Blood, 1958, Vol. 13, No. 11, pp. 1074-1084.
© 1958 American Society of Hematology, Inc.
Paroxysmal Nocturnal Hemoglobinuria: a Case Report
with a Negative Ham Presumptive Test Associated
with Serum Properdin Deficiency
RICHARD K. BLAISDELL 1,
ROBERT E. PRIEST 1, and
ERNEST BEUTLER 1
1 Department of Medicine and the Argonne Cancer Research Hospital, USAEC,
The University of Chicago, and the Department of Pathology, University of Washington
School of Medicine, Scattle.
1. A case of paroxysmal nocturnal hemoglobinuria (PNH) has been presented in which the Ham presumptive test for PNH was negative.
2. The basis for this false negative test and the atypical results of the Ham
complete acid hemolysis test for PNH appeared to be due to a loss of the in
vitro PNH hemolytic activity of the serum associated with a deficiency of
properdin.
3. The acidosis accompanying the renal failure in this patient did not appear to accelerate the hemolytic process, for there was no apparent reduction
of hemolytic activity in vivo when the acidosis was corrected by the administration of sodium bicarbonate.
4. The autopsy observations suggest that the protracted deposition of massive quantities of iron in the kidneys may play a robe in the not uncommon
occurrence of renal disease in PNH.
Submitted on November 12, 1957
Accepted on April 12, 1958
