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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.

Pelger-Huët anomaly (PHA), an autosomal dominant haematological trait is characterised by neutrophil nuclear hypolobulation and modified chromatin distribution. Mutations in the lamin B receptor gene, a member of the sterol reductase family have been identified as the underlying cause. Due to its asymptomatic nature or lack of observer familiarity, PHA is often overlooked. In this review, we give an overview of the main pathophysiological, clinical, morphological and functional aspects of PHA. Furthermore, we highlight the importance of a comprehensive approach to the assessment of this laminopathy.

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.

We have applied Mueller matrix ellipsometry to assess the change in the state of polarization of a light beam that has double passed the ocular media and is scattered at the fundus of the human eye in vivo. At several positions in the pupil plane, which together cover the area of the dilated pupil, Mueller matrices are assessed. From them the magnitude of the retardation and the orientation of the eigenvector are calculated. The properties of the retardation process are surveyed by measuring the retardation along a horizontal meridian as a function of wavelength, density of visual pigment, and location of retinal fixation. Furthermore, photographs are taken from the polarization patterns on the iris with circularly polarized light. We posit that the cornea behaves as a biaxial crystal with its fastest principal axis normal to its surface and its slowest nasally downward. The retardation of light by a model eye with such a cornea is calculated, and the results are compared with the data.