CH. 36 - Hemorrhagic Disorders and Laboratory Assessment

Localized bleeding

Localized bleeding

Bleeding from a single location that usually indicates injury, infection, tumor, or an isolated blood vessel defect.

Generalized bleeding

Bleeding from multiple sites, spontaneous and recurring bleeds, or a hemorrhage that requires physical intervention

Mucocutaneous bleeding

Bleeding into the skin that appears either as:

• Petechiae

• Purpura

• Ecchymoses

Anatomic bleeding

Type of bleeding that is seen in acquired or congenital defects in secondary hemostasis such as plasma coagulation factor deficiencies (coagulopathies)

TEG and TEM

These are coagulometers that measure whole blood clotting, a process called global hemostasis

Congenital bleeding disorders

They lead to recurrent hemorrhages that may be spontaneous or may occur after minor injury or in unexpected locations

Coagulopathy

It is defined as any single or multiple coagulation factor or platelet deficiency

Massive hemorrhage

It is defined as:

• Blood loss exceeding total blood volume within 24 hours

• Loss of 50% of blood volume within a 3-hour period

• Blood loss exceeding 150 mL/min

• Blood loss that necessitates plasma and platelet transfusion

Plasma

It is a key TIC management component

Thawed plasma

FP-24 may be subsequently thawed and stored at 1° C to 6° C for up to 5 days, a product officially named?

TRUE

TRUE OR FALSE

The bleeding associated with liver disease may be localized or generalized, mucocutaneous or anatomic

Liver

It is the organ that produces nearly all of the plasma coagulation factors and regulatory proteins

Liver disease

It alters the production of the vitamin K-dependent factors II (prothrombin), VII, IX, and X and control proteins C, S, and Z

Declining coagulation factor V activity

It is a more specific marker of liver disease than deficient factors

Fibrinogen

It is an acute phase reactant that frequently becomes elevated in early or mild liver disease.

Dysfibrinogenemia

Moderately and severely diseased liver produces fibrinogen that is coated with excessive sialic acid, a condition called?

• VWF

• Factor VIII

• Factor XIII

These are acute phase reactants that may be unaffected or elevated in mild to moderate liver disease:

VWF

It is synthesized in the endoplasmic reticulum of endothelial cells and stored in their cytoplasmic Weibel-Palade bodies

Reptilase time test

Occasionally, this test may be useful to confirm dysfibrinogenemia

Bothrops venom

It triggers fibrin polymerization by cleaving fibrinopeptide A but not fibrinopeptide B from the fibrinogen molecule

Nephrotic syndrome

It is a state of increased glomerular permeability associated with a variety of conditions, such as:

• Chronic glomerulonephritis

• Diabetic glomerulosclerosis

• Systemic lupus erythematosus

• Amyloidosis

• Renal vein thrombosis

Vitamin K

It is ubiquitous in foods, especially green leafy vegetables, and the daily requirement is small, so pure dietary deficiency is rare

Brodifacoum or Superwarfarin

Often used as a rodenticide

Nijmegen-Bethesda assay

Quantitation of autoanti-VIII inhibitor is accomplished using the?

Factor XIII inhibitors

• They are extremely rare but cause life-threatening bleeding

• They may arise spontaneously or in association with autoimmune or lymphoproliferative disorders

Acquired VWD

It manifests with moderate to severe mucocutaneous bleeding and may be suspected in any patient with recent onset of bleeding who has no hemorrhage-related medical history

Von Willebrand Disease (VWD)

It is the most prevalent inherited mucocutaneous bleeding disorder

Type 1 VWD

It is a quantitative VWF deficiency caused by one of several autosomal dominant frameshifts, nonsense mutations, or deletions that may occur anywhere in the VWF gene

Type 2 VWD

It encompasses four qualitative VWF abnormalities

PT-VWD or pseudo-VWD

A platelet mutation that raises GPIb affinity for normal HMW-VWF multimers creates a clinically similar disorder called?

Subtype 2M VWD

It describes a qualitative VWF variant that possesses poor platelet receptor binding despite generating a normal multimeric distribution pattern in electrophoresis

Type 3 VWD

• It is also known as the “Null allele”

• It is the rarest form of VWD, where the VWF concentration measured by immunoassay or by activity assay is less than 10%

Hemophilia A

It causes anatomic bleeds with deep muscle and joint hemorrhages; hematomas; wound oozing after trauma or surgery; and bleeding into the central nervous system, peritoneum, gastrointestinal tract, and kidneys

Hemophilia B

It also called Christmas disease

It is caused by deficiency of factor IX (FIX)

Factor IX

• It is one of the vitamin K-dependent serine proteases.

• It is a substrate for both factors XIa and VIIa because it is cleaved by either to form dimeric factor IXa

Hemophilia C

• It is also known as Rosenthal Syndrome

• It is an autosomal dominant hemophilia with mild to moderate bleeding symptoms common in Ashkenazi Jews

Trauma-induced coagulopathy

REVIEW QUESTION

What is the most common acquired bleeding disorder?

Central nervous system bleed

CH. 36 REVIEW QUESTION

Which is a typical form of anatomic bleeding?

Factor VII deficiency, the PT

CH. 36 REVIEW QUESTION

What factor becomes deficient early in liver disease, and what assay does its deficiency prolong?

Hypofibrinogenemia

CH. 36 REVIEW QUESTION

Which of the following conditions causes a prolonged thrombin time?

Subtype 2B

CH. 36 REVIEW QUESTION

In what type or subtype of VWD is the RIPA test result positive when ristocetin is used at a concentration of less than 0.5 mg/mL?

Vitamin K and four-factor PCC

CH. 36 REVIEW QUESTION

What is the typical treatment for vitamin K deficiency when the patient is bleeding?

Factor XIII

CH. 36 REVIEW QUESTION

If a patient has anatomic soft tissue bleeding and poor wound healing, but the PT, PTT, TT, platelet count, and platelet functional assay results are normal, what factor deficiency is possible?

rFVIIa

CH. 36 REVIEW QUESTION

What therapy may be used for a hemophilic boy who is bleeding and who has a high FVIII inhibitor titer?

Type 1

CH. 36 REVIEW QUESTION

What is the most prevalent form of VWD?

Factor V assay

CH. 36 REVIEW QUESTION

Which of the following assays is used to distinguish vitamin K deficiency from liver disease?

Defects in primary hemostasis

CH. 36 REVIEW QUESTION

Mucocutaneous hemorrhage is typical of: