q

What are the confirmatory labs for hemophilia when you have prolonged PTT?

A

(1) 1:1 PTT mixing study

(2) Coagulation factor assay

32
Q

What is the 1:1 PTT mixing study?

A
  • Patient’s plasma and normal pooled plasma
  • the clotting time corrects to 50%
  • it is enough to correct the PTT
33
Q

What is hemophilia management? What do you want to avoid?

A

Factor replacement; NSAIDS

34
Q

What is Von Willebrand Disease?

A

Inherited deficiency/defect in Von Willebrand’s factor

35
Q

What does VWF do? (2)

A

(1) Platelet adhesion to subendothelial collagen and platelet-platelet binding= primary hemostasis (platelet plug)
(2) Carrier molecule for Factor VIII in plasma= secondary hemostasis (fibrin clot)

36
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand Disease

37
Q

T/F Von Willebrand disease is an autosomal dominant inheritance.

A

TRUE

38
Q

What is one different to distinguish between Von WIllebrand Disease and Hemolysis.

A

VWD is seen in males and females equally. Hemolysis= males

39
Q

Which type of VWD is most common?

A

Mild (type 1) 70-75%

40
Q

What is the difference between types 1 and 3 VWD? between 1,3 and 2?

A

type 1=partial quantitative deficiency in VWF
type 3=complete deficiency of VWF
type 2= QUALITATIVE

41
Q

What are the symptoms of type 1 VWD?

A

PLT-like bleeding
- Epistaxis, gingival bleeding, easy brusing
(excessive menstrual or post-partum bleeding)
(bleeding after surgery or dental extraction)

42
Q

What are the symptoms of type 3 VWD?

A
  • GI bleeding, hemarthrosis, deep hematomas (female patient with “hemophilia”)
  • undetectable multimers
43
Q

VWD treatment

A

DDAVP

Heme/Onc (7 decks)

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