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Hematology > Chapter 141 Coagulation Disorders > Flashcards

Chapter 141 Coagulation Disorders Flashcards

1
Q

Top three most common causes of DIC

A

Bacterial sepsis
Malignancy / Acute promyelocytic leukemia
Abruptio placentae, dead fetus syndrome, amniotic fluid embolism

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2
Q

Severe form of DIC affecting predominantly young children following viral and bacterial infections, presenting as thrombosis of extensive areas of the skin?

A

Purpura fulminans

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3
Q

Central mechanism of DIC?

A

Uncontrolled generation of thrombin

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4
Q

Proinflammatory cytokines that play central roles in mediating coagulation defects in DIC?

A

IL-6

TNF alpha

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5
Q

How is bleeding in chronic DIC different from acute DIC?

A

Discrete bleeding and restricted to skin or mucosal surfaces

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6
Q

The most sensitive test for DIC is:

A

Fibrin degradation products level

Fragmented red cells (schistocytes) in blood smear + elevated FDP

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7
Q

Most common differential diagnosis for DIC that present similarly?

A

Severe liver disease

Also at risk for bleeding
Thrombocytopenia - platelet sequestration, portal hypertension, hypersplenism
Elevated FDP - poor hepatic clearance
Low synthesis of coagulation factors

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8
Q

How to differentiate liver disease and DIC?

A

Lab parameters in liver disease do NOT change rapidly

Look for presence of portal hypertension or signs of underlying liver disease

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9
Q

Treatment of DIC with active bleeding or is at high risk for bleeding?

A

Transfusion of FFP and/or platelet concentrates

- in preparation for surgery or after chemotherapy

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10
Q

What cutoff platelet level in DIC requires replacement therapy?

A

Platelet <10,000-20,000

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11
Q

Low levels of fibrinogen <100mg/dl or brisk hyperfibrinolysis will require infusion of:

A

Cryoprecipitate
(fibrinogen, factor VIII, vWF)

10 U Cryo for every 2-3 U of FFP is sufficient to correct hemostasis

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12
Q

Treatment of purpura fulminans?

A

Heparin

During surgical resection of tumor or removal of dead fetus

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13
Q

Which patients with DIC benefit from antifibrinolytic drugs / tranexamic acid?

A

Acute promyelocytic leukemia
Chronic DIC
Giant hemangioma

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14
Q

Coagulation disorder caused by deficiency in Factor VIII?

A

Hemophilia A

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14
Q

Coagulation disorder caused by deficiency in Factor IX?

A

Hemophilia B

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14
Q

Isolated abnormal prothrombin time suggests deficiency of what factor?

A

Factor VII (Extrinsic pathway)

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15
Q

Prolonged aPTT indicates deficiency in Factors:

A

Factors VIII and IX (Hemophilia) or Factor XI (Intrinsic Pathway)

16
Q

Prolongation of both PT and aPTT indicates deficiency in what factors?

A

Factors V, X, II (Thrombin) and Fibrinogen - Common Pathway

17
Q

Differentiate deficiency vs presence of inhibitor using mixing tests

A

Addition of the missing protein to the plasma of a subject with an inhibitor does NOT correct the abnormal PTT and PT - if there is an inhibitor

18
Q

One of the most common Hemophilia A mutations results from inversion of:

A

Intron 22 sequence

19
Q

What test for coagulation is prolonged (isolated) in patients with Hemophilia?

A

Isolated prolongation of aPTT

Bleeding time and Platelet counts are normal

20
Q

Most common bleeding manifestation in severe hemophilia

A

Recurrent hemarthroses - mainly knees elbows ankles shoulders hips

21
Q

Acute hemarthroses due to bleeding may be painful, patients would tend to adopt a fixed position leading to this complication:

A

Muscle contractures

22
Q

Hematomas into the muscle of patients with hemophilia may lead to:

A

Compartment syndrome

Recurrent bleeding in the joint (target joint) may need surgery

23
Q

Retroperitoneal hemorrhage in hemophilia may form masses of calcification and inflammatory tissue reaction producing:

A

Pseudotumor Syndrome

  • results to damage of the Femoral nerve
24
Q

What complication is expected in elderly patients diagnosed and treated for hemophilia in the 1970s

A

HIV and Hep C infections that may have contaminated blood products that were transfused

25
Q

Factor replacement for hemophilia has emerged in response to bleeding or as prophylaxis. What are the half lives and corresponding dose frequency of Factor VIII and IX?

A

FVIII half life 8-12 hours, injections BID

FIX half life 24 hours, injections OD

26
Q

What blood product is enriched with FVIII protein and was popularly used as treatment of hemophilia A?

A

Cryoprecipitate - approximately 80 IU of FVIII

27
Q

Risk of transfusing cryoprecipitate as treatment for hemophilia?

A

Risks of blood borne diseases increase

28
Q

Prophylactic replacement of factors prior to surgery requires normal factor levels 100% for how many days?

A

7-10 days

29
Q

Synthetic vasopressin analog that causes transient rise in FVIII and VWD by release from the endothelial cells

A

DDAVP

Does not improve FVIII levels in severe hemophilia A because there are no stores to release

30
Q

Complication of repeated dosing of DDAVP in Hemophilia?

A

Tachyphylaxis - mechanism is release of FVIII from endothelial cells vs de novo synthesis

CANNOT use DDAVP in Type IIB VWD

31
Q

Contraindication to starting tranexamic acid or any antifibrinolytic drug in preparation for surgery

A

Hematuria or GU bleed - may risk the formation of occlusive clot in the lumen of the GU tract

32
Q

Major complication of factor replacement therapy in Hemophilia A and B?

A

Formation of alloantibodies to both FVIII and FIX (inhibitors)

33
Q

Most effective way to eradicate inhibitors to FVIII and FIX:

A

Immune tolerance induction (ITI) +/- Rituximab (daily infusions for >1 year)

34
Q

Rare inherited platelet disorder with the absence of Platelet IIB/IIIA receptor?

A

Glanzmann’s Thrombasthenia

35
Q

Rare inherited platelet disorder with the absence of platelet IB-IX-V receptor?

A

Bernard Soulier Syndrome

36
Q

Expected PT PTT results in hemophilia?

A

PT normal

PTT prolonged

37
Q

Expected PT PTT in DIC?

A

PT PTT prolonged

38
Q

Expected findings in peripheral smear of DIC

A

Schistocytes

Hematology (9 decks)

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