Mrs Smart was a 47-year-old fit and healthy woman who was taking no medication. She first presented with dyspepsia that was worse with fatty foods.
I thought her pain might be due to a gallstone and this was reinforced shortly afterwards when she contacted the out-of-hours co-op with severe right upper quadrant pain, thought to be biliary colic. The following week I made a private referral to a consultant surgeon who arranged an ultrasound.
The ultrasound showed a pancreatic mass, which was confirmed by CT scan and thought to be a carcinoma in the head of the pancreas. She had also been referred to a tertiary centre for further investigation.
I had a couple of consultations with her in the following weeks as she awaited the appointment. Her pain was proving difficult to control.
Mrs Smart saw the upper-gastrointestinal specialist three weeks later. Her bloods at this time were abnormal: C-reactive protein 155, liver function tests abnormal with alkaline phosphatase of 491, bilirubin 22 and gamma-glytamyl transpeptidase 361. Her FBC was also abnormal with haemoglobin 17.2, packed cell volume 55, white cell count 13.3 and platelets of 765.
The abdominal ultrasound had noted that portal venous flow was absent and that the patient had an enlarged spleen and possible pancreatic mass. CT confirmed a grossly abnormal head of pancreas.
The patient obviously had a portal vein thrombosis and the specialist queried whether this may be secondary to polycythaemia vera.
The pancreatic mass might be due to dilated veins and collaterals, and the pain caused by poor small bowel perfusion. She was referred for an urgent haematological opinion.
Mrs Smart had an episode of melaena and an endoscopy showed oesophageal varices, which were banded.
During her admission, she was seen by the haematologists and a bone marrow confirmed essential thrombocythaemia.
At this time her platelet count was 1,211, she had 14.5cm of splenomegaly, her clotting screen was deranged, and she required a diamorphine pump to control her abdominal pain. Her acute management involved venesection and IV PPI therapy because of the GI bleed.
She was anticoagulated initially with heparin and then warfarin. She was also put on hydroxycarbamide 500mg twice daily.
Two months later she was feeling better. Her platelet count was 160, haemoglobin 13.3 and LFTs nearly normal. She continues on hydroxycarbamide but on a reduced dosage. Follow-up endoscopy shows no evidence of varices.
Thrombocythaemia is one of the group of myeloproliferative disorders, which include polycythaemia rubra vera (characterised by increased red cells), chronic myeloid leukaemia (raised white blood cell count), essential thrombocythaemia (raised platelets) and primary myelosclerosis (increased fibroblasts in bone marrow). All this group of disorders may present with fever, weight-loss, itching, night sweats and malaise.
Hyperviscosity causes impairment of the microcirculation and sometimes visual disturbance, retinal haemorrhage, headaches, coma and genitourinary or gastrointestinal bleeding.
The treatment is with venesection, and removal of a litre of blood may relieve symptoms. The cytotoxic treatment hydroxycarbamide slows marrow turnover and the production of the abnormal cells.
The prognosis is normally good, but the abnormal cells sometimes mutate and cause acute leukaemia.
Dr Warburton is a GP at Ironbridge, Shropshire