The humoral response of mRNA COVID-19 vaccine in hematological diseases: The HEMVACO study.

Objectives: The HEMVACO study evaluated the humoral response after mRNA anti-SARS-CoV-2 vaccination in an hematological cohort.

Methods: HEMVACO was a prospective, multicentric study registered in ClinicalTrials.gov, number NCT04852796.

Inflammatory optic neuropathy in granulomatosis with polyangiitis can mimick isolated idiopathic optic neuritis.

Objective: We describe a clinico-radiological presentation of inflammatory optic neuropathy that mimicked optic neuritis.

Methods: Retrospective single-center case series and literature review of optic neuropathy without orbital pseudotumor.

Results: Five local patients fulfilled the inclusion criteria.

Early discontinuation of empirical antibacterial therapy in febrile neutropenia: the ANTIBIOSTOP study.

Introduction: Immediate empirical antibiotic therapy is mandatory in febrile chemotherapy-induced neutropenia, but its optimal duration is unclear, especially in patients with fever of unknown origin (FUO).

Objectives: The primary objective of this 20-month prospective observational study was to evaluate the feasibility and safety of short-term antibiotic treatment in afebrile or febrile patients exhibiting FUO, irrespective of their neutrophil count. The secondary objective was to describe the epidemiology of all episodes of febrile neutropenia.

Observational Study of a French and Belgian Multicenter Cohort of 23 Patients Diagnosed in Adulthood With Mevalonate Kinase Deficiency.

The aim of this study was to describe the clinical and biological features of Mevalonate kinase deficiency (MKD) in patients diagnosed in adulthood. This is a French and Belgian observational retrospective study from 2000 to 2014. To constitute the cohort, we cross-check the genetic and biochemical databases.

Skin nodules in a patient with acute lymphoblastic leukaemia.

Opportunistic infections cause a significant morbidity and mortality in immunocompromised patients. We describe the case of a patient with skin fusariosis and a probable cerebral toxoplasmosis after UCB stem cell transplantation for B-cell acute lymphoblastic leukaemia. Fusarium species (spp) infections are difficult to treat.

Mononeuritis multiplex in a patient with B-cell prolymphocytic leukaemia: a diagnostic challenge.

B-cell prolymphocytic leukaemia (BPLL) is a haematological malignancy defined as lymphocytosis and splenomegaly with >55% circulating cells being clonal prolymphocytes of B-cell origin. The evolution of this disease is more aggressive than chronic lymphocytic leukaemia. We reported a case of a 62-year-old man with BPLL who, on treatment, attained cytological, immunophenotypic and complete cytogenetic remission.

Efficacy and safety of pegylated-interferon α-2a in myelofibrosis: a study by the FIM and GEM French cooperative groups.

Myeloproliferative neoplasm-related myelofibrosis is associated with cytopenic or proliferative phases, splenomegaly and constitutional symptoms. Few effective treatments are available and small series suggested that interferon could be an option for myelofibrosis therapy. We performed a retrospective study of pegylated-interferon α-2a (Peg-IFNα-2a) therapy in myelofibrosis.

Identification of patients with indolent B cell lymphoma sensitive to rituximab monotherapy.

The potential predictive value of tumor bulk, genetic, and immunological variants in patients with low-grade non-Hodgkin's lymphoma to respond to treatment with rituximab (RTX) monotherapy was evaluated. Thus, the value of assessing the effect of 18-fluoro-desoxy-D-glucose (FDG) uptake on PET scan, polymorphisms in Fc gamma receptor (FcγR) IIIa-158, FcγRIIa-131, and C1qA-276 genes in predicting the response to treatment were evaluated in 50 low-grade non-Hodgkin's lymphoma patients. The influence of RTX pharmacokinetics, plasma levels of the B cell-activating factor (BAFF), and human antichimeric antibodies was also investigated.

[Cutaneous periarteritis nodosa recurring over a period of 30 years in streptococcal infections and progressing toward systemic vasculitis].

Introduction: Periarteritis nodosa (PAN) is a form of vasculitis affecting the small and medium-sized arteries. Below, we report a case of cutaneous PAN relapsing in streptococcal infections over a period of 30 years and progressing towards systemic vasculitis.

Case Report: A 35-year-old man was hospitalised for a retro-pharyngeal access associated with fever, arthralgia, myalgia and inflammatory subcutaneous nodules.

[Voluminous nodular splenomegaly in Gaucher disease: a case report].

Patients affected by type 1 Gaucher disease (an autosomal recessive inheritance lysosome storage disorder) develop nodular splenomegaly in 20 to 30% of cases where imiglucerase therapy proves ineffective. The lack of response to imiglucerase therapy on spleen nodules could be an indication of the existence or development of a malignant spleen. We report a 47-year-old man with Gaucher disease who presented with a voluminous splenic nodule, in whom therapy was delayed.

[Epidemiology of candidemia: a one-year prospective observational study in the west of France].

Objective: A one-year prospective, observational study was conducted in the west of France, to evaluate the epidemiology of candidemia.

Method: During the year 2004, each patient with at least one blood culture yielding Candida sp. was included.

[Acquired factor XI inhibitor and chronic lymphocytic leukemia].

Autoimmune phenomena, most frequently autoimmune hemolytic anemia, is a well-known complication of lymphoproliferative diseases. We report a very rare association of a chronic lymphocytic leukemia with an acquired factor XI inhibitor. A 87-year-old man presented with auto-immune hemolytic anemia.

A conspicuous role for B cells In Sjögren's syndrome.

Although the relative contributions of T cells and B cells in Sjögren's syndrome (SS) are far from being settled, recent studies have suggested a crucial role for B cells in its pathophysiology. Early investigations have focused on the ability of B cells to produce autoantibodies, and new studies have enlarged the range of their functions. For example, beyond the paradigm that T lymphocytes maintain strict control over B cells, the latter cells are now acknowledged to solicit their own help from the former cells and release a flurry of cytokines.

BAFF-modulated repopulation of B lymphocytes in the blood and salivary glands of rituximab-treated patients with Sjögren's syndrome.

Objective: Treatment with rituximab depletes B cells from the peripheral blood (PB) and salivary glands (SGs) of patients with primary Sjögren's syndrome (SS). The purpose of this study was to track the repopulation of B cell subsets in PB as well as their subsequent homing into SGs in patients with primary SS treated with rituximab.

Methods: A series of 4-color flow cytometry experiments delineated B cell subsets in 15 patients with primary SS.

Aberrant expression of BAFF by B lymphocytes infiltrating the salivary glands of patients with primary Sjögren's syndrome.

Objective: To identify the cells that produce BAFF in the salivary glands of patients with primary Sjögren's syndrome (SS), and to analyze BAFF receptor expression by local T and B lymphocytes.

Methods: We used 3 methods to identify the source of BAFF: in situ hybridization of the transcripts for BAFF combined with staining of membrane markers, regular and real-time reverse transcription-polymerase chain reaction (RT-PCR) of cultured epithelial cells, and RT-PCR of sorted single-cell T and B lymphocytes eluted from salivary glands. Cells expressing TACI, BCMA, and B lymphocyte stimulator receptor 3 (BR-3) were disclosed by combining each specific staining of the receptors with each specific staining of the cells.

Identification of transitional type II B cells in the salivary glands of patients with Sjögren's syndrome.

Objective: To identify B cell subpopulations participating in the lymphocyte infiltrate of salivary glands from patients with primary Sjögren's syndrome. A special emphasis was placed on those B lymphocytes included in the ectopic germinal centers (GCs).

Methods: The presence of B cells in salivary glands and their polyclonality were ascertained by phenotyping and reverse transcription-polymerase chain reaction in salivary gland samples from 18 patients.