Bleeding disorder resident survival guide

Bleeding disorder Resident Survival Guide
Overview
Causes
FIRE
Diagnosis
Treatment
Do's
Don'ts

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Javaria Anwer M.D.[2]

Overview

Causes

Coagulopathy

The following are the causes of coagulopathy (defects in coagulation):

Genetic:

Hemorrhagic Disorders

• Factor VIII Deficiency
• Factor IX Deficiency
• Von Willebrand Factor Deficiency
• Factor XI Deficiency
• Factor II, V, VII, X Deficiency (Common Pathway Proteins)
• Factor XIII and Fibrinogen Deficiency

Hypercoaguable Diseases

• Antithrombin III Deficiency
• Protein C and S Deficiency

Acquired:

• Prohemorrhagic Liver Diseases
• Vitamin K Deficiency
• Drugs such as:
  • Warfarin
  • Heparin
  • Hemodilution and massive transfusion
  • Disseminated Intravascular Coagulation (DIC)
  • Immunoglobulin mediated Factor Deficiency (VIII, V, XIII, X)
  • Hyperfibrinolysis
  • Venom Induced

Prothrombotic:

• Heparin Induced Thrombocytopenia
• Antiphospholipid Antibody Syndrome
• Microvascular Thrombosis (Warfarin Induced Skin Necrosis)

Platelet Related Disorders

Congenital:

• Glanzmann's thrombasthenia
• Bernard-Soulier syndrome
• Von Willebrand's disease

Acquired

• Myeloproliferative Disorders
• Uremia
• Drugs (NSAIDs, Aspririn, Clopidogrel etc.)
• Neoplasia
• Monoclonal Gammopathies
• DIC
• Ehlrichiosis
• Retroviral Infection
• Snake Venom
• Cirrhosis

FIRE

A Focused Initial Rapid Evaluation (FIRE) should be performed to identify patients with severe bleeding and in need of immediate intervention.

Boxes in red signify that an urgent management is needed Abbreviations

Boxes in red signify that an urgent management is needed.

Assess the patient for bleeding and screen with CBC, PT, aPTT ❑ Clinical assessment of types and sites of bleeding ❑ Spontaneous or follows trauma ❑ Duration of bleeding (lifelong/recent) ❑ History of blood transfusion ❑ History of bleeding after surgical procedures ❑ Drug History ❑ Family history of bleeding disorders

Soft tissue hematoma, deep internal hemoorhage, hemarthrosis

Superficial cutaneous or mucous membrane bleeding

PT Normal, aPTT Prolonged

PT Prolonged, aPTT Normal

PT Prolonged, aPTT Prolonged

Platelet Count Low

Platelet Count Normal

•Factor VIII, IX, XI Deficiency •Von Willebrand Disease •Heparin Contamination

•Factor VII deficiency •Vitamin K Deficiency

•Check Thrombin Time

•Idiopathic Thrombocytopenic Purpura (ITP) •Hereditary Platelet Disorder •Bone Marrow Failure

•Check PFA-100

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Diagnosis

Abbreviations:HEENT: Head, Eyes, Ears. Nose, and Throat exam; CBC: Complete blood count; ESR: Erythrocyte sedimentation rate; CMP: Comprehensive metabolic panel; LFTs:Liver function tests

The algorithm illustrates the approach to the diagnosis of bleeding disorder.

History ❑ Demographics: Patient age, gender,and race to screen for inherited disorders. ❑ Bleeding history: ❑ Onset of bleed: Differentiate between spontaneous vs post-trauma or post-surgery bleed. Post-trauma may suggest an inherited bleeding disorder. ❑ Duration of bleed: Lifelong vs recent. coagulation factor defect. ❑ Type and site of bleed (skin or muscle): Petechiae, purpura, epistaxis, gingival bleeding, and bruises may suggest a vascular or platelet abnormality. Joint or muscle bleed may suggest coagulation factor abnormality. ❑ Past medical history: For the underlying disease. History of blood or blood components transfusion. Childhood history of epistaxis, bleeding post-circumcision, and umbilical stump bleeding may suggest an inherited bleeding disorder. ❑ Past surgical history: May reveal post-surgical bleed such as after a tooth extraction. History of poor wound healing. ❑ Drug history: For the drugs causing bleeding. Distinguish between drug induced thrombocytopenia and idiopathic thrombocytopenic purpura, or other forms of thrombocytopenia. ❑ Family history: Certain bleeding disorders. Consanguineous marriage history. ❑ Gynaecological history: Menorrhagia or hematuria may suggest vascular or platelet abnormality.

Physical exam Appearance of the patient Petechie, bruises, or hemorrhages ❑ Vital signs ❑ Temperature: High-grade / low-grade fever ❑ Heart rate: Tachycardia with regular pulse may demonstrate hypotension due to hypovolemia. ❑ Respiratory rate: Tachypnea may demonstrate respiratory system involvement sepsis and DIC. ❑ Blood pressure: Hypotension indicates hypovolemia , due to excessive bleeding. ❑ Oxygen saturation: may be low due to anemia. ❑ HEENT ❑ Cardiovascular examination ❑ Respiratory examination ❑ Gastrointestinal system exam includes oral examination, abdominal examination, and digital rectal exam. ❑ Splenomegaly) may demonstrate IM, hodgkin's/ non-Hodgkin's lymphoma, and sarcoidosis ❑ Extremities exam ❑ Skin exam: Evaluate for the petechie, bruises, hemorrhages. Location, symmetry, and pattern are important.

Labs ❑ CBC with differential ❑ ESR ❑ CMP ❑ Peripheral smaer ❑ LFTs Labs may be required at a later stage pf diagnosis

Treatment

Do's

• A study by Wahlberg et al. demonstrated that the patient's perception of his/her own bleeding may be understated or exaggerated, so labs vital in the assessment of bleeding disorders.

Don'ts

References