Intracranial Haemorrhage And Immune Thrombocytopenia
Professor Adrian Newland
17th Feb 2013
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
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
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.
Professor Adrian Newland CBE
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
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.
Academic Haematology Unit
Barts and The London School of Medicine and Dentistry
Queen Mary University of London
London E1 2AD