A case of HELLP syndrome: an immuno-"logical" approach.

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.

Autoimmune haemolytic anaemia associated with a thymoma: case report and review of the literature.

A 67-year-old female presents with a small mass in the anterior mediastinum on chest computed tomography. A biopsy proves the mass to be a spindle-cell-type or type A thymoma. Subsequently the patient develops fever and severe Coombs-positive haemolytic anaemia. She is initially treated with oral corticosteroids. Because of persistence of the haemolysis subsequent thymectomy is performed. Haemolysis disappears almost instantly and does not return after discontinuation of the oral corticosteroids. Review of the literature reveals only 17 other cases of thymoma-associated autoimmune haemolytic anaemia.

[Systemic lupus erythematosus over hemophagocytic lymphohistiocytosis]. / Systemische lupus erythematosus-geassocieerde hemofagocytaire lymfohistiocytose.

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.

Pelger-Huët anomaly: a critical review of the literature.

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.