Bleeding Disorders- Lecture Flashcards

1
Q

(Platelet bleeding/ factor bleeding) is spontaneous, immediate, superficial bleeding

A

Platelet bleeding is spontaneous, immediate, superficial bleeding
* Problem is with primary hemostasis

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2
Q

Skin bleeding like petechiae is usually a symptom of (platelet/ factor) bleeding

A

Skin bleeding like petechiae is usually a symptom of platelet bleeding
* Superficial

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3
Q

Mucous membrane bleeding like nosebleeds, heavy menstrual cycles, gingival bleeding are usually symptoms of (platelet/ factor) bleeding

A

Mucous membrane bleeding like nosebleeds, heavy menstrual cycles, gingival bleeding are usually symptoms of platelet bleeding

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4
Q

(Platelet bleeding/ Factor bleeding) is delayed bleeding after soft platelet plug washes away

A

Factor bleeding is delayed bleeding after soft platelet plug washes away

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5
Q

Hematoma (deep soft tissue bleeding) and hemarthrosis (joint bleeding) are signs of (platelet/ factor) bleeding

A

Hematoma (deep soft tissue bleeding) and hemarthrosis (joint bleeding) are signs of factor bleeding

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6
Q

Thrombocytopenia < 50,000 platelets is associated with _ symptoms

A

Thrombocytopenia < 50,000 platelets is associated with easy bruising, petechiae

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7
Q

Thrombocytopenia < 10,000 platelets is associted with _

A

Thrombocytopenia < 10,000 platelets is associted with spontaneous bleeding

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8
Q

Normal platelet count is _

A

Normal platelet count is 150-450 cells

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9
Q

The most common inherited bleeding disorder is _

A

The most common inherited bleeding disorder is von willibrand disease

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10
Q

von Willibrand disease is most commonly inherited in a _ pattern

A

von Willibrand disease is most commonly autosomal dominant

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11
Q

Name the primary triad of sx associated with von willibrand disease

A

vWBD is usually mild with mucocutaneous bleeding

  1. heavy menstrual bleeding
  2. epistaxis
  3. easy bruising
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12
Q

vWF is primarily produced in _ cells

A

vWF is primarily produced in endothelial cells within weibel-palade bodies
* Can also be made by platelets in alpha granules

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13
Q

Two functions of vWF

A
  1. vWF binds platelets to the SEC via GP1b
  2. vWF stabilizes VIII in the circulation and prevents its degradation
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14
Q

von Willebrand disease can be called a “mixed platelet and coagulation disorder” explain

A

Defect in vWF –> inability to form platelet plug
AND: reduced factor VIII levels

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15
Q

_ is the most common type of von Willebrand disease

A

Type I is the most common type of von Willebrand disease –> quantitative defect

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16
Q

Type I vWBD is caused by a _

A

Type I WBD is caused by a decreased production of vWF

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17
Q

Type 2A, 2B, 2M vWBD are considered _ disorders

A

Type 2A, 2B, 2M vWBD are considered qualitative disorders

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18
Q

Type 3 vWBD is associated with a _

A

Type 3 vWBD is associated with a complete lack of vWF
* Very rare- autosomal recessive

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19
Q

Type 2B vWBD is _

A

Type 2B vWBD is hyperfunctional high molecular weight multimers –> spontaneous platelet aggregation –> unintented decrease in vWF
* We end up using up all of our vWF

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20
Q

What lab values would we expect to see in vWBD?

A
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21
Q

_ is a medication that can be given to patients with von willebrand disease to increase the release of vWF from weibel palade bodies within the endothelial cells

A

Desmopressen is a medication that can be given to patients with von willebrand disease to increase the release of vWF from weibel palade bodies within the endothelial cells

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22
Q

If a patient with von Willebrand disease is being treated with desmopressin and is not improving it probably indicates _

A

If a patient with von Willebrand disease is being treated with desmopressin and is not improving it probably indicates patient has a qualitative defect in vWF
* Desmopressin is only useful in type I vWBD
* Only useful if patients have residual endothelial storage of vWF

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23
Q

The most common factor deficiency is _ which is _

A

The most common factor deficiency is factor 8 which is hemophilia A

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24
Q

Hemophilia B is a mutation in the factor _ gene

A

Hemophilia B is a mutation in the factor IX gene

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25
Q

Hemophilia C is a mutation in the factor _ gene

A

Hemophilia C is a mutation in the factor XI gene

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26
Q

Inheritance of hemophilia A and B

A

Inheritance of hemophilia A and B: x-linked recessive
* Heterozygous females are carriers but really they have mild hemophilia
* Most commonly seen in males

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27
Q

Females with more extreme low levels of VIII may have _

A

Females with more extreme low levels of VIII may have lyonization
(inactivation of one of the X chromosomes)

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28
Q

Hemophilia C has _ inheritence

A

Hemophilia C has autosomal recessive inheritence

29
Q

The _ can be prolonged in hemophilia A or B but will correct with a _

A

The PTT can be prolonged in hemophilia A or B but will correct with a mixing study

30
Q

Two options to treat hemophilias:

A
  1. Factor 8, 9, 11 concentrates
  2. Desmopressin (hemophilia A)- vWF increases the stability of vWF
31
Q

_ is an inherited defect in the GP1b receptor

A

Bernard-Soulier syndrome is an inherited defect in the GP1b receptor
* Prevents platelet binding to vWF

32
Q

_ is an inherited defect in the GPIIb/ IIIa receptor

A

Glanzmann thrombasthenia is an inherited defect in the GPIIb/ IIIa receptor
* Prevents fibrinogen from binding platelets together

33
Q

(Bernard-Soulier/ Glanzmann) will present with large platelets on blood smear

A

Bernard-Soulier will present with large platelets on blood smear

34
Q

_ is an inherited disorder that leads to reduced or absent alpha granules

A

Gray platelet syndrome is an inherited disorder that leads to reduced or absent alpha granules
* Cannot make platelet plugs
* Large, gray platelets on PB smear

35
Q

_ and _ are two inherited delta-granule disorders

A

Hermansky Pudlak syndrome and Chediak Higashi syndrome are two inherited delta-granule disorders
* Both are autosomal recessive

36
Q

Patient presents with hypopigmentation and dysfunction of their platelets (on PB smear), pulmonary fibrosis, colitis, neutropenia, and immunodeficiency

A

Patient presents with hypopigmentation and dysfunction of their platelets (on PB smear), pulmonary fibrosis, colitis, neutropenia, and immunodeficiency: Hermansky Pudlak syndrome
* Autosomal recessive delta granule deficiency

37
Q

Patient presents with recurrent bacterial infections, neuropathy, ataxia, and oculocutaneous albinism and platelet dysfunction

A

Patient presents with recurrent bacterial infections, neuropathy, ataxia, and oculocutaneous albinism and platelet dysfunction: Chediak higashi syndrome

38
Q

Uremia (typically from renal failure) has the effect of interrupting _ and can therefore increase bleeding risk

A

Uremia (typically from renal failure) has the effect of interrupting GPIIb/ GPIIIa (receptor aggregation) and can therefore increase bleeding risk

39
Q

_ is a medication that decreases thromboxane A2 and decreases platelet aggregation;
_ is a medication that prevents fibrinogen from binding GPIIb/IIIa receptors

A

Aspirin is a medication that decreases thromboxane A2 and decreases platelet aggregation;
Clopidogrel is a medication that prevents fibrinogen from binding GPIIb/IIIa receptors

40
Q

Bernard-Soulier

A
41
Q

Glanzmann thrombasthenia

A
42
Q

Gray platelet syndrome

A
43
Q

delta-granule deficiency

A
44
Q

vWBD

A
45
Q

Triad of HELLP syndrome

A

HELLP:
1. Hemolysis
2. Elevated Liver enzymes
3. Low Platelets

associated with pre-eclampsia in pregnant women

46
Q

HELLP syndrome, hypertensive emergencies, and prosthetic heart valves can all lead to _

A

HELLP syndrome, hypertensive emergencies, and prosthetic heart valves can all lead to microangiopathic hemolytic anemia –> schistocytes on PB smear

47
Q

Explain the mechanism of DIC

A
  1. Something triggers the coagulation cascade to be hyperactive
  2. Unnecessary fibrin forms in vessels all over the body
  3. Fibrin gets lodged in small vessels
  4. Platelets join fibrin and form small thrombi
  5. We begin to exhaust our platelets and clotting factors –> bleeding
  6. RBCs are getting ripped apart as they go past thrombi –> schistocytes
48
Q

We can identify DIC on peripheral blood smear by the presence of _

A

We can identify DIC on peripheral blood smear by the presence of helmet cells, schistocytes

49
Q

Clinical manifestations of DIC

A
  • Oozing from puncture sites
  • Petechiae
  • Thrombocytopenia
  • Respiratory failure
  • Seizures, coma
  • Acute renal failure, cardiovascular collapse
50
Q

Classic causes of DIC

A

Classical causes of DIC:
* Snake venom
* Gram-negative bacteria sepsis
* Trauma/ burns
* Acute promyelocytic leukemia
* Vasculitis

“MOST” : malignancy, obstetrics, spesis, trauma

51
Q

4 key laboratory findings associated with DIC

A
  1. Schistocytes
  2. Prolonged PT, PTT, INR, TT (due to the consumption of coagulation factors)
  3. Elevated D-dimers (breakdown of microthrombi)
  4. Decreased fibrinogen
52
Q

Thrombotic thrombocytopenia purpura is caused by a deficiency in _

A

Thrombotic thrombocytopenia purpura is caused by a deficiency in ADAMTS13
* Without ADAMTS13 we can’t cleave ultra-large vWF multimers
* We get excessive formation of microthrombi in small vessels –> deplete our platelets and we rip apart RBCs

53
Q

(Acquired/ Inherited) TTP is more common

A

Acquired TTP is more common
* Results from a formation of an autoantibody that prevents ADAMTS13 from functioning normally

54
Q

Symptoms of TTP

A

Symptoms of TTP:
* MAHA- anemia with schistocytes
* Thrombocytopenia- petechiae and purpura
* Acute renal failure- increased creatinine
* Fever
* Neurologic abnormalities- altered mental status

55
Q

One treatment option of TTP includes removing defective ADAMTS13 and replacing with normal ADAMTS13 via _

A

One treatment option of TTP includes removing defective ADAMTS13 and replacing with normal ADAMTS13 via therapeutic plasma exchange

56
Q

We can also treat TTP with a monoclonal ab fragment medication called _ that binds to vWF and blocks its interaction with platelets to prevent microthrombi formation

A

We can also treat TTP with a monoclonal ab fragment medication called caplacizumab that binds to vWF and blocks its interaction with platelets to prevent microthrombi formation

57
Q

We can also treat acquired TTP with a medication to suppress the immune system such as _ or _

A

We can also treat acquired TTP with a medication to suppress the immune system such as glucocorticoids or rituximab

58
Q

_ is a condition in which bacterial toxins cause damage to the endothelium and lead to platelet activation and aggregation

A

Hemolytic uremic syndrome is a condition in which bacterial toxins cause damage to the endothelium and lead to platelet activation and aggregation
* E.Coli O157:H7
* Shiga toxin

59
Q

Triad of Sx associated with HUS

A
  1. Hemolytic microangiopathic anemia
  2. Thrombocytopenia
  3. Acute renal failure

Shares all of these symptoms with TTP

60
Q

What is unique about HUS that distinguishes it from TTP?

A

HUS is usually associated with bloody diarrhea
* Question stems often include “child ate a burger”

61
Q

DIC:
Platelet count
PT
PTT
Fibrinogen

A

DIC:
Platelet count decreased
PT increased
PTT increased
Fibrinogen decreased

Note: DIC thrombi are composed of platelets and fibrin so we see consumption of coagulation factors

62
Q

TTP or HUS
Platelet count
PT
PTT
Fibrinogen

A

TTP or HUS
Platelet count decreased
PT normal
PTT normal
Fibrinogen normal

Note that the thrombi are mostly composed of platelets –> no consumption of coagulation factors

63
Q

Immune thrombocytopenia (ITP) is caused by _

A

Immune thrombocytopenia (ITP) is caused by autoantibodies to platelet glycoproteins (GPIIb/ GPIIIa)
* Platelets get sent to the splenic macrophages for destruction –> thrombocytopenia
* Mild cases: easy bruising
* Severe cases: large areas of bleeding in the skin

64
Q

Chronic ITP is most common in _ populations

A

Chronic ITP is most common in adult women < 40
* It is often secondary to lupus, HIV, infection
* Sx: petechiae, heavy menstrual bleeding, easy bruising

65
Q

Acute ITP is most common in _ populations

A

Acute ITP is most common in children following a viral illness
* The antibodies generated against infection are cross-reacting with platelets
* Usually self limiting
* Sx: petechiae, bruising, bleeding, abrupt onset

66
Q

How would ITP present on peripheral blood smear and bone marrow biopsy

A
  • Larger platelets on smear
  • Increased immature (small) megakaryocytes on bone marrow biopsy
67
Q

What does this indicate?

A

Error in blood collection

68
Q

Treatment options for ITP

A
  • Steroids
  • IVIG
  • Platelet transfusions
  • Thrombopoietin receptor agonist
  • Splenectomy