Age: 53.0
Sex: M
Vaccine: PPV
Symptoms: Laboratory test abnormal, Platelet count decreased, Thrombocytopenia, Thrombotic thrombocytopenic purpura
Clinical narrative: Information has been received from a published literature article, title as stated above, regarding an approx 53 year old male with single functional kidney and a history of thrombotic thrombocytopenic purpura (initial onset 2000), hospitalisation, confusion, aphasia, acute renal failure, fever, jaundice, haemolytic anaemia, thrombocytopenia, thrombotic microangiopathy and C reactive protein increased who was vaccinated with a dose of Pneumococcal 23v polysaccharide vaccine. Subsequently the pt was given a splenectomy to avoid plasma exchange dependence. The pt was administered anti pneumococcal injection before the intervention. No recurrence of TTP was observed for 18 months. Platelet count was 160 to 200. Two years 3 months after the onset of TTP, the first relapse occurred day 830. Clinical symptoms such as asthenia and bruises, and biological signs appeared (hemoglobin 10.0 g/dl (100 g/l), platelet count 10x10*3/ul, LDH 1050 u/l, schizocytes ++. No other symptoms were observed, especially none suggesting viral illnesses. on 10/15/02 the pt was administered influenza virus sAg 3v vaccine inactivated (Fluvirine) 3 months earlier (day 736). He had no vaccination for more than 5 months. Remission was obtained rapidly after 6 PEs, platelet count was stabilized to approx 250 x 10*3/ul. A similar scenario occurred with a second relapse 22 months later on day 1542 the pt on 10/25/04 was administered influenza virus sAg 3v vaccine inactivated (Agrippal) 2 months earlier day 1477 and DPT )Revaxis) vaccine twice on 11/4/04 and 12/4/04, respectively, 1 and 2 months before the relapse (day 1487 and 1517 respectively). Platelet count was 30 x 10*3/ul and LDH level was 1060 u/l. TTP resolved after 5 PEs. No influenza vaccination was performed during autumn 2003. ADAMTS 13 protease activity (thrombospondin type 1 motif isoform 13) was measured first during the second relapse on day 1542. ADAMTS 13 protease activity decreased dramatically (<5%), and a high level of ADAMTS 13 protease inhibitors were detected. Diagnosis of idiopathic TTP caused by the presence of ADAMTS 13 protease auto antibodies were established. It was also reported that in the present case, no initial factor for the onset of disease was identified (neither infection nor vaccination. Both vaccinations were followed with a relapse, although the delay might be a little long, it suggested the imputability of vaccinations as a trigger. A seasonal factor may have been assumed (physical, food), but never reported. It was reported that ie could be hypothesized that either cross reactive stimulation between vaccinal antigens (especially influenza vaccine) and ADAMTS 13 protease or bystander activation of vaccination with the ADAMST 13 specific immunoglobulin G causes a response. Absence of relapse after pneumococcal vaccine before splenectomy was in favor of the former hypothesis. Immediate and midterm control of TTP was difficult to obtain in the pt. Long term remission was obtained after many PEs, vincristine injections, and splenectomy. Vaccination induced relapses did not last long, and remission was obtained rapidly. It was reported that this possibly could be related to the absence of spleen in the pt. In conclusion the report emphasized the immunologic susceptibility of TTP and from this case suggested the potential role of vaccination. The pt needed vaccination because of splenectomy, but TTP relapses seemed to occur after each injection. Additional vaccinations probably should be discouraged. The reporter suggested performing vaccinations in pts who develop ADAMTS protease antibodies only when mandatory. If vaccination as mandatory, the pt should under go sustained clinical and biological follow up. Upon internal review thrombotic thrombocytopenic purpura was considered to be an other important medical event (OMIC). No further information is available. A copy of the published article is attached as further documentation of the pts experienced.