Coagulopathy in amyloidosis: combined deficiency of factors IX and X.

Combined severe deficiencies of blood clotting factors IX and X were observed in 2 patients who suffered from systemic amyloidosis. This unique deficiency state was marked by refractoriness to Vitamin K as well as to transfusion therapy. Increased antithrombin activity was present in both individuals and corresponded in time to the emergence of a monoclonal IgG kappa light chain paraprotein in 1. Both patients demonstrated profound bleeding disorders. It is hypothesized that the Vitamin K dependent factors have special affinity for amyloid deposits due to an unusual amino acid (gamma-carboxyglutamic acid) present in these factors.

Alpha-1-fetoprotein in a patient with a poorly differentiated carcinoma of the anterior mediastinum.

A case of a mediastinal tumor which was associated with the production of AFP is described. The diagnostic significance of an elevated serum concentration of AFP is reviewed in both neoplastic and non-neoplastic clinical situations.

Transferrin loss into the urine with hypochromic, microcytic anemia.

Anemia developing during the course of chronic renal disease is a frequent complication often necessitating periodic transfusion therapy. A number of etiologic factors have been implicated, including decreased production of erythropoietin; decreased erythrocyte life span secondary to uremia and splenomegaly; increased bleeding tendency due to platelet dysfunction; and acquired lack of folic acid and iron. This paper concerns the problem of acquired hypochromic, microcytic anemia secondary to heavy urinary loss of iron and transferrin in a child with the nephrotic syndrome. The patient had microcytic, hypochromic anemia with serum iron, 12 mug. per dl. and a serum iron-binding capacity of 12 mug. per dl. There was no evidence of major bleeding resulting in a chronic hemorrhagic anemia. Urinary iron was 64 mug. per dl., with a urinary iron-binding capacity of 366 mug. per dl. Renal biopsy showed mesangio-proliferative glomerulonephritis. Evaluation of any patient with the nephrotic syndrome should include careful analysis of the various serum and urinary proteins and determination of serum and urinary iron and iron-binding capacity. This information would offer a more precise evaluation of the underlying cause of anemia in the nephrotic patient who may develop urinary loss of iron and transferrin and subsequent hypochromic, microcytic anemia.