Hemophagocytic lymphohistiocytosis and dengue infection: rare case report.

We describe a 21-year-old female patient returning from a journey to Cambodia, who developed dengue fever complicated with hemophagocytic lymphohistiocytosis. Hectic fever, rash, leukopenia, thrombocytopenia, hepatocellular dysfunction, a markedly elevated ferritin level, and a bone marrow demonstrating abundant hemophagocytosis were present. The patient recovered within 14 days. To our knowledge, this is only the second reported case of dengue virus-associated hemophagocytosis in Europe. As dengue is a rising pathogen in tropical import diseases, clinicians must be aware of its rare but serious complications.

Soft tissue infections with atypical mycobacteria in two patients with inflammatory rheumatic diseases using TNF-inhibitors and/or leflunomide.

Infections of the soft tissues due to atypical mycobacteria are relatively uncommon. We describe two cases. A 61-year-old woman with rheumatoid arthritis (RA) who was treated with the combination of the TNF inhibitor etanercept (Enbrel) and leflunomide (Arava), developed paronychia and cellulitis of the index finger due to Mycobacterium chelonae/abscessus complex. The patient was successfully treated with clarithromycin and surgical debridement. A second case describes a 50-year-old man with ankylosing spondylitis, receiving infliximab (Remicade) and low dose corticosteroids, who developed a granulomatous infection of the right thumb and forearm due to Mycobacterium marinum.The patient was successfully treated with clarithromycin and ethambutol. The increased risk for subcutaneous mycobacterial infections in these cases are probably related to the DMARD treatment with a TNF-inhibitor and leflunomide.

Haemorrhagic diathesis in neonatal calves: an emerging syndrome in Europe.

In 2008 and 2009 a large number of cases of haemorrhagic diathesis (HD) in neonatal calves were reported in different European countries. In Flanders, 84 cases of neonatal HD in 30 herds were reported in this period. The disease typically affects calves younger than 1 month old from different breed and gender. Prominent clinical signs are cutaneous bleeding, petechiae on all mucosae, melena and often high fever. Early in the disease, the mental state of the animals is uncompromised. The typical haematological finding is pancytopenia, with severe to complete thrombocytopenia being the cause of the increased susceptibility to bleeding. In seven of the affected herds blood samples of calves of the same age group as the clinical case were collected and on six of those farms at least one subclinical case could be identified. Necropsy findings were generalized petechiae, ecchymoses or haemorrhages and variable lymphadenopathy. Histopathology of haemorrhagic lesions revealed multifocal extravasation of red blood cells (haemorrhage) with preservation of tissue architecture and absence of other abnormalities. Total bone marrow aplasia and depletion of all lymphoid tissue was the most prominent finding on histology. Activated macrophages and haemophagocytosis were seen on bone marrow cytology from two live calves. Polymerase chain reaction for bovine viral diarrhoea virus, bluetongue and epizootic haemorrhagic disease virus was negative. Several attempts to isolate a viral agent were unsuccessful.

Regulation of the alpha-fetoprotein promoter: Ku binding and DNA spatial conformation.

This work shows that the proximal promoter of the mouse Afp gene contains a Ku binding site and that Ku binding is associated with down-regulation of the transcriptional activity of the Afp promoter. The Ku binding site is located in a segment able to adopt a peculiar structured form, probably a hairpin structure. Interestingly, the structured form eliminates the binding sites of the positive transcription factor HNF1. Furthermore, a DNAse hypersensitive site is detected in footprinting experiments done with extracts of AFP non-expressing hepatoma cells. These observations suggest that the structured form is stabilised by Ku and is associated with extinction of the gene in AFP non-expressing hepatic cells.

Antineutrophil cytoplasmic antibodies (ANCA) and small vessel vasculitis.

We report on one patient with Wegener's granulomatosis (WG) and two patients with microscopic polyangiitis (MPA). The patient with WG had signs of a respiratory infection and showed a c-ANCA pattern with proteinase 3 (PR3) specificity. The patients with MPA presented with pulmonary haemorrhage and signs of renal damage and showed a p-ANCA pattern with myeloperoxidase (MPO) specificity. In the three patients histopathological findings confirmed the diagnosis. We discuss the clinical indications of ANCA testing and the current terminology for reporting ANCA results (c-ANCA, p-ANCA, c-ANCA (atypical) and atypical ANCA). The target antigens and diseases associated with these different patterns are considered. Finally we focus on the value of ANCA and more specific PR3-ANCA and MPO-ANCA in the diagnosis of WG and MPA. The new application domain of ANCA in Crohn's disease and ulcerative colitis is also discussed.

Mastoiditis caused by atypical mycobacteria.

Three case reports of mastoiditis caused by atypical mycobacteria are discussed and a review of the world literature is given. Our aim is to focus the attention of the ENT specialist and the pediatrician on these pathological agents in unilateral recurrent otitis media. The case reports illustrate the importance of histologic and microbiologic examination. Difficulties in the differential diagnosis are mentioned. The therapy is slightly different from lymphadenitis caused by atypical mycobacteria because for mastoiditis we prefer the combination of surgery with medical treatment.

Extreme deficiency of L-type pyruvate kinase with moderate clinical expression.

In the erythrocytes and liver of a patient with hereditary non-spherocytic hemolytic anemia and increased serum aminotransferases, almost complete deficiency of L-type pyruvate kinase was detected. The parents of the patient are second cousins and the pyruvate kinase activity in their erythrocytes was decreased to about half normal values. Pyruvate kinase from the patient is characterized by extreme lability. Pyruvate kinase from the parents' red cells showed no molecular abnormalities. No cross-reactive material could be precipitated with a monospecific antibody raised against L-type pyruvate kinase in the patient's erythrocytes. In the red cells of the parents a decreased amount of cross-reactive material against pyruvate kinase antibodies was found, indicating that the lowered pyruvate kinase activity in the erythrocytes of the parents is caused by a decreased level of the pyruvate kinase protein. In the liver of the patient no L-type pyruvate kinase activity and no immunologically recognizable L-type pyruvate kinase could be detected. The increased lability of the enzyme protein may explain the low residual activity. However, this decreased activity is shown to be sufficient to perform a normal glycolytic flux resulting only in moderate clinical expression.