R. Jean

Successful treatment with rituximab in an elderly patient with acquired factor VIII inhibitor

We report here on an elderly patient, aged 88 years, who developed acquired hemophilia, with a high titer of factor VIII inhibitors and severe hemorrhage, in the absence of a detectable cause. Marked and prolonged efficacy was observed after rituximab.

Still's-like disease, breast prosthesis, and collagen implants

Abstract. Silicone-induced connective tissue disease raises a controversial issue. We report a case of Stills disease associated with silicone and collagen implants that showed improvement on steroids, but remained steroid-dependent despite removal of the silicone implants. This observation complements four previous cases in the literature and questions the role of breast implants in the pathogenesis of Stills disease. The number of cases studied is insufficient for conclusions, but silicone-implant-associated syndrome may be confused with Stills disease. We consequently propose the use of ferritinemia and its serum glycosylated fraction level as discriminating factors. Collagen has been associated with some inflammatory diseases, but never previously with Stills disease. However, considering this observation and previous data in the literature, its role may be postulated as an exacerbating factor or a pathogenic agent.

Sarcoidosis Occurring After Solid Cancer: A Nonfortuitous Association

AbstractThe association between cancer and sarcoidosis is controversial. Some epidemiological studies show an increase of the incidence of cancer in patients with sarcoidosis but only few cases of sarcoidosis following cancer treatment have been reported.We conducted a retrospective case study from internal medicine and oncology departments for patients presenting sarcoidosis after solid cancer treatment. We also performed a literature review to search for patients who developed sarcoidosis after solid cancer. We describe the clinical, biological, and radiological characteristics and outcome of these patients.Twelve patients were included in our study. Various cancers were observed with a predominance of breast cancer. Development of sarcoidosis appeared in the 3 years following cancer and was asymptomatic in half of the patients. The disease was frequently identified after a follow-up positron emission tomography computerized tomography evaluation. Various manifestations were observed but all patients presented lymph node involvement. Half of the patients required systemic therapy. With a median follow-up of 73 months, no patient developed cancer relapse. Review of the literature identified 61 other patients for which the characteristics of both solid cancer and sarcoidosis were similar to those observed in our series.This report demonstrates that sarcoidosis must be considered in the differential diagnosis of patients with a history of malignancy who have developed lymphadenopathy or other lesions on positron emission tomography computerized tomography. Histological confirmation of cancer relapse is mandatory in order to avoid unjustified treatments. This association should be consider as a protective factor against cancer relapse.

JAW CLAUDICATION AS AN ATYPICAL MANIFESTATION OF AORTIC THORACIC DISSECTION IN ELDERLY PEOPLE

ventricular function returned to normal 7 days later. This report demonstrates that T-wave inversion on telemetry monitoring might be useful for detecting the onset of tako-tsubo cardiomyopathy in patients in a coma. Since tako-tsubo cardiomyopathy was first reported in 1990, this disorder has been recognized worldwide. Most patients have been elderly women, symptoms are similar to those of acute myocardial infarction, and patients have often experienced emotional or physical stress before onset. Tako-tsubo cardiomyopathy should be considered in the differential diagnoses of acute myocardial infarction. In our case, acute stress due to brain infarction could have been a precipitating factor leading to tako-tsubo cardiomyopathy. Because apical ballooning of tako-tsubo cardiomyopathy is transient and resolves dramatically in a short time, its prognosis is generally favorable, although recent reports have demonstrated early life-threatening complications, including left ventricular rupture, arrhythmia, heart failure, cardiogenic shock, and apical thrombus formation. Early diagnosis and subsequent treatment are required to prevent these complications even in patients with tako-tsubo cardiomyopathy. Tako-tsubo cardiomyopathy often occurs as a complication of noncardiac disorders. The development of takotsubo cardiomyopathy may be diagnosed at a later stage or overlooked, especially in patients who are in a coma. Clinicians should recognize that there is a possibility of the development of tako-tsubo cardiomyopathy when T-wave inversion appears in patients with noncardiac disorders during hospitalization. Transthoracic echocardiography or repeated electrocardiogram should be performed routinely in these patients.

Bilateral Breast Ulcers: Granulomatosis with Polyangiitis

Please cite this article as: Pierre-André Jarrot MD , Marie-Laure Pelletier MD , Marion Brun MD , Martin Penicaud MD , Karin Mazodier MD , Audrey Benyamine MD, PhD , Jean Del Grande MD , Rodolphe Jean MD , Dominique Casanova MD , Gilles Kaplanski MD, PhD , Pierre-Jean Weiller MD , Bilateral Breast Ulcers: Granulomatosis with Polyangiitis, The American Journal of Medicine (2018), doi: https://doi.org/10.1016/j.amjmed.2018.09.008

CASE REPORT: β-Carotene-Induced Hepatic Fibrosis

A 66-year-old woman, an alcohol drinker, presented with anicteric cholestasis of two years duration. She has been treated during the last 30 years for a pigmentary retinopathy with anthocyanosides associated with -carotene (total dose 165 g). The clinical examination was normal. Biology confirmed cholestatic changes: serum alkaline phosphatase 1364 IU/liter (normal 200), gammaglutamyltranspeptidase 144 IU/liter, (normal 35), aspartate aminotransferases 52 IU/ liter, and alanine aminotransferases 60 IU/liter, (normal 35). Ultrasonography and endoscopic retrograde cholangiopancreatography were normal. The liver biopsy showed a pronounced portal fibrosis. Bile ducts were normal; there was no evidence of portal inflammation. The usual causes of chronic hepatic diseases, especially primary biliary cirrhosis and chronic hepatitis C, were excluded. All infectious, immunologic, and metabolic investigations were normal. The blood level of vitamin A was 0.43 g/liter (normal range: 0.5–0.8g/ liter). We observed a favorable course within six months, after -carotene therapy was stopped and ursodeoxycholic acid supplementation was given (alkaline phosphatase 290 IU/liter, gammaglutamyltranspeptidase 44 IU/liter). Subsequently, the patient has remained stable for three years despite continued alcohol intoxication.

Danger des anticorps antiérythrocytaires au cours de la grossesse

Introduction Red cell autoantibodies are very rarely observed in pregnant women. We report one case related to the presence of a lupus anticoagulant. Exegesis A 32-year-old woman had a history of two intrauterine deaths that had occurred at weeks 32 and 33, respectively. Due to the presence of a lupus anticoagulant, prednisone (20 mg/day) and subcutaneous calcium heparin were administered during her third pregnancy. The direct antiglobulin test remained positive throughout the pregnancy; however, hemolysis could not be detected. Caesarean section was done in emergency at week 29, due to fetal bradycardia related to severe fetal hemolysis. Conclusion When autoimmune hemolysis occurs during pregnancy, risks for the fetus increase with the severity of hemolysis in the mother. It is thought that child mortality reflects the existence of anemia, rather than the existence of hemolysis. Our observation suggests that severe fetal hemolysis may occur, despite the absence of hemolysis in the mother.