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Heme and Lymph > RBCs 2 > Flashcards

RBCs 2 Flashcards

1
Q

Mammalian coagulation systems are based primarily upon what enzymes

A

serine proteases

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2
Q

a coagulopathy result in what

A

either bleeding or clotting or both

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3
Q

The primary pathway for the initiation of coagulation is what?

A

the tissue factor (extrinsic) pathway

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4
Q

What test measures intrinsic pathway?

extrinsic?

A

PTT

PT

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5
Q

PTT measures what molecule?

PT?

A

heparin

coumadin/warfarin

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6
Q

Abnormal platelet counts are best confirmed by inspection of what?

A

A peripheral blood smear

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7
Q

a calculation based on PT and is generally utilized to monitor individuals who are being treated with the (oral) anticoagulant warfarin (coumadin)

A

INR

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8
Q

DIC: PT?
PTT?
bleeding time?
Platelet count?

A
  • long
  • long
  • long
  • decreased
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9
Q

Von Willebrand disease: PT?
PTT?
bleeding time?
Platelet count?

A
  • unaffected
  • long or unaffected
  • long
  • unaffected
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10
Q

Hemophilia: PT?
PTT?
Bleeding time?
Platelet count?

A
  • Unaffected
  • long
  • unaffected
  • unaffected
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11
Q

PT and PTT in uremia

A

unaffected

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12
Q

PT and PTT in Factor V deficiency

A

long

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13
Q

How do bleeding disorders caused by vessel wall abnormalities usually present?

A
  • do not usually cause serious bleeding problems
  • most often they present with small hemorrhages (petechiae and purpura) in the skin or mucous membranes, particularly the gingivae
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14
Q

Platelet count and test of coagulation (PT and PTT) in bleeding disorders caused by vessel wall abnormalities

A

usually normal

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15
Q

These are associated with microvascular bleeding that results from collagen defects that weaken vessel walls

A

Scurvy and Ehlers-Danlos syndrome

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16
Q

Protein wasting effects of excessive corticosteroid production cause loss of perivascular supporting tissue

A

Cushing syndrome

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17
Q

Systemic IgA deficiency that in the kidney causes a mesangial glomerulonephritis

A

Henoch-Schonlein purpura

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18
Q

Henoch-Schonlein purpura is characterized by what?

A

a purpuric rash especially in buttocks and abdomen, colicky abdominal pain, polyarthralgia, and acute glomerulonephritis

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19
Q

An autosomal dominant disorder that can be caused by mutations in at least five different genes, most of which modulate TGF-B

A

Hereditary hemorrhagic telangiectasia, also know as Weber-Osler-Rendu syndrome

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20
Q

What is Hereditary hemorrhagic telangiectasia characterized by?

A

dilated, tortuous blood vessels with thin walls that bleed readily. Bleeding can occur anywhere, but it is most common under the mucous membranes of the nose (epistaxis), tongue, mouth and eyes, and throughout the GI tract

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21
Q

A platelet count less than what constitutes as thrombocytopenia

A

less than 100,000

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22
Q

Platelet counts in what range may aggravate post traumatic bleeding

A

20-50k

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23
Q

Platelets less than what can be associated with spontaneous (nontraumatic) bleeding? . .petichial and cutaneous

A

less than 20k

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24
Q

bleeding from thrombocytopenia has what kind of PT and PTT

A

normal

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25
Q

What is a potential complication of any patient with a markedly decreased platelet count and is the most feared complication

A

intracranial bleeding

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26
Q

What are the 4 categories of causes of thrombocytopenia?

A
  • Decreased platelet production
  • Decreased platelet survival
  • Sequestration
  • Dilution
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27
Q

caused by autoantibody mediated destruction of platelets

A

Chronic immune thrombocytopenic purpura (ITP)

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28
Q

The thrombocytopenia of chronic ITP is usually markedly imporoved by what?

A

splenectomy

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29
Q

Chronic ITP: spleen size?
Marrow?
Peripheral blood?

A
  • normal
  • modestly increased number of megakaryocytes
  • abnormally Large platelets (megathrombocytes) which are a sign of accelerated thrombopoiesis
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30
Q

Age and gender for ITP

A

adult women under 40

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31
Q

Chronic ITP is characterized by what?

A
  • -bleeding into the skin and mucosal surfaces
  • cutaneous bleeding is seen in the form of pinpoint hemorrhages which are especially prominent in dependent areas where capillary pressure is higher
  • petechiae can become confluenet giving rise to ecchymoses
  • history of easy bruising, nosebleeds, bleeding from gums, and hemorrhages into soft tissue from minor trauma
  • may manifest first with melena, hemoturia, or excessive menstrual flow
  • subarachnoid hemorrhage and intracerebral hemorrhage are serious and sometimes fatal complications
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32
Q

splenomegaly and lymphadenopathy in chronic primary ITP

A

uncommon . . . their presence should lead one to consider other diagnosis such as ITP secondary to a B cell neoplasm

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33
Q

ACUTE ITP is mainly a disease of what age?

After what?

A
  • a disease of childhood equally in both sexes

- symptoms appear abruptly often 1 to 2 weeks after a self limited viral illnes

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34
Q

Resolution of Acute ITP?

A

self-limited usually resolving spontenously within 6 months . .Glucocorticoids are given only if the thrombocytopenia is severe

35
Q

What are the most common drugs implicated in drug induced thrombocytopenia

A
  • Quinine
  • Quinidine
  • Vancomycin
36
Q

Describe the 2 types of HIT

A
  • Type I: rapidly after initiation of therapy. Generally resolves and typically is of little clinical importance
  • Type II: less common, more clnically important. Begins 5-14 days after initiation of therapy. Results in severe, sometimes life threatening venous and arterial thrombosis, despite the thrombocytopenia
37
Q

What is one of the most common hematologic manifestations of HIV infection

A

thrombocytopenia

38
Q

contrast TTP and HUS to DIC

A

IN TTP and HUS, activation of the coagulation cascade is not of primary importance, and hence laboratory assessments of coagulation such as PT and PTT are usually normal

39
Q

cause of typical HUS

A

E. coli 0157:H7

40
Q

What causes atypical HUS

A

-alternative complement pathway inhibitor deficiencies (complement factor H, membrane cofactor protein (CD46), or factor I . . especially during pregnancy

41
Q

deficiency is ADAMTS13

A

TTP: thrombotic thrombocytopenic purpura

42
Q

What does ADAMTS13 normally do?

A

normally degrades very high molecular weight multimers of von Willebrand factor (vWF). In its absence they can accumulate in plasma and tend to promote platelet activation and aggregation

43
Q

Inherited disorders of platelet function can be classified into 3 pathogenically distinct groups

A
  • defects of adhesion
  • defects of aggregation
  • disorders of platelet secretion
44
Q

inherited deficiency of the platelet membrane glycoprotein complex ib-IX. This glycoprotein is a receptor for vWF and is essential for normal platelet ADHESION to the subendothelial ECM

A

Bernard-Soulier syndrome

45
Q

autosomal recessive defect of platelet aggregation due to deficiency or dysfunction of glycoprotein IIb-IIIa

A

Glanzmann thrombasthenia

46
Q

The most common and important INHERITED deficiency of coagulation factors affect what factors? called what?

A
  • VIII (hemophilia A)

- IX (hemophilia B)

47
Q

A deficiency in this influences both coagulation and platelet function

A

vWF

48
Q

ACQUIRED deficiencies of coagulation factors usually involve ____

A

multiple

49
Q

vitamin K deficiency results in the impaired synthesis of which factors

A

II, VII, IX, X

50
Q

What is the most common inherited bleeding disorder

A

Von Willebrand disease

51
Q

What types of Von Willebrand Disease exhibit quantitative defects in vWF?
Qualitative?

A

I and III

II

52
Q

Which type of Von Willebrand Disease has very low levels of vWF and has severe clinical manifestations that may resemble hemophilia

A

Type III

53
Q

What measures the plasma level of active vWF

A

ristocetin cofactor activity . . reduced in Von Willebrand disease

54
Q

Because vWF stabilizes factor VIII, a deficiency in vWF gives rise to a secondary decrease in factor VIII levels, reflected as what?

A

increased PTT in types 1 and 3

55
Q

Persons with von Willebrand disease facing hemostatic challenges like dental work or surgery can be treated with what?

A

desmopressin which stimulates vWF release, or with infusions of plasma concentrates containing factor VIII and vWF

56
Q

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

A

Hemophilia A

57
Q

inheritance of hemophilia A

A

X linked recessive . .affects mainly males and homozygous females

58
Q

clinical features of Hemophilia A

A
  • easy bruising and massive hemorrhage
  • hemarthroses
  • petechiae ABSENT
  • Prolonged PTT
  • normal PT
59
Q

What particular sites do hemophiliacs tend to bleed

A

joints, muscles, CNS

60
Q

how is hemophilia A treated

A

infusions of recombinant factor VIII

61
Q

What is the crippling effect of repeated bleeds into a joint seen in hemophilia A

A
  • loss of range of motion
  • muscle loss
  • destruction of joints themselves
62
Q

Other names for Hemophilia B

A
  • Christmas Disease

- Factor IX deficiency

63
Q

Hemophilia A vs. B clinically

A

indistinguishable

64
Q

What can you look for with DIC

A

fibrin split products

65
Q

What are the two major mechanisms that trigger DIC?

A
  • release of tissue factor, or other poorly characterized procoagulants into circulation
  • widespread injury to endothelial cells
66
Q

About 50% of DIC patients are what?

A

obstetric pts having complications of pregnancy and the disorder tends to be reversible with delivery of the fetus

67
Q

What is acute DIC associated with

A

obstetric complications or major trauma for example is dominated by a BLEEDING diathesis

68
Q

What is chronic DIC associated with

A

cancer patients . . tends to present with thrombotic complication

69
Q

describe most of the complications of transfusion

A

minor or transient

70
Q

What is the most common complication of Transfusion?

Describe it

A
  • febrile nonhemolytic reaction
  • takes form of fever and chills, sometimes with mild dyspnea, within 6 hours of a transfusion of red cells or platelets
  • thought to be caused by inflammatory mediators derived from donor leukocytes
  • Symptoms respond to antipyretics and are short lived
71
Q

Severe, potentially fatal allergic reactions may occur when blood products containing certain antigens are given to previously sensitized recipients. These are most likely to occur in what patients?

A
  • IgA deficiency

- IgG antibodies recognize IgA in the infused blood

72
Q

This transfusion reaction may be triggered by the presence of an allergen in the donated blood that is recognized by IgE antibodies in the recipient

A

Urticarial allergic reactions

73
Q

describe the Urticarial allergic reactions

A

generally mild . . most respond to antihistamines and do not require discontinuation of the tranfusion

74
Q

These are usually caused by preformed IgM antibodies against donor red cells that fix complement

A

Acute hemolytic reactions . . ABO incompatibility

75
Q

Direct Coombs test with Acute hemolytic reactions

A

positive

76
Q

clinical symptoms of acute hemolytic reactions to transfusion

A

Fever, shaking chills, and flank pain appear rapidly

77
Q

Reaction caused by IgG antibodies to foreign protein antigens and are associated with a positive direct Coombs test and lab features of hemolysis (low haptoglobin and elevated LDH)

A

Delayed hemolytic reactions

78
Q

in a delayed hemolytic reaction to transfusion, antibodies to what often induce sufficient complement activation to cause severe and potentially fatal reactions identical to those resulting from ABO mismatches

A

Rh, Kell, Kidd

79
Q

A severe, frequently fatal complication in which factors in a transfused blood product trigger the activation of neutrophils in the lung microvasculature

A

TRALI: transfusion-related Actue Lung Injury

80
Q

By far, the most common antibodies associated with TRALI are those that bind what? found in what people?

A
  • MHC

- particularly MHC class I that are often found in multiparous women

81
Q

TRALI is most likely to occur with products containing high levels of donor antibodies such as what?

A

fresh frozen plasma and platelets

82
Q

What is the clinical presentation of TRALI

A
  • dramatic with sudden onset respiratory failure during or soon after a transfusion
  • Diffuse bilateral pulmonary infiltrates that do not respond to diuretics are seen on chest imaging
  • other associated findings include fever, hypotension, and hypoxemia
83
Q

Significant bacterial contamination in a transfusion is much more common in what preparations?

A

platelets rather than red cell preparations due in large part to the fact that platelets must be stored at room temp

Heme and Lymph (27 decks)

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