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We report on a 27-year-old woman who developed severe arterial hypertension on a background of general malaise within 48 hours after vaginal delivery, suggesting severe acute-onset pre-eclampsia. Concomitant biochemical observations of haemolysis, elevated liver tests and low platelets lead to the diagnosis of (post-partum) HELLP syndrome. Our patient was transferred immediately to the intensive care unit (ICU), where she underwent plasmapheresis in combination with intravenous glucocorticoids, nicardipine and labetalol. Our patient recovered fully after three plasmapheresis sessions. Genetic testing of mutations responsible for complement deficits was negative.

A woman was admitted to the hospital with lymphadenopathy, fever and a generalised exanthema. Laboratory examination revealed leucopenia, anaemia, high sedimentation, elevated CRP and a markedly elevated serum ferritin. Further exploration showed a positive anti-nuclear factor-titre with anti-double-stranded DNA antibodies, positive p-ANCA and a falsely positive syphilis-test. Bone marrow examination revealed an elevated number of phagocytizing macrophages. Diagnosis of secondary haemophagocytic lymphohistiocytosis in a patient with systemic lupus erythematosus was made, a serious and sometimes fatal condition with often repeated exacerbations of the systemic lupus erythematosus that stays active for long periods in spite of the use of immunosuppressive therapy. Haemophagocytic lymphohistiocytosis and systemic lupus erythematosus are sometimes difficult to differentiate because the clinical presentation and laboratory findings are frequently very similar. The diagnosis depends on the clinical picture, blood and bone marrow examination. Bone marrow reveals an elevated haemophagocytosis. In patients with secondary haemophagocytic lymphohistiocytosis, the treatment of the underlying disorder is sometimes sufficient. In some cases there is need for a specific treatment with corticosteroids, intravenous immunoglobulin, immunosuppressive therapy or etoposide.