Spontaneously Acquired Factor VIII Inhibitor in a Non-Haemophiliac Child

Abstract

Observations were made of the blood and bone marrow of a male infant with Down’s syndrome during the first year of life. At 4 days of age there were 36,500 myeloblasts/mm3 in the blood and 10.8% myeloblasts in the marrow. Initially it appeared the patient had acute myeloblastic leukemia. However, the clinical course and kinetic and electron microscopic studies of his bone marrow cell population indicate he did not have acute leukemia.

Case Details

Personal Characteristics

A three-year-old girl WHO had for two months suffered bruising after minimal injury

Clinical Characteristics

Diffuse ecchymoses and a large haematoma hindering elbow movement

Remission Characteristics

Although the inhibitor still persisted at high levels after two years, no further haemorrhage occurred, excepted haematomas three months after the onset of symptoms, in association with mumps

Treatment & Mechanisms

Proposed Remission Mechanisms

Not discussed

Additional Notes

No definite etiology was evident despite repeated immunological investigation. The patients and the family history were negative for autoimmune disorders or haemorrhagic diathesis. There had been no exposure to toxins or drugs except penicillin, given four months before. On admission, clinical examination showed extensive ecchymoses on the limbs, small bruises over the trunk and a large subcutaneous haematoma on one elbow. There was no lymphadenopathy or hepatosplenomegaly, nor fever. The large subcutaneous haematoma resolved after a week. The child has been followed for two years. No other bruising or bleeding has occurred.