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Blood & Lymph > Bleeding Disorders 1 > Flashcards

Bleeding Disorders 1 Flashcards

1
Q

What is the first question to ask when a patient has a bleeding disorder?

A

Is it a platelet problem? Or is it at coagulation problem?

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2
Q

2 events of the first phase of hemostasis

A
  1. Arterial vasoconstriction (endothelin mediated)
  2. Platelet adhesion → formation of platelet plug
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3
Q

Events of secondary hemostasis

A
  1. Coagulation
  2. Clot resorption
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4
Q

Classification of bleeding disorders

A
  1. Thrombocytopenia and platelet disorders
  2. Coagulation defects
  3. Fragility of vessel walls
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5
Q

What 2 labs would you order to determine whether the problem is platelet sarcoid relation?

A
  1. CBC
  2. Coagulation tests: PT, PTT
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6
Q

Normal number of platelets

A

150,000 to 400,000

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7
Q

Below _____ = post-traumatic bleeding; below _____= spontaneous bleeding

A
  • 50,000
  • 20,000

(platelets can function from 50,000-150,000)

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8
Q

Half-life of platelets

A

9 days

(2-3 if transfused)

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9
Q

Intrinsic system of coagulation is activated by _____ and involve factors _____.

A
  • Collagen exposure
  • XII, XI, IX, X
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10
Q

Regulation pathways converge at factor ______

A

X → Xa + V

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11
Q

The extrinsic system of coagulation is activated by ______

A

external trauma tissue factor

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12
Q

PT

A

Prothrombin time

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13
Q

PT monitors the ____ pathways by measuring the ______.

A
  • Extrinsic and common pathways
  • clotting of plasma after thromboplastin & Ca2+ ions
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14
Q

PTT monitors the _____ pathways by measuring the _____.

A
  • intrinsic and common
  • clotting of plasma after kaolin, cephalin & Ca2+ ions
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15
Q

Factor V, X, prothrombin and fibrinogen are all measured in BOTH PT & PTT, which factors are specific to the PT pathway? PTT pathway?

A
  • VII
  • VIII, IX, XI, XII
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16
Q

What is the main difference equation between platelets and coagulation problems clinically?

A

Platelet problems are mostly superficial bleeding

(skin & mucous sx)

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17
Q

What is the difference between petechiae, purpura and ecchymoses?

A
  • Petechiae: 1-2 mm
  • Purpura: 2mm - 1 cm
  • Ecchymoses: > 1cm
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18
Q

These are typical of which type of general bleeding disorders?

A

Platelet disorders → superficial bleeding

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19
Q

What is the biggest threat a platelet disorders?

A

Intracranial bleeding

(rare, but most serious)

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20
Q

Once you determine the patient has a platelet dysfunction what is the next question to ask?

A

Too few platelets or defective platelets

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21
Q

4 Causes of thrombocytopenia

A
  1. Decrease survival
  2. Decrease production
  3. Sequestration
  4. Dilution

(MC)

22
Q

Decrease production of platelets is commonly due to ______ (6).

A
  1. Aplastic anemia
  2. B12, folate deficiency
  3. Drugs/EtOH
  4. Infection
  5. Leukemia
  6. myelodysplastic syndrome (ineffective hematopoiesis)
23
Q

How does heparin cause decreased platelet production?

A

anti-heparin-PF4 ab → activate platelets & pro-thrombotic state → depletion

24
Q

List the drugs that can cause a decreased production of platelets (6).

A
  1. Chemotherapy
  2. Heparin
  3. Quinidine
  4. Radiotherapy
  5. Sulfur compounds
  6. Thiazides
25
Q

Why is concomitant thrombocytopenia a common problem in cancer patients _______?

A
  1. Bleeding risk
  2. Thrombocytopenia limits chemotherapy schedule
26
Q

In evaluating a thrombocytopenic cancer patient you must check for ______ (6).

A
  1. Coagulopathy
  2. Drug reactions
  3. Infection
  4. ITP
  5. Post-transfusion purpura
  6. TTP & HUS
27
Q

Which chemotherapy drugs have the highest rate of inducing thrombocytopenia (2)?

A
  1. Gemcitabine
  2. Platinum-based regimens
28
Q

How do the following chemotherapy agents → thrombocytopenia:

  1. alkylating agents affect ______.
  2. cyclophosphamide agents affect _______.
  3. bortezomib prevents ______.
A
  1. stem cells
  2. later megakaryocyte progenitors
  3. platelet release from megakaryocytes

(some treatments promote platelet apoptosis)

29
Q

The main regulator of platelet production is ______ and is therefore used as a treatment for thrombocytopenic patients with cancer to receive chemo.

A

thrombopoietin

30
Q

Classical causes of thrombocytopenia (decreased survival of platelets)

A
  1. ITP (immune thrombocytopenia)
  2. TTP
  3. HUS
31
Q

In ITP (immune thrombocytopenia), antibodies destroy the platelets. What are the secondary causes?

A
  1. SLE
  2. HIV
  3. CLL
32
Q

In ITP, IgG anti-platelet antibodies opsonize the ______.

A

platelets → phagocytosis of platelets/megakaryocytes in the spleen

(GpIIb/GpIIIa on platelets which mediates primary hemostasis)

33
Q

ITP is most commonly seen in which patient population

A

women under 40

34
Q

ITP s/sx

A
  1. petechiae
  2. mucosal bleeding (nose bleed, excessive bleeding from gums)
  3. ecchymoses
35
Q

What is thrombotic thrombocytopenic purpura

A

excessive platelet activation → deposit as thrombi in small blood vessels

36
Q

Pentad for TTP

A

Mn: “Nasty Fever Ruined My Tubes”

  • N – Neurological symptoms,
  • F – Fever
  • R – Renal function impairment
  • M – Microangiopathic hemolytic anemia
  • T – Thrombocytopenia
37
Q

TTP is associated with an enzyme deficiency of ______ which leads to _____.

A
  • ADAMTS13 (vWF metalloprotease)
  • vWF accumulate → platelet activation & aggregation

(acquired auto-ab or inherited)

38
Q

TTP patients must avoid

A

transfusion of blood cells (especially platelets)

39
Q

TTP tx (3)

A
  1. plasma transfusions (plasmapheresis)
  2. ASA & persantine
  3. prednisone

(blood will increase the progression of disease)

40
Q

Typical HUS is caused by ______

A

shiga-like toxin of E. coli O157:H7

41
Q

In typical HUS, shiga-like toxin of E. coli O157:H7 damages _____.

A

endothelial cells, activates platelets → aggregation

42
Q

Symptoms of HUS

A

bloody diarrhea

(after HUS exposure)

43
Q

Atypical HUS is caused by _____

A

deficiency of alternative pathway complement inhibitor (CD46 or factor I)

(inherited or acquired by antibody production)

44
Q

Atypical HUS may be triggered by _____ (4).

A
  1. Rx
  2. Radiation
  3. Infection: HIV, pneumococcal
  4. SLE, lymphoid neoplasms
45
Q

How do you distinguish from DIC & HUS?

A
  1. TTP & HUS is a platelet problem
  2. DIC is a coagulation + platelet problem

(PT & PTT is normal in TTP & HUS)

46
Q
A

HUS: glomerulus w/capillary fibrin thrombi, karyorrhexis & entrapped fragmented RBCs

47
Q
A

HUS : Diffuse glomerular basement membrane multilayering

(PAS staining)

48
Q

In addition to ITP, TTP & HUS, what are the 2 other causes of thrombocytopenia?

A
  1. Sequestration of platelets in spleen
  2. transfusions w/non-viable platelets → dilution
49
Q

Spleen findings in thrombocytopenia

A

normal size, but congested sinusoids & enlarged follicles

50
Q

bone marrow findings in thrombocytopenia

A

increased number of megakaryocytes

51
Q

peripheral blood findings of thrombocytopenia

A

megathrombocytes

Blood & Lymph (29 decks)

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