[Haemophagocytic syndrome as a complication of acute pancreatitis during systemic lupus erythematosus]. / Syndrome hémophagocytaire compliquant une pancréatite aiguë dans le cadre d'un lupus érythémateux systémique.

BACKGROUND: Acute pancreatitis and haemophagocytic syndrome (HS) are rarely seen in systemic lupus erythematosus (SLE). PATIENTS AND METHODS: We report the case of a young female patient without any noteworthy prior history, who was hospitalised for abdominal pain associated with acute pancreatitis possibly related to SLE with associated haemophagocytic syndrome. DISCUSSION: Screening for cutaneous symptoms of lupus in patients with pancreatitis can help avoid diagnostic errors.

[Ulcerative colitis: exceptional consequence after rituximab therapy]. / La colite ulcérative : une conséquence exceptionnelle après traitement par rituximab.

INTRODUCTION: Possible adverse complications related to rituximab (RTX) are low, some of which are extremely rare. The authors describe one situation visibly waning exceptional treatment with RTX for SLE refractory to conventional therapies. COMMENT: The authors report a patient of 34 years followed for months for an illness in its bullous lupus, with cutaneous, articular, hematologic and immunologic. Given a corticosteroid resistance, several therapeutic background based hydroxychloroquine, cyclophosphamide and methotrexate, were initiated without any improvement. Immunomodulatory therapy type RTX was introduced to this form refractory at a rate of 375mg/m(2)/week. The waning of the second infusion, the patient presented a sudden intense abdominal pain syndrome, revealing an acute catarrhal appendicitis. At distance from the appendectomy, the consequences of which were favorable, treatment with RTX was resumed. In the aftermath of the third infusion, the patient presented in table tract marked by profuse watery diarrhea whose explorations reveal a morphological endoscopic appearance of erythematous, ulcerative colitis, reversible upon discontinuation of treatment. Histological data revealed important infiltrates composed mainly of CD8T lymphocytes. CONCLUSION: Gastrointestinal immunological consequences to the requirements of the targeted therapies deserved very careful and rigorous monitoring. However, at the slightest sign of digestive, a detailed morphological exploration is essential, to avoid in particular surgical emergency, evolution without treatment could engage in short-term vital prognosis.

[Durable efficacity and remission after treatment with imatinib mesylate for FIP1L1-PDGFRA transcript negative associated eosinophilic cardiomyopathy]. / Efficacité et rémission durables après traitement par imatinib mésylate pour une cardiomyopathie éosinophilique à transcrit FIP1L1-PDGFRA négatif.

INTRODUCTION: The cardiac involvement in hypereosinophilia remains a major cause of morbidity and mortality. Recent advances have identified new molecular mechanisms responsible for the expansion of the eosinophilic lineage, allowing a better classification of the different forms of Hypereosinophilic syndrome (HES) and especially targeted therapy. Since the discovery of the involvement of deregulated tyrosine kinases in the pathophysiology of these diseases, and particularly the identification of the fusion gene FIP1L1-PDGFRA, new molecules inhibiting specifically this signaling pathway (imatinib) were individualized, leading to dramatic therapeutic benefits in proliferative forms of HES considered before that of very poor prognosis. CASE REPORT: We report here the dramatic effectiveness of imatinib used as second line therapy for dilated cardiomyopathy revealing a hypereosinophilic syndrome in a patient in whom the search for FIP1-L1-PDGFRA fusion gene was negative. CONCLUSION: If hypereosinophilia has varied clinical and morphological outcome, its clinical consequences, particularly on heart function, are sometimes dreadful, and are not correlated either with blood eosinophil levels or with a specific etiology. We report here a case of HES lacking the FIP1-L1-PDGFRA fusion gene showing that despite the absence of this molecular defect, imatinib mesylate may have therapeutic interest in those cases of HES resistant to first line therapies.

[Efficacity of rituximab in hemolytic anemia with cold autoantibodies case]. / Efficacité exemplaire du rituximab dans un cas d'anémie hémolytique à autoanticorps froids.

INTRODUCTION: Autoimmune hemolytic anemia with cold autoantibodies or cold agglutinin disease is a rare chronic disorder in which no treatment has, until now, evidence of its effectiveness. CLINICAL CASE: We report a patient who successfully responded to rituximab for a cold agglutinin disease refractory to conventional therapy with very good tolerance and a complete remission. CONCLUSION: There are only few observations that have been reported in the literature regarding the efficacity of rituximab in the treatment of cold agglutinin disease. This promising therapy could, in the future, constitute a real alternative.

[Acute recurrent hemolytic anemia as the first manifestation of Wilson's disease: Report of a case]. / Anémie hémolytique récidivante révélatrice d'une maladie de Wilson (une nouvelle observation et revue de la littérature).

INTRODUCTION: The liver and central nervous system are the usual targets of Wilson's disease, an inherited disorder of copper metabolism. Severe hemolytic anemia is an unusual complication of Wilson's disease. EXEGESIS: We report a case of Wilson's disease revealed by acute intravascular repeating hemolytic anemia associated with liver failure. The initially negative etiologic investigation was directed by occurred of liver failure. The genetic study allowed to discover an other similar case. The evolution was favourable under treatment with zinc sulfate and penicillamine. DISCUSSION: Diagnosis of Wilson's disease must be considered in case of acute hemolytic anemia associated with liver failure in young adults.

[A case of bronchopulmonary sequestration]. / Une séquestration pulmonaire particulière.

INTRODUCTION: Bronchopulmonary sequestration is a rare congenital abnormality characterized by the presence of a non-functioning mass of lung tissue which receives an aberrant arterial supply from systemic circulation. CASE REPORT: We describe the case of a 30-year-old man who presented with recurrent episodes of mild hemoptysis. CT angiography revealed a right inferior lobar sequestration supplied by three systemic arteries. The patient underwent thoracotomy with right lower lobectomy and section-ligature of the three abnormal vessels. CONCLUSION: This case highlights the radiological features as well as the intraoperative appearances of this rare anomaly.

Allopurinol-induced Drug Reactions with Eosinophilia and Systemic Symptoms Syndrome with Interstitial Nephritis.

Allopurinol-induced drug reactions with eosinophilia and systemic symptoms (DRESS) is a severe illness related to hypersensitivity syndrome characterized by fever, skin rash, lymph node enlargement, hematological abnormalities, especially eosinophilia and atypical lymphocytosis, and single or multiple organ involvement. The syndrome is difficult to diagnose in view of its clinical heterogeneity and long latency period within 8 weeks after start treatment. We report a case of DRESS syndrome in a 64-year-old man, induced by allopurinol treatment for asymptomatic hyperuricemia, started 8 weeks earlier but stopped only 3 days after because of the onset of rash. The diagnosis was retained due to combining of interstitial nephritis with the clinical findings of fever, skin rash, cervical lymphadenopathy, eosinophilia, and reactivation of human herpesviruses specifically HHV-6. The glucocorticoids were started to relieve hypersensitivity. Five days later, the patient became afebrile, and the rash improved significantly. However, interstitial nephritis with renal function impairment progressed to severe azotemia, and even anuria requiring hemodialysis. Allopurinol-induced DRESS syndrome is associated with significant mortality, and care must, therefore, be exercised when given this drug.

[Ocular involvement in polyarteritis nodosa: two cases]. / Atteinte oculaire au cours de la périartérite noueuse. Deux observations.

INTRODUCTION: Polyarteritis nodosa (PAN) is a systemic vasculitis with polymorphic manifestations. Ocular involvement is rare; we report two such cases. CASES: The first case was a 56-year-old man with PAN treated with corticosteroids. A year and a half later, during a disease outbreak associated with decreased visual acuity, ocular examination objectified a localized pigment epithelial detachment in the right eye. The outcome was favorable with corticoid bolus followed by azathioprine. The second case was a 41-year-old man presenting with PAN and decreased visual acuity. Ophthalmologic examination showed inferotemporal branch vein occlusion in the right eye with serous retinal detachments in the left eye. The outcome was favorable with intravenous cyclophosphamide followed by azathioprine. DISCUSSION: The incidence of ocular involvement in PAN varies from 10 to 20%. It is most often associated with vasculitis of retinal and choroidal arteries. Fluorescein angiography is the gold standard of diagnosis. Multifocal acute ischemia of the choriocapillaris is very pathognomonic. Immunosuppressant drugs are effective and should be reserved for severe forms.

[Septic pylephlebitis associated with Enterobacter cloacae septicemia]. / Pyléphlébite septique associée à une septicémie à Enterobacter cloacae.

Septic pylephlebitis or purulent thrombosis of the portal venous system generally results from a progressive extension of suppurated thrombophlebitis, secondary to an intrabdominal infection. Germs most often found are Escherichia coli and Streptococcus, isolation of Enterobacter cloacae is unusual. We report a particular observation of septic pylephlebitis associated with E. cloacae bacteremia, without biliary, digestive or pancreatic lesion on the CT-scan. The antibiotic sensitivity pattern of the isolated germ and the negative epidemiologic investigation pled in favour of community acquired infection. The infection resolved with antibiotics and anticoagulation, followed by total repermeation of the portal system.