Figure 1
Figure 1. Blood smears and osmotic gradient ektacytometry. Blood smears (A) and osmotic gradient ektacytometry (B). (A) P indicates proband. Many red blood cells were spherocytes (➡). The remaining showed further changes, as if some fragmentation had occurred. There was a pronounced anisocytosis. A few red cells verged on stomatocytes (→). Erythroblasts were present (17%). F indicates father. Presence of many spherocytes without anisocytosis. M indicates mother. Presence of many spherocytes without anisocytosis. (B) In both parents (M and F), there was an increased osmotic fragility, a reduced maximum deformability index, and a decreased dehydration, a situation typical of HS.11 These features were dramatically enhanced in the proband (P). C indicates control. The maximum deformability index (DImax; normal values, 0.41-0.53 AU) is the maximum value of the deformability index. The “hypo-osmotic point” (Omin; normal values, 143-163 mOsm/L) is the osmolality at which the deformability index reaches a minimum in the hypotonic region; it is the same as the osmolality at which 50% of the erythrocytes hemolyze in a standard osmotic resistance test. This index thus provides a measure of the average surface area–to-volume ratio of erythrocytes. The “hyper-osmotic point” (O′; normal values, 325-375 mOsm/L) is the osmolality in the hypertonic region (right leg of the curve) at which the deformability index reaches half its maximum value. It provides information on the erythrocyte hydration.

Blood smears and osmotic gradient ektacytometry. Blood smears (A) and osmotic gradient ektacytometry (B). (A) P indicates proband. Many red blood cells were spherocytes (➡). The remaining showed further changes, as if some fragmentation had occurred. There was a pronounced anisocytosis. A few red cells verged on stomatocytes (→). Erythroblasts were present (17%). F indicates father. Presence of many spherocytes without anisocytosis. M indicates mother. Presence of many spherocytes without anisocytosis. (B) In both parents (M and F), there was an increased osmotic fragility, a reduced maximum deformability index, and a decreased dehydration, a situation typical of HS.11  These features were dramatically enhanced in the proband (P). C indicates control. The maximum deformability index (DImax; normal values, 0.41-0.53 AU) is the maximum value of the deformability index. The “hypo-osmotic point” (Omin; normal values, 143-163 mOsm/L) is the osmolality at which the deformability index reaches a minimum in the hypotonic region; it is the same as the osmolality at which 50% of the erythrocytes hemolyze in a standard osmotic resistance test. This index thus provides a measure of the average surface area–to-volume ratio of erythrocytes. The “hyper-osmotic point” (O′; normal values, 325-375 mOsm/L) is the osmolality in the hypertonic region (right leg of the curve) at which the deformability index reaches half its maximum value. It provides information on the erythrocyte hydration.

Close Modal

or Create an Account

Close Modal
Close Modal