Paroxysmal Cold Hemoglobinurias

Autoimmune Haemolysis Associated with Donath-Landsteiner Antibodies

“…In other series the total incidence has been 1.7% [5] and 5.1% [6], Previous reports [3] suggest that the disease was more common before effective treatment early in syphilis was available and our series of 14 patients is a large one for the present times. Males are affected more frequently than females and young children more than adults.…”

“…Our fig ures for children under 10 years suggest that the male:female ratio is about 2.5:1 and that the incidence per year per 100,000 in age and sex matched populations is 0.001 and 0.0005, respectively. The frequency of Donath-Landsteiner haemolysis in child hood has been noted previously [5,7], but the male predominance has not been stressed and on occasion even stated not to exist [5]; in case reports of 20 patients less than 10 years old, twice as many males as females were affected [4,[8][9][10][11][12][13][14][15][16], Similar sex differences in chronic PCH secondary to syphilis have been noted [3]. Occasionally clustering of cases occurs, 5 of our patients presented within 10 months, 2 of these within the same week.…”

“…Nomenclature Paroxysmal cold haemoglobinuria (PCH) was the name given to a rare but characteristic clinicopathological syndrome usually [2] or always [3] secondary to sy philis. When it was recognized that cases need not be related to syphilis, the essential diagnostic parameter was considered to be the presence of Donath-Landsteiner anti bodies.…”

“…A review of PCH 50 years ago [2] stressed the importance of syphilis as the main aetiological agent, however, even then evidence was beginning to accrue that the disease could occur in non-syphilitic individ uals, and that the Wassermann reaction could be falsely positive in these cases. A further review 20 years later [3] firmly implicated syphilis (particularly the late quiescent form) as the cause of PCH, the congenital form being responsible for PCH in young children. In none of our patients was there any suspicion of syphilis but in three ins tances false positive Wassermann reactions were found (table III).…”

The Destruction of Red Cells by Antibodies in Man. II. Pyrogenic, Leukocytic and Dermal Responses to Immune Hemolysis1

“…Taken together, the following observations strongly suggest that certain isoantibodies may, in causing hemolysis, provoke the release of histamine, serotonin and possibly other related substances from leukocytes and platelets: 1) the finding of others (56)(57)(58) that antigen-antibody reactions with the probable involvement of complement cause a release of histamine and of serotonin from leukocytes, platelets and certain other cells; 2) the observation of others (11) (60) and may reasonably be attributed to the sequence of events postulated to occur during hemolysis by isohemolysins.…”