Bleeding Disorders Flashcards

1
Q

Hemophilias

A

hemophilia A - factor 8 (VIII) deficiency
-MC*

hemophilia B - factor 9 (IX) deficiency
-Christmas disease

congenital clotting disorders

  • X-linked recessive (so–>affects males)
  • Severe pain in weight-bearing joint (hemophilic arthropathy)*

Labs: Instrinsic pathway affected so aPTT is long. Bleeding time is normal in hemophilia

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2
Q

Platelet disorders

A
  1. Dysfunction (acquired or congenital)
  2. Splenic spequestration
  3. Increased destruction
  4. Impaired production
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3
Q

Signs of bleeding

A

Petechiae (<2mm)

Purpura (2-10mm)

Ecchymosis (>1cm)

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4
Q

What are the 4 components of a basic screen for a patient you suspect has a bleeding disorder?

A
  1. CBC
  2. Peripheral smear
  3. PT/INR, aPTT
  4. Bleeding time
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5
Q

Platelet counts

A

Normal:
150,000 -450,000/mm3

Thrombocytopenia:
<100,000/mm3

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6
Q

When does spontaneous bleeding occur?

A

<20,000/mm3 platelets

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7
Q

Bleeding time

A

the measure of time from bleeding to hemostasis (coagulation)

Prolonged bleeding time:

  1. Platelet disorder (ex. von Willebrand’s Disease)
  2. Severe thrombocytopenia
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8
Q

What are the 3 stages of hemostasis

A

Just after vessel injury…

  1. Vasoconstriction
    “vascular spasm”
    -more effective in small vessels
  2. PRIMARY Hemostasis/Platelet plug formation:
    - activated by von Willebrand factor
    - platelets adhere to exposed collagen, release ADP and thrombaxane A2
  3. SECONDARY Hemostasis/Coagulation cascade:
    - formation of fibrin from fibrinogen
    - Extrinsic, Instrinsic, and Common pathways
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9
Q

What do Protein C, S, and ATIII do?

A

regulate clot formation.

If you don’t have these, you clot TOO MUCH!

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10
Q

Prothrombin Time (PT)

A
  • Extrinsic pathway (or common)

- For monitoring Warfarin

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11
Q

PTT or aPTT (Activated partial thromboplastin Time)

A
  • Intrinsic pathway (or common)
  • For monitoring heparin therapy (not LMWH)

-Significant for Hemophilia, vW disease, Vitamin K deficiency

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12
Q

INR (International Normalized Ratio)

A

-more accurate, standardized way of looking at PT (extrinsic pathway)

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13
Q

MC sites of bleeding for hemophilias A & B

A
  • Joints (spontaneous hemarthrosis, is sign of severe disease)
  • Muscles
  • Skin
  • GI
  • GU
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14
Q

Hemophilia A: Labs

A
  • Platelets and PT are normal

- aPTT will be prolonged**

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15
Q

Hemophilia A: treatment

A
  • Factor VIII infusions
  • Desmopressin
  • AVOID aspirin
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16
Q

Dysfunctional platelets

A

Acquired versus congential

  • Acquired (MC)**
    ex. Drugs, uremia, alcoholism, myeloproliferative disease

LABS: prolonged bleeding time (otherwise normal)

TX:

  • stop offending drug
  • hemodialysis in uremic pt
  • platelet transfusion if serious bleeding
17
Q

Platelets and the spleen

A

Splenomegaly/hypersplenism = thrombocytopenia

Post-splenectomy = (reactive) thrombocytosis

18
Q

Platelet disorders: Increased destruction

A

ITP (idiopathic thrombocytopenic purpura)

-autoimmune destruction

19
Q

Platelet disorders: Platelet consumption

A
  1. TTP (thrombotic thrombocytopenic purpura)
  2. HUS (hemolytic-uremic syndrome)
  3. DIC (disseminated intravascular coagulation)
20
Q

ITP (idiopathic thrombocytopenic purpura)

A

-antibody mediated destruction of platelets

Primary ITP: not related to another condition
Secondary ITP: related to another condition (ex. CLL, SLE, HIV)

21
Q

Acute ITP

A
  • kids 2-4
  • IgG associated
  • preceeded by viral URI
  • Self-limited
22
Q

Chronic ITP

A

Females> males
20-50 yrs
-insidious onset
-usually secondary ITP, associated with another condition

23
Q

ITP:signs

A
  • petechiae, purpura
  • epistaxis, oral bleeding
  • NO splenogmegaly
24
Q

ITP: labs

A

Platelets often below 20,000

Coagulation studies normal
BM–> increased megakaryocytes

25
Q

ITP: treatment

A

Acute: usually children don’t need treatment

Chronic ITP: HIGH dose dexamethasone or prednisone***

26
Q

HIT (heparin-induced throbocytopenia)

A
  • IgG autoantibody against complexes of heparin and platelet factor 4
  • Associated with arterial and venous thrombosis**

Tx: stop heparin

27
Q

TTP (thrombotic thrombocytopenic purpura)

A

Microangiopathic hemolytic anemia with thrombocytopenia***
-RBCs are getting sliced on the fibrin and platelets in vessels

Symptoms:

  • abdominal pain (pancreatitis is common)
  • Jaundice
  • fever, fatigue, toxic-appearing
  • Neuro findings
28
Q

IF a child presents with what looks like TTP folllowing E. coli infection what could it be?

A

HUS (hemolytic uremic syndrome)

Sx: bloody diarrhea before onset

-microangiopathic hemolytic anemia + thrombocytopenia**

  • NO neuro findings
  • Renal failure predominates**
29
Q

TTP and HUS: Labs

A
  • Microangiopathic hemolytic anemia
    1. Increased retics
    2. Increased LDH
    3. Increased indirect bilirubin
    4. Negative Coombs test

Schistocytes (helmet cells) on peripheral smear

-Normal coagulation tests

30
Q

TTP-HUS: treatment

A

Corticosteroids***

  • plasmapheresis
  • Rituximab if refractory
31
Q

DIC (Disseminated Intravascular Coagulation)

A

First, extensive thrombosis

Second, bleeding/hemorrhage once all the platelets are used up

32
Q

DIC: labs

A

Decreased platelets
Decreased fibrinogen
Increased PT and PTT
Increased D-Dimer

-Schistocytes on peripheral smear

33
Q

DIC: treatment

A

Transfusion support**

  • Cryoprecipitate
  • FFP
  • Platelet transfusion
  • RBC transfusion
34
Q

Von Willebrand’s Disease

A
  • Autosomal dominant
  • MC coagulopathy
  • Reduced levels of factor VIII antigen or ristocetin cofactor

Type 1 is most common (decreased vWF)

Acquired vW disease –> bone marrow malignancy MC*

35
Q

What does Von Willebrand Factor do?

A
  1. makes platelets adherent to subendothelium

2. Plasma carrier of factor VIII

36
Q

Von Willebrand disease: Labs

A

Prolonged bleeding time
(hemophilia has normal bleeding time)

  • Low vWF
  • INR normal
37
Q

Von Willebrand disease: treatment

A
  1. Desomopressin for type

- releases stored vWF from endothelial cells