Acquired Hemophilia Differential Diagnoses

  • Author: Sara J Grethlein, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
Updated: Mar 24, 2016

Diagnostic Considerations

Diagnosis of acquired hemophilia can be difficult, both because the condition is rare and because the patient does not have the usual personal or family history of bleeding episodes, such as is seen in congenital hemophilia.[1] Moreover, the clinical signs and symptoms of acquired hemophilia differ from those of hereditary hemophilia.

In addition to the differentials (see below), conditions to be considered include the following:

  • Heparin administration
  • Lupus anticoagulant

Please see the following for more information:

  • Hemophilia A
  • Hemophilia B
  • Hemophilia C

Differential Diagnoses

Contributor Information and Disclosures

Sara J Grethlein, MD Associate Dean for Undergraduate Medical Education, Indiana University School of Medicine

Sara J Grethlein, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American Society of Hematology, American Society of Clinical Oncology

Disclosure: Nothing to disclose.


Craig M Kessler, MD MACP, Professor, Department of Medicine and Pathology, Division of Hematology/Oncology, Georgetown University School of Medicine; Director, Clinical Coagulation Laboratory, Lombardi Comprehensive Cancer Center, Georgetown University Hospital

Disclosure: Received honoraria from NovoNordisk for consulting; Received grant/research funds from NovoNordisk for other; Received honoraria from Baxter-Immuno for consulting; Received honoraria from Octapharma for speaking and teaching; Received grant/research funds from Octapharma for none; Received consulting fee from Amgen for consulting; Received honoraria from Bayer for review panel membership.

Chief Editor

Srikanth Nagalla, MBBS, MS, FACP Associate Professor of Medicine, Division of Hematology and Oncology, UT Southwestern Medical Center

Srikanth Nagalla, MBBS, MS, FACP is a member of the following medical societies: American Society of Hematology, Association of Specialty Professors

Disclosure: Nothing to disclose.


Marcel E Conrad, MD Distinguished Professor of Medicine (Retired), University of South Alabama College of Medicine

Marcel E Conrad, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association of Blood Banks, American Chemical Society, American College of Physicians, American Physiological Society, American Society for Clinical Investigation, American Society of Hematology, Association of American Physicians, Association of Military Surgeons of the US, International Society of Hematology, Society for Experimental Biology and Medicine, and Southwest Oncology Group

Disclosure: No financial interests None None

Pradyumna D Phatak, MBBS, MD Chair, Division of Hematology and Medical Oncology, Rochester General Hospital; Clinical Professor of Oncology, Roswell Park Cancer Institute

Pradyumna D Phatak, MBBS, MD, is a member of the following medical societies: American Society of Hematology

Disclosure: Novartis Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

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Clinical presentation of acquired hemophilia.
Sites of bleeding in patients with acquired hemophilia (n = 149). This research was originally published in Blood. Collins PW, Hirsch S, Baglin TP, et al. 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):1870-7. © American Society of Hematology.
Workup for acquired hemophilia.
Management of bleeding in acquired hemophilia.
Eradication of the inhibitor for acquired hemophilia.
Table 1 below illustrates the frequency of underlying diagnoses in 3 cohort studies of patients with acquired hemophilia A.[10, 13, 14, 15, 16]
Disease AssociationGreen 1981 (N = 215), %Morrison 1993 (N = 65), %Collins 2007 (N = 172), %
Collagen, vascular, and other autoimmune diseases18.017.016.7
Skin diseases4.52.03.3
Possible drug reaction5.63.0NR
*In this trial, idiopathic and other were combined.

NR—not reported.

Table 2. Acquired Bleeding Disorders Associated With Inhibitors of Factors Other Than FVIII
Coagulation Factor InhibitedMost Commonly Associated DisordersTreatment
VLymphoproliferative disorders, adenocarcinoma, tuberculosis, aminoglycosides, topical thrombinFFP, rFVIIa
IXSystemic lupus erythematosus, acute rheumatic fever, hepatitis, collagen vascular diseases, multiple sclerosis, postprostatectomy, and postpartumFIX concentrates, APCCs, rFVIIa, corticosteroids
XIAutoimmune diseases, prostate carcinoma, chronic lymphocytic leukemia, chlorpromazineFFP, FXI concentrates, rFVIIa, tranexamic acid, fibrin glue
XIIIIdiopathic, isoniazid, penicillinFXIII concentrate, FFP, stored plasma, cryoprecipitate
VWF‡Autoimmune disorders, monoclonal gammopathies, lymphoproliferative diseases, epidermoid malignancies, hypothyroidism, myeloproliferative disorders, and certain medicationsDesmopressin, infusion of FVIII that contains vWF, IVIG, plasma exchange
IITopical thrombin, idiopathic, autoimmune diseases, procainamideAPCC, FFP
VIIBronchogenic carcinoma, idiopathicFIX concentrates, APCC, FVIII concentrates, rFVIIa, fibrin glue, tranexamic acid
XAmyloidosis, carcinoma, acute nonlymphocytic leukemia, acute respiratory infections, fungicide exposure, idiopathicAPCC, tranexamic acid, fibrin glue, FFP
APCC—activated prothrombin complex concentrate; FFP—fresh frozen plasma; IVIG—intravenous immunoglobulin; vWF—von Willebrand factor.
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