Cause of death in hemophilia a patients in the United States from 1968 to 1979

Abstract

Death data on 949 hemophiliacs for the years 1968-1979 have been analyzed. The median age at death has increased from 33 to 55 years. There was no evidence of new diseases caused by the more intensive therapy during this time interval. The leading infectious disease was hepatitis, accounting for eight deaths. Only one acute hepatitis death was listed after 1974, when sensitive tests for hepatitis B antigen screening of plasma were implemented. Cirrhosis was a primary or associated cause of death in 76 cases (8%) and pneumonia was a primary or associated cause of death in 62 deaths (6.5%). The types of malignancies in hemophiliacs were similar to those in the male US population with no evidence of excessive retrovirus malignancies prior to infection with HIV-1.

Hemophilia treatment in historical perspective: a review of medical and social developments

“…The most common cause of death in hemophilia still is hemorrhage, although it no longer constitutes the sole death cause: in our study it accounted for half of the deaths [78,93,102,112,129,148]. In a recent report on almost a thousand deaths of hemophilia patients in the USA from 1968 to 1979, it was observed that the ratio of the number of deaths by ischemic heart disease over the number of cancer deaths was much smaller than in the general population [7]. It was not possible from this study to conclude whether this was caused by an increased mortality from cancer or a decreased mortality from ischemic heart disease, since this was not a follow-up study: the number of deaths could only be compared relative to each other [proportional mortality ratios (PMR)].…”

“…Evidence of cirrhosis has been reported in 15-38% of hemophilia patients [4,57]. In the USA liver disease accounted for 9% of the deaths in hemophilia patients between 1968 and 1979 [7]; surprisingly, in our more recent study of mortality over the period 1973 -1986 no deaths from liver disease were noted [112].…”

Causes of Death Among 379 Patients With Hemophilia: A Developing Country’s Report

“…[16][17][18] There were noticeable numbers of the fatal spontaneous CNS bleeding events in the surveyed patients with hemophilia. This may be due to limited access to the coagulation factor concentrates in their country in the past decades.…”

Effects of HIV infection on age and cause of death for persons with hemophilia A in the United States

“…In the 1960s and 1970s, the introduction of factor replacement therapy, the development of comprehensive hemophilia treatment centers, and the implementation of home care for this population decreased the number and proportion of deaths due to hemorrhage [7], dramatically increasing their median age at death [33,34]. In this study, these perturbations are underscored by the disparity in the median age at death in 1995-1998 for persons with hemophilia A between those with HIV-related disease (33 years) and those without HIV-related disease (72 years).…”