CASE HISTORY

A married woman aged 64 years at death. 1947, attended Hospital with sore tongue. Diagnosis: Polycythaemia rubra vera. Treated with repeated venesections for next four years. Remained pretty well. 1956 - Recurrent pain in left abdomen. Nov 1959: readmitted with tense ascites. 7 litres taken off. Investigation of ascites and haematemesis: Clinical examination: no flap or foetor. No spider naevi. Liver and spleen as before. Prominent abdominal veins. Liver function tests: Bilirubin 0.3mg. Cholestoerol 152mg. Alkaline phosphatase 28 KA units. Thymol 6. ZnSO410 units. GOT 35 units, GPT 25 units, Alb 2.7 -3.3g%, Glob 3.1g%. BSP retention 1 and 3%. Liver biopsy small and fragmented. Some excess fibrous tissue. Liver cells healthy. Barium swallow - suggests oesophageal varices. Splenic venogram - large splenic and portal veins, both widely patient. No collaterals. Splenic pressure 25mm.Hg (normal <14mm). Ascities fluid - protein 3.1g%. One sample malignant cell seen. Conclusion: Portal hypertension; probable cirrhosis of liver. Treatment; diuretics (mersalyl, chlorothiazide and aldactone) and low salt diet. High protein diet (100g) caused no cerebral deterioration - EEG's normal. Further progress: very slow response to diuretics. Blood urea 91mg%. Persistent pyuria - culture: B.coli resisitant to sulphonamides, sensitive to chloramphenicol and furadantin. Treated first with gantrisan, then chloramphenicol, then furadantin. Urine never sterile. Gradual downhill course during next three months. Ascites cleared. Then given low protein diet. 2.2.60: blood urea 118mg%, Hb 43%. Transfused 2 pints packed cells. 18.2.60: Sudden onset of acute dyspnoea. No pain. BP 1870/100. Pulse regular 120/min. VVP + 8cmm. Generalised crepitations over both lungs. ECG normal. Treated with digoxin, mersalyl, aminophylline. Cuffs on limbs. Steady deterioration and died twelve hours later. Clinical diagnosis: Polycythaemia rubra vera progressing to myelosclerosis. 2. Probable hepatic cirrhosis. 3. Pyelonephritis and uraemia. 4. Terminal pulmonary oedema - Pulmonary embolus or myocardial infarction.

PATHOLOGY

The splenic vein was grossly dilated and tortuous. Both veins were quite patent and free from thrombus. The spleen was enlarged to about 15 times normal size and weighed 2165g. It was adherent to surrounding viscera. The capsule was grossly thickened up to 4mm at the upper pole where it formed a layer of icing – sugar beneath which, after reflection there were rusty deposits of iron pigment. The pulp was a rather uniform, fleshy, pinkish-brown colour with inconspicuous follicles. Several infarcts, mostly old, were present. Some showed central fibrous scarring with an irregular reddish brown-edge. The surface of the healed infarcts was depressed with a corresponding localised thickening of the capsule filling the depression. Microscopic examination: showed marked extramedullary haemopoiesis,with great increase of reticulin. Moderate amounts of iron present in reticulo-endothelial cells.