Elevation of alkaline phosphatase in a pregnant patient with antiphospholipid syndrome: HELLP syndrome or not?

Hep2), anti-dsDNA antibody positive (Crithidia assay), anti-Ro-positive antibody and hypocomplementaemia (C3 0.45 g/l, C4 0.17 g/l)]. The patient received prednisolone, azathioprine and hydroxychloroquine. Two years later she developed an infectious meningo-encephalitis, and tragically serious cerebral complications have resulted in the development of a persistent neuro-vegetative state. A 14-yr-old Asian female presented with fever, cough, weight loss and left-sided supraclavicular lymphadenopathy. Infection was excluded and the autoimmune profile was non-diagnostic [ANA 1:1280 (Hep2)]. Lymph node biopsy was typical of KFD. Shortly after the biopsy she developed a widespread inflammatory polyarthritis, malar rash and headaches. Simultaneously, immu-nological features of SLE were detectable with positive anti-dsDNA antibodies and hypocomplementaemia and she was commenced on appropriate therapy. Her progress has been complicated by episodes of arthritis, pancytopenia secondary to active SLE, fevers and migraines requiring pulses of i.v. methylpredniso-lone. She developed diffuse proliferative glomerulonephritis [World Health Organisation (WHO) Class IV], which following intolerable side-effects with mycophenolate and later cyclopho-sphamide, was successfully treated with B-cell depletion therapy. Since 1991, 32 reports of KFD associated with SLE have been published [5, 6]. On review, many describe the association of KFD diagnosed concomitantly with or following the diagnosis of SLE [4, 7], which in all probability represent lupus lymphadenitis. We expand the literature with the report of four cases in which KFD predated a clinical and immunological diagnosis of SLE by between 3 and 14 months. Unfortunately, however, there are currently no predictive markers to identify as to which of the patients with KFD will develop SLE [6–8]. Clinically, KFD is typically characterized by lymphadenopathy (predominantly cervical), acute fevers and other systemic features. Extra-nodal involvement is less common, although reported [9]. ANA is usually negative and whilst the natural history can occasionally be unpredictable in relation to severity and complications , most cases are self-limiting, improving within 6 months [5, 10]. The clinical and immunological features required for diagnosis of SLE are well documented and specific. Lupus lymphadenitis has been reported in between 12% and 59% of patients with SLE, but in contrast to KFD, is rarely the presenting feature [10]. Careful examination of lymph node histopathology in correlation with clinical features is the most reliable way to differentiate the two entities. KFD is typified by cortical and paracortical necrotizing nodules, apoptotic debris, proliferation of histiocytes and immuno-blasts, abundant CD8þ T cells, and an absence/paucity of neutrophils [2, 6]. In contrast, lupus lymphadenitis is diagnosed in patients …