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We present the case of an 8-year-old girl with chronic idiopathic thrombocytopenic purpura (ITP) and a short history suggestive of raised intracranial pressure. Urgent computed tomography scan of the head showed a large bleed into a left cerebellar lesion. She underwent treatment with intravenous immunoglobulin (IVIg) and steroids to increase her platelet count, followed by excision of the lesion, which was found to be a benign pilocytic astrocytoma. The patient made a complete recovery and shortly afterwards underwent splenectomy, following which there was complete resolution of her thrombocytopenia.
There have been a number of case reports documenting the association between solid tumours and idiopathic thrombocytopenic purpura (ITP) in adults, some even suggesting a causal relationship. This is the first report in a child of bleeding into an intracranial tumour secondary to ITP. This case illustrates the importance of looking for dual pathology when a patient with a known chronic condition presents with new symptoms. In particular, for children with bleeding tendencies who present with an acute neurological deficit, there could be a danger of assuming the bleeding was solely due to spontaneous haemorrhage. Our case highlights the necessity of detailed intracranial imaging in such situations.
An 8-year-old girl, with a long history of ITP, presented as an emergency with a 5 week history of worsening left sided headache. During the 6 days before admission she had also developed vomiting and was unsteady on her feet, veering to the right. Earlier on the day of presentation she had developed vertical diplopia in all directions of gaze. Notable findings on examination were that her left eye was medially deviated, though with a full range of movement, and papilloedema was noted on fundoscopy. Coordination of her left arm and leg was impaired, with past-pointing and mild ataxia, but the remainder of her neurological examination was normal.
The patient had initially been diagnosed with ITP aged 18 months when she presented with excessive bruising and petechiae. Platelet count ranged on average between 8–20×109/l with no spontaneous remission since diagnosis. Since diagnosis she had suffered sporadically from nose bleeds, some requiring admission though none severe, and occasionally required intravenous immunoglobulin (IVIg) and oral steroid treatment as well as tranexamic acid to reduce mucosal bleeding. Bone marrow aspirate showed increased megakaryocytes, consistent with peripheral destruction, but was otherwise normal; other parameters were also persistently normal. After assessment by two paediatric haematologists it was decided that splenectomy should not be pursued.
Shortly after admission she underwent an urgent computed tomography (CT) scan of the head, which showed a large left cerebellar bleed and raised the suspicion of an underlying lesion. This was further investigated by magnetic resonance imaging (MRI) (fig 1), which revealed a 5.4 × 3.6 cm mass lesion in the left cerebellar hemisphere, with associated mass effect and hydrocephalus.
On this admission her initial platelet count was 5×109/l. The patient was treated with 2×0.8g/kg IVIg and oral steroids (40 mg/day prednisolone initially, later changed to dexamethasone to reduce peritumoural oedema), and after 2 days her platelet count had risen to 112×109/l—sufficient to allow excision of the cerebellar mass. At operation (posterior fossa craniectomy) the tumour was macroscopically excised, and confirmed on histology as World Health Organization grade I pilocytic astrocytoma (fig 2).
A postoperative MRI showed complete resection of the tumour and haematoma (fig 3), and her postoperative recovery was smooth. The patient was discharged 7 days after the operation, with no residual neurological deficit. Six months later she underwent laparoscopic splenectomy, following which there was complete remission of the ITP with a platelet count 6 months following this of 357×109/l.
Intracranial bleeds secondary to immune thrombocytopenic purpura in children are very rare, occurring in only 0.1–0.5% of children diagnosed with ITP.1 This is the first report in the literature of intracranial bleeding into a solid tumour in a child with ITP. Astrocytomas account for 50% of paediatric primary central nervous system tumours, and of these pilocytic astrocytomas are the most common type.2
Juvenile pilocytic astrocytomas are usually benign, with transformation to a high grade malignant tumour being rare. In this case the tumour was probably beginning to become symptomatic, causing hydrocephalus and papilloedema, but the bleed was the final precipitating event that caused her to come to medical attention.
There are reports in the literature of solid tumours in adults precipitating the development of ITP,3 or of ITP improving after tumour resection.4 In this case there is no evidence that the two conditions are related in causal terms, although as is seen in the case report they impact strongly on each other’s management. It would not be possible to determine which of the two conditions arose first, but the platelet count was not affected in the long run by the removal of the tumour.
When a child with ITP presents with features suggestive of an intracranial bleed it may be assumed to be a spontaneous bleed due only to the thrombocytopenia. This case illustrates the importance of arranging detailed intracranial imaging in such a case to look for other underlying causes, which may require separate management. We recommend MRI with contrast administration, if necessary repeated when the blood products have cleared, as the cost of missing an expanding intracranial mass lesion could be very high.
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.