Pneumococcal septicemia in functional asplenia: first manifestation of systemic autoimmune disease?

A 35 year old female was admitted into the hospital because of rapid onset fever and chills. Streptococcus pneumoniae could be isolated from blood as the responsible pathogen for septicemia. Necroses of fingers and feet occurred. The clinical signs of an overwhelming post-splenectomy infection (OPSI) were evident. High-dose penicillin was administered and the patient recovered. Howell-Jolly-bodies were seen in peripheral blood smears. A spleen within normal size could be demonstrated in CT and sonography. Angiologic findings showed intact splenic arteries and a normal vein, whereas the small splenic vessels were rare. MRT of the spleen using Endorem (Fe), showed only a minimal uptake of the RES. In a scintigram of spleen and liver using 320 MBq Tc-99m nanokoll, the spleen was not visible. Thus, functional asplenia was demonstrated by Howell-Jolly-Bodies and by image methods. An increased antinuclear antibody level and a Sm-antibody lead to the diagnosis of undifferentiated connective tissue disease. As far as we know this is the first case that functional asplenia was the first symptom of a systemic autoimmune disease.