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La rate occupe un rôle central dans le système de filtration et de défense sanguin. La pulpe rouge permet notamment l'élimination des globules rouges altérés, via son réseau microcirculatoire unique ; tandis que la pulpe blanche est un organe lymphoïde secondaire, directement connecté à la circulation sanguine, dont la spécificité est la défense contre les bactéries encapsulées via la production d'IgM « naturelles » dans la zone marginale. Une altération acquise de la fonction splénique (ou hyposplénisme) peut résulter de diverses maladies et/ou d'une splénectomie thérapeutique. L'hypo/asplénie se complique d'une susceptibilité accrue aux infections à germes encapsulés, mais un excès de risque de thrombose et d'hypertension pulmonaire est également rapporté après une splénectomie chirurgicale. La drépanocytose homozygote est la plus fréquente des maladies associée à une asplénie fonctionnelle. Cette dernière apparaît précocement, dans l'enfance, vraisemblablement du fait d'altérations ischémiques répétées dues à la falciformation des hématies. De plus, des complications spécifiques telles que l'hypersplénisme et les séquestrations spléniques aiguës peuvent survenir et menacer le pronostic vital. Nous proposons ici une mise au point sur le rôle et la physiologie de la rate, ce qui permettra de mieux comprendre la physiopathologie de l'atteinte splénique et ses conséquences au cours de la drépanocytose. The spleen filters blood cells and contributes to the immune defense. The red pulp clears the blood from altered red blood cells via its unique microcirculatory network ; while the white pulp is a secondary lymphoid organ, directly connected to the bloodstream, whose specificity is the defense against encapsulated bacteria through the production of "natural" IgM in the marginal zone. Various health conditions can cause acquired impairment of the splenic function (or hyposplenism) directly and/or through therapeutic splenectomy. Hypo/asplenia is complicated by an increased susceptibility to encapsulated germ infections, but an increased risk of thrombosis and pulmonary hypertension has also been reported after surgical splenectomy. Homozygous sickle cell disease is the most common disease associated with functional asplenia. The latter appears early in childhood likely through repeated ischemic alterations caused by the sickling of red blood cells. In addition, specific complications such as hypersplenism and acute splenic sequestration can occur and may be life-threatening. We provide here an update on the role and physiology of the spleen, which will allow a better understanding of the pathophysiology of spleen damage and its consequences in sickle cell disease. [ABSTRACT FROM AUTHOR]

The article presents a case report of a 26-year old patient suffering from the chronic pulmonary disease associated with an unusual dysgammaglobulinaemia. The patient had had chronic atopic dermatitis since approximately age one month. Shortly after the onset of the atopic dermatitis, his mother noted the first appearance of wheezing. Since that time, the patient has had multiple attacks of wheezing. At the age of 6 years, he had pneumonia for the first time. Since then he has had repeated bouts of pneumonia.

Competing Interests: Conflict of interest: None declared.

Background and Purpose: Ischemic stroke has been reported in various conditions associated with eosinophilia. FIP1L1-PDGFRA fusion ([Fip1-like 1-platelet-derived growth factor receptor alpha]; F/P) leads to the proliferation of the eosinophilic lineage and thus to a clonal hypereosinophilic syndrome that is highly responsive to imatinib., Methods: We previously reported on a nationwide retrospective study of 151 patients with F/P-associated clonal hypereosinophilic syndrome. Patients from this cohort with a clinical history of ischemic stroke (as well as 2 additional cases) were further analyzed to better define their clinical picture and outcomes., Results: Sixteen male patients (median age, 51 [43–59] years) with low-to-intermediate cardiovascular risk were included. Median National Institutes of Health Stroke Scale was 4 (range, 1–6). Most cerebral imaging disclosed multiple bilateral infarctions of watershed distribution (69%). Despite frequent cardiac involvement (50%), cardiac thrombus was evidenced in a single patient and, according to the TOAST classification (Trial of ORG 10172 in Acute Stroke Treatment), 62.5% of strokes were presumably of undetermined etiology. Among the 15 patients treated with imatinib, and after a median follow-up of 4.5 years, stroke recurred in only 3 patients (consisting of either cardio embolic or hemorrhagic events, unrelated to the first episode)., Conclusions: F/P+ clonal hypereosinophilic syndrome is a diagnosis to consider in patients with unexplained ischemic stroke and hypereosinophilia (especially in the setting of multiple cortical borderzone distribution) and warrants prompt initiation of imatinib.

Primary Sjögren's syndrome (pSS) is a common systemic autoimmune disease characterised by exocrinopathy resulting in dryness of the mouth and eyes, unexplained fatigue and diffuse pain. Neurological involvement is uncommon in pSS, involving the central nervous system in 2-5% of cases and more frequently the peripheral nervous system in 5-15% of cases. The diagnosis of pSS is to be considered when confronted with symptoms such as mouth and eye dryness, fatigue and pain, the most frequent of pSS symptoms. Objective measures of oral and eye dryness may help assert the diagnosis of pSS, as well as ACR/EULAR criteria. Differential diagnoses have to be excluded in patients exhibiting neurological symptoms, such as cryoglobulinaemic vasculitis or multiple sclerosis, before considering a neurological involvement specific to pSS. The treatment of these neurological manifestations takes into account different parameters, such as the presence of cryoglobulinaemic vasculitis, the severity of the symptoms, a rapidly progressing evolution and the failure of previous symptomatic treatments.

Fraction A, a concentrate derived from short ragweed extract by (NH4)2SO4 precipitation, contains all major allergens including antigen E. Therapeutic efficacy and safety of this alum-adsorbed product was evaluated in a multicenter double-blind clinical trial by comparing it to placebo in 131 randomly matched ragweed hay fever patients who were not treated for at least 1 year. Therapeutic response to fraction A injections was more significant than placebo (p less than 0.05) by investigators' evaluation, by average daily symptom score (1.097 vs. 1.378, p less than 0.05) during the peak ragweed season, or by average proportion of days in which medication was required (0.411 vs. 0.584, p = 0.01). Fraction A patients exhibited a significantly higher (p less than 0.0005) increase in hemagglutinating titer after treatment than placebo controls. Radioallergosorbent binding, evaluated in a subgroup of 31 patients before and after the ragweed season, was unchanged or decreased in 12 of 15 patients treated with fraction A. In contrast, 9 of 16 placebo-treated patients showed an increase in this index. Systemic effects were noted after 42 (1.5%) of 2.641 injections, while local reactions occurred at the rate of 2.3%. It is concluded that a single course of preseasonal immunotherapy wih alum-adsorbed fraction A is effective and relatively safe.

The serums of dogs that are allergic to ragweeed can passively sensitize normal dogs. Cutaneous reactions or systemic anaphylaxis may be produced by appropriate challenge with ragweed extract. Canine antiserum produced by immunization of normal dogs with ragweed extract is shown to inhibit these reactions of the passively sensitized dogs.