Disorders of Hemostasis Flashcards
What is hemostasis?
Hemostasis is a dynamic process that is geared to preventing blood from escaping the boundaries of the vessel.
What are the phases of hemostasis?
The phases of hemostasis are:
* Maintenance of vessel integrity
* Formation of a platelet plug
* Propagation of the coagulation cascade
* Clot development
* Fibrinolysis and clot disintegration
What are the two types of hemostasis?
The two types of hemostasis are:
* Primary hemostasis (platelet plug formation)
* Secondary hemostasis (coagulation process)
What maintains vascular integrity?
Vascular integrity is maintained by a lining of overlapping endothelial cells supported by a basement membrane, connective tissue, and smooth muscle.
What substances do endothelial cells secrete to regulate clot formation?
Endothelial cells secrete:
* Tissue factor pathway inhibitor (TFPI)
* Heparin sulfate
* Prostacyclin
* Nitric oxide
What role does CD39 play in hemostasis?
CD39 degrades ATP and ADP to AMP and adenosine, which are potent antiplatelet and antithrombotic agents.
How does von Willebrand factor (vWF) contribute to hemostasis?
vWF links to platelet glycoprotein Ib (gpIb) receptors allowing platelet adhesion to the intravascular surface.
What are platelets and their role in hemostasis?
Platelets are complex cytoplasmic fragments released from bone marrow megakaryocytes, playing roles in adhesion and aggregation.
Largely regulated by Thrombopoeitin
What is the coagulation cascade?
The coagulation cascade is a complex system that results in controlled formation of a fibrin clot.
What are the two traditional pathways of the coagulation cascade?
The two traditional pathways are:
* Intrinsic pathway
* Extrinsic pathway
What initiates the clot process in the coagulation pathway?
The extrinsic pathway initiates the clot process through exposed tissue factor at the site of vessel injury.
What is the role of tissue factor in hemostasis?
Tissue factor is a critical cofactor required for activation of factor VII.
What is the most commonly encountered disorder of hemostasis?
The most commonly encountered disorder of hemostasis is antithrombotic drug administration.
Fill in the blank: Hemostasis is dependent on normal functioning and integration of the ______, platelets, and coagulation pathway.
[vasculature]
Endothelium
Role of Platelets in Hemostasis
- Adhesion to subendothelial connective tissue: Collagen, basement membrane, and noncollagenous microfibrils; serum factor VIII and von Willebrand factor (vWF) permit this function; adhesion creates the initial bleeding arrest plug
- Release of adenosine diphosphate, the primary mediator and amplifier of
aggregation; release of thromboxane A, another aggregator and potent
vasoconstrictor; release of calcium, serotonin, epinephrine, and trace thrombin - Platelet aggregation over the area of endothelial injury
- Stabilization of the hemostatic plug by interaction with the coagulation system:
* Platelet factor 3, a phospholipid that helps accelerate certain steps in the coagulation system
* Platelet factor 4, a protein that neutralizes heparin
* Pathway initiation and acceleration by thrombin production
* Secretion of active forms of coagulation proteins - Stimulation of limiting reactions of platelet activity
Coagulation Factors
Factor I. Fibrinogen
Factor II. Prothrombin
Factor III. Tissue thromboplastin
Factor IV. Calcium
Factor V. Labile factor (proaccelerin)
Factor VI. Not assigned
Factor VII. Proconvertin
Factor VIII. Antihemophilic A factor
Factor IX. Antihemophilic B factor (plasma thromboplastin component, Christmas factor)
Factor X. Stuart-Prower factor
Factor XI. Plasma thromboplastin antecedent
Factor XII. Hageman factor (contact factor)
Factor XIII. Fibrin-stabilizing factor
Normal Controls of Coagulation
- Removal and dilution of activated clotting factors through blood flow, which also mechanically opposes growth of the hemostatic plug
- Alteration of platelet activity by endothelium-generated nitric oxide and prostacyclin
- Removal of activated coagulation components by the reticuloendothelial system
- Regulation of the clotting cascade by antithrombin III, protein C, protein S, and tissue factor pathway inhibitor (TFPI)
- Activation of the fibrinolytic system
Coagulation Cascade
Clinical evaluation of bleeding patient
Features of Coagulation disorders that differentiate from platelet disorders
DDx of Platelet Disorders
DDx of Vascular disorders
DDx of Coagulation Disorders
Diagnostic Hemostasis tests
What initial assessment is required when a bleeding disorder is diagnosed?
Stabilization, which may include intravenous fluids, RBC transfusion, or factor replacement
What are common initial symptoms of platelet disorders?
Epistaxis, menorrhagia, gastrointestinal bleeding
How are platelet disorders typically manifested?
Acquired petechiae, purpura, or mucosal bleeding
What distinguishes purpura associated with platelet disorders from that associated with vasculitis?
Purpura in platelet disorders is usually asymptomatic and not palpable; vasculitis purpura can burn or itch and is palpable.
What types of bleeding are characteristic of coagulation disorders?
Delayed deep muscle or joint bleeding
What are the key laboratory tests for diagnosing hemostatic disorders?
Complete Blood Count, Blood Smear, Platelet Count, Bleeding Time, Prothrombin Time, Partial Thromboplastin Time, Anti-Xa Assay, Fibrinogen, Thrombin Time, Clot Solubility, Factor Level Assays
What does a complete blood count assess in the context of bleeding episodes?
Degree of anemia
What is defined as thrombocytopenia?
Platelet count of less than 100,000/mm3
What is the normal bleeding time range?
8 minutes
What does the Prothrombin Time (PT) test?
Factors of the extrinsic and common coagulation pathways
What can cause a prolonged Prothrombin Time?
Vitamin K deficiency, warfarin use, liver disease
What factors does the Partial Thromboplastin Time (PTT) test exclude?
Factor VII and factor XIII
What are the two groups of inherited disorders that manifest as isolated elevation in PTT?
- Deficiencies of contact factors (e.g., factor XII)
- Deficiencies of intrinsic coagulation factors (e.g., factors VIII, IX, XI)
What is the role of the anti-Xa assay?
Monitoring unfractionated and low-molecular-weight heparin levels, quantifying direct factor Xa inhibitors
What does fibrinogen reflect in the coagulation process?
Balance between production and consumption
What does the thrombin time measure?
Conversion of soluble fibrinogen to insoluble fibrin
What is a key feature of disorders involving factor XIII deficiency?
Clot solubility testing may show abnormal results
How are factor levels determined in laboratory tests?
- Bioassay
- Immunologic assay
What do inhibitor screening tests reveal?
Antibodies in plasma that prolong the normal plasma clotting time
What is thrombocytopenia defined as?
A platelet count of less than 100,000 micro/mm3
What are the two main causes of thrombocytopenia?
- Decreased production
- Increased destruction
What conditions can lead to decreased bone marrow production causing thrombocytopenia?
- Chemotherapeutic drugs
- Myelophthisic disease
- Alcohol
- Thiazides
What is splenic sequestration in relation to thrombocytopenia?
A rare cause seen primarily with hypersplenism due to malignant hematologic disease, portal hypertension, or increased splenic RBC destruction
In what scenarios are platelet transfusions commonly indicated?
- Primary bone marrow disorders (e.g., aplastic anemia, acute leukemia)
At what platelet count is spontaneous bleeding more likely to occur?
Below 10,000/mm3
What is the risk of hemorrhage attributed to platelet deficiency at counts higher than 50,000 mm3?
Unlikely
What is immune thrombocytopenia (ITP)?
An acquired condition resulting from autoantibodies against platelet antigens
What are the categories of immune thrombocytopenia?
- Primary ITP
- Secondary ITP
- Drug-induced thrombocytopenia (DITP)
What characterizes severe ITP?
A platelet count of less than 20,000 micro/mm3
What age group is most commonly affected by acute ITP?
Children aged 2 to 6 years old
What is the typical outcome for acute ITP in children?
Greater than 90% rate of spontaneous remission
What are common manifestations of chronic ITP in adults?
- Easy bruising
- Prolonged menses
- Mucosal bleeding
What is secondary ITP associated with?
- Autoimmune diseases
- Infectious diseases
- Malignancies
Which drug is an important cause of drug-induced thrombocytopenia in hospitalized patients?
Heparin
What is the recommended treatment for newly diagnosed adult patients with ITP and platelet counts less than 30,000/mm3?
Corticosteroids
What is the preferred glucocorticoid for ITP treatment?
Dexamethasone, 40 mg PO or IV daily for 4 days
What treatment should be considered for patients with platelet counts less than 10,000/mm3 or significant bleeding?
IVIG in addition to corticosteroids
What is a thrombopoietin receptor agonist (TPO-RA) used for?
Life threatening bleeding in inadequate response to corticosteroids, IVIG, and platelet transfusion
What are the common drugs associated with drug-induced thrombocytopenia?
Quinine
Phenytoin / carbamazepine / VPA
Heparin
ASA
Digoxin
Sulfa / vanco
QPHADS
What is the mechanism by which quinine and quinidine cause thrombocytopenia?
Through an ‘innocent bystander’ mechanism
The platelet is coated with a drug-antibody complex, complement is
fixed, and intravascular platelet lysis occurs.
What characterizes heparin-induced thrombocytopenia (HIT)?
An immune-mediated process associated with unfractionated heparin and low-molecular-weight heparin.
Heparin is thought to pair with platelet factor 4 (PF4), a procoagulant substance found in platelet granules.
This paired complex results in rapid generation of antibodies, which coat platelets resulting in thrombocytopenia
What is the 4Ts Score used for?
Differentiating patients with HIT from those with alternative causes of thrombocytopenia
What should be done if HIT is clinically suspected?
Immediate cessation of heparin
What is the most widely utilized anticoagulant for the treatment of HIT?
Argatroban
What is the initial dose of argatroban for patients with normal hepatic function?
2 mcg/kg/min IV
What are alternative treatments for HIT aside from argatroban?
- Bivalirudin
- Danaparoid
- Fondaparinux
- Direct oral anticoagulants (DOAC)
What is the risk of HIT related to?
- Drug and dosage factors
- Duration of exposure(risk greatest after 5 days of Rx)
- Heparin type(UFH higher risk than LMWH)
- Patient gender(Females twice at risk)
- Surgical and Trauma patients
Can occcur a median of 14 days to upto 40 days post initiation of heparin
True or False: HIT can occur in the absence of exposure to heparin.
True
Immune Thrombocytopenia Chronicity
Describe 4Ts scoring system
4Ts Point Risk Stratification
What is HITT
Heparin Induced Thrombocytopenia with Thrombosis develops in 33% to 50% of patients with HIT.
The antibodies crated in HIT also activate platelets, creating hypercoagulable state.
What is post-transfusion purpura (PTP)?
A rare disorder causing a precipitous fall in platelets approximately one week following transfusion.
What antigen is frequently linked to post-transfusion purpura?
Human platelet antigen 1 (HPA1).
When an HPA1 antigen–negative patient receives a platelet transfusion, the platelets with attached HPA1
antibodies provoke an anamnestic response, though the actual mechanism of platelet destruction is uncertain. Patients are usually middle aged women with a history of prior pregnancy, during which they may have been previously sensitized to the HPA1 antigen.
What is the typical platelet count in patients with post-transfusion purpura?
Often falls below 10,000/mm3.
What is the first-line treatment for severe thrombocytopenia in post-transfusion purpura?
High-dose IVIG, 1g/kg IV.
Glucocorticoids may be given with IVIG. Plasma exchange therapy is no longer
considered a preferred therapy
True or False: Platelet transfusion is generally recommended for post-transfusion purpura.
False.
Transfused platelets are often rapidly destroyed, thus platelet transfusion is generally not recommended
except in the setting of severe thrombocytopenia and life-threatening bleeding, preferably with HPA1-negative blood products
What are thrombotic microangiopathies (TMA) characterized by?
Microangiopathic hemolytic anemia (MAHA) and thrombocytopenia.
What are the most common primary TMA disorders?
- Thrombotic thrombocytopenic purpura (TTP)-most common
- Hemolytic Uremic Syndrome(HUS)
What causes TTP?
Autoantibodies to ADAMTS13, an endothelial protein.
What are the classic symptoms of TTP?
F ever
A nemia
T hrombocytopenia
R enal failure (more kids, HUS)
N eurologic (more adults, TTP)
Petechiae and purpura 50% but overt bleeding rare
What is the standard treatment for TTP?
90% mortality if not treated
** Plasma exchange**( Standard)
Glucocorticoids- Prednisone 1 mg/kg PO daily
Rituximab- consider if refratory to standard Rx
Caplacizumab- 11 mg IV followed by 11 mg sc x 10 days
Eculizumab (HUS)
Avoid platelets if no life-threatening bleeding
(may increase microthrombi)
Plasma contains ADAMTS13
Avoid platelets if no life-threatening bleeding (may increase microthrombi)
What is dilutional thrombocytopenia?
A complication of massive transfusion, exchange transfusion, or fluid resuscitation.
Current guidelines for 1 :1 : 1 ratio of fresh frozen plasma (FFP), platelets, and RBCs may assist
in alleviating dilutional thrombocytopenia in the setting of massive transfusion.
What is the significance of hereditary thrombocytopenia?
As many as 1 in 7 patients initially diagnosed with ITP may have a hereditary thrombocytopenic syndrome.
What is Bernard-Soulier Syndrome (BSS)?
A defect in the platelet gpIb complex affecting platelet adhesion to vWF.
What is Glanzmann thrombasthenia (GT)?
An autosomal-recessive disorder due to a defect in the glycoprotein IIb/IIIa complex.
What are secretory defects in thrombocytopathy?
Defective platelet granule formation or secretory machinery.
What medication blocks thromboxane A2 formation?
Aspirin.
What is thrombocytosis?
Defined as a platelet count of greater than 600,000/mm3.
What are common causes of secondary thrombocytosis?
- Infection
- Iron deficiency.
What is primary thrombocytosis associated with?
- Polycythemia vera
- Myelofibrosis
- Chronic myelogenous leukemia.
What is the recommended evaluation for primary thrombocytosis?
A thorough hematologic evaluation.
Describe Plasmic Score?
What are six potential treatments for ITP?
- Corticosteroids : (can be PO or IV, often high dose and pulse requiring admission)
- Immunosuppression: IVIG, rituximab
- RhoGAM/WinRho (must be Rh+ to work; spleen has less ability to destroy platelets covered in RhIG)
- Thrombopoeitin receptor agonist (TPO-RA – romiplastim, eltrombopag)
- Emergency splenectomy
- Platelet transfusion for life-threatening bleeding, target >50
Pediatric: Mild bleeding typically no medication, even with platelets <20
Consult for workup (rule out sepsis/HUS-TTP, malignancy ie leukemia)
When should thrombocytopenia be treated in the ED?
- 100 : SAH/ICH, neurosurgery
- 50 : LP, Active bleeding (GIB, intraabdominal) , ITP with life-threatening bleeding
- 30 : Paracentesis
- 20 : Large laceration, central line
“A platelet count of >10 usually provides adequate hemostasis…”
List 5 adverse effects of platelet transfusion
What causes Hemophilia A?
A deficiency of factor VIII.
Hemophilia A is primarily sex-linked recessive as it is carried on the X chromosome.
What is the prevalence of Hemophilia A in males?
Approximately 17 cases per 100,000 males.
This translates to around 1,125,000 affected individuals worldwide.
How many people in the United States have Hemophilia A?
Approximately 13,500 people.
X linked recessive, only males will have clinical disease
How is the severity of Hemophilia A categorized?
By factor VIII activity level:
* Severe: less than 1%
* Moderate: 1% to 5%
* Mild: 5% to 40%.
What percentage of Hemophilia A cases are considered severe?
Approximately 42%.
What is the risk associated with severe Hemophilia A patients?
Increased risk to develop alloantibodies, termed inhibitors, that may inactivate factor VIII.
What is the primary source of factor VIII for replacement therapy?
Recombinant DNA technology.
What is Emicizumab?
A recombinant immunoglobulin that substitutes for part of the function of activated factor VIII.
How does Emicizumab function?
By bridging activated factor IX and factor X to restore the function of missing activated factor VIII.
What is the typical concentration of factor VIII in plasma?
Circulates in low concentrations bound to vWF.
What are common sites of bleeding in Hemophilia A?
Deep muscles, joints, urinary tract, and intracranial sites.
What are major causes of morbidity in Hemophilia A?
Recurrent hemarthrosis and progressive joint destruction.
What is the probability of intracranial hemorrhage (ICH) in male patients with Hemophilia A?
1 in 50,000 to 1 in 140,000 male patients.
What is the mortality rate from ICH in hemophiliacs?
Around 20%.
What type of bleeding is rare in Hemophilia A?
Mucosal bleeding such as epistaxis, oral bleeding, or menorrhagia unless associated with comorbid conditions.
What is a common initiator of bleeding in Hemophilia A?
Trauma.
What is involved in the comprehensive management of Hemophilia A?
A team effort including physicians, specialized nursing, physical therapy, social work, patients, and caregivers.
What should emergency clinicians have rapid access to for managing Hemophilia A?
Information such as primary care physician, hematologist, diagnosis, factor VIII activity level, blood type, and inhibitor status.
What is the typical factor VIII activity goal for minor bleeding?
40% to 50%.
What is the typical factor VIII activity goal for serious or life-threatening bleeding?
80% to 100%.
How much does 1 U/kg of factor VIII increase the circulating factor VIII level?
By 2%.
What should be done if there is no response to factor VIII administration?
Raise suspicion for the presence of inhibitors.
What is the safest immediate action in emergency care for patients with inhibitors?
Administer recombinant factor VIIa at a dose of 90 mcg/kg IV.
What is Desmopressin used for in Hemophilia A?
For patients with documented mild hemophilia A without inhibitors who are experiencing non-life-threatening bleeding.
What effect does Desmopressin have on factor VIII levels?
Raises factor VIII by three to six times baseline levels.
Indications for Factor Replacement in Hemophilia
Recommended Factor VIII Therapy and Dosing for Complications Associated
With Hemophilia
What is Hemophilia B?
A deficiency of factor IX activity.
How does the incidence of Hemophilia B compare to Hemophilia A?
Its incidence is only a fifth that of hemophilia A.
What type of protein is factor IX?
A vitamin K–dependent glycoprotein.
How is a deficiency of factor IX diagnosed?
By a factor IX assay after a normal factor VIII assay.
What treatment strategies for hemophilia A generally apply to hemophilia B?
Clinical presentations and treatment strategies.
What is the replacement factor therapy for hemophilia B?
Use of a recombinant factor IX preparation.
When is plasma-derived concentrate used in hemophilia B?
In an emergency situation when recombinant factor IX is unavailable.
Is fresh frozen plasma recommended for hemophilia B treatment?
No, it is not routinely recommended.
What is the maintenance factor dosing schedule for factor IX?
Every 24 hours.
What is the appropriate dose of factor IX for severe bleeding?
100 to 140 units/kg IV.
What should be administered for hemophilia B with inhibitors?
Recombinant factor VIIa at a dose of 90 mcg/kg IV.
What is von Willebrand disease?
What is the role of vWF?
**The most common hereditary bleeding disorder,caused by quantitative and qualitative deficits in vWF, with an estimated prevalence of 1%.
VWF does two things:
Role: Bridges platelets to damaged vascular endothelium;Brings factor VIII to the site of injury to generate fibrin
Inheritance pattern for main variants is autosomal dominant
What is the typical factor VIII activity level in von Willebrand disease?
In the 6% to 50% range.
What is the preferred treatment for mild to moderately severe von Willebrand disease?
Desmopressin, 0.3 mcg/kg IV.
What is the replacement therapy for severe von Willebrand disease?
Factor VIII in the form of lyophilized concentrate at a dose of 50 IU/kg.
What can cause an altered level or abnormal function of fibrinogen?
Deficiencies in the coagulation pathway.
What are the inherited forms of coagulation disorders associated with?
Rare deficiencies in components of the common pathway (factors II, V, and X).
What common causes are associated with acquired forms of coagulation disorders?
Vitamin K deficiency, hepatic insufficiency, or massive transfusion of stored blood.
Clinical Features of VWF
More common: GIB, mennorhagia, PPH, epistaxis
Less common: Joint and muscle bruising (unlike Heme A/B)
Types of von Willebrand disease?
MANY polymorphisms, but 3 main types
Type 1 – reduced amount - mostly asymptomatic, 75% of cases, autosomal dominant
Type 2 – functional issues (platelet or factor VIII)
Type 3 – rare – total deficiency, lifethreatening bleeding early in life, autosomal recessive
Acquired:
Malignancies (MGUS, myeloma, NHL, leukemia, Wilms tumor)
Lupus
High states of vascular flow: VSD, CHD with high flow, LVAD/ECMO
Hypothyroidism
Drugs: valproate, ciprofloxacin
von Willebrand Disease Management
- Desmopressin 0.3mcg/kg IV/intranasal/SC for mild to moderate disease (Induces synthesis and release of VWF from platelets/endothelial cells)
- TXA
- FFP or cyro may be used
- Replace with VWF/factor VIII product 50 U/kg.
- Recombinant VWF exists which requires giving factor VIII as well.
- Giving plasma would not require factor VIII
- Factor VIII actually accumulates and can cause thrombosis
- Can get inhibitors as well, similar treatment to heme A/B give factor VIIa
Review lab and coagulopathy diagnosis
What are the two main types of oral anticoagulants?
Vitamin K antagonists (e.g., warfarin) and direct-acting oral anticoagulants (DOACs)
DOACs include dabigatran, rivaroxaban, and apixaban.
What is the mechanism of action of dabigatran?
Dabigatran is a direct thrombin inhibitor
It was the first DOAC to gain FDA approval.
Name two common DOACs and their specific targets.
- Rivaroxaban: selective factor Xa inhibitor
- Apixaban: selective factor Xa inhibitor
What complications may arise from the use of DOACs in patients with impaired renal or liver function?
Excessive anticoagulation
What is the primary concern that brings patients to the ED regarding anticoagulants?
Supratherapeutic dosing or hemorrhage
How can the effects of heparin be urgently reversed?
With protamine sulfate
1 mg of protamine for every 100 units of heparin.
What is the off-label use of protamine in relation to LMWH?
To reverse effects of LMWH in life-threatening bleeding
What is the recommended dose of protamine for LMWH reversal depending on timing?
- 1 mg for ≤8 hours since last administration
- 0.5 mg for >8 hours since last administration
What should not exceed 50 mg over 10 minutes when administering protamine?
Administration of protamine
What is the FDA-approved reversal agent for dabigatran?
Idarucizumab, 5 g IV
What is the alternative to idarucizumab for dabigatran-associated bleeding?
Activated prothrombin complex concentrate (aPCC) at 50 units/kg IV
What factors are included in 4F-PCC?
- Factors II
- VII
- IX
- X
- Protein C
- Protein S
How much of dabigatran is removed with 4 hours of dialysis?
Approximately 57%
What is the FDA-approved reversal agent for apixaban or rivaroxaban in life-threatening bleeds?
Andexanet alfa
What is an alternative to andexanet alfa for apixaban or rivaroxaban?
4F-PCC at 25 to 50 units/kg IV or a fixed dose of 2000 units IV
What is the management approach for excessive anticoagulation from warfarin with INR below 4.5?
Withhold additional warfarin
What is the recommendation for INR levels between 4.5 and 10 without bleeding?
Hold additional warfarin doses for 1 to 2 doses and consider vitamin K
What treatment is recommended for INR levels above 10 but not bleeding?
Hold warfarin and treat with 5 mg of oral vitamin K
What is the treatment for patients with elevated INR and active bleeding?
Cessation of warfarin, 10 mg of vitamin K IV, plus 4F-PCC or fresh frozen plasma
What is the most effective route for administering vitamin K?
Orally
How should intravenous vitamin K be administered?
As a slow infusion over 20 to 30 minutes
True or False: Subcutaneous dosing of vitamin K is the preferred method of administration.
False
Review labs and anticoagulation
Adjuncts and reversal agents
Critical Sites for Hemorrhage
in Anticoagulated Patients
Treatment for
Supratherapeutic INR
4F-PCC dosage for Warfarin reversal in major bleeding
DIC Abnormal Clotting Sequence
What does DIC stand for?
Disseminated Intravascular Coagulation
DIC is an acquired coagulopathy reflecting dysregulated coagulation and fibrinolytic pathways.
What is the primary cause of DIC?
DIC is caused by multiple pathologic processes that disturb the balance between procoagulants and inhibitors.
What are the clinical consequences of DIC?
- Life-threatening bleeding from loss of platelets and clotting factors
- Fibrinolysis and fibrin degradation product interference
- Small-vessel obstruction and tissue ischemia from fibrin deposition
- RBC injury and anemia from microvascular hemolysis
Which patients should be considered for DIC diagnosis?
Any patient with purpura, a bleeding tendency, and signs of organ injury, especially in the central nervous system or kidney.
How is the clinical diagnosis of DIC confirmed?
By laboratory testing.
What conditions may be confused with DIC?
- Severe liver disease
- Primary fibrinolysis
What are the manifestations of severe liver disease?
Clinical jaundice and splenomegaly.
What is primary fibrinolysis?
A rare disorder affecting fibrinogen and fibrin while preserving other coagulation components.
What is the initial treatment focus for DIC?
Reversal of the triggering mechanism.
When is specific management of DIC warranted?
In cases of active bleeding, significant risk of bleeding, invasive procedures, arterial or venous thromboembolism, skin necrosis, or acral ischemia.
What should be included in replacement therapy for active bleeding in DIC?
- Platelets
- Fresh frozen plasma
- Cryoprecipitate
What is the goal of replacement therapy in DIC?
To avoid depletion of clotting factors.
What monitoring indicators are useful in DIC management?
- Slowing of bleeding
- Decrease in fibrin degradation products
- Rise in platelet count and fibrinogen level
When can heparin be selectively utilized in DIC treatment?
When fibrin deposition and thrombosis predominate the pathologic picture.
What are some disease states associated with fibrin deposition?
- Purpura fulminans
- Retained nonviable fetus before delivery
- Giant hemangioma
- Acute promyelocytic leukemia
In which conditions is heparin generally of little benefit?
- Meningococcemia
- Abruptio placentae
- Severe liver disease
- Trauma
What other therapeutic agents have been evaluated for DIC?
- Antithrombin III
- PCC
- Recombinant factor VIIa
- Activated protein C
What are the goals of emergency care for patients with DIC?
- Early recognition and close monitoring
- Focus on mitigation of the precipitating condition
- Identifying potential life-threatening complications
- Initiation of blood product or anticoagulation therapy only rarely
Laboratory Diagnosis of Disseminated Intravascular Coagulation
