Platelet transfusion in adults: An update.

Many patients worldwide receive platelet components (PCs) through the transfusion of diverse types of blood components. PC transfusions are essential for the treatment of central thrombocytopenia of diverse causes, and such treatment is beneficial in patients at risk of severe bleeding. PC transfusions account for almost 10% of all the blood components supplied by blood services, but they are associated with about 3.25 times as many severe reactions (attributable to transfusion) than red blood cell transfusions after stringent in-process leukoreduction to less than 106 residual cells per blood component. PCs are not homogeneous, due to the considerable differences between donors. Furthermore, the modes of PC collection and preparation, the safety precautions taken to limit either the most common (allergic-type reactions and febrile non-hemolytic reactions) or the most severe (bacterial contamination, pulmonary lesions) adverse reactions, and storage and conservation methods can all result in so-called PC "storage lesions". Some storage lesions affect PC quality, with implications for patient outcome. Good transfusion practices should result in higher levels of platelet recovery and efficacy, and lower complication rates. These practices include a matching of tissue ABH antigens whenever possible, and of platelet HLA (and, to a lesser extent, HPA) antigens in immunization situations. This review provides an overview of all the available information relating to platelet transfusion, from donor and donation to bedside transfusion, and considers the impact of the measures applied to increase transfusion efficacy while improving safety and preventing transfusion inefficacy and refractoriness. It also considers alternatives to platelet component (PC) transfusion.

[Myelofibrosis: A review]. / Les myélofibroses.

Myelofibrosis is a BCR-ABL1-negative chronic myeloproliferative neoplasm that includes primary myelofibrosis, post-polycythemia vera myelofibrosis, and post-essential thrombocythemia myelofibrosis. It is characterized by stem cell-derived clonal proliferation that is often, but not always, accompanied by somatic mutations, which are classified into driver mutations (JAK2, CALR, or MPL), subclonal mutations and fibrosis on bone marrow biopsy. Myelofibrosis commonly demonstrates splenomegaly, constitutional symptoms, anemia, thrombocytosis, or thrombocytopenia. Patients may also be asymptomatic. Complications as thromboembolic or hemorrhagic events can reveal the disease. Primary myelofibrosis is the least common myeloproliferative neoplasm but is associated with poor survival and acute leukemic transformation. In contrast to the significant progress made in understanding the disease's pathogenesis, treatment for myelofibrosis remains largely palliative. The JAK2 inhibitor, ruxolitinib is not sufficient in eliminating the underlying myeloid progenitor clone, as disease inevitably returns with therapy discontinuation. Allogeneic hematopoietic stem cell transplantation is the only therapeutic option that offers potential cure. The development of novel treatment strategies aimed at slowing or even reversing disease progression, prolonging patient survival and preventing evolution to blast-phase are still lacking.

Bone marrow involvement in systemic lupus erythematosus.

BACKGROUND: Besides peripheral cytopenias, bone marrow abnormalities, such as fibrosis, pure red cell aplasia and aplastic anemia have been reported in patients with systemic lupus erythematosus (SLE), suggesting that bone marrow may be a 25 target organ in SLE. AIM: Our objective was to describe this bone marrow involvement. METHODS: This registry is a nationwide retrospective study. Centers provided data concerning medical history, SLE manifestations, type of hematologic disorder, treatments and outcome. Bone marrow aspirations and/or biopsies were transferred for centralized review. RESULTS: Thirty patients from 19 centers were included. Central hematologic manifestations comprised bone marrow fibrosis (n = 17; 57%), pure red cell aplasia (n = 8; 27%), myelodysplastic syndrome (n = 3; 10%), aplastic anemia and agranulocytosis (n = 1; 3% each). Bone marrow involvement was diagnosed concomitantly with SLE in 12 patients. Bone marrow biopsies showed fibrosis in 19 cases, including one case of pure red cell aplasia and one case of agranulocytosis and variable global marrow cellularity. Treatments included corticosteroids (90%), hydroxychloroquine (87%), rituximab (33%), intravenous immunoglobulins (30%), mycophenolate mofetil (20%) and ciclosporine (20%). After a median follow-up of 27 months (range: 1-142), 24 patients manifested complete improvement. No patient died. CONCLUSIONS: This registry comprises the largest series of SLE patients with bone marrow involvement. It demonstrates the strong link between SLE and bone marrow fibrosis. Patients with atypical or refractory cytopenia associated with SLE should undergo bone marrow examination to enable appropriate, and often effective, treatment. Long-term prognosis is good.

A Hospice and Palliative Care Bed Dedicated to Patients Admitted to the Emergency Department for End-of-Life Care.

A hospice and palliative care (PC) bed was created in 2006, located within a quiet area of our intensive care unit, in order to admit terminally ill patients sent to the emergency department (ED) for end-of-life care. We retrospectively analyze the records of the 342 terminally ill patients sent to the ED from 2007 to 2011. Among them, 176 (51.5%) were admitted to our hospice and PC bed, where 114 died. Besides, 99 (28.9%) of them died on stretchers in the ED. Our intervention led to a significant decrease in the number of terminally ill patients dying on stretchers in the ED. It also allowed both patients and families to have access to a more suitable environment.

Sarcoidosis and lymphoma: a comparative study.

AIM: To assess the clinical features and outcome of lymphoma when associated with sarcoidosis and to determine whether this association gives lymphoma a better prognosis. DESIGN: Multicentre retrospective cohort study. METHODS: Retrospective chart review. RESULTS: Twenty-one patients were included (9 males, 12 females). Median age at sarcoidosis diagnosis was 48 years (range: 24-68 years). In 14 cases, lymphoma occurred within a previously known sarcoidosis. Five patients received a concomitant diagnosis of sarcoidosis and lymphoma, whereas lymphoma preceded sarcoidosis in two patients. Three patients were diagnosed with Hodgkin's lymphoma and 18 patients with non-Hodgkin's lymphoma (diffuse large B-cell lymphoma (DLBCL) (n = 11), follicular lymphoma (n = 2), chronic lymphocytic leukemia/small lymphocytic lymphoma (n = 2), anaplastic large cell lymphoma ALK + (n = 1), angioimmunoblastic T-cell lymphoma (n = 1) and T-cell prolymphocytic leukemia (n = 1)). Thirteen patients were alive and in complete remission. Median age at the time of diagnosis of sarcoidosis was lower in patients with concomitant lymphoma compared with patients with sarcoidosis preceding lymphoma (34 years vs. 51 years, P = 0.01). Patients presenting with DLBCL associated with sarcoidosis were compared with DLBCL without sarcoidosis. No statistical difference was found in the risk of death or progression between the two groups (P = 0.685). CONCLUSIONS: We report here the largest series of lymphoma associated sarcoidosis patients. As opposed to previous studies, we observed a predominance of patients with DLBCL. Our study confirms the concept of the sarcoidosis-lymphoma syndrome. Large B-cell lymphoma does not have a better prognosis when associated with sarcoidosis.

[Newly diagnosed multiple myeloma: Do we need to propose thromboprophylaxis?]. / Myélome multiple de novo : faut-il proposer une prophylaxie antithrombotique ?

The incidence of venous thromboembolism in multiple myeloma depends on the disease characteristics that include recent diagnosis, persistent or recurrent multiple myeloma, patient characteristics, and the type of treatment received such as thalidomide or lenalidomide especially in combination with high-dose dexamethasone, or combined chemotherapy. Currently, recommendations could be challenged by the results of the first randomized study evaluating aspirin, low molecular weight heparins and vitamin K antagonists in the antithrombotic prophylaxis. The recent data from the literature show that it is not possible to propose a therapeutic management for venous thromboembolism prophylaxis in multiple myeloma and that the use of antithrombotic prophylaxis may not be mandatory.