Immune thrombocytopenia (ITP) presents very variably. Petechiae, mucous membrane bleeding, and GI/CNS haemorrhages have all been reported as presentations of the disease.
In children with ITP 80% experience a complete sustained remission within a few weeks or months of initial presentation, irrespective of any therapy given, although at the time of presentation bleeding manifestations are most marked and can be quite alarming.
In adults the disease is more chronic and in many there may be few symptoms or signs of bleeding. Not all require treatment, but complete remissions are rare without some form of therapy. The major concern in both children and adult groups is the small but finite (0.1-0.9%) risk of intracranial haemorrhage (ICH), which occurs in general when the platelet counts are very low (< 10,000/mm3 or 10 x 109/l).
ICH is usually intraparenchymal, i.e. into the substance of the brain, and usually presents with a relatively rapid onset. With older patients it may take the form of a subdural haemorrhage, which is on the surface of the brain. Although the latter may be less serious, it often presents slowly and insidiously, may be difficult to recognise and may often be associated with little more than minor mental clouding.
The risk of ICH tends to be mainly in patients with very low counts. Nevertheless, bleeding may occur at higher platelet levels if the patient has any other condition that may increase the bleeding risk, or is taking drugs or medications that affect platelet function.
Aspirin and the non-steroidal anti-inflammatory drugs (e.g. Nurofen) are the ones most commonly associated with damaging platelets, but the possibility should always be considered for any drug offered.
Any drug that can lower the platelet count should be used with caution in thrombocytopenia. Where treatment is necessary for another condition this needs to be closely monitored. Numerous drugs have been associated with thrombocytopenia; these include certain cytotoxic drugs, antimalarial agents, quinine, anti-epileptic medications, frusemide diuretics and digoxin. This list is by no means exhaustive.
Rarely, patients with coronary artery disease may receive anti-platelet agents and other anticoagulant drugs (such as heparin and warfarin). These can be used, but need extreme caution and close collaboration between the haematologist and the cardiologist.
Excessive alcohol intake may also increase the bleeding risk by its effect on the bone marrow, the liver and also directly on the platelets. Patients with renal problems are also at risk of bleeding as the urea level, which is cleared by the kidneys, accumulates and prevents platelets working effectively.
Children Born To Mothers With ITP
The other group that needs to be carefully monitored for the risk of developing ICH are children born to mothers with ITP. Platelets levels need to be monitored for approximately 1 week after birth so that appropriate treatment can be given to prevent ICH or any other bleeding episode.
Urgent Neurological Imaging
In the more acute presentation rapid onset of neurological abnormalities and decreased level of alertness (including unconsciousness) are of paramount importance in suggesting ICH. After obtaining a history and performing a physical examination, the diagnosis of ICH requires urgent neurological imaging.
Computerised Tomography (CT) scanning has revolutionised the management of brain haemorrhage by demonstrating the site and extent of bleeding quite clearly. It also helps to localise precisely the haematoma and outline the degree of brain oedema (swelling) and mass effect. This can help treatment planning. The CT scanning study can be repeated rapidly and as often as may be needed to evaluate the subsequent clinical course.
Magnetic Resonance Imaging (MRI) is also increasingly available and is effective in identifying small haemorrhages in the brain that may not be obvious clinically.
A lumbar puncture procedure is of no use in intracranial haemorrhage and may be hazardous.
Loss Of Consciousness
Patients with large ICHs present with a decreased level of consciousness due to increased intracranial pressure caused by the bleed. Approximately 25% of patients with ICH who are originally alert will experience secondary deterioration in the level of consciousness within 24 hours. This is normally due to continuing bleeding and worsening cerebral oedema.
More localised bleeding may develop slowly and may be associated with more specific problems, such as cranial nerve defects affecting the face, gaze abnormalities (squints), walking difficulties and some degree of paralysis of an arm or leg, as can be seen following a stroke.
Patients may also experience nonspecific symptoms, which include headache, vomiting and epileptic seizures. These may occur in up to 25% of cases.
Intracranial haemorrhage is fatal in the first 6 months in 23%-58% of patients. When associated with thrombocytopenia it requires rapid diagnosis to enable early and effective treatment. Prompt diagnosis is imperative because a delay in treatment can lead to secondary brain tissue damage.
Early diagnosis and treatment increases the chances of full recovery and minimises longer term problems. The goal of surgery is the rapid evacuation of the haematoma while causing minimal brain injury from the surgery itself. This can be done safely if haemostasis can be assured by raising the platelet count and transfusing platelets. If the bleed is only small then raising the platelet count may suffice and surgery can be avoided.
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