History: Case 1. Thrombotic-thrombocytopenic purpura (TTP, Moschkowitz' disease) in a 57-year-old woman had for one year been treated conservatively. But when daily plasmapheresis was temporarily discontinued she developed behavioural changes and impaired speech, providing an indication for splenectomy. Case 2. A 53-year-old woman with TTP had been similarly treated for one month. Splenectomy was indicated when neurological symptoms rapidly developed.
Investigations: At admission, creatinine 110 mg/d, white cell count (WBC) 12.4 G/l haemoglobin 10.1 g/dl, haematocrit 0.29, platelets 91 G/l. Prothrombin time (PTT) and thromboplastin time were normal. Patient 2. At admission, platelet count was below 10 G/l and she had various neurological abnormalities. Haemoglobin was 9.0 f/dl, haematocrit 0.27. Platelet count, PTT, thromboplastin time and renal functions were normal.
Treatment and course: Case 1. After plasmapheresis and administration of cryoprecipitate-free fresh frozen plasma (FFP) excess, laparoscopic splenectomy was performed. On the third postoperative day WBC count was 11.5 G/l, haemoglobin level was unchanged, but platelet count was now normal, as were PTT and thromboplastin time and renal functions. 8 and 32 months after the operation WBC count, haemoglobin, haematocrit and platelets were all normal. There were no neurological abnormalities postoperatively. Case 2. Laparoscopic splenectomy was performed after intensive haematological preparation. The pre- and postoperative course was uneventful and she was discharged on the 8th postoperative day, at which time her haemoglobin was 8.4 g/dl, haematocrit 0.25, while platelets, PTT, thromboplastin time and renal functions were all normal and remained so at follow-up 11 months later. There have been no neurological symptoms after the splenectomy.
Conclusion: Laparoscopic splenectomy is a haematologically and surgically safe treatment of TTP and should be considered for all cases of TTP that fail to respond to conservative management.