Paroxysmal nocturnal hemoglobinuria is a rare acquired stem cell disorder characterized by intravascular hemolysis, aplasia and an increased risk of thrombosis. We describe a patient under treatment with the anti-complement antibody eculizumab who developed pancytopenia, requiring blood transfusions, due to massive splenomegaly. The patient underwent two separate splenic embolizations, which reduced the size of the spleen and improved his blood count to the point that blood transfusions were no longer necessary. Splenic embolization was chosen over splenectomy due to the potential postoperative complications of splenectomy, especially that of thrombosis.
机构:
Japanese Red Cross Med Ctr, Dept Hematol, Shibuya Ku, 4-1-22 Hiroo, Tokyo 1508935, JapanJapanese Red Cross Med Ctr, Dept Hematol, Shibuya Ku, 4-1-22 Hiroo, Tokyo 1508935, Japan
Iki, Seiko
Ohgi, Kazuyuki
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Japanese Red Cross Med Ctr, Dept Radiol, Shibuya Ku, 4-1-22 Hiroo, Tokyo 1508935, JapanJapanese Red Cross Med Ctr, Dept Hematol, Shibuya Ku, 4-1-22 Hiroo, Tokyo 1508935, Japan
Ohgi, Kazuyuki
Suzuki, Kenshi
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Japanese Red Cross Med Ctr, Dept Hematol, Shibuya Ku, 4-1-22 Hiroo, Tokyo 1508935, JapanJapanese Red Cross Med Ctr, Dept Hematol, Shibuya Ku, 4-1-22 Hiroo, Tokyo 1508935, Japan