[Carbimazole can be the cause of iron deficiency anemia?]. / Le carbimazole peut-il être à l'origine d'une anémie férriprive ?

Iron deficiency anemia as a hematologic complication of the antithyroid medication (ATS) that has not been already described in the literature. We report on two exceptional cases: the first case concerns a 24 years old man admitted for an anemic syndrome. He was treated with carbimazole for Graves' disease. The blood count showed a non-regenerative microcytic anemia. Serum ferritin was severely decreased. The etiologic searching for bleeding, hemolysis, malabsorption or iron deficiency was negative. Treatment with iron salts was introduced without any real improvement. Given this situation, and given the negativity of the etiologic investigations, the decision to stop carbimazole was taken. Since that, the clinical and biological evolutions have been favorable. The second observation is much more original and concerns a 35 years old woman. The clinical, laboratory, etiological and treatment data are similar to those of the first observation. The evolution after withdrawal of carbimazole was favorable. The originality of this observation is that a reintroduction test of carbimazole was performed and allowed to reproduce the same haematological effects. These findings led us to hold the diagnosis of anaemia due to carbimazole. In this occasion, and in the light of the data in the literature, we underline the exceptional character of these two cases and we raise the possibility of an etiopathogenic link between administration of ATS and the occurrence of anaemia by iron deficiency.

[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.

[The methods for screening pulmonary hypertension related to systemic sclerosis in France. Descriptive survey of the French Research Group on Sclerosis]. / Pratiques françaises pour le dépistage de l'hypertension artérielle pulmonaire associée à la sclérodermie systémique. Enquête descriptive du Groupe de Recherche Français sur la Sclérodermie.

CONTEXT AND OBJECTIVES: Pulmonary hypertension (PHT) represents one of the severest complications and is life-threatening for patients suffering from systemic sclerosis (SSc). In France, the modalities for screening and treating PHT related to SSc are not well codified and no consensus has been reached. We conducted a survey among physicians inscribed on the list of the French Research Group on Sclerosis (GRFS - Groupe de Recherche Francais sur la Sclerodermie) to gather information on the status of the management of PHT related to SSc. METHODS: In 2002, we sent a questionnaire to 160 physicians, members of the GRFS, to assess the epidemiology and clinical profile of SSc patients as well as the modalities of screening and management of PHT in these patients. RESULTS: Eighty-eight physicians in 71 centres replied to the questionnaire. Each centre followed-up a mean of 33 SSc patients, with a global distribution of 53% limited and 47% diffused SSc. These physicians saw a mean of 5 new cases of SSc per year. The patients had been referred by town practitioners (53%) or from the hospital (47%). The mean number of SSc patients with PHT was of 5.1 per physician (1.5 new SSc + PHT patients per year). Almost all the centres (65/67) who replied systematically screened for PHT in SSc patients using Doppler echocardiography a mean of every 1.3 years. For the management of the patients exhibiting PHT, the majority (41/63) of centres collaborated with a specialized unit. Around one third of the centres treated these patients with calcium channel inhibitors (82%) and/or prostacyclin (90%). All the patients were followed-up by Doppler echocardiography. The majority of the physicians (72%) were interested in a research protocol on the subject and each could have included 4 patients, i.e., a total of 160. CONCLUSION: Pulmonary hypertension, a severe complication of SSc is screened for by the physicians of the GRFS using echocardiography with a frequency similar to Who guidelines (1.3 versus once/year).

[Severe Moorens ulcer: efficacy of monthly cyclophosphamide intravenous pulse treatment]. / Ulcères de Mooren sévères: efficacité du traitement par cyclophosphamide en bolus intraveineux mensuels.

PURPOSE: Mooren's ulcer (MU) is a chronic peripheral corneal ulceration featuring conjunctival immunoglobulin deposits. It is considered as the result of a limbic immune process with hyperactivation of T and B lymphocytes. The etiology remains unknown. The response to topical steroid therapy and surgical procedures usually poor and the visual outcome can be devastating. METHODS: Clinical follow-up of 3 patients who had rebel MU to conventional therapy, and were treated with 1g monthly intravenous cyclophosphamide. RESULTS: First patient was a 24-years-old man who had MU in his left eye. The response to surgical procedure and intravenous steroid treatment was poor and corneal perforation occurred. The affected cornea healed after 9 months of Cy treatment. The second patient was a 50-years-old man who had MU in his left eye, which did not improved with lamellar keratoplasty and topical steroid therapy. Corneal healing was obtained after 20 months of Cy treatment. The third patient was a 70-years-old man who presented with a furrowed MU in his right eye which healed with conjunctival resection and 4 months of Cy perfusion. No adverse effects of Cy was noted as opposed to Cy given orally. CONCLUSION: We report the effectiveness of 1g monthly intravenous cyclophosphamide (Cy) treatment in rebel MU. We suggest that immunosuppressive therapy using IV monthly Cy may be proposed in severe rebel MU.