Idiopathic non-syphilitic paroxysmal cold haemoglobinuria in children.

Abstract

Three examples of non-syphilitic paroxysmal cold haemoglobinuria (PCH) in children are described which occurred, within a period of 16 days, in association with a febrile illness. No definite viral aetiology or obvious epidemiological association could be established. A Donath-Landsteiner antibody of anti-P specificity was demonstrated in all three patients. The serological aspects of PCH are critically discussed.

Autoimmune Haemolysis Associated with Donath-Landsteiner Antibodies

“…This picture is similar to that in other reports [4,8,13,16] and Donath-Landsteiner haemolysis secondary to cytomegalovirus [4], measles [14], mea sles immunization [10], mumps [11] and infectious mononucleosis [18] has occurred. Although one authority [8,19] feels that most, if not all, non-syphilitic examples are associated with (often unknown) viral disor ders, non-viral infections, e.g. Haemophilus influenzae and Mycoplasma, have apparent ly caused Donath-Landsteiner haemolysis on occasions [4,9,20] and H. influenzae but no virus was isolated from our patient C.H.…”

“…Occasionally clustering of cases occurs, 5 of our patients presented within 10 months, 2 of these within the same week. Similarly re ports of 3 cases presenting within 16 days [8], 5 within 8 months (3 in 1 month) [4] and 3 in 7 months [12] are found.…”

“…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.…”

“…All pa tients were very ill, in most the rate of hae molysis was marked and in 7 patients (table III) the Hb fell to less than 5 g/dl. In patient I. W. the rate of fall was greater than 1 g/dl/ day; similar or even higher rates of haemo lysis are common [4,8,12,14,15], There may be an associated reticulocytopoenia which has been attributed either to suppres sion of marrow erythropoiesis or to the Donath-Landsteiner antibody having stronger activity against reticulocytes [4]. The reticu'.ocyte count may not reach its maximum for several days (patients S.H., J. W.) and in one reported patient whose re ticulocyte count was 0.2°/o at presentation, the peak response of 52°/o did not occur un til the 18th day [15].…”

“…presented with a characteristic history of ep isodic haemoglobinuria and systemic symp toms on exposure to cold -this case was very easy to diagnose. Donath-Landsteiner antibodies usually show specificity within the P blood group system in both acute and chronic forms of the disease [4,10,[14][15][16][30][31][32], Anti-P specificity, which has been regarded as being essential for diagnosis [8], was found in patient I.W. Testing for antibody specif icity is often difficult, as other tests have precedence for the small blood samples ob tainable from children, and red cells of rare genotype are not always available.…”