Recruiting refugees and migrants as potential hematopoietic stem cell donors to serve patients of comparable ethnicities with rare human leucocyte antigen patterns - The BluStar.NRW project in North Western Germany.

Currently, approximately 19 million people with a migration background live in Germany. The majority of those descend from regions where the population has a genetically different distribution of HLA antigens when compared to the HLA frequencies usually found in North Western Europe. In case of severe haematological disorders of these individuals, allogeneic stem cell transplantation may be the treatment of choice. However, finding appropriate histocompatible hematopoietic stem cell donors continues to be a major challenge. If no matching sibling donors are available, there are only few suitable donors with a similar genetic background available in international blood stem cell donor registries. The "BluStar.NRW" project aimed to recruit new blood and hematopoietic stem cell donors with a migration background and to noticeably increase the number of suitable donors for patients within this group. Since December 2017, a total number of 9100 blood and stem cell donors with a migration background were recruited and typed for this project. HLA typing for HLA-A, -B, -C, -DRB1, -DQB1, and -DPB1 was performed by Next Generation Sequencing. We assessed the proportion of rare alleles according to HLA frequency tables, as defined by a frequency of <1:1000. The rare HLA allele frequencies according to HLA frequency tables of the BluStar.NRW cohort were compared with a matched control donor cohort: Rare HLA-A, -B, -C, -DRB1 and -DQB1 alleles occurred three times more frequent than in the control group, but rare HLA-DPB1 alleles occurred more frequently in the control cohort. This difference was highly significant for all HLA alleles (p < 0.0001 for HLA-A, -B, -C, -DRB1, -DPB1; p = 0.0002 for HLA-DQB1). In addition, the distribution of rare alleles differed between the two groups. To date, 29 work-ups were initiated, 12 PBSC, one BM and three DLI were collected so far out of the BluStar.NRW cohort. The apheresis probability is twofold higher (0.18% vs. 0.07%) compared to the control group which clearly shows a serious medical need. However, 13 work-ups were cancelled in the BluStar.NRW donor cohort which represents an almost twice as higher cancellation rate (45% vs. 25%). This single registry analysis with a large sample cohort clearly indicates that hematopoietic stem cell donors with a migration background represent an adequate donor pool to serve patients of comparable ethnicity.

Posterior cervical haemangiopericytoma with intracranial and skull base extension. Diagnostic and therapeutic challenge of a rare hypervascular neoplasm.

Haemangiopericytomas are rare hypervascular tumors arising from pericytes. They may occur anywhere in the body, but posterior cervical location is rather uncommon. A case of posterior cervical haemangiopericytoma with posterior fossa and temporal bone extension is reported. Although the patient had undergone preoperative endovascular embolization and surgical resection on three separate occasions, control of the skull base extension was not successful. Following endovascular embolization combined with radiotherapy, the patient has been asymptomatic for 48 months. Angiographic features may help in differentiating haemangiopericytomas from other hypervascular lesions. Preoperative endovascular embolization is recommended due to the pronounced tendency for haemorrhage throughout biopsy and surgical procedures.

[Brain MR imaging of chronic alcoholism]. / Imagerie par résonance magnétique (IRM) des complications encéphaliques de l'alcoolisme.

Brain complications from chronic alcoholism (Wernicke encephalopathy, central pontine myelinolysis, Marchiafava-Bignami disease, Korsakoff's syndrome, hepatic encephalopathy, cerebellar atrophy, hemorrhagic and ischemic brain lesions) may be diagnosed by MR imaging.

[Siderosis of the brain and spinal cord. Report of two cases]. / Sidérose cérébrale et médullaire. A propos de deux observations.

Two cases of superficial siderosis of the brain and spinal cord with cochleovestibular and cerebellar symptoms are diagnosed on brain and spinal MRI scans. Low signal intensity lines are noted on the surface of the brainstem, cerebellum, spinal cord and within the interhemispheric and sylvian fissures. In one case, no brain or vascular malformation is identified; in the second case, two cavernous angiomas are noted on the MRI study. 3D CISS may visualize thickening of the cochleovestibular nerve.

[Epidermoid cyst of the fourth ventricle: four case reports]. / Les kystes épidermoïdes du quatrième ventricule: à propos de quatre cas.

Patients with epidermoid cyst of the fourth ventricle usually present with headaches and/or disequilibrium. These cysts are characterized by a focal lesion that is nearly isodense to CSF at CT and nearly isointense to CSF on T1W and T2W MR images. MRI using FLAIR and diffusion weighted images as well as 3D CISS acquisitions is useful to better characterize the lesions and their relation with the vermis, foramen magnum and CP angle cisterns. DWI images are useful for postsurgical evaluation of residual tumor. Extension of the cyst into the CP angle cisterns usually precludes complete surgical resection.

Plasma levels of D-dimer and circulating endothelial adhesion molecules in veno-occlusive disease of the liver following allogeneic bone marrow transplantation.

Veno-occlusive disease (VOD) of the liver is a frequent and life-threatening complication of BMT. Recently, successful treatment by t-PA has been reported but has been compromised by fatal bleeding events. Therefore, t-PA application should be restricted to patients with severe VOD. However, moderate and severe forms of VOD are difficult to distinguish in early stages. We analyzed plasma levels of cross-linked fibrin degradation products (D-dimer) and soluble endothelial adhesion molecules such as sE-selectin, sVCAM-1 and sICAM-1 in 10 consecutive patients undergoing allogeneic BMT to evaluate their use in identifying severe forms of VOD. During the observation period, 4 episodes of VOD occurred, 2 of which were fatal due to early onset of multiorgan failure. Concentrations of D-dimer generally increased after transplantation. However, there was an additional significant increase in D-dimer levels during severe VOD. Thus, D-dimer levels above 1000 microg/l were only found in 2 cases with severe VOD and fatal outcome. When compared with bilirubin concentrations substantial increases of D-dimers appeared earlier during the course of severe VOD. In contrast, VOD episodes were not accompanied by significant increases in sE-selectin, sVCAM-1 and sICAM-1 levels. It is concluded that measurement of D-dimer concentrations may aid accuracy to the early diagnosis of severe VOD.