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Year 2 Heme lymph > RBC II, Putthoff, Hemophilias > Flashcards

RBC II, Putthoff, Hemophilias Flashcards

1
Q

What is the most common hereditary disease assoc with life threatening bleeding

A

hemophilia A

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2
Q

what is the mutation in hemophilia A

A

mutations in facotr VIII

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3
Q

inhertiatnce hemophilia A

A

X linked recessive

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4
Q

The severity of clinical signs of hemophilia A depends on what

A

level of facto VIII activity

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5
Q

what percent of VIII activity constitutes moderately severe and mild levels hemophilia A

A

2-5% is moderately severe

6-50% mild

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6
Q

most mutations with VIII causing hemophilia A are what

A

inversion involving the X chromosome that abolishes synthesis of factor VIII

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7
Q

If immunoassay levels of VIII are normal is suspected hemophilia A case does this rule it out? why or why not?

A

no because can have point mutations that impair function

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8
Q

Clinical Sx of hemophilia A

A

easy bruising and massive hemorrhage after trauma or operative procedures
spontaneous hemorrhages in areas susceptible to trauma, like joints

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9
Q

PT and PTT of hemophilia A

A

prolonged PTT

normal PT

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10
Q

what does the complex VIIIa/IXa do

A

activate X

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11
Q

Tx hemophilia A

A

infusions of recombinant factor VIII

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12
Q

therapeutic challenge in Tx hemophilia A

A

can develop Ab to the factor VIII since “never seen before”

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13
Q

What is Hemophilia B causedby

A

factor IX deficiency

clinically indistinguishable from hemophilia A

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14
Q

inheritance hemophlia B

A

x linked recessibe trait

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15
Q

PT and PTT hemophilia B

A

PTT prolonged and PT normal

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16
Q

how to Dx hemophilia B

A

assay of factor levels

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17
Q

Tx hemophilia B

A

Tx with infusions of recombinant factor IX

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18
Q

What is DIC

A

acute, subacute or chronic thrombohemorrhagic disorder characterized by the excessive activation of coagulation and the formation of thrombi in the microvasculature of the body

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19
Q

What occurs to blood products in DIC

A

consumption of platelets, fibrin, coagulation factors

activation of fibrinolysis

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20
Q

Signs and Sx DIC

A

tissue hypoxia and infarction caused by myriad microthrombi

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21
Q

Is DIC a primary disease

A

no coagulopathy that occurs in course of variety of clinical conditions

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22
Q

describe clotting in vivo

A

TF which combines with VII to activate X and IX
X–> thrombin which activates fibrinogen to fibrin which activates IX VIII and V to stimulate crosslinking and activate platelets

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23
Q

what occurs to thrombin at distal sites from acute injury of vessels wall

A

binds thrombomodulin and becomes an anticoagulant

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24
Q

what does thrombin-thrombomodulin do

A

activates protein C which inhibits V and VIII

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25
Q

What factor is in blood to decrease blood clots

A

plasmin

26
Q

what 2 major mech trigger DIC

A

release of TF or other procoagulants and widespread injury to endothelial cells

27
Q

what is a state taht is at high risk DIC

A

widespread cancer

pregnancy

28
Q

What is an important mediator of endothelial injury

A

TNF

29
Q

DIC is most likely assoc with what

A

obstetric complications
malignant neoplasms
sepsis
major trauma

30
Q

how can infections lead to DIC

A

endotoxins

Ag-Ab complexes that activate C’

31
Q

what cancers are most freq assoc with DIC

A

acute promyelocytic leukemia, adenocarcinomas of lung, pancrease, colon and stomach

32
Q

What are signs DIC

A

dyspnea, cyanosis, respiratory failure, convulsions, coma, oliguria, acute renal failure
sudden progressive circulatory failure and shock

33
Q

Dx DIC based on

A

lab studies
fibrinogen levels
PT and PTT
fibrin split products

34
Q

what are the consequences of DIC

A

1) deposition fibrin in microvasculature leading to ischemia and microangiopathic hemolytic anemia from fragmentation RBC
2) consumption of factors leads to hemorrhagic diathesis

35
Q

What is kasabach-Merritt syndrome

A

unusual form DIC in assoc with giant hemagiomas

thrombi form in neoplasm because stasis and recurrent trauma to fragile blood vessels

36
Q

who are more severely affected by hemophilia disorders male or females?

A

males because only have one X

37
Q

most common adverse reaction to transufions

A

febrile nonhemolytic reaction

38
Q

what are signs febrile nonhemolytic reaction from transfusion

A

fever chills

mild dyspnea within 6 hrs

39
Q

what causes febrile nonhemolytic reaction from transufion

A

inflammatory mediators from donot leukocytes

40
Q

what is Tx for febrile nonhemolytic reaction

A

antipyretics

shortlived Sx

41
Q

When can severe allergic reactions occur after transufion

A

when blood with Ag is given to previously sensitized patient

42
Q

most common patients to have severe allergic rxn to transfusion

A

IgA deficiency

IgG Ab recognize IgA in infused blood

43
Q

What cause the urticarial allergic reactions after transfusions

A

donated blood recognized by IgE Ab in recipient

44
Q

Acute hemolytic reactions to transfusions are caused by what

A

preformed IgM Ab against donor RBC that fix C’

usually from error in tube labeling (ABO compatability)

45
Q

IgM Ab in acute hemolytic rxns cause what

A

C’ mediated lysis, intravascular hemolysis, hemoglobinuria

46
Q

what are Sx acute hemolytic rxn

A

fever, shaking chills and flank pain appear rapidly

47
Q

Dx of acute hemolytic rxn?

A

direct Coombs test

48
Q

rapidly progressing acute hemolytic rxn can lead to

A

DIC shock acute renal failure and death

49
Q

What are delayted hemolytic reactions to transfusions caused by

A

Ab that recognize RBC Ag that recipient was previously sensitized to
IgG

50
Q

Dx delaye hemolytic rxns transfusion

A

direct coombs

laboratory features of hemolysis: low haptoglobin and elevated LDH

51
Q

Ab to what Ag can cause severe near fatal reactions from transfusions

A

Rh Kell and Kidd

activate C’

52
Q

What is Transfusion related Acute Lung Injury

A

severe frequently fatal complication from activation of neutrophils in lung microvasculature

53
Q

What patients are at higher risk for transfusion lung injury

A

preexisiting lung disease

54
Q

What is current idea behind trasnfusion lung injury

A

2 hit hypothesis

1) priming that inc sequestration and sesitization of neutrophils in microvasc of lung
2) neutrophils activated by factor in the trasnfused blood product

55
Q

What are the most common Ab associated with transfusion acute lung injury

A

those that bind MHC Ag (MHC I)

usually multiparous women

56
Q

What types of blood products have higher neutrophil specific Ag

A

fresh frozen plasma and platelets

57
Q

what is clnical presentation of transfusion related acute lung injury

A

dramatic with sudden onset respiratory failure, during or soon after a transfusion
diffuse b/l pulmonary infiltrates that do not respond to diuretics
fever hypotension, hypoxemia

58
Q

Tx transfusion related lung injury

A

supportive and outcome is guarded (>50% in severely ill patients)

59
Q

What type infections are complications in transfusions

A

infectious-bacterial or viral

60
Q

significant bacterial infections in transfusions mroe likley in what prepartaion

A

platelet because stored at room temp

61
Q

Sx bacterial infection from transfusion

A

fever chills hypotension

62
Q

what viruses can be trasnmitted in transfusions

A

HIV Hep C and B

West nile, trypanosomiasis and babesiosis

Year 2 Heme lymph (28 decks)

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