1. Some anticoagulants; 2. Decreased synthesis of clotting factors (liver disease, Vitamin K deficiency including malnutrition, malabsorption, antibiotics); 3. Increased consumption of clotting factors (sepsis/DIC)

1. Some anticoagulants; 2. Von Willebrand disease 3. Hemophilia 4. Antiphospholipid antibodies 5. Sepsis/DIC

Bleeding disorders Flashcards by Qian Wang

HIgh INR

Etiology

Some anticoagulants;
Decreased synthesis of clotting factors (liver disease, Vitamin K deficiency including malnutrition, malabsorption, antibiotics);
Increased consumption of clotting factors (sepsis/DIC)

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What does it test for?

Extrinsic and common pathway

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PTT

What does it test for?

Intrinsic and common pathway

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High PTT

Etiology?

Some anticoagulants;
Von Willebrand disease
Hemophilia
Antiphospholipid antibodies
Sepsis/DIC

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HIgh fibrinogen

Etiology

Acute phase reactant

Pregnancy

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Low fibrinogen

Etiology

DIC

Liver failure

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Acute phase reactant

What does it include?

C3, C4, fibrinogen, ESR, CRP, ferritin, hepcidin

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D-dimer–high

Etiology

PE/DVT
Arterial clots
Sepsis/DIC
Malignancy
Recent surgery or trauma
Liver disease
Pregnancy

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Coagulation profile

Plt, PT, PTT, D-dimer, fibrinogen

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Algorithm for investigation of abnormal coagulation tests.

If PT or PTT and PT both prolonged, what’s your next step?

Give Vitamin K

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Algorithm for investigation of abnormal coagulation tests.

PT or PTT and PT both prolonged s/p Vitamin K

If responded–>Vitamin K deficiency

2. If not responded–>Liver disease assessment

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Algorithm for investigation of abnormal coagulation tests.

PT or PTT and PT both prolonged 2/2 not vitamin K nor liver disease

Check mixing study

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Algorithm for investigation of abnormal coagulation tests.

If Mixing study corrected

Due to factor deficiency

Von Willebrand disease;
Specific factor activity levels

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Algorithm for investigation of abnormal coagulation tests.

If Mixing study not corrected

Due to antibody presentation

Check antiphospholipid antibodies;
Specific factor inhibitors.

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Normal hemostasis

Intrinsic pathway factors

XII, XI, IX, VIII

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Normal hemostasis
Intrinsic pathway factors
What is the test for?

PTT

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Normal hemostasis

Extrinsic pathway factors

VII

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Normal hemostasis
Extrinsic pathway factors
What is the test for?

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Normal hemostasis

What is the common pathway factors

X, V, II, fibrinogen

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Prolonged PT, normal aPTT

DDx

Factor VII deficiency
DIC
Liver disease
Vitamin K deficiency
Warfarin ingestion

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Normal PT, prolonged aPTT

DDx

Deficiency of VIII, IX, XI or XII;
von Willebrand disease (if severe and factor VIII level is low);
Heparin exposure

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Prolonged PT and aPTT

DDx

Deficiency of factor V, X, II or fibrinogen;
Severe liver disease;
DIC;
Vitamin K deficiency;
Warafin use;
Heparin overdose;

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Normal PT and aPTT

DDx

Platelet dysfunction (acquired and congenital);
von Willebrand disease (if mild and factor VIII level is not too low);
Scurvy;
Ehlers-Danlos syndrome;
Hereditary hemorrhagic telangiectasia;
Deficiency of factor XIII

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Bleeding into muscles and joints

Humoral clotting factor disorders

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Mucosal bleeding

Primary hemostasis

Bleeding disorders | What signs in women menstrual history indicating underlying bleeding disorder?

Nighttime "flooding"; Passing clots larger than a quarter; Duration longer than 8 days; Development of iron deficiency.

Congenital bleeding disorder Hemophilia A and B What is the genetic?

X-linked recessive

Congenital bleeding disorder Hemophilia A and B What are the deficient factors?

Hemophilia A: VIII | Hemophilia B: XI

Congenital bleeding disorder Hemophilia A and B Sx of severe hemophilia?

Factor level <1% Joint bleeding, retroperitoneal bleeding, intramuscular bleeding and intracranial bleeding; Children: bleeding after circumcision or loos of deciduous teeth.

Congenital bleeding disorder Hemophilia A and B Sx of moderate hemophilia?

Factor level between 1% to 5% | Present in adult, unlikely to be spontaneous

Congenital bleeding disorder Hemophilia A and B Sx of mild hemophilia?

Factor level>5% | Present in adult, unlikely to be spontaneous

Congenital bleeding disorder Hemophilia A and B What are the concerns for older patients?

HIV and HCV infection from blood product transfusion

Congenital bleeding disorder Hemophilia A and B Rx

Replacing factor VIII and IX

Congenital bleeding disorder Hemophilia A and B What are the complications 2/2 to treatment?

Antibody development to factor VIII and IX.

Congenital bleeding disorder Hemophilia A and B How to test alloantibodies against VIII and IX?

Bethesda titer

Congenital bleeding disorder Hemophilia A and B What is the typical lab results show?

Prolonged aPTT and normal PT; aPTT mixing study will fully correct; Measure baseline factor activity/

Congenital bleeding disorder von Willebrand disease Epi:

Most common inherited bleeding disorder.

Congenital bleeding disorder von Willebrand disease Sx:

Nosebleeds in children, easy bruising, bleeding gums, postsurgical bleeding, GYN problems

Congenital bleeding disorder von Willebrand disease What are the GYN presentations?

Heavey menstrual bleeding; Endometriosis; Miscarriages Postpartum hemorrhage

Congenital bleeding disorder von Willebrand disease How many types of VW disease are there?

Congenital bleeding disorder von Willebrand disease Dx:

``` vWF antigen and activity level <30%; Factor VIII levels may be normal or low; Platelet function analyzer-100 (PFA-100) is prolonged; PT normal; PTT may be normal or slightly prolonged. ```

Congenital bleeding disorder von Willebrand disease Treatment for heavy menstrual bleeding?

Estrogen-containing oral contraceptives; Desmopressin; Antifibrinolytic agents such as tranexamic acid or e-aminocaproic acid during menses

Congenital bleeding disorder von Willebrand disease Rx:

Desmopressin preoperative at surgical prophylaxis | If severe bleeding episodes or prophylaxis for major surgeries- give factor VIII and vWF

Congenital bleeding disorder von Willebrand disease Type 2

Production of an abnormally functioning vWF molecules

Congenital bleeding disorder von Willebrand disease Type 3

Near lack of vWF, very low factor VII levels

Acquired bleeding disorder Coagulopathy of liver disease Which factor is not produced in liver?

Factor VIII, produced in extrahepatic endothelia cell.

Acquired bleeding disorder Coagulopathy of liver disease What is the lab results?

Prolonged PTT and PT; Low fibrinogen level; Supranormal factor VIIIlevels

Acquired bleeding disorder Coagulopathy of liver disease Rx

If bleeding: Cryoprecipitate, FFP and platelet If vitamin K deficiency--> Vitamin K

Acquired bleeding disorder Acquired hemophilia Presentation

Elderly people without family or PMHx of bleeding disorder; Acute new onset bleeding; Mucocutaneous or intramuscular

Acquired bleeding disorder Acquired hemophilia Lab findings

Normal PT; Significantly prolonged aPTT; 1:1 mixing study unsuccessful at completely correting the aPTT

Acquired bleeding disorder Acquired hemophilia Mechanism

Autoantibody against factor VIII

Acquired bleeding disorder Acquired hemophilia Etiology

``` Associated with underlying disease: SLE Lymphoproliferative disorder Solid tumors More common idiopathic ```

Acquired bleeding disorder Acquired hemophilia Rx

Immunosuppression to eradicate inhibitor; | Recombinant activated factor VII

Acquired bleeding disorder DIC Lab features:

Elevated PT (due to consumption of factor VII); Elevated PTT; Low fibrinogen; Elevated D-dimers Low plt; Microangiopathic hemolytic anemia with schistocytes on the peripheral blood smear

Acquired bleeding disorder DIC Rx

1. Underlying causes; 2. If bleeding or at risk for bleeding--> plt, ffp, or replacing clotting factors or cyroprecipitate to replace fibrionoten

ITP | What to test if found to have ITP

HIV, hepatitis (HBV, HCV), MPNs, Collagen vascular disease

ITP | What to do if plt<10

Spontaneous bleeding, bleeding precautions

ITP | What to do if plt>30

NTD

ITP | what to do if plt<30

Dexamethasone->IVIG->Rituximab->Romiplostin/Splenectomy

ITP | What to do if plt<50 and needs surgery

IVIG and transfuse

ITP | What to do if plt<50 and bleeding

IVIG and plt transfuse

ITP | What to do if plt<100 with intracranial bleeding

IVIG and plt transfusion

ITP | Plt threshold for central line

>20

ITP | plt threshold for abdominal tap

>30

ITP | Plt threshold for spinal tap

>50

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Bleeding disorders Flashcards

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