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Aplastic Anaemia and Paroxysmal Nocturnal Haemoglobinuria

The unit CME this week was prepared by Dr Fatimah Jaafar Purba.

She was given a couple of topics to cover. She started off with aplastic anaemia. She emphasised the important point that aplastic anaemia is a diagnosis of exclusion. Once a patient with pancytopenia presents, early investigation was geared up to identify any underlying reversible condition. These include infections - especially viral infection such as hepatitis virus and parvovirus. The next group of condition needed exclusion would be connective tissue disease, drugs and underlying cancer or leukaemia. Often, a period of six weeks were lapsed prior to aplastic anaemia being assumed as the underlying cause.

Aplastic anaemia is thought to have an autoimmune origin, mediated by cytotoxic T lymphocytes. Currently, there are two main treatment modalities - 1) immunosuppression with combination of anti-lymphocyte globulin (ATG) and cyclosporin A (CSA); and 2) stem cell transplant. In the younger population, the risk of transplant is lower than the benefit of possible cure, hence transplant seemed to be amore attractive choice especially if the condition was detected early and patient has only been exposed to minimal prior blood transfusion. Otherwise, combination of CSA and ATG is used, with response rate as high as 70%.

However, the actual treatment method and statistic was not discussed. Dr Fatimah then went on to describe the pathophysiology and presentation of paroxysmal nocturnal haemoglobulinopathy (PNH), a condition in which the patient acquired a genetic abnormality on the surface of blood cells making them prone to be activated by the immune system (via the complement system). If the red cells were activated, the patient will suffer haemolytic anaemia, and if the platelet is involved, patient suffers a blood clot. The patient may also present in a picture not dissimilar to aplastic anaemia.

Treatment also follow almost an identical route to aplastic anaemia although the decision to try immunosuppresion as first-line is more straight-forward. The session was then completed with question and answers. Later after the session, Dr Edmund presented a paper on polycythaemia as part of the Journal Club.

Lunch for the session was provided by Novartis.

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