Blood, 1972, Vol. 39, No. 3, pp. 398-406.
© 1972 American Society of Hematology, Inc.
Oxygen Affinity in Hemoglobin Köln Disease
Frank G. de Furia 1 and
Denis R. Miller 1
1 Department of Pediatrics and Medicine, New York Hospital, Cornell University
Medical College, New York.
Oxygen affinity studies in a splenectomized patient with sporadically occurring Hb Köln disease revealed high
whole blood oxygen affinity (P50 O2
17.6 mm Hg) with increased 2, 3-diphosphoglycerate (DPG), low ATP, and
normal RBC
pH. Isolated electrophoretically slow migrating Hb Köln
had a high oxygen affinity, decreased
Hills number, and normal DPG reactivity. Functional evidence for hybrid
tetramers with normal mobility is presented. Partial deoxygenation may
play a role in the denaturation of the
Hb Köln molecule and thus account
for a higher oxygen affinity (low P50
O2), measured by the mixing technique,
than the actual values for P50 that
exist in vivo. Increased oxygen affinity
and decreased P50 O2 would result in
increased erythropoiesis and account
for a well-compensated hemolytic
process in this patient with a normal
red cell mass and normal values of
hemoglobin.
Submitted on August 9, 1971
Revised on September 15, 1971
Accepted on September 20, 1971