Factor X deficiency is usually inherited.1 Of the acquired forms some are transient, possibly associated with infection, and, in these, spontaneous intracerebral haemorrhage does not seem to have been reported.
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Copyright © 2003, The Royal Society of Medicine Intracerebral haemorrhage in an adult due to transient factor X deficiency Neurosurgery Unit, King's College Hospital, London, UK Correspondence to: Dr T M Young, Flat 6, Monroe House, 7 Lorne Close, London NW8 7JN, UK E-mail: dtimyoung/at/hotmail.com Factor X deficiency is usually inherited.1 Of the acquired forms some are transient, possibly associated with infection, and, in these, spontaneous intracerebral haemorrhage does not seem to have been reported. CASE HISTORY A previously well man of 66 attended his local accident and emergency unit after two weeks of fevers and two days of apparent confusion. His general practitioner had started him on erythromycin a week previously for a presumed chest infection (he was penicillin allergic). On examination the chest was clear clinically and radiologically. When he proved to have frank haematuria a urinary tract infection was suspected, but the midstream urine revealed no organisms and produced no growth. Blood cultures were also negative. The international normalized ratio (INR) was high at 5.4 (normal 0.9–1.2) and the activated partial thromboplastin time (APTT) ratio was 2.6 (0.9–1.2). The platelet count was within the normal range at 3426109/L. A value of 0.2 mg/mL for latex D-dimers (normal 50.5) excluded disseminated intravascular coagulopathy. Liver function was normal. There was no personal or family history of bleeding diathesis and he had never taken warfarin. A 50/50 mix of the patient's plasma with normal plasma corrected the clotting indices to INR 1.7, APTT ratio 1.2. Coagulation factor assays (in iU/dL, normal range for all 50–200) were: factor II, 37; factor V, 38; factor VII, 48; factor VIII 158; factor IX, 147; factor X, 3. Thus factor X was very low, with factors II, V and VII just below normal. Acquired factor X deficiency was diagnosed and the patient was treated with 10 mg intravenous vitamin K, 15 mL/kg of fresh frozen plasma, and 3180 iU factor IX concentrate (HIPFIX-SNBTS), which also contains factor X. His factor X rose to 26 iU/dL. After a further dose of 3000 iU factor IX concentrate the INR was 1.9, APTT ratio 1.6, and factor X 52 iU/dL. Over the two days from presentation the patient showed worsening expressive dysphasia, and CT of the brain revealed a moderate-sized left (dominant) hemisphere intracerebral haemorrhage without significant midline shift. By the time of transfer to the neurosurgical unit he was almost aphasic. Since he was fully alert, initial management was conservative, while his clotting was further corrected with coagulation factor infusions. After 5 days on the unit, factor X was 51 iU/dL and INR 1.29. Nevertheless, the patient's level of consciousness dropped rapidly on day five and repeat CT (Figure 1  ) showed extension of the original haemorrhage, this time with midline shift. The haematoma was evacuated the same day and the patient made an excellent recovery, becoming fully alert and mobile with only mild residual expressive dysphasia. Throughout the recovery phase his INR, APTT ratios and factor X concentrations remained normal without further infusions.
COMMENT The cause of the factor X deficiency in this case is uncertain. Amyloidosis2,3 seems unlikely both on clinical grounds and because the deficiency proved to be reversible. Might an infection have caused it? Although the initial chest X-ray was clear and the blood/urine cultures were negative, the patient had just completed a course of erythromycin for suspected upper respiratory tract infection. This antibiotic might have cured an atypical pneumonia (such as mycoplasma pneumonia) of a kind associated with some other cases of transient acquired factor X deficiency.4,5 However, convalescent serology for mycoplasma and other atypical pneumonia showed no evidence of recent infection by such agents. A final possible culprit was the antibiotic.5 One other case of transient factor X deficiency has been reported after a course of erythromycin, in a woman of 67 treated for a presumed chest infection. But this patient, unlike ours, displayed inhibitor activity against factor X. References 1. Sandler E, Gross S. Prevention of recurrent intracranial hemorrhage in a factor X deficient infant. Am J Pediatr Hematol Oncol 1992;14: 163-5 [PubMed] 2. Zeitland KD, Blatt PM. Amyloidosis and factor X deficiency. Southern Med J 1982;75: 306-8. 3. Greipp PR, Kyle RA, Bowie EJ. Factor X deficiency in amyloidosis: a critical review. Am J Hematol 1981;11: 443-50 [PubMed] 4. Currie MS, Stein AM, Rustagi PK, et al. Transient acquired factor X deficiency associated with pneumonia. N Y State J Med 1984;84: 572-3 [PubMed] 5. Pescher FV, van Aken WG, van Mourik JA, et al. Acquired transient factor X (Stuart factor) deficiency in patients with mycoplasma pneumonial infection. Scand J Haematol 1979;23: 257-64 [PubMed] 6. Mulhare PE, Tracy PB, Golden EA, Branda RF, Bovill EG. A case of acquired factor X deficiency with in vivo and in vitro evidence of inhibitor activity directed against factor X. Am J Clin Pathol 1991;96: 196-200 [PubMed] |
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Am J Pediatr Hematol Oncol. 1992 May; 14(2):163-5.
[Am J Pediatr Hematol Oncol. 1992]Am J Hematol. 1981 Dec; 11(4):443-50.
[Am J Hematol. 1981]N Y State J Med. 1984 Nov; 84(11):572-3.
[N Y State J Med. 1984]Scand J Haematol. 1979 Oct; 23(4):257-64.
[Scand J Haematol. 1979]