Bruising and Bleeding

Last updated 31 May 2026 · Haematology

📋 Key Information Summary

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Bleeding disorders are classified into three broad categories: vascular (vessel wall defects), platelet (quantitative or qualitative), and coagulation (clotting factor deficiencies or inhibitors).
A structured bleeding history is the single most important diagnostic tool — distinguish mucocutaneous bleeding (platelet/vascular) from deep tissue/haemarthrosis bleeding (coagulation).
The Purpura Diagnostic Model differentiates petechiae, purpura, and ecchymoses by size and aetiology to guide the initial investigation pathway.
Von Willebrand disease (VWD) is the most common inherited bleeding disorder (prevalence ~1%), presenting with mucocutaneous bleeding; Type 1 accounts for 70–80% of cases.
Haemophilia A (Factor VIII deficiency) and Haemophilia B (Factor IX deficiency) are X-linked recessive; severity is classified by factor level (<1% severe, 1–5% moderate, 5–40% mild).
First-line for VWD Type 1: desmopressin (DDAVP) 0.3 µg/kg IV or intranasal; for Types 2/3, use von Willebrand Factor/Factor VIII concentrate (e.g., Biostate®, Wilate®).
Haemophilia A bleeding is managed with recombinant Factor VIII (e.g., Advate®, Kogenate®); Haemophilia B with recombinant Factor IX (e.g., BeneFIX®). Emicizumab (Hemlibra®) is a non-factor prophylaxis option for haemophilia A with inhibitors.
Patients on anticoagulants (warfarin, DOACs) or antiplatelet agents commonly present with bruising — always consider drug-induced bleeding before investigating for intrinsic disorders.
Initial investigations: FBC with film, APTT, PT/INR, fibrinogen. If abnormal or clinical suspicion is high, proceed to specific factor assays, von Willebrand panel, and platelet function testing.
Aboriginal and Torres Strait Islander Australians have higher rates of rheumatic heart disease and may be on anticoagulation; remote communities face barriers to specialist haematology access and factor concentrate availability.
Refer to a haematologist for unexplained bleeding diathesis, suspected inherited coagulopathy, severe/prolonged bleeding, or prior to invasive procedures in at-risk patients.

Introduction & Australian Epidemiology

Bruising and bleeding are among the most common presenting complaints in primary care and emergency medicine. While most episodes are benign and self-limiting, some may signal an underlying inherited or acquired bleeding disorder requiring timely diagnosis and specialist management. The clinical challenge lies in distinguishing normal physiological bruising from pathological bleeding diatheses.

In Australia, inherited bleeding disorders affect approximately 30,000 people, with von Willebrand disease (VWD) being the most prevalent at a population prevalence of roughly 1%. Haemophilia A affects approximately 1 in 5,000 males and Haemophilia B approximately 1 in 25,000 males. The Australian Bleeding Disorders Registry (ABDR), managed by the National Blood Authority, maintains data on over 3,000 people with severe bleeding disorders.

Acquired causes of abnormal bleeding are considerably more common and include anticoagulant and antiplatelet medication use (affecting an estimated 750,000 Australians on anticoagulants), chronic liver disease, vitamin K deficiency, disseminated intravascular coagulation (DIC), and immune thrombocytopenic purpura (ITP). Drug-induced bleeding — particularly from warfarin, direct oral anticoagulants (DOACs), aspirin, and clopidogrel — should always be considered early in the differential.

Aboriginal and Torres Strait Islander Australians have a disproportionately higher burden of conditions predisposing to bleeding complications, including rheumatic heart disease requiring long-term anticoagulation, chronic kidney disease, and chronic liver disease. Access to specialist haematology services remains limited in remote and very remote areas.

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Clinical pearl: Always take a thorough bleeding history before ordering investigations. The clinical context — mucocutaneous vs. deep tissue bleeding, age of onset, family history, and medication use — will direct your investigation pathway more efficiently than a battery of screening tests.

Classification of Bleeding Disorders

Bleeding disorders are broadly classified into three categories based on the component of haemostasis that is disrupted. This classification directly guides clinical assessment, investigation selection, and management.

Vascular Disorders (Vessel Wall Defects)

Abnormal bleeding due to structural or functional defects in blood vessel walls. These typically present with easy bruising, petechiae, and purpura that are often localised rather than systemic.

Condition	Mechanism	Key Features
Hereditary haemorrhagic telangiectasia (HHT / Osler-Weber-Rendu)	AD; defective endoglin/ALK-1 → abnormal vessel formation	Epistaxis, telangiectasia on lips/tongue/hands, AVMs (lung, liver, brain)
Ehlers-Danlos syndrome (vascular type IV)	COL3A1 mutation → defective type III collagen	Easy bruising, arterial dissection, organ rupture; thin translucent skin
Senile purpura	Dermal atrophy, loss of perivascular connective tissue support	Ecchymoses on sun-exposed forearms in elderly; benign
Steroid purpura	Exogenous corticosteroids → dermal thinning	Easy bruising in patients on long-term oral or topical corticosteroids
Scurvy (vitamin C deficiency)	Impaired collagen synthesis	Perifollicular haemorrhages, gum bleeding, poor wound healing
Henoch-Schönlein purpura (IgA vasculitis)	IgA-mediated small vessel vasculitis	Palpable purpura (lower limbs/buttocks), arthralgia, abdominal pain, nephritis

Platelet Disorders

Defects in platelet number (quantitative) or function (qualitative). Clinical presentation is characteristically mucocutaneous: petechiae, purpura, epistaxis, gingival bleeding, menorrhagia, and prolonged bleeding from superficial cuts.

Category	Examples	Key Features
Thrombocytopenia (↓ count)	ITP, TTP, HIT, drug-induced (heparin, quinine, valproate), marrow failure, hypersplenism, DIC, sepsis	Spontaneous bleeding typically <20 × 10⁹/L; petechiae, purpura; bleeding risk increases with severity
Platelet dysfunction (qualitative)	Bernard-Soulier syndrome (GPIb defect), Glanzmann thrombasthenia (GPIIb/IIIa defect), storage pool disease, uraemic platelet dysfunction	Prolonged bleeding time despite normal platelet count; mucocutaneous bleeding
Drug-induced platelet dysfunction	Aspirin, clopidogrel, ticagrelor, prasugrel, NSAIDs, SSRIs, dipyridamole	History often reveals antiplatelet or NSAID use; bruising, prolonged bleeding from cuts

Coagulation Disorders

Deficiencies or inhibitors of clotting factors. Characteristically present with delayed bleeding into deep tissues: haemarthroses, deep muscle haematomas, prolonged bleeding post-surgery or trauma, and intracranial haemorrhage.

Condition	Inheritance / Cause	Key Features
Haemophilia A (Factor VIII deficiency)	X-linked recessive	Haemarthroses, deep muscle bleeds, prolonged post-operative bleeding; severity correlated with factor level
Haemophilia B (Factor IX deficiency)	X-linked recessive	Clinically indistinguishable from haemophilia A; requires specific factor assay
Von Willebrand disease	Usually AD	Mucocutaneous bleeding; mucosal surfaces; may have features of both platelet and coagulation disorder
Vitamin K deficiency	Acquired — malnutrition, malabsorption, prolonged antibiotics, neonatal	Prolonged PT/INR; corrects with vitamin K administration
Liver disease coagulopathy	Acquired — reduced synthesis of all factors except VIII	Prolonged PT, APTT; often thrombocytopenia; rebalanced haemostasis
DIC	Acquired — sepsis, trauma, malignancy, obstetric complications	Consumption of factors and platelets; microangiopathic bleeding and thrombosis
Acquired haemophilia (Factor VIII inhibitors)	Autoantibodies; associated with malignancy, autoimmune disease, pregnancy, drugs	Spontaneous soft-tissue bleeding in adults without prior history; prolonged APTT not correcting on mixing study

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Distinguishing the pattern: Mucocutaneous bleeding (petechiae, purpura, epistaxis, gingival bleeding, menorrhagia) → think platelet or vascular. Deep tissue bleeding (haemarthroses, muscle haematomas, delayed post-surgical bleeding) → think coagulation factor deficiency.

Checklist for a Bleeding History

A structured bleeding history is the cornerstone of evaluating patients with suspected bleeding disorders. The International Society on Thrombosis and Haemostasis (ISTH) Bleeding Assessment Tool (BAT) provides a validated framework. The following checklist should be systematically assessed:

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Red flags requiring urgent haematology referral: Unprovoked intracranial haemorrhage, compartment syndrome from deep muscle bleed, life-threatening haemorrhage on anticoagulation, suspected DIC, or new-onset severe bleeding in a previously well patient.

Bleeding History Checklist

Site and Pattern of Bleeding

Epistaxis (frequency, duration, need for packing/cautery), gingival bleeding (spontaneous vs. brushing), skin bleeding (petechiae, purpura, ecchymoses), GI bleeding, haematuria, menorrhagia (pad/tampon count, clot passage, duration), haemarthroses, deep muscle bleeds, CNS bleeding.

Timing and Onset

Age of onset (childhood suggests inherited; adulthood suggests acquired). Lifelong vs. recent onset. Neonatal bleeding (cord separation, cephalohaematoma) may indicate inherited coagulopathy.

Provocation and Severity

Spontaneous vs. trauma/surgery-provoked. Bleeding after dental extractions, tonsillectomy, circumcision, childbirth, or minor surgery. Severity: need for transfusion, hospitalisation, or surgical intervention.

Family History

Bleeding symptoms in first-degree relatives. Pattern of inheritance (X-linked for haemophilia, AD for VWD and most vascular disorders). Consanguinity. Family history of thrombocytopenia or easy bruising.

Medication and Supplement History

Antiplatelet agents (aspirin, clopidogrel, ticagrelor), anticoagulants (warfarin, apixaban, rivaroxaban, dabigatran, enoxaparin), NSAIDs, SSRIs, fish oil, ginkgo biloba, garlic supplements, vitamin E in high doses. Duration of use and compliance.

Comorbidities

Chronic liver disease, chronic kidney disease (uraemic platelet dysfunction), autoimmune conditions (SLE → acquired inhibitors), malignancy, myelodysplastic syndromes, HIV, malabsorption syndromes.

Obstetric History (Females)

Menorrhagia since menarche, postpartum haemorrhage, recurrent pregnancy loss. Heavy menstrual bleeding is the most common presentation of VWD in women.

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ISTH-BAT scores: A score ≥4 in adults (≥3 in children) is considered abnormal and warrants further investigation. Scores are age- and sex-specific. The BAT is available through the World Federation of Haemophilia (WFH) website.

Purpura Diagnostic Model

The purpura diagnostic model provides a systematic clinical framework for evaluating patients presenting with skin bleeding. Purpura is defined as extravasation of blood into the skin that does not blanch with pressure, distinguishing it from erythema and telangiectasia.

Classification by Size

Type	Size	Clinical Significance	Common Aetiologies
Petechiae	<3 mm	Pinpoint, non-blanching red/purple spots	Thrombocytopenia, platelet dysfunction, vasculitis (early), meningococcaemia, raised venous pressure (coughing/vomiting)
Purpura	3 mm – 1 cm	Non-blanching; may be palpable (vasculitis) or flat (non-palpable)	Palpable: vasculitis (HSP, IgA, PAN). Non-palpable: thrombocytopenia, senile purpura, steroid purpura, scurvy
Ecchymoses	>1 cm	Large areas of bruising; may evolve through colour changes (red → purple → green → yellow)	Trauma, coagulation factor deficiencies, severe thrombocytopenia, vasculopathy, amyloidosis

Palpable vs. Non-Palpable Purpura

This distinction is clinically critical and directs the diagnostic approach:

Non-Palpable Purpura

Caused by haematological or coagulation defects — thrombocytopenia, platelet dysfunction, coagulation factor deficiency, vasculopathy, or increased vascular fragility.

Investigations: FBC with film, coagulation screen (APTT, PT/INR, fibrinogen), platelet function assay.

Palpable Purpura

Caused by vasculitis — inflammation and damage to vessel walls allows blood extravasation. The purpura can be felt on palpation.

Investigations: Skin biopsy, ANA, ANCA, complement levels (C3/C4), cryoglobulins, IgA levels, hepatitis B/C serology, urinalysis.

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Meningococcal petechiae: Petechiae in a febrile, unwell child or adult — particularly in a centripetal distribution — is meningococcaemia until proven otherwise. Treat empirically with IV benzylpenicillin (or ceftriaxone) and transfer urgently. This is a medical emergency.

Non-Vascular Causes of Purpura to Exclude

Factitious purpura: Self-inflicted; often in a distribution accessible to the patient; dermatitis artefacta.
Solar purpura: UV-related; on sun-exposed areas; common in elderly.
Increased intravascular pressure: Petechiae above nipple line after vomiting, coughing, prolonged tourniquet use (Valsalva-related).
Dependent purpura: Lower limb purpura in immobile or venous insufficiency patients.
Drug-related: Corticosteroids, anticoagulants, SSRIs; consider iatrogenic causes first.

Haemophilia A & B and Von Willebrand Disease

Von Willebrand Disease (VWD)

Von Willebrand disease is the most common inherited bleeding disorder, affecting approximately 1% of the population, though clinically significant disease occurs in a smaller proportion. Von Willebrand factor (VWF) serves two key functions: (1) mediating platelet adhesion to damaged vessel walls and (2) protecting Factor VIII from premature proteolytic degradation.

Type	Prevalence	Pathophysiology	VWF:Ag	VWF:RCo	FVIII
Type 1 (quantitative partial deficiency)	70–80% of VWD	Reduced synthesis/secretion of qualitatively normal VWF	↓	↓ (proportional)	↓ or normal
Type 2A (qualitative — ↓ large multimers)	10–15%	Defective VWF secretion or increased proteolysis; loss of high-molecular-weight multimers	↓ or normal	↓↓ (disproportionate)	Variable
Type 2B (qualitative — ↑ platelet binding)	Rare	Increased affinity of VWF for platelet GPIb; consumption of platelets and large multimers	↓ or normal	↓	Variable
Type 2M (qualitative — ↓ platelet binding)	Rare	Decreased platelet-dependent function without loss of large multimers	Normal or ↓	↓↓	Normal or ↓
Type 2N (Normandy — ↓ FVIII binding)	Rare	Defective binding of VWF to Factor VIII; mimics mild haemophilia A	Normal	Normal	↓↓
Type 3 (quantitative — virtually absent)	Rare (1 in 500,000)	Virtual absence of VWF; AR inheritance	Undetectable	Undetectable	↓↓↓ (<10%)

VWD Management

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Desmopressin (DDAVP)

Octostim® · Synthetic vasopressin analogue

Adult dose 0.3 µg/kg IV in 50 mL NaCl 0.9% over 20–30 min; or 300 µg intranasal (150 µg each nostril)

Paediatric dose 0.3 µg/kg IV over 20–30 min; intranasal for children >5 years (150 µg for <50 kg)

Indication First-line for VWD Type 1 (confirmed response via DDAVP trial); minor bleeding/procedures

Contraindications Type 2B (may worsen thrombocytopenia); Type 3 (no VWF to release); hyponatraemia risk — fluid restrict for 24 h

PBS status ✔ PBS General Benefit

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VWF/FVIII Concentrate

Biostate® · Wilate® · Immunate® · Plasma-derived

Adult dose Dose (IU) = body weight (kg) × desired VWF:RCo rise (IU/dL) × 0.5; typically 40–80 IU/kg for major bleeding/surgery

Paediatric dose Same formula; adjusted to clinical response

Indication VWD Types 2 and 3; Type 1 non-responsive to DDAVP; major bleeding/surgery in all types

PBS status ⚠ Authority Required — available through Australian Haemophilia Treatment Centres (HTCs)

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Tranexamic Acid

Cyklokapron® · Antifibrinolytic

Adult dose 1 g PO/IV TDS; or 15 mg/kg IV/PO TDS; mouthwash: 5 mL (500 mg) gargle QID for oral bleeding

Paediatric dose 15 mg/kg PO/IV TDS (max 1 g per dose); mouthwash: 10 mg/kg QID

Indication Adjunctive for mucosal bleeding (oral, nasal, menorrhagia); useful as monotherapy for mild VWD Type 1; dental procedures

Renal adjustment eGFR 30–60: reduce dose to 10 mg/kg TDS; eGFR <30: 5 mg/kg TDS or avoid (accumulation risk → seizures)

PBS status ✔ PBS General Benefit

Haemophilia A

Haemophilia A is an X-linked recessive bleeding disorder caused by deficiency of coagulation Factor VIII. It affects approximately 1 in 5,000 males worldwide and is classified by severity based on residual Factor VIII activity.

Mild

Factor VIII 5–40%

Bleeding only with significant trauma or surgery. May not be diagnosed until adulthood. No spontaneous haemarthroses.

Setting: General practice with haematology oversight

Moderate

Factor VIII 1–5%

Bleeding with minor trauma; occasional spontaneous haemarthroses (1–4 per year). Joint disease may develop over time.

Setting: Haemophilia Treatment Centre (HTC) co-management

Severe

Factor VIII <1%

Frequent spontaneous haemarthroses (≥5 per year), deep muscle bleeds, intracranial haemorrhage risk. Target joints develop. Requires lifelong prophylaxis.

Setting: Haemophilia Treatment Centre (primary management)

Haemophilia A Treatment

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Recombinant Factor VIII

Advate® · Kogenate® FS · Xyntha® · Recombinate®

Prophylaxis dose 25–40 IU/kg IV three times weekly or every other day; tailored to trough levels (target trough ≥1%)

Acute bleeding dose Dose (IU) = body weight (kg) × desired FVIII rise (%) × 0.5; joint bleed: 25–50 IU/kg; life-threatening: 50–100 IU/kg

Duration Joint bleed: 1–3 days; major surgery: 7–14 days; life-threatening: 10–14 days minimum

PBS status ⚠ Authority Required — funded through the NBA/IHCF programme at HTC

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Emicizumab

Hemlibra® · Bispecific monoclonal antibody (non-factor)

Dose Prophylaxis: 1.5 mg/kg SC weekly, or 3 mg/kg SC every 2 weeks, or 6 mg/kg SC every 4 weeks (after loading)

Indication Prophylaxis in haemophilia A with or without Factor VIII inhibitors; breakthrough bleeds managed with activated FVIIa (not aPCC due to thrombotic risk)

Key safety point Do NOT use aPCC (FEIBA®) concomitantly — risk of thrombotic microangiopathy (TMA)

PBS status ⬤ Authority Required (Specialist) — PBS-listed since 2020 for patients with inhibitors or severe haemophilia A

Haemophilia B

Haemophilia B (Christmas disease) is an X-linked recessive disorder caused by Factor IX deficiency. It is clinically indistinguishable from Haemophilia A but is approximately five times less common (1 in 25,000 males). Severity classification is identical to Haemophilia A, using Factor IX levels.

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Recombinant Factor IX

BeneFIX® · Rixubis® · Alprolix® (extended half-life)

Prophylaxis dose Standard half-life: 40–60 IU/kg IV twice weekly; Extended half-life (Alprolix®): 50 IU/kg every 1–2 weeks

Acute bleeding dose Dose (IU) = body weight (kg) × desired FIX rise (%) × 1.0 (note: higher volume of distribution than FVIII); joint bleed: 40–80 IU/kg

Key difference from FVIII FIX has a larger volume of distribution — the dosing multiplier is 1.0 (not 0.5)

PBS status ⚠ Authority Required — funded through the NBA/IHCF programme

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Inhibitor development: Approximately 25–30% of severe haemophilia A patients and 3–5% of severe haemophilia B patients develop neutralising antibodies (inhibitors) against infused factor. This renders standard factor replacement ineffective. Suspect inhibitors if bleeding fails to respond to adequate factor doses. Confirm with Bethesda assay. Refer urgently to HTC for immune tolerance induction (ITI) and/or emicizumab initiation.

Investigations

Investigation should be guided by the clinical picture from the bleeding history and physical examination. A stepwise approach avoids unnecessary testing and reduces healthcare costs.

First-Line Screening Tests

Essential

Full Blood Count (FBC) with Blood Film

Platelet count, morphology, and size. Rule out thrombocytopenia, pseudothrombocytopenia (EDTA clumping), leukaemia, MDS. MBS Item 65070.

Essential

Activated Partial Thromboplastin Time (APTT)

Screens the intrinsic pathway (Factors VIII, IX, XI, XII) and common pathway. Prolonged in haemophilia A/B, VWD (if FVIII low), lupus anticoagulant, heparin. MBS Item 65100.

Essential

Prothrombin Time (PT) / INR

Screens the extrinsic pathway (Factor VII) and common pathway. Prolonged in warfarin use, liver disease, vitamin K deficiency, DIC. MBS Item 65100.

Essential

Fibrinogen (Clauss method)

Reduced in DIC, liver disease, congenital hypofibrinogenaemia. Part of the coagulation screen. MBS Item 65105.

Second-Line / Specific Tests

Available

Von Willebrand Panel

VWF antigen (VWF:Ag), VWF ristocetin cofactor activity (VWF:RCo), Factor VIII level. Confirms VWD type. Repeat testing required (VWF is an acute phase reactant). MBS Item 65122.

Available

Factor VIII / IX Assays

Specific factor activity levels to diagnose and grade haemophilia severity. MBS Item 65122.

Available

Bethesda Assay (Inhibitor Screen)

Quantifies neutralising antibodies against Factor VIII or IX. Titre ≥0.6 Bethesda Units (BU) is significant. >5 BU = high-titre inhibitor. MBS Item 65125.

Available

Mixing Study (APTT 1:1 Mix)

Mix patient plasma 1:1 with normal pooled plasma. Correction suggests factor deficiency; failure to correct suggests an inhibitor (lupus anticoagulant or specific factor inhibitor).

Available

Platelet Function Analyser (PFA-100/PFA-200)

Point-of-care screening for platelet and VWF dysfunction. Sensitive but not specific. Not a substitute for formal platelet aggregometry. Available at major pathology providers.

Referral

Platelet Aggregometry (LTA)

Gold standard for platelet function testing. Performed at specialist reference laboratories. Diagnoses Bernard-Soulier, Glanzmann, storage pool disease. Requires specialist referral.

Specialist

Thromboelastography (TEG) / Rotational Thromboelastometry (ROTEM)

Viscoelastic point-of-care testing assessing global haemostasis. Used in major trauma, cardiac surgery, and liver transplant settings. Available at tertiary hospitals.

Investigation Pathway by Clinical Pattern

Clinical Pattern	Likely Category	First-Line Tests	Second-Line Tests
Mucocutaneous bleeding (petechiae, purpura, epistaxis, menorrhagia)	Platelet or vascular	FBC + film, coagulation screen, APTT	VWD panel, PFA-100, platelet aggregometry, skin biopsy (if vasculitis suspected)
Deep tissue bleeding (haemarthrosis, muscle haematoma, delayed surgical bleeding)	Coagulation factor deficiency	APTT, PT/INR, fibrinogen, FBC	Factor VIII/IX/XI assays, mixing study, Bethesda assay
Both mucocutaneous AND deep tissue	VWD, DIC, severe liver disease	FBC, APTT, PT/INR, fibrinogen, D-dimer	VWD panel, liver function, factor assays
Isolated prolonged APTT (normal PT)	Intrinsic pathway defect	Mixing study → if corrects: factor assay (VIII/IX/XI); if fails: lupus anticoagulant	Factor VIII, IX, XI, XII assays; LA screen/confirm
Isolated prolonged PT/INR (normal APTT)	Extrinsic pathway / warfarin / liver	Check warfarin use; vitamin K level; LFTs	Factor VII level (rarely needed)

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Repeat testing: VWF levels are influenced by stress, exercise, oestrogen, pregnancy, inflammation, and blood group (type O has lower baseline VWF). A single normal result does not exclude VWD. Repeat abnormal results on at least two occasions before confirming the diagnosis.

Special Populations

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Pregnancy

VWD in pregnancy

VWF and FVIII levels rise 2–3 fold by the third trimester in most Type 1 VWD patients. Type 2 and 3 VWD require factor concentrate cover. Monitor levels every 4 weeks from 28 weeks. Target VWF:RCo and FVIII >50 IU/dL at delivery. Tranexamic acid can be used postpartum (compatible with breastfeeding). Epidural anaesthesia requires levels >50 IU/dL — liaise with obstetric anaesthesia.

Haemophilia carrier status

Female carriers of haemophilia may have reduced factor levels and be at risk of postpartum haemorrhage. Check factor levels in the third trimester. Factor levels <50% require factor cover at delivery. All male neonates of carrier mothers should have cord blood FVIII/IX levels checked.

DDAVP in pregnancy

Desmopressin can be used in pregnancy (Category B2); fluid restriction is critical due to hyponatraemia risk, especially in the peripartum period when oxytocin also affects water balance.

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Paediatrics

Neonatal bleeding

Vitamin K deficiency bleeding (VKDB) — prevented by IM vitamin K at birth (all Australian states recommend this). Late VKDB (2–12 weeks) occurs in exclusively breastfed infants who did not receive vitamin K. Present with intracranial haemorrhage. Also consider haemophilia in male neonates with prolonged bleeding from heel prick, intracranial haemorrhage, or excessive cephalohaematoma.

Haemophilia in children

Prophylaxis is the standard of care for severe haemophilia. Commence with first joint bleed or by age 1–2 years. Peripheral IV access can be challenging — consider port-a-cath or subcutaneous emicizumab. Vaccination: avoid IM injections in severe haemophilia; use subcutaneous route or give factor cover 30 min before IM injection.

ITP in children

Most common cause of isolated thrombocytopenia in children. Usually post-viral, self-limiting (resolves within 6 months in 80%). Bleeding without trauma: observe. Significant bleeding: IV immunoglobulin (IVIG) 0.8–1 g/kg or methylprednisolone 30 mg/kg/day for 3 days.

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Elderly

Drug-induced bleeding

Polypharmacy is the most common cause of bruising and bleeding in the elderly. Review all antiplatelet agents, anticoagulants, NSAIDs, SSRIs, and supplements. Falls risk combined with anticoagulation significantly increases bleeding risk. Consider HAS-BLED score for anticoagulated patients.

Senile purpura

Benign; chronic sun damage to dermal connective tissue. Ecchymoses on dorsal forearms and hands. No investigation required unless bleeding is widespread or atypical in distribution.

Acquired haemophilia

Peak incidence in the elderly (median age 70 years). Presents with spontaneous soft-tissue bleeding (bruising, muscle haematomas, retroperitoneal bleeding) without prior bleeding history. Prolonged APTT not correcting on mixing study. Requires urgent haematology referral and immunosuppression.

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Renal Impairment

Uraemic platelet dysfunction

Impaired platelet adhesion and aggregation in chronic kidney disease. Bleeding time may be prolonged despite normal platelet count. Management: optimise dialysis, correct anaemia (target Hb >100 g/L with EPO/transfusion), DDAVP 0.3 µg/kg IV, cryoprecipitate if severe, conjugated oestrogen 0.6 mg/kg/day IV for 5 days.

Anticoagulation in CKD

DOACs require dose adjustment or avoidance in severe CKD (eGFR <15–30 mL/min depending on agent). Apixaban is the preferred DOAC in advanced CKD. Warfarin is used in dialysis patients with mechanical heart valves or atrial fibrillation with high thrombotic risk, with careful INR monitoring.

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Hepatic Impairment

Liver disease coagulopathy

Chronic liver disease produces a "rebalanced" haemostatic state — reduced procoagulant AND anticoagulant factors. INR alone does not predict bleeding risk accurately. Thrombocytopenia from hypersplenism and reduced thrombopoietin. Fibrinolysis may be accelerated. Avoid routine FFP — it is ineffective and risks fluid overload. Treat bleeding-specific: vitamin K IV, platelet transfusion if <50 × 10⁹/L, cryoprecipitate if fibrinogen <1.5 g/L, tranexamic acid for hyperfibrinolysis.

Avoid in liver disease

NSAIDs — increased GI bleeding and renal injury risk. Paracetamol is preferred analgesic (max 2 g/day in severe liver disease). DDAVP — less effective due to reduced VWF stores.

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Immunocompromised

HIV-associated thrombocytopenia

Immune-mediated platelet destruction, distinct from ITP. Responds to antiretroviral therapy. IVIG and corticosteroids for severe bleeding. Avoid splenectomy (risk of overwhelming sepsis).

Drug-induced thrombocytopenia

Common culprits in immunocompromised patients: cotrimoxazole, linezolid, valproic acid, vancomycin, heparin (HIT). Heparin-induced thrombocytopenia (HIT) — stop all heparin immediately, commence argatroban or fondaparinux, haematology referral.

Aboriginal and Torres Strait Islander Health Considerations

Aboriginal and Torres Strait Islander Health

Aboriginal and Torres Strait Islander Australians experience a significantly higher burden of conditions predisposing to bleeding and bruising complications. A culturally safe, trauma-informed approach to assessment is essential, particularly when bruising may raise concerns about differential diagnosis including non-accidental injury in children.

Rheumatic Heart Disease (RHD)

Aboriginal and Torres Strait Islander Australians have the highest rates of RHD globally. Long-term warfarin anticoagulation for mechanical heart valves or secondary prophylaxis increases bleeding risk. INR monitoring is challenging in remote communities. Point-of-care INR testing (CoaguChek®) is available in some communities through RHDAustralia programmes. Ensure access to vitamin K (IV/PO) for emergency reversal.

Chronic Kidney Disease

CKD prevalence is 2–3 times higher in Indigenous Australians, contributing to uraemic platelet dysfunction and increased bleeding risk. Renal anaemia management with EPO can help improve haemostasis. Factor concentrate dosing requires renal adjustment for some products (tranexamic acid dose reduction when eGFR <30 mL/min).

Chronic Liver Disease

Higher rates of hepatitis B and C, harmful alcohol use, and non-alcoholic fatty liver disease contribute to liver-related coagulopathy. Hepatitis B vaccination is funded under the National Immunisation Program. Liver function tests and coagulation screening should be part of routine health assessments for at-risk individuals.

Remote Access to Specialist Care

There are limited Haemophilia Treatment Centres (HTCs) — primarily in capital cities. The National Haemophilia Foundation and Australian Haemophilia Centre Directors' Organisation (AHCDO) support telehealth models. Factor concentrates must be stored at 2–8°C — cold chain logistics in remote areas are critical. The NBA manages the national blood product distribution network, including to remote areas.

Cultural Safety in Bleeding Assessment

Bruising in children must be assessed with cultural sensitivity. Aboriginal and Torres Strait Islander children may be cared for by extended family (kinship care) — involve the appropriate cultural liaison. The "purple cards" / healing bruises pattern guide should not be applied uncritically across all skin tones — petechiae and purpura may be less visually apparent on darker skin. Examine palmar surfaces, soles, oral mucosa, and conjunctivae for petechiae.

Vitamin K Deficiency

Remote communities may have limited access to fresh produce, increasing the risk of vitamin K deficiency. Neonatal vitamin K administration rates are lower in some Indigenous communities. Health promotion and ensuring vitamin K is given at birth is a critical preventive measure. Community health workers and Aboriginal Health Practitioners are essential in this education.

📚 References

1. National Blood Authority (NBA). Australian Bleeding Disorders Registry (ABDR) Annual Report 2022–2023. Canberra: NBA; 2023. Available from: www.blood.gov.au
2. Australian Haemophilia Centre Directors' Organisation (AHCDO). Guidelines for the management of haemophilia in Australia. Melbourne: AHCDO; 2020.
3. Laffan MA, Lester W, O'Donnell JS, et al. The diagnosis and management of von Willebrand disease: a United Kingdom Haemophilia Centre Doctors Organization guideline approved by the British Committee for Standards in Haematology. Br J Haematol. 2014;167(4):453–465.
4. Srivastava A, Santagostino E, Dougall A, et al. WFH Guidelines for the Management of Hemophilia, 3rd edition. Haemophilia. 2020;26(Suppl 6):1–158.
5. Rodeghiero F, Tosetto A, Abshire T, et al. ISTH/SSC bleeding assessment tool: a standardized questionnaire and a proposal for a new bleeding score for inherited bleeding disorders. J Thromb Haemost. 2010;8(9):2063–2065.
6. National Heart Foundation of Australia and Cardiac Society of Australia and New Zealand. Australian Clinical Guidelines for the Diagnosis and Management of Atrial Fibrillation 2018. Sydney: NHFA; 2018.
7. Australian Institute of Health and Welfare (AIHW). Aboriginal and Torres Strait Islander Health Performance Framework 2023 — Summary Report. Canberra: AIHW; 2023.
8. RHDAustralia (Rheumatic Heart Disease Australia). The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
9. Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report. Haemophilia. 2008;14(2):171–232.
10. Australian Commission on Safety and Quality in Health Care (ACSQHC). Blood and Blood Products — National Safety and Quality Health Service Standards. 2nd ed. Sydney: ACSQHC; 2021.
11. Mannucci PM, Franchini M. Old and new antihemophilic factor concentrates for the treatment of hemophilia A: pharmacokinetic and clinical considerations. Blood Transfus. 2018;16(2):150–157.
12. Oldenburg J, Mahlangu JN, Kim B, et al. Emicizumab prophylaxis in hemophilia A with inhibitors. N Engl J Med. 2017;377(9):809–818.
13. Rodeghiero F, Pabinger I, Ragni M, et al. Fundamentals for a systematic approach to mild and moderate inherited bleeding disorders: an EHA consensus report. Hemasphere. 2019;3(6):e295.
14. Baglin T. The role of the laboratory in treatment with new oral anticoagulants. J Thromb Haemost. 2013;11(Suppl 1):122–128.
15. Watson HG, Greaves M. Can we predict bleeding? Semin Thromb Hemost. 2008;34(1):97–103.

for PBS scripts. Utilise ACCHS pharmacies and Remote Area Aboriginal Health Worker programs for medication supply in remote areas. Avoid initiating benzodiazepines; support holistic pain management including community-based exercise programs.

Preventive health

Promote bone health: encourage vitamin D supplementation (1000 IU daily in deficient individuals), smoking cessation support, reduction of alcohol intake, and weight-bearing exercise. MBS Item 715 health checks provide a structured opportunity to assess bone health, screen for osteoporosis risk factors, and discuss musculoskeletal health in a culturally safe context.

Quick Reference: Differential Diagnosis at a Glance

Costovertebral dysfunction

Paracetamol ± NSAID; manual therapy

2–6 weeks

Provocable on palpation; no red flags

Thoracic compression fracture

Paracetamol; ± calcitonin; DXA + osteoporosis Rx

6–12 weeks healing

Elderly; osteoporosis; acute onset

ACS (posterior MI)

Aspirin 300 mg, GTN, heparin; urgent PCI

Time-critical

ECG, troponin; CV risk factors

Aortic dissection

IV labetalol; urgent CT aortogram; surgery (Type A)

Time-critical

Tearing pain; BP differential >20 mmHg

Vertebral osteomyelitis

IV antibiotics (vancomycin + ceftriaxone initially); ID consult

6 weeks IV antibiotics

Fever, elevated CRP, IV drug use

Biliary colic / cholecystitis

Paracetamol ± morphine; lap cholecystectomy

Surgical within 72 h (cholecystitis)

RUQ/infrascapular; post-prandial; RUQ US

📚 References

1. Briggs AM, Smith AJ, Straker LM, Bragge P. Thoracic spine pain in the general population: prevalence, incidence and associated factors in children, adolescents and adults. A systematic review. BMC Musculoskelet Disord. 2009;10:77.
2. National Health and Medical Research Council (NHMRC). Evidence-based management of acute musculoskeletal pain. Canberra: NHMRC; 2003 (updated 2020).
3. Australian Institute of Health and Welfare (AIHW). Aboriginal and Torres Strait Islander Health Performance Framework: Summary report 2023. Canberra: AIHW; 2023.
4. Deyo RA, Rainville J, Kent DL. What can the history and physical examination tell us about low back pain? JAMA. 1992;268(6):760–765.
5. Stochkendahl MJ, Kjaer P, Hartvigsen J, et al. National Clinical Guidelines for non-surgical treatment of patients with recent onset low back pain or lumbar radiculopathy. Europ Spine J. 2018;27(1):60–75.
6. Erwin WM, Jackson PC, Homonko DA. Innervation of the human costovertebral joint: implications for clinical back pain syndromes. J Manipulative Physiol Ther. 2000;23(6):395–403.
7. Royal Australian College of General Practitioners (RACGP). Guidelines for preventive activities in general practice. 9th edn. Melbourne: RACGP; 2018 (updated 2023).
8. Hirsch JA, Singh V, Falco FJE, et al. Thoracic facet joint interventions. Pain Physician. 2016;19(4):E581–E593.
9. Erwin WM, Jackson PC. The costovertebral joint: anatomy, biomechanics, and clinical significance in thoracic back pain syndromes. J Can Chiropr Assoc. 2003;47(2):112–120.
10. Strayer RJ, Gunnerson JM, Brown LH, et al. Aortic dissection: clinical features, diagnosis, and management. Aust Crit Care. 2019;32(2):144–153.
11. Ombregt L. A system of orthopaedic medicine. 3rd edn. Edinburgh: Churchill Livingstone Elsevier; 2013. Chapter 18: Thoracic spine.
12. Lin CC, Chen KH, Li DM, et al. Characteristics and outcomes of patients presenting with thoracic back pain to the emergency department. Emerg Med Australas. 2020;32(5):805–811.

for PBS-listed medicines at participating pharmacies.

Cultural safety

Engagement with Aboriginal Community Controlled Health Organisations (ACCHOs) is essential. Cultural safety training for non-Indigenous clinicians, use of Aboriginal Health Workers and Liaison Officers, and incorporation of traditional healing practices alongside Western medicine improve treatment adherence and outcomes. Avoidance of eye contact, respect for gender-sensitive examination practices, and understanding of sorry business protocols are critical elements of culturally safe care.

Medication adherence

Complex DMARD regimens with frequent monitoring requirements present adherence challenges. Long-acting depot injections (e.g., methotrexate SC) may improve adherence compared to oral regimens. Community pharmacy partnerships through the Indigenous Pharmacy Programmes improve medication management.

Specific conditions

Rheumatic heart disease (RHD) requires secondary prophylaxis with benzathine penicillin G (BPG) 1.2 MU IM every 3–4 weeks for a minimum of 10 years or until age 21 (whichever is longer). RHD registers (e.g., NT RHD Register) facilitate recall and follow-up. The Australian RHD Endgame Strategy targets elimination by 2031.

Referral pathways

Referral through ACCHOs and Aboriginal Hospital Liaison Officers (AHLOs) improves engagement. The Specialist Outreach Assistance Programme provides funded specialist visits to remote communities. NT, WA, and QLD have specific rheumatology outreach programmes targeting Indigenous communities.

📚 References

1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).

for PBS-listed medicines at participating pharmacies.

Cultural safety

Medication adherence

Specific conditions

Referral pathways

📚 References

1. Australian Institute of Health and Welfare (AIHW). Autoimmune disease in Australia. Cat. no. PHE 312. Canberra: AIHW; 2023.
2. Fraenkel L, Bathon JM, England BR, et al. 2021 American College of Rheumatology guideline for the treatment of rheumatoid arthritis. Arthritis Care Res. 2021;73(7):924–939.
3. Fanouriakis A, Kostopoulou M, Alber K, et al. 2019 update of the EULAR recommendations for the management of systemic lupus erythematosus. Ann Rheum Dis. 2019;78(6):736–745.
4. Chung SA, Langford CA, Maz M, et al. 2021 American College of Rheumatology/Vasculitis Foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Care Res. 2021;73(11):1583–1599.
5. Smolen JS, Landewé RBM, Bijlsma JWJ, et al. EULAR recommendations for the management of rheumatoid arthritis with synthetic and biological disease-modifying antirheumatic drugs: 2022 update. Ann Rheum Dis. 2023;82(1):3–18.
6. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook. Australian Government Department of Health; 2024. Available from: immunisationhandbook.health.gov.au.
7. Rheumatic Heart Disease Australia (RHDAustralia). The 2020 Australian guideline for prevention, diagnosis, and management of acute rheumatic fever and rheumatic heart disease. 3rd ed. Darwin: Menzies School of Health Research; 2020.
8. Pharmaceutical Benefits Scheme (PBS). PBS Schedule. Australian Government Department of Health. Available from: pbs.gov.au. Accessed 2024.
9. Agarwal S, Cunnington J, Nossent J. Autoimmune disease in Indigenous Australians: a systematic review. Int J Rheum Dis. 2021;24(12):1487–1498.
10. Pisetsky DS. Antinuclear antibody testing — misunderstood or misused? Clin Immunol. 2023;255:109717.
11. Bertsias GK, Tektonidou M, Amoura Z, et al. Joint European League Against Rheumatism and European Renal Association–European Dialysis and Transplant Association (EULAR/ERA-EDTA) recommendations for the management of adult and paediatric lupus nephritis. Ann Rheum Dis. 2012;71(11):1771–1782.
12. Ledingham J, Deighton C; British Society for Rheumatology Standards, Audit and Guidelines Working Group. Update on the British Society for Rheumatology guidelines for prescribing TNFα blockers in adults with rheumatoid arthritis. Rheumatology. 2005;44(2):155–158.
13. National Health and Medical Research Council (NHMRC). National statement on ethical conduct in human research. Canberra: NHMRC; 2023 (updated).