Diagnosing acquired hemophilia

When unexplained bleeding with no previous history occurs, urgent laboratory testing to aid in diagnosis is recommended. Approximately 70% of acquired hemophilia (AH) patients experience severe, unexplained bleeding at diagnosis, with widely varying bleeding patterns.¹

Diagnosing acquired hemophilia

When unexplained bleeding with no previous history occurs, urgent laboratory testing to aid in diagnosis is recommended. Approximately 70% of acquired hemophilia (AH) patients experience severe, unexplained bleeding at diagnosis, with widely varying bleeding patterns.¹

How do patients with AH present?

Acquired hemophilia signs and symptoms

Purpura (bruising) and soft-tissue hemorrhage²

Bleeding sites atypical of congenital hemophilia³

Gastrointestinal, urological, or retroperitoneal bleeding^4,5

Prolonged bleeding following surgery²

Compartment syndrome (compression of blood vessels and nerves)³

Isolated prolonged activated partial thromboplastin time (aPTT)²

Purpura (bruising) and soft-tissue hemorrhage²

Bleeding sites atypical of congenital hemophilia³

Gastrointestinal, urological, or retroperitoneal bleeding^4,5

Prolonged bleeding following surgery²

Compartment syndrome (compression of blood vessels and nerves)³

Isolated prolonged activated partial thromboplastin time (aPTT)²

Significant dark bruising along patient’s chest and abdomen

Signficant dark bruising along patient’s back and left hip

Importance of early diagnosis

Patients frequently experience delays in diagnosis for acquired hemophilia. Prompt diagnosis is vital to minimize a patient’s risk of bleeding complications and avoid non-essential invasive procedures.²

Parameter

Normal
range^7,a

Normal range^6,a

Acquired
hemophilia⁶

Acquired
hemophilia³

aPTT

25-38
seconds

25-38 seconds

Above
normal

Above normal

PT

11-13
seconds

11-13 seconds

Normal

FVIII

50%-150%
activity

50%-150% activity

Below
normal

Below normal

aPTT=activated partial thromboplastin time; PT=prothrombin time.
^aNormal ranges may vary among different laboratories.

Consult a hematologist immediately if lab results show an unexplained, isolated, prolonged aPTT.^3,4,7

Consult a hematologist immediately if lab results show an unexplained, isolated, prolonged aPTT.^4,6

Confirm an acquired hemophilia diagnosis through lab testing

Explore an interactive clinical pathway for patients with acquired hemophilia

Review an example of treating acquired hemophilia in the emergency department.

Go to clinical pathway

References:

Knoebl P, Marco P, Baudo F, et al; EACH2 Registry Contributors. Demographic and clinical data in acquired hemophilia A: results from the European Acquired Haemophilia Registry (EACH2). J Thromb Haemost. 2012;10(4):622-631.
Collins PW, Percy CL. Advances in the understanding of acquired haemophilia A: implications for clinical practice. Br J Haematol. 2010;148(2):183-194.
Giangrande P. Acquired Hemophilia: Revised Edition: Treatment of Hemophilia No. 38. Montréal, Québec: World Federation of Hemophilia; 2012:1-2.
Tiede A, Collins P, Knoebl P, et al. International recommendations on the diagnosis and treatment of acquired hemophilia A. Haematologica. 2020;105(7):1-16.
Collins PW, Hirsch S, Baglin TP, et al; for UK Haemophilia Centre Doctors’ Organisation. Acquired hemophilia A in the United Kingdom: a 2-year national surveillance study by the United Kingdom Haemophilia Centre Doctors’ Organisation. Blood. 2007;109(5):1870-1877.
Introduction to normal values (reference ranges). In: Wallach J, ed. Interpretation of Diagnostic Tests. 8th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2007:3-25.