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SBL > Pathology of Red Blood Cells and Bleeding Disorders (Part 3 of 3) > Flashcards

Pathology of Red Blood Cells and Bleeding Disorders (Part 3 of 3) Flashcards

1
Q

What components are involved in primary bleeding disorders?

A

platelets, vWF, and vessel wall

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2
Q

what components are involved in secondary bleeding disorders?

A

coagulation

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3
Q

what components are involved in tertiary bleeding disorders?

A

fibrinolysis factors

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4
Q

what is the timing of bleeding for primary bleeding disorders?

A

immediate

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5
Q

what is the timing of bleeding for secondary bleeding disorders?

A

delayed

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6
Q

what is the inheritance pattern for primary bleeding disorders?

A

autosomal dominant

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7
Q

what is the inheritance pattern for secondary bleeding disorders?

A

autosomal or x-linked recessive

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8
Q

what is an example of a more extreme vessel wall disorder?

A

hereditary hemorrhagic telangiectasia

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9
Q

perivascular amyloid is a red flag for what?

A

myeloma

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10
Q

what is the technical value for thrombocytopenia?

A

platelet count less than 150,000

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11
Q

when do symptoms of thrombocytopenia typically present?

A

when platelet count is less than 50,000

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12
Q

when might you get dangerous spontaneous bleeding with thrombocytopenia?

A

when platelet count is less than 20,000

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13
Q

what could be happening to cause thrombocytopenia?

A

decreased production, decreased survival, or sequestration

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14
Q

what occurs with HIV-associated thrombocytopenia?

A

HIV can suppress marrow by infection of HSC or autoantibodies can form against platelets

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15
Q

what could cause myelosuppression?

A

chemotherapy, chloramphenicol, penicillamine, or gold salts

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16
Q

what could cause drug-induced immune thrombocytopenia?

A

quinidine, vancomycin, and heparin

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17
Q

What is an example of immunologic destruction of platelets?

A

immune thrombocytopenic purpura (ITP)

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18
Q

what is immune thrombocytopenic purpura (ITP)?

A

when antibodies are created against platelets- a sensitized platelet is covered with these opsonizing antibodies and then our own spleen sees the antibody and the splenic macrophages clear them

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19
Q

how does ITP present?

A

petechiae, purpura, CBC will show thrombocytopenia, platelet antibodies may be present, bone marrow will show increased megakaryocytes

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20
Q

how do you treat ITP?

A

by reducing the immune response using corticosteroids, IV Ig, and anti-CD20

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21
Q

what is the medication name for anti-CD20?

A

rituximab

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22
Q

What are two examples non-immunologic destruction of platelets?

A

thrombotic microangiopathies: 1. thrombotic thrombocytopenic purpura (TTP) 2. hemolytic uremic syndrome (HUS)

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23
Q

what are the symptoms of TTP?

A

the pentad: fever, thrombocytopenia, microangiopathic hemolytic anemia (MAHA), neurologic defects, renal failure

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24
Q

what are the symptoms of HUS?

A

there is an overlap of symptoms with TTP, but less neuro manifestations and more renal; HUS is more likely to be seen in kids

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25
Q

what occurs during TTP/HUS?

A

there is an exaggerated platelet plug that is activated- so instead of just a controlled normal platelet plug formation, there is a large inappropriate platelet plug; as you consume these platelets by formation of this platelet plug- thats where the thrombocytopenia is coming in; as these red cells go by, they shear against these little thrombotic foci and as they shear that’s where the actual hemolytic anemia comes from–> forms schistocytes

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26
Q

most cases of TTP are associated with a defect in what?

A

the metalloproteinases ADAMTS13

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27
Q

what do most patients with TTP respond well to and why?

A

plasma exchange therapy- because most patients with TTP have the form with the autoantibodies against ADAMTS13

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28
Q

how does plasma exchange therapy work with patients with TTP?

A

it takes away the multimers and antibodies and provides the patients with ADAMTS

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29
Q

How is HUS different than TTP?

A

it is still a TMA (thrombotic microangiopathy) but it is not associated with ADAMTS13

30
Q

what is the most likely etiology of a typical case of HUS?

A

a shiga-like toxin elaborated by E. coli O157:H7

31
Q

how does typical HUS caused by shiga-like toxin present?

A

first with bloody diarrhea, but then when you do further evaluation, you may find the triad of symptoms associated with HUS

32
Q

what is the HUS triad?

A

microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and renal insufficiency

33
Q

what is the atypical form of HUS linked to?

A

complement dysfunction

34
Q

how do you treat HUS?

A

supportive therapy often leads to full remission

35
Q

what is the general description of bernard-soulier?

A

no adhesion

36
Q

what is the general description of glanzmann thrombasthenia?

A

no aggregation

37
Q

what is the general description of storage pool disorders?

A

there is no granule (ADP/thromboxane) release

38
Q

what are two examples of acquired forms of defective platelet function?

A

aspirin use and uremia

39
Q

what is grey platelet syndrome?

A

when you don’t have alpha granules in your platelets, you’ll have a bleeding tendency, the platelets are big and gray in appearance and lack granules

40
Q

von willebrand diseases present more like platelet disorders, but one von willebrand disease will be more like the type of bleeding you get in coagulation disorders. Which type?

A

type 3

41
Q

what is the vWF-FVIII complex?

A

secreted vWF has an important role- it is going to meet up with factor VIII and takes it where it needs to go- onto the surface of the platelet plug

42
Q

Von willebrand disease is going to fall into 1 of 2 major categories. what are these categories?

A

quantitative defects and qualitative defects

43
Q

in what types of von willebrand disease are the quantitative defects seen?

A

type I and type 3

44
Q

which type of quantitative von willebrand disease is the most severe?

A

type 3- there is severely decreased/ absent vWF

45
Q

what is type 1C von willebrand disease?

A

vWF is actually created, but it is so unstable that it is rapidly degraded- so there is increased clearance of vWF in type 1C von willebrand disease

46
Q

in what types of von willebrand disease are the qualitative defects seen?

A

type 2

47
Q

how are type 2 von willebrand diseases defined?

A

a lack of appropriate interaction with ligands

48
Q

what is the most common type of qualitative von willebrand disease?

A

type 2A

49
Q

what occurs in type 2A von willebrand disease?

A

there is a lack of multimer assembly- so vWF binding to other vWF does not happen with this type

50
Q

Type 3 von willebrand disease resembles what other disorder?

A

hemophilia

51
Q

what is the inheritance pattern of hemophilia?

A

x-linked recessive

52
Q

what is the main difference between hemophilia A and hemophilia B?

A

hemophilia a is lacking FVIII; hemophilia B is lacking FIX

53
Q

how do you treat hemophilia a? how do you treat hemophilia b?

A

hemophilia a treatment: replacement factor VIII; hemophilia b treatment: replacement factor IX

54
Q

what might you have if the labs showed: increased PT, increased PTT, and decreased platelet count?

A

DIC, liver disease, or lupus

55
Q

what occurs in DIC?

A

coagulation activates, thrombi form, everything gets used up, bleeding occurs

56
Q

what is the common finding in DIC?

A

there is too much tissue factor being released and tissue factor is a very potent prothrombotic factor

57
Q

activation of plasmin causes what?

A

fibrinolysis and proteolysis of clotting factors

58
Q

what is the syndrome called when the adrenal glands hemorrhage due to DIC?

A

waterhouse-friderichsen syndrome

59
Q

with the pathophysiology of DIC, as you are consuming your clotting factors and your platelets, what will you identify?

A

increased PT/PTT and thrombocytopenia

60
Q

what does increased d-dimer reflect in a patient with DIC?

A

reflects the fibrin split products that are occurring from fibrinolysis

61
Q

Why is bacterial infections not tested in donated blood?

A

because bacterial sepsis is not going to go unnoticed by the person who has it/ donated the blood

62
Q

donated blood used for transfusion could cause an infection. What is it?

A

transfusion transmitted bacterial infection (TTBI)

63
Q

how does TTBI present and what is it most commonly associated with and why?

A

dramatic presentation of fever and hypotension during transfusion; mostly associated with platelets because they are stored at room temperature

64
Q

what is the reaction called when a person with type a blood receives type b blood?

A

acute immune hemolytic reaction

65
Q

what antibodies are responsible for the acute immune hemolytic reaction?

A

preformed IgM antibodies

66
Q

what is the reaction called when someone with rh negative blood is exposed to rh positive blood?

A

delayed immune hemolytic response

67
Q

what antibodies are responsible for the delayed immune hemolytic reaction?

A

reactive IgG antibodies

68
Q

what should you suspect if a patient has an anaphylactic reaction while being transfused?

A

IgA deficiency

69
Q

what antibodies are responsible for the anaphylactic reaction that occurs in an IgA deficient patient undergoing a transfusion?

A

IgG

70
Q

What are two examples of transfusion-related phenomena?

A

transfusion related acute lung injury (TRALI) and transfusion related immune modulation (TRIM)

71
Q

How does TRALI present?

A

acute respiratory failure during or after transfusion; CXR shows diffuse bilateral pulmonary infiltrates; may have a fever and hypotension; may be fatal

72
Q

what is transfusion related immune modulation?

A

when there is a pro-inflammatory response and an immune down regulation occurring at the same time

SBL (14 decks)

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