No abstract

In the UK, diagnosis of syphilis among women of child-bearing age has more than doubled between 1999 and 2007, and each year, around ten cases of congenital syphilis have been reported. Doctors should, therefore, be aware of the potential presentation of congenital syphilis, a deadly disease yet curable when managed correctly. We present a rare case of a premature neonate of 29 weeks displaying manifold clinical signs of congenital syphilis acutely after delivery, including coagulopathy, hepatosplenomegaly, intraventricular ischaemic lesions and metaphyseal bone changes, not previously described in the literature at this age in the UK.

Paroxysmal cold haemoglobinuria in syphilis patientsParoxysmal cold haemoglobinuria (PCH) was first described by Donath and Landsteiner in 1904' and is one of the rarest autoimmune haemolytic anaemias. Acute intermittent attacks of intravascular haemolysis following exposure to cold and the presence of a biphasic haemolysin, the Donath-Landsteiner (DL) antibody, are the classical clinical manifestation and pathognomonic laboratory finding of PCH.2 The autoantibody is typically IgG, usually showing anti-P specificity. The antibody is described as biphasic because it sensitises red cells at O-4°C and causes complement-mediated haemolysis when the temperature is raised to 370C.3 The association of PCH with syphilis, particularly the congenital form has been described earlier. 4 Although there has been a decline in the prevalence of syphilis subsequent to the advent of antibiotics, there are still certain regions in India with a high prevalence of the disease.We undertook a study on 26 patients with syphilis (four primary and 22 secondary syphilis) over a period of six months. The study of the incidence of PCH in syphilis patients and to seek a correlation between DL antibody and evidence of haemolysis. The patients were aged 12 to 36 years and 18 of them were males. Twenty five healthy controls were also studied. The diagnosis of syphilis was based on clinical and serological findings. All patients were given penicillin therapy 10-20 million units over a period of 21 to 28 days. The environmental temperature to which the patients were exposed varied from 10-36°C.A 12 (46%) could be followed up after treatment, including all 7 DL+ cases. In three of these seven patients the DL antibody disappeared immediately after treatment. In the remaining four cases, one showed a decrease in DL antibody titre from 1:2 to 1:1 while in the rest it remained unchanged. DL antibody titres ranged from 1:1 to 1:4 and anti-P specificity was demonstrated in all seven DL positive cases. Direct antiglobulin test was found to be positive in four out of the seven DL positive cases showing complement component on the red cells, while none of the DL antibody negative patients had a positive DAT. This study showed that the presence of DL antibody in syphilis patients can lead to paroxysmal cold haemoglobinuria, especially on exposure to cold, which may go unnoticed in the absence of a typical history. Detection of DL antibody may thus have therapeutic implications in syphilitic patients, especially in avoiding exposure to cold temperature; however, in severe haemolysis steriods may be helpful in some cases.