Bleeding disorders

Keywords: Coagulation disorders

Causes

Appropriate Tests

Causes of acquired bleeding disorders

Investigation is guided by clinical and family history and examination. There are published bleeding assessment tools available (see below). Relevant information from clinical history includes:

  • Personal bleeding history (site and nature of previous bleeding, age of onset of symptoms, spontaneous or excessive bleeding following trauma, surgery, dental extractions or post-partum)
  • Interventions to stop bleeding or treat anaemia
  • Medication history (including antiplatelet agents, anticoagulants, complementary medicine)
  • Medical history (eg, liver, renal disease, haematological disease, thrombocytopenia, thrombocytosis)
  • Family history of bleeding disorder or undiagnosed excessive bleeding

Initial investigations if positive personal or family history:

Full blood count and Blood film (Platelet count, platelet morphology, evidence of Anaemia or Iron deficiency), Prothrombin time / INR, APTT, Blood film, Fibrinogen, Thrombin time.  

Additional laboratory testing may be indicated if personal or family bleeding history is strong, including testing for VWD and FVIII.

Normal initial results do not absolutely exclude the possibility of a clinically significant inherited bleeding disorder. Further investigation will be guided by initial laboratory results and clinical and family history – Specialist advice may be appropriate.

See also Bruising, Purpura, Fibrinolysis.

  • Advanced and/or metastatic carcinoma

 

  • Massive transfusion

See Blood transfusion.

  • DIC

Fibrinogen, D Dimer

See Disseminated intravascular coagulation.

  • Drug-related bleeding, especially
 
  • Anticoagulant therapy

See Anticoagulant monitoring.

  • Fibrinolytic therapy

See Thrombolytic therapy (Thrombolysis).

  • Aspirin, other NSAID, COX-2 inhibitors

Predictable abnormality of bleeding time and platelet aggregation; these tests do not predict the risk of bleeding and are not indicated.

  • Thrombocytopenia

 

  • Liver disease

See Bleeding under Cirrhosis - consequences.

  • Myeloproliferative neoplasms, especially Essential thrombocythaemia and Primary myelofibrosis

 

  • Neonatal alloimmune thrombocytopenia

Platelet antigen testing

  • Obstetric (peripartum) bleeding

INR / PT, APTT, Fibrinogen, Full blood count

See under Pregnancy.

  • Renal failure

See Bleeding under Renal failure - chronic (complications).

  • Rodenticide poisoning

See under Poisoning.

  • SLE

 

  • Paraproteinaemia, Plasma cell myeloma, Waldenström's macroglobulinaemia / Lymphoplasmacytic lymphoma

 

  • Renal failure

See Bleeding under Renal failure - chronic (complications).

  • Rodenticide poisoning

See under Poisoning.

  • SLE
 
  • Thrombocytopenia
 

Inherited bleeding disorders

 

  • von Willebrand disease

 

  • Haemophilia A

See under Haemophilia.

  • Haemophilia B

See under Haemophilia.

  • Factor XI deficiency
Coagulation factors (XI) assay.
  • Factor XII deficiency

Is associated with prolonged APTT that corrects when mixed with normal plasma. Coagulation factors (XII) assay indicates low level, however this deficiency is not associated with an increased risk of bleeding.

  • Factor XIII deficiency

Factor XIII screening test; consult pathologist.

  • Hypofibrinogenaemia/ afibrinogenaemia

Thrombin time. Fibrinogen assay.

  • Dysfibrinogenaemia

Thrombin time. Reptilase time; consult pathologist.

Inherited disorders of platelet function, including

A Platelet function analyser (PFA) may suggest a platelet function disorder. This should be confirmed by formal platelet aggregation studies (Platelet aggregometry). Consult pathologist.

  • Release disorders, eg, Storage pool disease

Platelet aggregometry. For definitive platelet membrane, granule, and prostaglandin studies - consult pathologist.

  • Bernard Soulier syndrome

Full blood count, Blood film

 
  • Hereditary haemorrhagic telangiectasia

Clinical diagnosis. Mucocutaneous telangiectasia with recurrent bleeding from mucosal surfaces. See also Telangiectases.

References:

Rodeghiero F et al. on behalf of the ISTH/SSC Joint VWF and Perinatal/Pediatric Hemostasis Subcommittees Working Group. 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: 2063-2065.

National Heart, Lung, and Blood Institute and National Institutes of Health. The Diagnosis, Evaluation, and Management of von Willebrand Disease, 2007.