Online edition:ISSN 2758-089X

Acquired α2 Plasmin Inhibitor Deficiency with Manifestation of Hemarthrosis in a Patient with Spur Dell Anemia

We saw a male patient of alcoholic hepatic cirrhosis with spur cell anemia manifesting accelerated hemolysis and spontaneous hemarthrosis. His plasma antihemophilic factor level was within the normal limits. He was 45 years old, had a history of 20 years of alcoholism and transferred to our hospital because of progressive anemia and bleeding tendency. About 30 per cent of the red cells of his peripheral blood smear were spur cells. The diagnosis of spur cell anemia was made on the basis of elevated free cholesterol contents and high value of free cholesterol/phospholipid (FC/PL) ratio of the red cell membrane lipid. One day during the course of his illness, spontaneous hemarthrosis appeared suddenly in his right knee joint, but his plasma antihemophilic factor level was normal. On the contrary, the level of the α2-PI was as low as 0.98 mg/dl at that time. It was evident that α2- PI deficiency was the most important factor for causing hemarthrosis in this patient with hepatic cirrhosis.

Takemoto Y, et al