A rare case of Immune Thrombocytopenic Purpura

An eleven year old girl presented to the emergency, Colombo South Teaching Hospital with acute onset headache and weakness of left upper limb. Parents recalled a trivial fall two days prior to admission. Level of consciousness deteriorated rapidly with left sided focal fits. She was electively intubated. Cranial imaging revealed acute right sided intra-cerebral hemorrhage. Investigations revealed a haemoglobin of 5.9g/dl and platelets of 8000. She received numerous transfusions of blood and platelets, IV immunoglobulin and Methyl-prednisolone. She was transferred to Neurosurgical Unit – National Hospital. She passed away 24-hours later due to uncontrollable intracranial hemorrhage. She was diagnosed with Immune Thrombocytopaenic Purpura (ITP) 3 years back, when she presented with ecchymotic patches. During her initial presentation, she developed severe headache and was found to have bilateral hematomas of right & left fronto-parietal regions. She received IV Immunoglobulin and was started on long term Prednisolone. She was followed up at Paediatric clinic as well as Haematology clinic. She continued to have persistently low platelets while on low dose Prednisolone without any significant bleeding manifestations. ITP is a bleeding disorder due to immune mediated platelet destruction. Autoantibodies are formed against platelet membrane antigens. Antibody coated platelets are sequestrated in the spleen and phagocytized by mononuclear macrophages. Antibody formation is usually triggered by viral infections. Diagnosis is established by excluding other causes of thrombocytopaenia such as myelo-infiltrative disorders, auto immune disorders and other causes. 20% of patients progress to chronic ITP. Intracranial bleeding is extremely rare even at very low platelet levels.