Blood, 1973, Vol. 42, No. 5, pp. 771-781.
© 1973 American Society of Hematology, Inc.
Hemoglobin Köln in a Black: Preand PostSplenectomy Red Cell Survival (DF32P and 51Cr)
and the Pathogenesis of Hemoglobin Instability
Paul R. Pedersen 1,
Paul R. McCurdy 1,
R. N. Wrightstone 1,
J. B. Wilson 1,
L. L. Smith 1, and
T. H. J. Huisman 1
1 Georgetown University Medical Division, D.C. General Hospital, and the Division of
Hematology, Walter Reed Army Medical Center, Washington, D.C.; and the Department of Cell and
Molecular Biology, Medical College of Georgia, Augusta, Ga.
A 17-yr-old black male with hemolysis
and pigmenturia but no anemia was found
to have hemoglobin Köln (
2
298 val
met
[FG5]). Splenectomy was done because of
complicating thrombocytopenia. Thrombokinetic studies with 51Cr tagged platelets
suggested hypersplenism, and after surgery
the platelet count returned to normal. The
red cell t 
51Cr was more than doubled,
but the red cell life span (DF32P) was
more modestly improved (30.6 47.2
days). The "elution" of 51Cr from the red
cells presplenectomy was 5.6%/day,
whereas after surgery it was normal
(1.9%/day), accounting for the disparity
between the survival methods. Study of the
isolated cyanferri derivative of hemoglobin
Köln by ultracentrifugation at various salt
concentrations and various pH’s indicated
an increased tendency to dimer formation
under conditions where normal hemoglobin is a tetramer. This results from the
site and type of amino acid substitution and
accounts in part for its instability.
Submitted on January 8, 1973
Revised on April 30, 1973
Accepted on May 4, 1973
