Idiopathic thrombocytopenic purpura occurring in a subject previously splenectomized for traumatic splenic rupture. Role of splenosis in the pathogenesis of thrombocytopenia.
A 25 year old woman presented with idiopathic thrombocytopenic purpura. She had undergon splenectomy 12 years previously for traumatic splenic rupture. Thrombocytopenia was ultimately resistant to steroid therapy. Howell-Jolly bodies were absent from the peripheral smear and 99mTC-spleen scan demonstrated foci of increased uptake thought consistent with accessory spleens. However, splenosis alone was demonstrated at laparotomy, and all visible splenotic tissue was surgically removed. The patient responded and adequate platelet counts were maintained after discontinuation of steroid therapy. The functional capacity of splenic implants has been previously demonstrated both in animal and man. However, reports linking splenosis to hematologic disease are rare. In the present case, characteristic splenic function was demonstrated by both the the -9mTc-spleen scan and the absence of the typical peripheral blood findings of asplenia. The hematologic response to the removal of the splenotic tissue attests to its importance in maintaining the thrombocytopenic state. In the setting of prior splenectomy for splenic trauma, splenosis may contribute to hematologic disease. Removal of this splenotic tissue may result in hematologic remission.
D000328:Adult; D005260:Female; D006801:Humans; D011696:Purpura, Thrombocytopenic / Q000209:etiology*; D013154:Spleen / Q000637:transplantation; D013156:Splenectomy / Q000009:adverse effects*; D013158:Splenic Diseases / Q000150:complications*; D014182:Transplantation, Autologous