Janaan heath case report of the week 3 November 2016

A 86 years old lady was brought in to the emergency department by ambulance having complaining of the right leg swelling and lower abdominal bruising for 3 days.

She was conscious with GCS 15/15, Blood pressure 100/80 mmhg, pulse 100 sinus, ECG NAD, saturations 100% ambient air

Haemodynamically stable in ED

Examination by ED registrar revealed

Swelling of the right leg up to the mid thigh, very painful to touch with limited movements due to pain

She also had left flank bruising.

Rest of the system examinations were unremarkable.

An initial diagnosis of right leg DVT were

She was given a stat dose of LMWH Dalteparin 17000unit sc and an urgent US Doppler was requested,

She had blood tests

HB 120 WCC 5.6 PLT 340, neutrophils4.5 lymphocytes2.8, LFTs normal, Creatinine 120, urea 12.0, calcium 2.36

APTT 120, PT 12, Fibrinogen 5.9,

Thrombin time 20,

D-dimmer :normal.

Laboratory contacted the responsible medical team on call and

Her case was then discussed with haematologist registrar in her first year of training

Following the discussion with attending consultant haematologist the patient was seen immediately by haematology team

A clinical diagnosis of acquired haemophilia was made.

Patient was resuscitated with fluid resuscitations, adequate analgesia

Secondary investigations warranted immediately, includes

APTT 50/50 mixing study

Factor 8 assay

Inhibitor assay

Patient was given bypassing agent FEIBA (activated PCC) 50unit/kg stat and BD

Further detailed history and CT chest neck abdomen and pelvis revealed no significant abnormalities

Auto antibodies were negative

Viral serology were all negative

The clinical diagnosis of Acquired haemophilia was confirmed by inhibitor assay (Bethesda inhibitor assay)

Factor 8 level was undetectable

Learning points:

  1. A) Acquired haemophilia is a very rare condition
  2. B) 2.5 patients per million populations
  3. C) High degree of clinical suspicion is needed
  4. D) Elderly patients with unilateral leg swelling and bruising needs careful consideration before LMWH is given for suspected clinical DVT
  5. E) Early haematology expert opinion is crucial
  6. F) 30 % patients have underlying malignancy and or auto immune condition hence screening is essential
  7. G) Patients must be treated in a comprehensive care centre
  8. H) There is a role of immunosuppressive usually steroid and cyclophosphamide, occasionally Rituximab is used in relapsed setting.


Dr Amin Islam MRCP, FRCPath

London, 19th November 2016