Association myopathie inflammatoire et hépatocarcinome : à propos d’une observation et revue de la littérature

Association inflammatory myopathies and tumors are not fortuitous but association with hepatocellular carcinoma is rarely reported in literature. We described a case of association polymyositis hepatocellular carcinoma in 37-year-old black African patient, with fatal issue.     

Hémophilie A acquise découverte au cours de la grossesse : à propos d’un cas et revue de la littérature

Introduction

Acquired hemophilia A (AH) is a rare hemorrhagic disorder, secondary to the occurrence of factor VIII inhibitor. In young patients, this disorder is commonly observed during the post-partum period, and has been rarely documented in the prepartum. We report a new case of a prepartum AH and review literature data.

Case report

An isolated prolongation of the activated partial thromboplastin time (APTT) was fortuitously discovered in a 31-year-old pregnant women, with spontaneous ecchymosis of her lower limbs few days prior to delivery. Coagulation tests revealed decreased factor VIII activity (18%) and the presence of factor VIII inhibitor (1,4 Bethesda unit). In order to eradicate the autoantibody, the patient was first treated with prednisone and then with rituximab.

Conclusion

Prepartum factor VIII inhibitors need to be precociously recognized to allow prophylactic management of the delivery bleeding.     

Vascularite cutanée à anticorps anti-cytoplasme des polynucléaires neutrophiles induite par le benzylthio-uracile : à propos d’un cas et revue de la littérature

Introduction

Vasculitis associated to antineutrophil cytoplasmic antibodies is a rare complication of therapy with antithyroid medication. They were mainly reported in patients treated with propylthiouracil and rarely with benzylthiouracil.

Case report

We report a 22-year-old woman treated with benzylthiouracil for Graves’ disease, who developed a vasculitic skin involvement. The presence of antineutrophil cytoplasmic antibodies with anti-myeloperoxidase specificity was documented. The discontinuation of benzylthiouracil was followed by a complete disappearance of skin lesions and of antineutrophil cytoplasmic antibodies.

Conclusion

To our knowledge, only ten cases of antineutrophil cytoplasmic antibodies vasculitis induced by benzylthiouracil have been previously reported in the literature. Our patient was characterized by the occurrence of isolated cutaneous vasculitis, without renal involvement. Early discontinuation of benzylthiouracil may have prevented the occurrence of severe visceral complication.     

Amylose cardiaque : à propos d’une série de 14 patients,description et facteurs pronostiques

Purpose

Cardiac amyloidosis is rare. The objective of this study was to report on a case series of 14 patients with cardiac amyloidosis and to study the prognostic factors.

Methods

Monocentric retrospective study of all adult patients who presented with cardiac amyloidosis, diagnosed at the Georges-Pompidou European hospital in Paris between 2003 and 2011.

Results

Fourteen patients were identified (10 men and four women). Median age at diagnosis was 66.5 years. Twelve patients were diagnosed with AL amyloidosis, one with AA amyloidosis, and one with transthyretin amyloidosis. All patients presented cardiac manifestations: heart failure (n = 9), rhythm disorders (n = 6). Eight patients presented extra-cardiac manifestations of amyloidosis: renal (n = 8), gastrointestinal (n = 5). Troponin serum level was increased in eight patients and BNP level was superior to 400 pg/L in 12 patients. When performed, the cardiac magnetic resonance imaging (MRI) showed, in six patients out of seven, chamber dilatation, concentric hypertrophy or late enhancement. Among patients with cardiac failure at diagnosis (n = 9), seven died with a median survival of 1 month duration. Factors of poor prognosis were, in our study, heart failure, elevated levels of troponin and BNP, and the AL amyloidosis subtype.

Conclusion

Cardiac amyloidosis, especially the AL type, has a very poor prognosis, essentially because of an underlying multiple myeloma and heart failure.     

Nécrose médullaire avec sepsis compliquant une thrombocytémie essentielle : à propos d’une observation et revue de la littérature

Introduction

Bone marrow necrosis is a very rare condition which is characterized by a necrosis of hematopoietic progenitors, adipocytes and reticulin network.

Case report

We report a 62-year-old woman admitted to an intensive care unit for an essential thrombocytemia associated with bone marrow necrosis complicated by septic shock and progressive multi-organ failure. To our knowledge, this is the second case reported in the literature. The clinical presentation of bone marrow necrosis includes non-specific symptoms such as fever, bone pain and sometimes a clinically significant medullar insufficiency syndrome. Biology can reveal cytopenias, elevated LDH and alkaline phosphatase serum levels. The diagnosis is confirmed by bone marrow trephine biopsy. Bone marrow necrosis is classified as extensive if more than 50% of the bone marrow biopsy show necrosis. Haematological malignancies (particularly leukaemia), and solid malignant tumours (particularly gastro-intestinal or lung cancers) represent up to 90% of aetiologies and must be actively researched. Also, sickle cell disease and catastrophic anti-phospholipid syndrome must also be investigated. Essential thrombocytemia remains an exceptional cause of bone marrow necrosis.

Conclusion

Overall the prognosis of bone marrow necrosis is poor unless appropriate and intensive treatment, especially for sickle cell disease in which complete medullar regeneration has been observed.     

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.     

Manifestations cardiaques au cours la granulomatose de Wegener : à propos de quatre observations et revue de la littérature

Introduction

Discordance exists between the results of post-mortem studies and the low number of clinical reported cases of cardiac involvements in Wegener's granulomatosis.

Case reports

Data from four patients were studied retrospectively. Three patients had associated airway localization and three had kidney involvement. All patients had positive test for anti-PR3 antineutrophil antibodies. Two patients presented with dilated cardiomyopathy (one with terminal cardiac failure), another patient with complete atrioventricular block and pericarditis, and the remaining one with myopericarditis. One patient was asymptomatic. For three of these patients, the cardiac manifestations were contemporary of the diagnosis of Wegener's granulomatosis and had a severe disease course.

Conclusion

Cardiac events in Wegener's granulomatosis are probably underestimated, given the various type of heart damage and the clinical presentation. Cardiac involvement seems to be associated with a poor prognosis. Thus, we recommend systematic and regular cardiac assessment in the follow-up of patients with Wegener's granulomatosis.     

Efficacité clinique et biologique du tocilizumab au cours de la maladie de Horton : à propos de trois observations et revue de la littérature

Introduction

Treatment of giant cell arteritis is based on prolonged corticosteroid therapy but adverse side effects are common especially in the elderly.

Case reports

We report three patients with giant cell vasculitis treated by tocilizumab, an interleukin-6 receptor antibody, owing to resistance or intolerance to corticosteroid therapy. A favorable outcome was rapidly observed both on clinical and biological data allowing a corticoid therapy sparing.

Conclusion

Tocilizumab is a promising treatment of giant cell arteritis but controlled trials are needed to confirm its efficacy.     

Eczéma de contact à l’or : une revue de la littérature à propos d’un cas d’allergie professionnelle

Gold is an inert and stable precious metal, and is usually well tolerated. Allergic adverse events have been reported in patients treated with gold salts, but the immunologic consequences of skin exposure to gold metal are not precisely known. The authors report a case of gold-induced contact eczema and mouth lesions in a jeweller woman, and review the literature on gold-induced contact allergy. Although the results of epidemiological studies are highly variable, contact allergy to gold seems to be more frequent than usually suspected. The diagnostic value of epicutaneous tests (patch) with gold sodium thiosulfate (GST) is controversial. Thus, diagnosis of gold-induced contact allergy is based on positive skin tests in patients with a suggestive clinical history only. Prevention is based on the eviction of gold, which may be difficult to realize and expensive in patients with dental prosthesis.     

Arthrites réactionnelles secondaires à la BCG-thérapie. Deux observations et revue de la littérature

Introduction

Intravesical bacillus Calmette-Guérin (BCG) therapy-associated articular complications are uncommon, occurring in only 0.5 to 1% of the patients.

Observations

We report two patients who were given intravesical BCG therapy for superficial bladder cancer. Both patients developed polyarthritis and fever related to intravesical BCG instillation. The outcome of articular manifestations was favorable after administration of nonsteroidal anti-inflammatory therapy.

Conclusion

Intravesical BCG therapy-associated articular complications should not be overlooked, as they may result in high morbidity. Nevertheless, the diagnosis of intravesical BCG therapy-related reactive arthritis should be discussed after excluding infectious arthritis due to Mycobacterium bovis. Therefore, joint fluid microbiological tests (cultures, PCR) are required in the patients receiving intravesical BCG who develop arthritis.     

Pseudomaladie de Still et néoplasie : une observation de mélanome et revue de la littérature

Introduction

A malignancy must be carefully excluded before ruling in the diagnosis of adult onset Still's disease (AOSD). However, an occult or poorly symptomatic malignancy can easily be overlooked.

Case report

We report a 50-year-old female patient who presented with features of adult onset Still's disease (AOSD), in fact heralding a malignant melanoma with fatal outcome since discovered lately, at a metastatic stage. In retrospect, the only significant atypical feature was cholestatic hepatitis, which soon disappeared upon institution of glucocorticoid treatment. The literature review identified 27 additional cases of AOSD-like disease associated with malignancy published since 1980 including solid cancer in 61% of the cases (especially breast and lung) and haematological malignancies in 39% of the cases (especially malignant lymphoma). The interval between OASD-like symptoms and malignancy averaged 8 months, and AOSD most often preceding malignancy. Although idiopathic AOSD and neoplastic AOSD-like disease are often indistinguishable initially, some features could point toward the latter: an onset of AOSD after the age of 40 years, the presence of atypical clinical, biological, or immunological features in less than one third of the cases, and a poor response to NAIDS or systemic glucocorticoids in 61% of the cases.

Conclusion

Making the differential diagnosis of malignancy-associated AOSD in a timely fashion remains a primary goal, even in the most typical cases and those showing good initial therapeutic response.     

Le kyste hydatique costovertébral primitif : une observation et revue de la littérature

The bone involvement is uncommon in hydatid disease and represents less than 2 % of cases. Vertebral hydatidosis is the most common bone localization (44 %). The severity of vertebral echinococcosis is related to the neurological complications and therapeutic problems especially in advanced stages. The treatment relies on the actual surgical removal of hydatidosis. In endemic countries, prevention and health education are the best measures. We report a 17-year-old male who presented with an incomplete paraplegia with thoracic deformation, revealing a costovertebral hydatidosis.