Visceral leishmaniasis presenting with paroxysmal cold haemoglobinuria.

Abstract

Paroxysmal cold haemoglobinuria, first described by Donath and Landsteiner in 19041,2, is a rare autoimmune haemolytic anaemia, defined by the presence of an IgG biphasic haemolysin. It is generally considered an acute self-limited anaemia caused by an IgG biphasic auto-anti-P antibody (Donath-Landsteiner [DL]-haemolysin). The autoantibody is called biphasic because it sensitises red cells at 0–4 °C and causes complement-mediated haemolysis at higher temperatures. The generally reported incidence ranges from 1.6 to 5.1% in childhood haemolytic anaemia3, although an incidence of as high as 32% was found in one small series4. Most of the data are probably too old to reflect the true incidence at present; the haemolysis caused by DL antibody could be underestimated since proper testing is rarely performed. In the past years, a positive DL test has characterized a chronic disorder known as cold haemoglobinuria, usually secondary to syphilis. Currently, most cases of DL-haemolytic anaemia occur as acute transient episodes in children and young adults after and/or concomitant with a variety of viral and bacterial infections including measles, mumps, chickenpox, cytomegalovirus, Epstein-Barr virus, adenovirus type 2, influenza A, Klebsiella spp., Haemophilus influenzae, Mycoplasma and E. Coli infections, or as a complication of vaccinations2–6. We report here the case of a very young Caucasian child with acute haemolytic anaemia due to a DL red cell autoantibody, as the first sign of leishmaniasis.