Splenectomy as an effective treatment for macrothrombocytopenia in
Takenouchi-Kosaki syndrome
Abstract
Takenouchi-Kosaki syndrome (TKS) exhibits clinical features represented
by macrothrombocytopenia, developmental delay, dysmorphic facial
features, and deafness. There is little information on the treatment for
macrothrombocytopenia in TKS. Splenectomy has been a contraindication
for inherited thrombocytopenia. In the case of autoimmune haemolytic
anaemia (AIHA) with TKS that we previously reported, AIHA initially
resolved with prednisolone; however, it gradually became resistant to
drug therapy. We performed splenectomy, and post-operatively, both
anaemia and macrothrombocytopenia improved. This is a novel effect of
splenectomy for thrombocytopenia in TKS, which suggests that splenectomy
could be a treatment option for thrombocytopenia in TKS.