[Granulomatous hepatitis revealing a Mycobacterium bovis widespread infection following intravesical BCG therapy]. / Hépatite granulomateuse révélant une infection disséminée à Mycobacterium bovis après BCG-thérapie intravésicale.

INTRODUCTION: Intravesical therapy with bacillus Calmette-Guérin (BCG) has proved to be effective in the treatment of superficial bladder tumors. Side-effects include local infections and rarely disseminated BCG infection with multiple end organ complications such as granulomatous hepatitis, pneumonitis, aortitis and bone marrow involvement. CASE REPORT: We report an 83-year-old man who presented with chronic granulomatous hepatitis. This was related to intravesical BCG therapy received two years earlier for superficial bladder cancer. Aortitis, splenic infarction and hematopoietic involvement were also diagnosed. Outcome was favorable following adapted antibiotic course. CONCLUSION: This case report highlights the possibility of widespread BCG infection following intravesical treatment, and the need for vigilance in patients with a history of such a therapy even several years later.

Hypereosinophilic syndrome - lymphocytic variant transforming into peripheral T-cell lymphoma with severe oral manifestations.

Hypereosinophilic syndrome (HES) is a rare disease defined by organ damage directly attributable to hypereosinophilia of any type. Here, we report for the first time the case of a patient with a lymphocytic type of HES (HES-L) who had liver, skin, spleen, lung, bone marrow, digestive track, and mouth involvement. Associated T-cells displayed an aberrant CD30+ phenotype and were monoclonal. Thymus activated and regulated chemokine serum level was positive. Despite steroids (Cortancyl 20 mg [Sanofi Aventis, France], imatinib mesylate [Glivec 400 mg; Novartis Europharm], interferon alpha 2A [Roferon-A 3 MUI/0.5 ml; Roche]) and other lines of therapy including imatinib mesylate treatment, an oral necrotic lesion developed, and finally progressed into a peripheral CD30+ T-cell lymphoma. CHOP chemotherapy (cyclophosphamide, hydroxydoxorubicin, oncovin, prednisone), interferon-α, and mepolizumab were ineffective. Although progression into peripheral T-cell lymphoma is documented as a rare complication of HES-L, severe oral extension of HES-L is described for the first time.

[Non-fortuitous dynamin II mutation-related association: neutropenia and Charcot-Marie-Tooth disease]. / Association non fortuite d'une neutropénie chronique et d'une maladie de Charcot-Marie-Tooth en rapport avec une mutation de la dynamine 2.

Charcot-Marie-Tooth (CMT) disease or hereditary motor and sensory neuropathy is a genetically and clinically heterogeneous group of disorders of the peripheral nervous system. Mutations in multiple genes are currently known. We report an original case of CMT associated with chronic neutropenia in a patient with a K562del mutation in the dynamin 2 (DNM2) gene in a patient presenting with alterated cognitive function. Associated manifestations may guide molecular study.

[Antisynthetase syndrome: a retrospective study of 14 patients]. / Le syndrome des antisynthétases : étude rétrospective à propos d'une série de 14 patients.

PURPOSE: Antisynthetase syndrome is a rare entity characterized by myositis (dermatomyositis or polymyositis), interstitial lung disease, arthritis, Raynaud's phenomena and mechanic's hands skin manifestation, and the presence of autoantibodies against aminoacyl-transfer RNA synthetase. PATIENTS AND METHODS: Fourteen patients with antisynthetase syndrome followed-up between 1997 and 2009 were included. We studied retrospectively their clinical, radiological, and pathological findings. RESULTS: The sex ratio women/men was 2.5. Mean age at disease onset was 46 years. Arthritis (43%) and interstitial lung disease (38%) were the most frequent features at disease onset. Seven patients had myositis. Ten patients had anti-Jo1 autoantibodies, three had anti-PL7 and one anti-PL12. Corticosteroid therapy was given in all cases, immunosuppressive drugs in 12 cases, due to initial severity (n=8), disease relapse (n=3) or corticosteroid dependence (n=1). After a mean follow-up of 64 months, nine patients improved, four stabilized and one patient died after lung transplantation, required for pulmonary hypertension. CONCLUSION: The diffusion of immunologic assay will help us in the future to identify the specificity of this syndrome in order to improve care.

[Lipoatrophia semicircularis: a case report]. / La lipoatrophie semi-circulaire des cuisses : à propos d'un cas.

We report a 54-year-old man who presented bilateral, symmetric depressions on the anterior aspect of his thighs corresponding to lipoatrophia semicircularis. The origin of this distinctive variant of lipoatrophy is unknown. Repeated external microtraumatisms were considered to be the most plausible explanation. Recently, as many recently affected subjects shared a status of administrative employee, the role of electromagnetic fields generated by computers and their wirings has been discussed. Prevention proposal includes adapting work environment related to the use of computerized devices.

[Macroglobulinosis cutis revealing Waldenström macroglobulinemia]. / Une maladie de Waldenström révélée par une macroglobulinemia cutis.

Waldenström macroglobulinemia is defined by a bone marrow lymphoplasmacytic infiltration associated with serum IgM monoclonal gammopathy. Specific properties of the IgM gammopathy induce the main clinical manifestations revealing the disease: hyperviscosity syndrome, autoimmune peripheral neuropathy, cryoglobulinemia or hemolysis, and exceptional skin deposit such as macroglobulinosis cutis that we here report. Physicians should be aware of these clinical manifestations to avoid diagnostic delay.

[Wegener's granulomatosis with anti-MPO c-ANCA revealed by uveitis]. / Maladie de Wegener à c-ANCA de type anti-MPO révélée par une uvéite.

Wegener's granulomatosis (WG) is a rare systemic necrotizing granulomatous vasculitis affecting small- to medium-sized vessels, associated with antineutrophil cytoplasm antibodies (ANCA), mainly anti-proteinase 3. Rarely, ANCA may be directed against myeloperoxidase. We report a 58-year-old woman who developed an uveitis as the presenting manifestation of Wegener's granulomatosis who highlight the usefulness of internist and ophthalmologist collaboration.

[Splenic marginal zone lymphoma and autoimmunity: report of six cases]. / Lymphomes spléniques de la zone marginale et auto-immunité: à propos de six cas.

INTRODUCTION: Autoimmune manifestations are common in splenic marginal zone lymphoma (SMZL) and are sometimes the presenting feature of the disease. Autoimmune cytopenia (anemia, thrombocytopenia) are the most frequently reported autoimmune conditions. However, other immunological manifestations may be associated with SMZL. METHODS: We report a retrospective case series of six patients with SMZL associated with autoimmunity. RESULTS: Auto-immune manifestations were the presenting feature of lymphoma in four cases. Auto-immune manifestations included auto-immune cytopenia in three cases (two hemolytic anemia and one pancytopenia), thyroiditis in two cases, systemic lupus and Still's disease in one case each. Antinuclear antibodies were detected with a titre of 1/250 in three cases, and with a titre of 1/32,000 in the patient with systemic lupus. Testing for DNA antibodies was negative in all cases. Two patients had a circulating lupus anticoagulant, with portal venous thrombosis following splenectomy in one case. One patient had hypogammaglobulinemia. A monoclonal gammopathy was detected in three patients. All patients had spleen enlargement. Immunophenotyping of blood peripheral lymphocyte was typical in five out of the six cases. Bone marrow was infiltrated in five out of the six cases. Diagnosis was obtained by the combination of immunophenotyping and bone marrow histopathology in five cases, and by splenic histopathology in the remaining case. Hepatitis C virus serology was negative in all patient. CONCLUSION: Autoimmune disease as systemic lupus or Still's disease may be associated with SMZL before its tumoral manifestations are evident. In this mode of presentation, spleen enlargement, hypogammaglobulinemia, monoclonal gammopathy, and multiple autoimmune diseases, should alert the physician.

[Human immunoglobulins, adverse drug reaction, prevention]. / Effets indésirables liés aux immunoglobulines humaines normales: intérêt des mesures de prévention.

PURPOSE: Adverse drug reactions occur during 10% of immunoglobulin treatment. Risk factor of adverse drug reactions have been identified. STRONG POINT: Simple measures during prescription, administration and supervision of immunoglobulin treatment should prevent most of serious and non-serious adverse drug reactions. Patients with risk factor of adverse drug reactions may be identified. Hydration before and during immunoglobulin treatment is recommended. Low dose of immunoglobulin and slow rate of infusion associated with hydration should prevent many adverse drug reactions. Anyway follow-up of tolerance during infusion is necessary. Changing immunoglobulin preparation does not prevent adverse drug reactions although measures of safe prescription and administration do. CONCLUSION: Careful prescription, administration and supervision of immunoglobulin therapy should prevent most of immunoglobulin-related adverse drug reactions.

[The fate of the abstracts presented to the biannual meetings of the French National Society of Internal Medicine]. / Devenir des travaux publiés sous forme de résumés dans les actes des congrès semestriels de la Société nationale française de médecine interne.

PURPOSE: Medical meetings give the opportunity to present oral communications or posters to the attending participants. However, the peer-reviewed publication of a full article allows to reach a wide readership. KEY POINTS: The survey that was performed on the oral communications and posters presented at the 43rd meeting of the French National Society of Internal Medicine, December 2000, showed that amongst the 303 selected podium presentations and posters, only 82 (27%) were published during the five following years. Podium presentations were more likely to be published than posters (36% versus 22%). CONCLUSION: Many oral communications and posters that are presented in medical meeting are not followed by the publication of a peer-reviewed full article despite the modern means of communication. However, this issue is of paramount importance as beyond the legitimate personal satisfaction of a publication, the scientific and academic recognition are the ground of medical career achievement for many physicians.

Extra-pulmonary paragonimiasis with unusual arthritis and cutaneous features among a tourist returning from Gabon.

Paragonimiasis is a helminthic disease that affect accidentally man after consumption of raw or poorly cooked crustacean dishes. The clinical feature is represented mainly by pulmonary signs. Extra-pulmonary manifestations including arthritic and skin attempt remain less frequent. The case is described of a young white French woman who become infected with Paragonimus while travelling to Gabon for a tourist trip. Clinical presentation accounted for extensive recurrent pruritic urticarian subcutaneous induration, permanent assymetrical pauciarthritis associated with joint swelling, and marked eosinophilia. Diagnosis was reached using serological testing showing seroconversion for specific antibodies. The patient was cured with a single oral dose of praziquantel. Even if the condition is rare among tourists to endemic zones, it must be considered when hypereosinophilia occurs in the returning traveller and migrant.

[Lymphoma with initial renal involvement: four cases]. / Lymphomes à manifestations initiales rénales: à propos de quatre observations.

PURPOSE: To present a comprehensive description of the clinical features of patients with renal manifestations during lymphoma. METHODS: Retrospective review of medical records from all patients diagnosed with lymphoma associated with kidney involvement in our hospital between 1996 to 2004. Four cases were identified and analysed. RESULTS: Four patients presented a non-Hodgkin's lymphoma. One patient showed intravascular large B-cell lymphoma, revealed by proteinuria. Another patient had a nephrotic syndrome, and two had a renal mass. Renal histology allowed diagnosis of lymphoma in 3 cases. CONCLUSION: The diagnosis of lymphoma associated with renal involvement is rather difficult, and more specifically in case of intravascular large B-cell lymphoma, or even primary renal lymphoma. We present here a comprehensive review of the literature and we discuss pathogenesis of these conditions.

[Exophtalmia revealing a mantle cell lymphoma]. / Exophtalmie révélant un lymphome du manteau: à propos d'un cas.

INTRODUCTION: Mantle cell lymphoma reached rarely ophtalmic sphere and salivary glands. CAS REPORT: We reported a dry syndrome seen in a 67 year-old patient. The first patological analysis of accessory salivary glands evoked a primary Gougerot-Sjögren syndrome. Secondary, he presented a mantle cell lymphoma. DISCUSSION: The pathological lack of specifity and the discovery of atypical Gougerot-Sjögren syndrome must encourage complementary immunohistochemical study of salivary glands biopsy.

[Pulmonary plasmacytoma: about two new cases and review of the literature]. / Plasmocytome pulmonaire: à propos de deux nouvelles observations et revue de la littérature.

INTRODUCTION: Extramedullary plasmacytoma is an uncommon plasma cell malignancy mainly located to the upper aerodigestive tract. Primary pulmonary plasmacytoma is extremely rare. EXEGESIS: We report two new cases of primary pulmonary plasmacytoma and then proceed to a review of the literature concerning 35 similar cases previously described. CONCLUSION: Complete or partial responses were obtained in 24 cases (65%). Five patients (14%) have developed multiple myeloma within 3 years following plasmacytoma diagnostic. In spite of sustained responses with radiotherapy or chemotherapy, surgical resection while feasible remains the first therapeutic option.

[Nephrocalcinosis: initial manifestation of primary Sjögren's syndrome]. / La néphrocalcinose : manifestation inaugurale d'un syndrome de Gougerot-Sjögren primitif.

INTRODUCTION: Nephrocalcinosis is a rare complication of chronic tubulointerstitial nephritis observed in primary Sjögren's syndrome. It can precede subjective sicca symptoms. OBSERVATION: We report the case of a 50-year-old woman who presented with a primary Sjögren's syndrome. The first symptoms appeared 10-years-ago while she was affected with a nephrocalcinosis. CONCLUSION: Autoimmune investigations for Sjögren's syndrome should be initiated in any patient presenting with nephrocalcinosis and distal renal tubular acidosis.

[Common variable immunodeficiency: 17 observations in the adult]. / Le déficit immunitaire commun variable de l'adulte: étude clinique, biologique et immunologique chez 17 patients.

PURPOSE: Common variable immunodeficiency (CVID) is an immune defect characterized by primary hypogammaglobulinemia. Most of the time, clinical manifestations that reveal CVID are recurrent bacterial infections, but auto-immune or granulomatous events may occur. METHODS: This retrospective study was conducted on 17 patients fulfilling the classical CVID definition. Lymphocyte activation level was evaluated in 12 patients through HLA-DR expression on lymphocytes subsets. RESULTS: This study includes 17 patients, 7 men and 10 women. The mean age at the first clinical manifestation is 23 years and the mean age at diagnosis is 39 years. Recurrent upper and lower bacterial respiratory tract infections are common to all patients. Abdominal infection due to Mycobacterium avium-intracellulare complex is found in one patient. Digestive events are dominated by chronic diarrhea caused by giardiasis, nodular lymphoid hyperplasia or villous atrophy. Seven patients developed auto-immune conditions (insulin dependent diabetes, idiopathic thrombocytopenic purpura (ITP), rheumatoid arthritis) and 7 patients have a splenomegaly. Non caseating granulomas in the spleen or in lymph node biopsies are found in 3 patients. Ten patients have a T lymphopenia, 2 have a B lymphopenia, 5 have a CD4/CD8 ratio <1, and 6 have T CD4(+) lymphocytes <400/mm(3). The study of HLA-DR expression on lymphocytes subsets shows that 7/12 patients have activated T CD4(+) and/or CD8(+) cells and these patients have auto-immune or tumoral manifestations. The other 5 patients do not have activated T lymphocytes but present with infectious events only. CONCLUSIONS: Our study allows the separation of patients with CVID according to their T lymphocytes activation level. A patient's classification is necessary to define homogeneous groups of patients to perform genetic and functional studies which will probably reveal heterogeneous molecular abnormalities.