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]
Synonyms and keywords: Coagulopathy, platelet disorders.

Overview

The bleeding disorder can be due to coagulopathy, platelet dysfunction, or vessel pathology. They can be genetic or acquired. History of the site, duration, associated symptoms, and a thorough physical exam to evaluate the type of bleeding (subcutaneous vs deep tissue) are essential to diagnosis. Screening tests include CBC with peripheral smear, platelet count, PT, aPTT, PFA-100.

Causes

Common causes of bleeding (bleeding disorders) are enlisted below.

Causes of bleeding disorders

Platelet disorders

Coagulopathy

Vessel/ Supporting tissue defect

Acquired

Genetic

❑Aging ❑Corticosteroid use

❑ Myeloproliferative disorders ❑ Uremia ❑ Drugs (NSAIDs, asprin, clopidogrel, etc.) ❑ Neoplasia ❑ Monoclonal gammopathies ❑ DIC ❑ Ehrlichiosis ❑ Retroviral infection ❑ Snake venom ❑ Cirrhosis

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

Genetic

Acquired

Prothrombotic

❑ Prohemorrhagic liver diseases ❑ Vitamin K deficiency ❑ Drug-induced such as warfarin and heparin ❑ Hemodilution and massive transfusion ❑ Disseminated Intravascular Coagulation (DIC) ❑ Immunoglobulin mediated Factor Deficiency (VIII, V, XIII, X) ❑ Hyperfibrinolysis ❑ Venom induced

❑ Heparin Induced Thrombocytopenia ❑ Antiphospholipid Antibody Syndrome ❑ Microvascular Thrombosis (warfarin induced skin necrosis)

Hemorrhagic disorders

Hypercoaguable disease

❑ Factor VIII deficiency ❑ Factor IX Deficiency ❑ Von Willebrand factor deficiency ❑ Factor XI deficiency ❑ Factor II, V, VII, X deficiency (Common Pathway Proteins) ❑ Factor XIII deficiency and fibrinogen deficiency

❑ Antithrombin III deficiency ❑ Protein C deficiency and protein S deficiency

Diagnosis

The algorithm illustrates the approach to the diagnosis of bleeding disorder.
Abbreviations: HEENT: Head, Eyes, Ears. Nose, and Throat exam; CBC: Complete blood count; APTT Partial thromboplastin time; CMP: Comprehensive metabolic panel; LFTs:Liver function tests
Boxes in red signify that an urgent management is needed.

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; heart rate (tachycardia with regular pulse may demonstrate hypovolemia); respiratory rate, blood pressure (hypotension); and oxygen saturation may be low due to anemia. ❑ Assess the sites and severity of the bleeding. ❑ Assess if the bleeding is due to systemic disorder, local defect, or hemostatic disorder; inherited or acquired; platelet abnormality, coagulation disorder, or vascular defect. ❑ HEENT ❑ Cardiovascular examination ❑ Respiratory examination ❑ Gastrointestinal system exam includes oral examination, abdominal examination, and digital rectal exam. ❑ Extremities exam ❑ Skin exam: Evaluate for the petechie, bruises, hemorrhages. Location, symmetry, and pattern are important.

Screening Labs ❑ CBC with differential ❑ Platelet count ❑ Prothrombin time (PT) ❑ APTT ❑ Peripheral smear ❑ Thrombin time ❑ Bleeding time/ PFA-100

Soft tissue hematoma, deep internal hemorrhage, hemarthrosis

Superficial cutaneous or mucous membrane bleeding

PT normal, aPTT prolonged

PT prolonged, aPTT normal

PT prolonged, aPTT prolonged

Low platelet count

Normal platelet count

❑ Factor VIII, factor IX, factor XI, factor XII, and prekallikrien deficiency ❑ Von Willebrand Disease ❑ Heparin contamination ❑ Lupus anticoagulant

❑ Factor VII deficiency ❑ Vitamin K deficiency ❑ Early anticoagulation therapy

❑ Check thrombin time

❑ Idiopathic Thrombocytopenic Purpura (ITP) ❑ Hereditary platelet disorder ❑ Bone marrow failure

Check PFA-100

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Prolonged

Normal

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❑ Fibrinogen deficiency ❑ Liver disease ❑ Heparin

❑ Factor II, factor V, factor X deficiency ❑ Vitamin K deficiency ❑ Liver disease

Management

The following tables illustrates the treatment to common bleeding disorders.

Disorder	Labs	Treatment
Hemophilia A and Hemophilia B	Factor VIII and factor IX assay	Factor VIII concentrate (20-50 IU/ml) BPAs (bypass agents) help get around factor VIIa and Factor IX to generate thrombin. Immune tolerance induction (ITI) is the standard of care for inhibitor eradication predominantly among patients with severe hemophilia A.
Hemophilia C	Factor XI assay	Fresh frozen plasma
Von Willebrand disease		Bleeding prevention: 0.3 mcg/kg desmopressin acetate (DDAVP), 90 min before surgery helps prevent bleeding (increases the level of VWFAg and factor VIII to normal). Minor bleed: desmopressin acetate is the first-line agent. Tranexamic acid can be used. Major bleed: Replacing VWF and utilizing plasma-derived products. Antifibrinolytic drugs may be utilized as a hemostatic adjunct. Transfuse platelets only if other treatments fail. Factor VIII concentrates and plasma products also help overcome bleeding.
Factor XI deficiency (Hemophilia C)		Fresh frozen plasma, factor XI is rarely required
Microangiopathic hemolytic anemia	Platelet count] (also look for the clinical signs such as neurological deficits among TTP and renal dysfunction among HUS patients.	Total plasma exchange, cryoprecipitate lacking HMV VW multimers. 0.4 mg/kg IVIG for five days with a target platelet count to be 80,000. Among pregnant females with TTP/ HUS administer FFP and avoid RPR.
Thrombocytopenia of pregnancy	Platelet count	Mild: Corticosteroids Severe: Intervenous methyl prednisolone
SLE	Platelet count	RPR
Liver disease	LFTs, APTT, platelet count	Vitamin K if the patient has vitamin K deficiency. Treat per deficiency.

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.
• The initial clinical impression based on the baseline screening tests should direct specialized laboratory tests to save the time, effort and money.

Don'ts

References