Dr Ryan Agnew
A 20 year old female presented to hospital with a scattered history of abdominal pain, fatigue and a recent event of haematemesis. She described a sensation of tightness in her epigastric region, that was inconsistent in nature, did not move anywhere else and did not impact significantly on her working in the garden. She had a single episode of haematemesis 5 days ago – frank blood mixed with gastric content, no coffee ground type content. She had travelled about a days walk to arrive to Kompiam and could only speak a small amount of pidgin, speaking only Engan. Therefore it was difficult to establish a past medical history or much of a further history.
She was tachycardic, normotensive and afebrile. She had pallor of the palmar creases and sclera. Her chest was clear and although rapid, her heart sounds were dual without an added sound. On abdominal exam she had a distended abdomen and a large mass extending inferiorly to below the umbilicus. This mass was consistent with an enlarged spleen, firm in nature, moved with breathing and seemed to arise from the right side of her abdomen.
A pregnancy test was performed which was negative. Her point of care haemaglobin was found to 25g/L, we assumed this to be incorrect, however on macroscopic examination of her blood 80% of the blood content appeared to be serum. Her total white cells were 1.0 x 109/L with no specific dominance of subtypes.
A transfusion was arranged immediately, although she was clinically well.
She was reviewed the next day with an Engan translator. She reported chronic abnormal bleeding and fatigue for the past 7 years. The splenomegaly has been monitored and she had previous similar blood tests. She had no history of weight loss or enlarged nodes and was otherwise well. She had to reduce the amount of garden work she could do and had to take her time walking distances.
It was assumed that due to chronic nature of her symptoms and multiple episodes of severe anaemia for the past 7 years that a diagnosis of a leukaemia or another primary bone marrow disease was unlikely. It was likely her spleen that was causing the pancytopaenia rather than the process causing the pancytopaenia to be enlarging her spleen. Therefore, these symptoms were attributed to tropical splenomegaly.
Tropical splenomegaly is a hyperactive spleen which is effective at protecting against encapsulated organisms and malaria, however can get carried away and start behaving in an autoimmune type fashion. In this case the over-zealous tropical insurance was causing pancytopaenia.
We considered whether removal of the spleen was the best course of management. Due to the size of the spleen, this was anticipated to be a complicated surgery. It also was uncertain if the removal of her spleen would improve or in fact worsen her immune function. Was it worse to have the leukopaenia from the splenomegaly or the disarmed immune defenses secondary to splenectomy?
The decision was made after consultation with multiple surgeons around PNG, to manage conservatively with multiple transfusions.