Severe auto-immune hemolytic anemia in a fingolimod-treated multiple sclerosis patient.

Journal ArticleSCOPUS: le.jinfo:eu-repo/semantics/publishe

Induction de tolérance: De la délétion à la régulation

info:eu-repo/semantics/publishe

Alloreactive IL-17 production escapes to FOXP3posTreg mediated suppression

info:eu-repo/semantics/nonPublishe

Chronic myeloid leukemia may no longer be a contraindication to lung transplantation

SCOPUS: le.jinfo:eu-repo/semantics/publishe

Targeted treatment options in mastocytosis

Mastocytosis refers to a heterogeneous group of disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin (cutaneous mastocytosis when only in the skin, CM) or in various organs (systemic mastocytosis, SM). This leads to a wide variety of clinical manifestations resulting from excessive mediator release in CM and benign forms of SM (indolent SM, ISM) and from tissue mast cell infiltration causing multiorgan dysfunction and failure in more aggressive subtypes (aggressive SM, ASM, or mast cell leukemia). In addition, SM may be associated with hematological neoplasms (AHN). While treatment of ISM primarily aims at symptom management with anti-mediator therapies, cytoreductive and targeted therapies are needed to control the expansion of neoplastic mast cells in advanced forms of SM, in order to improve overall survival. Mast cell accumulation results from a gain-of-function mutation (mostly the D816V mutation) within the KIT tyrosine kinase domain expressed by mast cells and additional genetic and epigenetic mutations may further determine the features of the disease (ASM and AHN). Consequently, tyrosine kinase inhibitors and targeted therapies directed against the oncogenic signaling machinery downstream of KIT are attractive therapeutic approaches. A better understanding of the relative contribution of these genetic and epigenetic events to the molecular pathogenesis of mastocytosis is of particular interest for the development of targeted therapies and therefore to better choose patient subgroups that would best benefit from a given therapeutic strategy.SCOPUS: re.jinfo:eu-repo/semantics/publishe

An Unusual Case of Delayed Hemolytic Transfusion Reaction With Hyperhemolysis Syndrome Due to Anti-Jkb and Anti-Fya Alloantibodies.

Delayed hemolytic transfusion reaction (DHTR) is a complication appearing a few days to weeks due to alloimmunization following packed red blood cells (RBCs) transfusion, a pregnancy, or transplantation. Hyperhemolysis syndrome (HS) is a severe form of DHTR defined by a drop of hemoglobin to a level lower than before the transfusion, reflecting a destruction of the patient's own RBCs not presenting the targeted antigen as well as the transfused RBCs. Usually seen in sickle cell disease (SCD) patients, HS remains very rare in patients without a hematologic disorder. We report the case of an 82-year-old Caucasian woman who presented with a DHTR with HS after being transfused packed RBC twice in the context of rectal bleeding. The patient was not known for any hemoglobinopathy and did not have a history of massive transfusions nor multiple pregnancies putting her at risk of alloimmunization. Our patient developed anti-C, anti-Fya and anti-Jkb antibodies, known to be harmful antibodies. First line of treatment after avoidance of further transfusions is intravenous immunoglobulins for 3 to 5 days and high-dose corticosteroids. Exceptional in the non-SCD population, this complication should be recalled by clinicians as it can be fatal if not treated appropriately. We performed a review of the literature using the words "delayed hemolytic transfusion reaction" and "hyperhemolysis syndrome" for similar cases. Finally, we describe how to diagnose, manage, and prevent this potentially fatal complication, which is still underrecognized even within the SCD population.info:eu-repo/semantics/publishe

Recommendations on the use of ruxolitinib for the treatment of myelofibrosis

Objectives: Myelofibrosis (MF) is a severe disease, with decreased life expectancy and heavy symptom burden. Ruxolitinib is the only approved pharmacotherapy for the treatment of MF patients. In Belgium, ruxolitinib is only reimbursed for MF patients with splenomegaly for whom the disease is categorized as intermediate-2 or high risk. The improvement of symptoms without spleen volume reduction is not considered sufficient to continue treatment. The aim of this manuscript is to provide guidance for the safe and effective administration of ruxolitinib, considering the particularities of the Belgian reimbursement criteria. Methods: Our recommendations are based on a consensus reached during two meetings, where available data and observations derived from clinical experience were discussed. Results and discussion: We recommend changing the current Belgian reimbursement conditions to include the evaluation of disease-related symptoms along with splenomegaly to decide whether ruxolitinib treatment should be continued or not. Indeed, the decrease in disease-related symptoms seems to be an equally important parameter as the decrease in splenic volume in the evaluation of the response to ruxolitinib. We also advocate for the treatment with ruxolitinib of MF patients in lower-risk categories with severe disease-related symptoms, as this drug could greatly improve their quality of life. Optimization of the ruxolitinib dose is recommended to avoid an unnecessary decrease in platelet count or hemoglobin that may jeopardize treatment continuation. Conclusion: With the aim to optimize the treatment of MF patients, the Belgian regulation for ruxolitinib should be revised in terms of reimbursement criteria, dose titration, stopping rules, and patient follow-up.SCOPUS: ar.jinfo:eu-repo/semantics/publishe

Targeted Treatment Options in Mastocytosis

Mastocytosis refers to a heterogeneous group of disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin (cutaneous mastocytosis when only in the skin, CM) or in various organs (systemic mastocytosis, SM). This leads to a wide variety of clinical manifestations resulting from excessive mediator release in CM and benign forms of SM (indolent SM, ISM) and from tissue mast cell infiltration causing multiorgan dysfunction and failure in more aggressive subtypes (aggressive SM, ASM, or mast cell leukemia). In addition, SM may be associated with hematological neoplasms (AHN). While treatment of ISM primarily aims at symptom management with anti-mediator therapies, cytoreductive and targeted therapies are needed to control the expansion of neoplastic mast cells in advanced forms of SM, in order to improve overall survival. Mast cell accumulation results from a gain-of-function mutation (mostly the D816V mutation) within the KIT tyrosine kinase domain expressed by mast cells and additional genetic and epigenetic mutations may further determine the features of the disease (ASM and AHN). Consequently, tyrosine kinase inhibitors and targeted therapies directed against the oncogenic signaling machinery downstream of KIT are attractive therapeutic approaches. A better understanding of the relative contribution of these genetic and epigenetic events to the molecular pathogenesis of mastocytosis is of particular interest for the development of targeted therapies and therefore to better choose patient subgroups that would best benefit from a given therapeutic strategy

Eosinophils and Allograft Rejection

info:eu-repo/semantics/publishe