Sixth Åland Island Conference on von Willebrand disease.

INTRODUCTION: The sixth Åland Islands Conference on von Willebrand disease (VWD) on the Åland Islands, Finland, was held from 20 to 22 September 2018. AIM: The meeting brought together experts in the field of VWD from around the world to share the latest advances and knowledge in VWD. RESULTS AND DISCUSSION: The topics covered both clinical aspects of disease management, and biochemical and laboratory insights into the disease. The clinical topics discussed included epidemiology, diagnosis and treatment of VWD in different countries, management of children with VWD, bleeding control during surgery, specific considerations for the management of type 3 VWD and bleeding control in women with VWD. Current approaches to the management of acquired von Willebrand syndrome were also discussed. Despite significant advances in the understanding and therapeutic options for VWD, there remain many challenges to be overcome in order to optimise patient care. In comparison with haemophilia A, there are very few registries of VWD patients, which would be a valuable source of data on the condition and its management. VWD is still underdiagnosed, and many patients suffer recurrent or severe bleeding that could be prevented. Awareness of VWD among healthcare practitioners, including non-haematologists, should be improved to allow timely diagnosis and intervention. Diagnosis remains challenging, and the development of fast, simple assays may help to facilitate accurate and rapid diagnosis of VWD.

Effects of molgramostim, filgrastim and lenograstim in the treatment of myelokathexis.

Myelokathexis is a very rare form of chronic hereditary neutropenia resulting from impaired neutrophil releasing mechanism in the bone marrow. The recombinant human granulocyte-macrophage (molgramostim) and granulocyte (filgrastim, lenograstim) colony stimulating factors release the mature granulocytes from the bone marrow. We describe a 43-year-old woman suffering from myelokathexis, with the absolute neutrophil count ranging between 0.03 and 1.35 × 109/L. In the period before the introduction of cytokines, the patient had more than 80 major infectious episodes. Since 1991, infections in this patient have been treated with cytokines, given in conjunction with antibiotics. Initially, she received molgramostim in a daily dose of 5 µg/kg subcutaneously, which stimulated the release of granulocytes from her bone marrow, thereby allowing successful treatment of infection. After the development of hypersensitivity, molgramostim was substituted with filgrastim. Finally, lenograstim was given a trial. With all three cytokines, the patient's neutrophil count always attained normal values already 4 hours after subcutaneous application of the drug in a dose of 5 µg/kg, the highest neutrophil levels were measured at 24 hours post-injection, and the neutrophil count was again close to the baseline value 72 hours after the treatment. A slight neutropenia was present 48 hours after the application of filgrastim. We believe that all three cytokines are equally effective in increasing the neutrophil count in venous blood of patients with myelokathexis.

Association between platelet-related hemostasis impairment and functional iron deficiency in hemodialysis patients.

Hemostasis impairment and iron deficiency are relatively frequent in hemodialysis patients. Both conditions may contribute to anemia. The aim of our study was to explore possible associations between hemostasis impairment and iron deficiency by employing recently introduced methods for measurement of both conditions. Sixty-three hemodialysis patients were studied, with 30 age-matched, healthy controls. Hemostasis impairment was detected by in vitro closure time tests (collagen/epinephrine cartridge: CEPI; collagen/adenosine diphosphate (ADP) cartridge: CADP), whereas (functional) iron deficiency was measured by reticulocyte hemoglobin content (CHr) and the percentage of hypochromic red cells (HRC). We found that the patient group (N=14) with functional iron deficiency (CHr<29) had significantly delayed in vitro closure times in comparison to the patients (N=49) without functional iron deficiency. Furthermore, both types of closure time (CEPI and CADP) correlate highly significantly with CHr (P=0.002, and P=0.001). Such an association was not observed between in vitro closure time and HRC. We found a significant correlation between hemostasis impairment (measured by in vitro closure time) and iron deficiency (measured by CHr) in hemodialysis patients. This correlation has not previously been reported. It seems that in hemodialysis patients the hemostasis impairment affects (functional) iron deficiency, most likely by facilitating excessive blood loss and consequent iron deficiency. Thus, it appears that a delayed in vitro closure time along with decreased CHr may identify hemodialysis patients who suffer (occult) blood loss and/or excessive blood loss during hemodialysis procedure. The clinical value of this finding should be tested in larger studies.