Inherited phosphofructokinase deficiency in dogs with hyperventilation-
induced hemolysis: increased in vitro and in vivo alkaline fragility of
erythrocytes
U Giger, JW Harvey, RA Yamaguchi, PK McNulty, A Chiapella and E Beutler
Two male English springer spaniel dogs with a chronic hemolytic anemia and
sporadic hemolytic crises, historically related to "stress" situations,
were studied. Although canine erythrocytes are in general known to be more
alkaline fragile, erythrocytes from both patients began to lyse earlier, at
significantly lower pH values (near pH 7.4 at 37 degrees C), than
erythrocytes from control dogs. Hyperventilation induced by 30 minutes of
exercise, placement in a 39 degrees C water bath, or intravenous doxapram
increased venous blood pH in dog 1 and control dogs, but transient
hemoglobinemia, hemoglobinuria, and severe bilirubinuria occurred only in
the studied patient. The erythrocyte phosphofructokinase (PFK) activity was
severely decreased in both dogs (10% of controls). The erythrocyte
2,3-diphosphoglycerate content was markedly reduced and the cell chloride
content was consequently increased. This change in cell chloride content is
related to an increase in the erythrocyte pH, which may partially explain
the pathogenesis of hemolysis in canine PFK deficiency. Thus, these studies
demonstrate a presumably inherited erythrocyte PFK deficiency in English
springer spaniels, which causes an increased in vitro and in vivo
erythrocyte alkaline fragility. Dogs with PFK deficiency and inducible
hemolytic crises may become a valuable genetic animal model in which to
study the pathophysiology of hemolysis.
Volume 65,
Issue 2,
pp. 345-351,
02/01/1985
Copyright © 1985 by The American Society of Hematology
