Letter to the Editor


9 February 2009

Dear Editor,


Immune thrombocytopenic purpura (ITP) is an autoimmune disorder characterised by a low platelet count caused by the destruction of antibody-sensitised platelets in the reticuloendothelial system, such as the spleen.1 Overall, the key element is the loss of self-tolerance, leading to the production of autoantibodies directed against platelet antigens. In children with ITP, the clinically important subgroup is those who have platelet counts less than or equal to 20 × 109/L and require ongoing platelet-enhancing therapy because of bleeding symptoms. In these patients, second-line therapies are indicated. Laparoscopic splenectomy has now been accepted as an effective treatment with minimal risk of serious complications in expert hands.

Rarely, ITP can be complicated by intracranial haemorrhage, and this usually occurs in the setting of extremely low platelet counts of <10 × 109/L.2 Although rare, ITP is associated with mortality rates of approximately 50%, while among the survivors, neurological deficits are common. Emergent splenectomy can result in immediate improvement in platelet counts in approximately 80% of patients.3 Nevertheless, this procedure carries the risk of perioperative bleeding, especially in haemodynamically unstable patients. Here, we present the successful use of splenic artery embolisation as a temporising measure before laparoscopic splenectomy in a patient who has chemo-resistant ITP and presented with intracranial haemorrhage.

A 12-year-old boy with a previous history of diffuse large B cell lymphoma, in remission first presented with a two-month history of increased generalised petechiae and gum bleeding. He was diagnosed with ITP after laboratory investigations. However, treatment with prednisolone, cyclosporine, intravenous immunoglobulins and rituximab all failed to increase his platelet count. Subsequently, he was admitted as an emergency with sudden onset of frontal headache and altered consciousness. Physical examination showed a reduced Glasgow Coma Score of 12/15, right upper limb weakness, signs of meningeal irritation and aphasia. Computed tomography of the brain showed a crescent rim of extra-axial collection causing mild inward displacement of the cerebral hemisphere over the left frontal and temporoparietal region, suggesting subacute haematoma (Fig. 1). Platelet count at admission was 7 × 109/L.

Figure 1.

An axial computed tomography of the brain showing intracranial haemorrhage in the left parietal/occipital area (arrow).

In view of the serious nature of the patient's symptoms and the very low platelet count, we decided to proceed with splenic artery embolisation on the day of admission with a combination of stainless steel coils and polyvinyl alcohol particles (Fig. 2). The boy's platelet count rose rapidly the following day to above 100 × 109/L. Laparoscopic splenectomy was performed two days later with no complications. No blood products were transfused intra- or perioperatively. Platelet count continued to rise to 226 × 109/L, two days after the operation. The patient made a full neurological recovery and was discharged seven days after his operation. At last review, his platelet count was 380 × 109/L, and the patient was taken off all medication.

Figure 2.

A fluoroscopic image taken after splenic artery embolisation with coils. The celiac artery is highlighted with contrast.

In patients with acute ITP, splenectomy is indicated in those with bleeding symptoms with platelet counts of less than 20 × 109/L.4 Laparoscopic splenectomy is currently the preferred method for the surgical management of haematologically related splenic disorders as it has been shown to result in faster recovery periods and reduced post-operative pain.5 However, haemorrhagic complications remain a concern for this procedure given the spleen's rich vascularity and manoeuvres required for the operation.6,7 The risk of such events occurring may be increased in patients with very low platelet counts. Splenic artery embolisation has previously been used to treat various conditions, which include chronic ITP, hereditary spherocytosis, splenic trauma in haemodynamically unstable patients, and also in patients with liver cirrhosis with hypersplenism.8–11 It has been demonstrated to result in significantly reduced blood loss during subsequent laparoscopic splenectomy compared with patients who received laparoscopic splenectomy as a sole treatment.12 In our patient, we carried out complete splenic artery embolisation in an acute setting, with splenectomy in a staged fashion, with both procedures yielding a considerable improvement in platelet count. The rationale for performing complete embolisation was because of the unlikelihood of success for partial embolisation in our patient, due to the refractory nature of his disease. Splenectomy after embolisation is required to reduce the chances of developing post-infarction syndrome (fever, left upper quadrant pain, risk of infection and abscess formation). Other potential complications associated with embolisation are related to migration or inappropriate placement of embolic material. Although it has recently been suggested that splenic artery embolisation preceding laparoscopic splenectomy should not be carried out routinely as appropriate use of blood products and correct surgical technique will be adequate in controlling blood loss,13 in this patient, an extremely low platelet count and intracranial bleeding necessitated immediate intervention. As emergent splenectomy had a significantly higher risk of haemorrhagic complications, splenic artery embolisation could provide a temporary bridge before splenectomy. We therefore propose that in the treatment of children with life-threatening acute ITP, splenic artery embolisation preceding laparoscopic splenectomy may be a viable method of stabilisation.