可逆性機械的溶血性貧血を呈した重症大動脈弁膜症の一治験例

A cured case of severe aortic valve disease demonstrating reversible mechanical hemolytic anemia

　人工弁置換術後に溶血性貧血を生じうることは広く認められているが，非手術弁膜症例において重篤な溶血性貧血を来たすことは極めて稀である^1）。われわれは，大動脈弁に著明な石灰化を伴う重症大動脈弁膜症に重篤な溶血性貧血を合併した一治験例について，文献的考察を加えて報告する。

A 57-year-old woman was admitted to our clinic with easy fatigability, anginal pin and pale face. She appeared markedly anemic, slightly icteric, and had signs of severe aortic valve stenosis with mild aortic regurgitation. Chest X-ray showed an extensive calcification of the aortic valve. Cardiac catheterization demonstrated a marked pressure gradient (180 mmHg) across the aortic valve associated with mild aortic regurgitation. Her hemoglobin was 6.3 g/dl, hematocrit 21 per cent,and the red cell count 220×10⁴. The peripheral blood film showed fragmented red cells. The sternal marrow examination revealed normoblastic erythro-poietic hyperplasia, but no evidence of malignant infiltration. Red cell survival was markedly short-ened with the T 1/2 ⁵¹Cr of 6.5 days. Further hematological examination showed an evidence of intravascular hemolysis. The correctoin of the hemolysis was obtained after insertion of a pros-thetic aortic valve.

It is well known that hemolytic anemia may occur in patients who have had insertion of pros-thetic valves. Recently the association of a com-pensated hemolysis has been described in patients with chronic valvular heart disease without pros-thesis, and the mechanism of the hemolysis is generally thought to be a mechanical one due to extreme intracardiac turbulence. There have been, however, few previous reports of patients in whom intravascular hemolysis in association with chronic valvular disease have developed to severe hemolytic anemia as seen in our patient.