[Inherited primitive and secondary polycythemia]. / Polyglobulies héréditaires primitives et secondaires.

Myeloproliferative disorders and secondary polycythemia cover most of the polycythemia cases encountered in daily practice. Inherited polycythemias are rare entities that have to be suspected when the classical causes of acquired polycythemia have been ruled out. Recent advances were made in the understanding of these pathologies, which are still little known to the physicians. This review reports the state of knowledge and proposes an algorithm to follow when confronted to a possible case of inherited polycythemia.

Thymoma associated with autoimmune diseases: 85 cases and literature review.

OBJECTIVES: To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma. DESIGN: Pathological records from three university hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD. RESULTS: 47/85 (55%) cases of thymoma had AD, including myasthenia gravis (MG) (n=33), Hashimoto's thyroiditis (n=4), Isaac's syndrome (n=3), Morvan syndrome (n=2), pure red cell aplasia (n=2), systemic lupus (n=2), lichen planus (n=2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good's syndrome, pemphigus, autoimmune hepatitis, Graves' disease, limbic encephalitis, and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median duration of follow-up after surgery was 60 months (40-78 months). In 32 patients, the diagnosis of AD preceded the diagnosis of thymoma, in 9 patients, thymoma was diagnosed at the same time as the AD and 7 patients had been operated on when they developed an AD. We found a significative difference on the Masaoka stage between the MG patients and the patients who present another AD (p=0.028). No risk factor for developing an AD after thymectomy was identified. CONCLUSIONS: We describe here the long-term follow-up of a large series of AD related to thymoma. Our results confirm previous data concerning AD occurrence in patients with thymoma and suggest that preexisting autoimmunity is not a risk factor for developing autoimmune manifestations after thymectomy.

[Combined variable immunodeficiency with unusal features. A case report]. / Présentation atypique d'un déficit immun commun variable. À propos d'un cas.

INTRODUCTION: The association granulomatosis - combined variable immunodeficiency (CVID) - is well known from the clinicians. However, the association with a large granular lymphocyte (LGL) leukemia has not been yet reported. CASE REPORT: We report a 50-year-old woman, followed for CVID associated with a granulomatous disease. During the follow-up, the patient developed a granulomatous lymphocytic interstitiel lung disease (GLILD). Secondarily, she presented a LGL leukemia. CONCLUSION: To our knowledge, this is the first reported case of an association between CVID and LGL leukemia.

[Auto-immune diseases and cancers. Second part: auto-immune diseases complicating cancers and their treatment]. / Maladies auto-immunes et cancers. Deuxième partie: maladies auto-immunes au cours des cancers et de leur traitement.

Autoimmune diseases may reveal or occur during the course of a neoplasia or its treatment. Autoimmune cytopenia, especially haemolytic anaemia, is common in lymphoproliferative disorders such as chronic lymphoid leukemia. The link between cancer and myositis is well established. Dermatomyositis is associated with an increased relative risk of cancer of 3.4 to 4.4. A combination of detection of antibodies against p155 and TEP-computed tomography may be the best approach to ascertain the presence of occult malignancy in patients with dermatomyositis. A cutaneous or a systemic vascularitis may reveal a cancer, most often a haematological malignancy such as hairy cell leukemia. Paraneoplastic polyarthritis have been described in particular with adenocardinoma of the lungs. Underlying neoplasia should be considered in male smokers patients with new onset polyarthritis and poor health status. The prevalence of autoimmune conditions in myelodysplastic syndromes is 10 to 30%. Vasculitis and relapsing polychondritis are the most commonly reported manifestations. Immune manifestations can also be related to treatment. The most common treatment complications are autoimmune haemolytic anaemia with fludarabine and thyroiditis related to interferon and cervical radiotherapy.

[Complicated course of eosinophilic gastroenteritis: a case report and literature review]. / Complications de la gastroentérite à éosinophiles: une observation et revue de la littérature.

INTRODUCTION: Eosinophilic gastroenteritis is an unusual disease characterized by an eosinophilic infiltration of the gastrointestinal tract. The esophageal location of this disorder is uncommon and is usually revealed by dysphagia. Diagnosis is obtained by histology during endoscopy after exclusion of differential diagnosis. Treatment is based on systemic corticosteroids, which improve dramatically symptoms and endoscopic lesions. CASE REPORT: We report an 88-year-old man who presented eosinophilic gastroenteritis with esophageal injury complicated by gastrointestinal haemorrhage and fistule. CONCLUSION: Eosinophilic gastroenteritis may have a potentially unfavourable outcome. The treatment of complicated forms is not codified and often empirical.

[Autoimmune diseases and cancers. Part I: cancers complicating autoimmune diseases and their treatment]. / Maladies auto-immunes et cancers. Première partie : cancers au cours des maladies auto-immunes et de leur traitement.

The link between systemic disease and cancer is not fortuitous. An autoimmune disease can represent the starter for developing a non-Hodgkin lymphoma. This is particularly true for Sjögren's syndrome that is associated with the highest risk of lymphoma (odds ratio up to 44). Other systemic autoimmune diseases concerned are systemic lupus with an odds ratio of 4.5 and rheumatoid arthritis with an odds ratio of 2 to 3. It is now well established that high inflammatory activity, rather than immunosuppressive treatment, is the major risk determinant. The association between solid cancer and autoimmune systemic disease is uncommon and concerns in particular scleroderma and lung cancer. Concerning biotherapy-induced cancers, there is no demonstrated increased risk with anti-TNFα (except for cutaneous carcinoma and maybe melanoma) or with tocilizumab and abatacept even if studies with longer follow-up are needed at least for these two last drugs.

[Understand the neurodevelopment of language: a necessity to prevent learning disabilities in children]. / Comprendre le neurodéveloppement du langage, une nécessité pour prévenir les troubles des apprentissages de l'enfant ?

Clinical and radiological knowledge of language development in the former premature infant compared to the newborn allows us to argue for exploration of the sensorimotor co-factors required for proper language development. There are early representations of the maternal language in the infant's visual, auditory, and sensorimotor areas, activated or stabilized by orofacial and articulatory movements. The functional architecture of language is different for vulnerable children such as premature infants. We have already mentioned the impact of early dysfunction of the facial praxis fine motor skills in this population presenting comprehension disorders. A recent meta-analysis confirms the increasing difficulty of understanding between 3 and 12 years, questioning the quality of the initial linguistic processes. A precise analysis of language, referenced from 3 years of age, should be completed by sensorimotor tests to assess possible constraints in automating neurolinguistic foundations. The usual assessment at this age can exclude sensory disturbances and communication and offers guidance and socialization. However, a recent study shows the ineffectiveness of "language-reinforced immersion" at 2 and 3 years in a population of vulnerable children. The LAMOPRESCO study of language and motor skills in the premature infant (National PHRC 2010) has assessed language and sensorimotor skills of preterm-born (<33 weeks) 3.5-year-old children without cerebral palsy. Fragile children were randomized into 2 groups, 1 stimulated by a specific individual protocol, the other given guidance. The primary endpoint was phonology, assuming that it is composed of very early good-quality sensorimotor integration stabilized by the child's oral facial motor skills before 5 years of age. This developmental integrative dynamic validates the "motor theory of speech perception." Early and accurate assessment of language and the patient's constraints should differentiate and specify management strategies for all children, whatever their background and pathologies.

Gelatin-thrombin matrix: a new and simple way to manage recurrent epistaxis in hematology units.

Introduction. In case of thrombopenia and/or thrombopathy, epistaxes are very difficult to manage. Case Series. Two patients, one with a thrombocytopenia, the other with a thrombopathy, were hospitalized because of repeated active epistaxes after failure of packing. Both patients were successfully treated with an application of Surgiflo without side effects and left the hospital without recurrence of epistaxis. Discussion. Being a subject of many studies dealing with epistaxis, Surgiflo is a simple treatment that seems to be very effective and without side effects to treat acute epistaxis in fragile patients with coagulation disorders. Prospective studies of tolerance and efficiency in such situations should be performed.

[Granulomatosis and primary immunodeficiency in adulthood]. / Granulomatoses au cours des déficits immunitaires primitifs de l'adulte.

Primary immunodeficiency with granulomatosis in the adulthood mainly concern common variable immunodeficiency (CVID). Hypogammaglobulinemia in the adulthood is usually related to a secondary immunodeficiency. When a patient presents with the association of a hypogammaglobulinemia and a granulomatosis, an opportunistic infection must first be ruled out. For unknown reasons, about 10% of the patients affected by CVID also present with granulomatosis. Lesions usually affect the pulmonary tract or the mediastinum. Half of these patients are also affected by an autoimmune cytopenia. Treatment is not codified. Severe pulmonary complications can occur in about 50% of the patients.

[Plasmacytosis and dengue fever: an underestimated abnormality?]. / Plasmocytose sanguine et dengue: une anomalie biologique sous-estimée?

INTRODUCTION: A significant peripheral blood plasmacytosis is a rare finding associated with viral infections. We reported five consecutive cases of dengue virus infection, with circulating plasma cells. CASE REPORTS: Three women and two men, aged 26 to 75 years, had returned from French West Indies less than one week before the onset of the symptoms (mean: 2.5 days). The transient blood plasmacytosis was variable in intensity (0.1 to 0.8 G/L) with a maximal level between the fourth and the seventh day following the onset of the symptoms, and was associated in four patients, with activated lymphocytes and lympho-plasma cells. CONCLUSION: Reactive plasmacytosis during dengue fever is common and probably underestimated because it is transient and only identified by careful microscopic examination of a blood smear. Plasmacytosis could be explained by the intensity of the immunological response and the production of large amount of interleukins.

[Delayed haemolytic transfusion reaction due to anti-JK1 antibody]. / Accident hémolytique retardé post-transfusionnel par allo-immunisation anti-JK1.

INTRODUCTION: The sensitivity of the detection of irregular antibodies (DIA) is one of the fundamental basis of transfusion safety. The production of alloantibodies is the first cause of adverse events following transfusion. CASE REPORT: We report a 77-year-old woman who was transfused and presented with a delayed haemolytic anemia due to anti-JK1 alloimmunization. This event highlights the limits of DIA performed before a transfusion, the hazard of this specific type of antibody and the difficulties of the diagnosis of haemolytic anaemia. The preventive measures necessary to avoid this undesirable effect are reminded. CONCLUSION: Despite the sensitive routine test method, the anti-JK1 antibodies could be missed. We should keep in mind the possibility of an anaemia due to alloantibodies we confronted to an unexplained haemolytic episode.

[Ischemic stroke as the first manifestation of Wegener's granulomatosis]. / Une manifestation inaugurale inhabituelle de la granulomatose de Wegener : l'accident vasculaire cérébral ischémique.

The most characteristic clinical manifestations of Wegener's granulomatosis are upper and lower respiratory tract and renal involvement. The central nervous system manifestations are uncommon and occur usually late in the course of the disease. We report a 48-year-old man who presented with an ischemic stroke as the first manifestation of Wegener's granulomatosis. Wegener's granulomatosis should be considered in the differential diagnosis of ischemic stroke even in the absence of extraneurological involvement.

[Thyrotoxic hypokaliemic periodic paralysis revealing Graves' disease in a male Caucasian]. / Paralysie périodique thyréotoxique hypokaliémique révélatrice d'une maladie de Basedow chez un caucasien.

OBJECTIVES: Hypokaliemic thyrotoxic periodic paralysis (TPP) is an uncommon complication of hyperthyroidism. Mostly described among Asian patients, it is rare in other ethnic groups, in particular in Caucasian people. CASE REPORT: We present the case of a Caucasian male admitted to our unit after several paretic episodes. Tachycardia, goiter and mild proptosis led to the diagnosis of Graves' disease. CONCLUSION: Rare in the Caucasian population, TPP involves dysfunction of the NA-K-ATPase pump. Beta-blockers should be associated with medical or surgical treatment of hyperthyroidism.

[Etiology and prognosis of highly elevated C-reactive protein levels (≥500 mg/L): a retrospective study about 168 measures in a series of 113 patients]. / Profil étiologique et pronostique des valeurs extrêmes (≥500 mg/L) de protéine C-réactive : étude rétrospective de 168 valeurs chez 113 patients.

PURPOSE: The C-reactive protein (CRP) is a useful inflammatory marker with a rapid kinetics during the inflammatory process. The objective of this study was to determine the etiology and prognosis of extremely elevated CRP values greater or equal to 500 mg/L. METHODS: We performed an exhaustive retrospective study from January 2004 to July 2009, in a general hospital, of all patients with a CRP value above 500 mg/L, admitted in all clinical departments. Clinical data were collected by a single observer using a standardized questionnaire. RESULTS: One hundred and sixty-eight CRP values greater or equal to 500 mg/L were identified amongst 106,758 tests (0.16%) corresponding to 113 patients: 51% were men and their mean age was 59.5 years. Mean CRP value was 561 mg/L (500-772). An immunocompromised condition was observed in 52% of the patients. All but 13 patients presented an infectious disease. Microbiological analysis of the infected patients identified 59 Gram-positive cocci (20 Staphylococcus spp., 35 Streptococcus spp. including 21 Streptococcus pneumoniae), two Gram-negative cocci, 48 Gram-negative bacilli (including 19 Escherichia coli), three Gram-positive bacilli, 16 fungal infections, one viral infection. Site of infection was respiratory in 63%, urinary in 17% and abdominal in 16%. At day 30, mortality rate was 27% and only 41% of the patients were discharged at home. CONCLUSION: CRP value above 500 mg/L is highly related to bacterial infections, without over-representation of a given microorganism. One-month mortality is high (27%).

[Seriousness of AL amyloidosis of the digestive system]. / Gravité de l'amylose AL de localisation digestive.

Systemic amyloidosis usually does not spare the digestive tract but its involvement is rarely symptomatic. The clinical manifestations are not specific. We report a 64-year-old patient, presenting with a weight loss related to an AL amyloidosis. The amyloidosis was apparently limited to the digestive tract. We discuss the various presentations of the digestive amyloidosis and we insist on the seriousness of this localization.