A. Hot

Malignant mesothelioma of the peritoneum as the cause of a paraneoplastic syndrome: detection by 18F-FDG PET

Malignant mesothelioma of the peritoneum is a rare neoplasm with a rapidly fatal course. The tumour arises from the mesothelial cells lining the pleura and peritoneum or, rarely, in the pericardium or tunica vaginalis [1]. This neoplasm is characterised by being difficult to diagnose and having a rapid evolution, a poor response to therapy and a very high prevalence of thrombocytosis [2, 3]. We report a case in a 45-year-old female who presented with recurrent jugular vein thrombosis associated with weight loss, weakness, anaemia and an inflammatory biological syndrome. Given this clinical and biological picture, a paraneoplastic syndrome was considered. An aetiological investigation was performed, including ionogram, blood cultures, assays for biological tumour markers, colonoscopy, broncho-alveolar lavage (including investigations for tuberculosis), protein immunoelectrophoresis and a thoracoabdomino-pelvic contrast-enhanced CT scan. The findings from this set of investigations were: (a) an elevated CA 125 level and (b) on the axial contrast-enhanced CT scan, hypodense infiltration of the thymus without contrast uptake but responsible for a mass effect on the superior vena cava, mediastinal lymph nodes smaller than 1 cm, a right pleural effusion at the thoracic level, moderate ascites around the liver, spleen, right paracolic gutter and pouch of Douglas, and retroperitoneal lymph nodes smaller than 1 cm at the abdomino-pelvic level. The other explorations were negative. 18F-FDG PET was then performed and showed: – Right cervical chain lymph node uptake and right and left subclavian nodal uptake – Intense diffuse uptake outlining the liver, spleen, uterus and ovaries corresponding to the peritoneum (A, C) This appearance is consistent with peritoneal carcinomatosis with lymph node involvement. A retrospective analysis of the CT scan of the abdomen revealed an inspissated peritoneum and multiple intra-abdominal lymph nodes in addition to moderate ascites and and retroperitoneal lymph nodes (B). Laparoscopy revealed a thick peritoneal infiltration covering the viscera and abdomino-pelvic wall. Histology with immunohistochemistry confirmed malignant mesothelioma. This patient suffered from a malignant peritoneal mesothelioma with lymph node involvement which presented with recurrent jugular vein thrombosis and was detected by FDG PET. Two distinct scintigraphic patterns of glucose metabolism have been identified that appear to predict either diffuse, as in this case, or nodular peritoneal pathology and should alert the clinician to the possibility of peritoneal carcinomatosis [4].

Mise au pointDonnées actualisées sur la physiopathologie, les phénotypes et les traitements de la maladie de Still de l’adultePathophysiology, subtypes, and treatments of adult-onset Still's disease: An update

Adult-onset Stills disease is a rare and difficult to diagnose multisystemic disorder considered as a multigenic autoinflammatory syndrome. Its immunopathogenesis seems to be at the crossroads between inflammasomopathies and hemophagocytic lymphohistiocytosis, the most severe manifestation of the disease. According to recent insights in the pathophysiology and thanks to cohort studies and therapeutic trials, two phenotypes of adult-onset Stills disease may be distinguished: a systemic pattern, initially highly symptomatic and with a higher risk to exhibit life-threatening complications such as reactive hemophagocytic lymphohistiocytosis, where interleukin-1 blockade seems to be very effective, a chronic articular pattern, more indolent with arthritis in the foreground and less severe systemic manifestations, which would threat functional outcome and where interleukin-6 blockade seems to be more effective. This review focuses on these data.

Maladies auto-immunes et cancers. Première partie : cancers au cours des maladies auto-immunes et de leur traitement

The link between systemic disease and cancer is not fortuitous. An autoimmune disease can represent the starter for developing a non-Hodgkin lymphoma. This is particularly true for Sjögrens syndrome that is associated with the highest risk of lymphoma (odds ratio up to 44). Other systemic autoimmune diseases concerned are systemic lupus with an odds ratio of 4.5 and rheumatoid arthritis with an odds ratio of 2 to 3. It is now well established that high inflammatory activity, rather than immunosuppressive treatment, is the major risk determinant. The association between solid cancer and autoimmune systemic disease is uncommon and concerns in particular scleroderma and lung cancer. Concerning biotherapy-induced cancers, there is no demonstrated increased risk with anti-TNFα (except for cutaneous carcinoma and maybe melanoma) or with tocilizumab and abatacept even if studies with longer follow-up are needed at least for these two last drugs.

Mise au pointMaladies auto-immunes et cancers. Première partie : cancers au cours des maladies auto-immunes et de leur traitementAutoimmune diseases and cancers. First part: Cancers complicating autoimmune diseases and their treatment

The link between systemic disease and cancer is not fortuitous. An autoimmune disease can represent the starter for developing a non-Hodgkin lymphoma. This is particularly true for Sjögrens syndrome that is associated with the highest risk of lymphoma (odds ratio up to 44). Other systemic autoimmune diseases concerned are systemic lupus with an odds ratio of 4.5 and rheumatoid arthritis with an odds ratio of 2 to 3. It is now well established that high inflammatory activity, rather than immunosuppressive treatment, is the major risk determinant. The association between solid cancer and autoimmune systemic disease is uncommon and concerns in particular scleroderma and lung cancer. Concerning biotherapy-induced cancers, there is no demonstrated increased risk with anti-TNFα (except for cutaneous carcinoma and maybe melanoma) or with tocilizumab and abatacept even if studies with longer follow-up are needed at least for these two last drugs.

Horton ? C’est bien ma veine…

Un homme, âgé de 62 ans, était hospitalisé en médecine interne n mai 2012 pour prise en charge diagnostique d’une fièvre au ong cours évoluant depuis trois mois. Il présentait de lourds ntécédents médicaux, avec un séminome testiculaire à l’âge de 3 ans, traité par orchidectomie par voie haute et une radiothéapie rétropéritonéale. Il était également suivi en hématologie epuis 1994 pour une maladie de Waldenström, révélée par un yndrome de l’homme raide. Il avait eu, entre 1995 et 2004, des changes plasmatiques bimensuels avec une bonne efficacité sur e plan neurologique. L’hémopathie s’était également compliquée n 2001 d’un purpura thrombopénique auto-immun, traité par plénectomie. En 2005, devant une progression de l’hémopathie, uatre cures de rituximab (375 mg/m2) à un mois d’intervalle vaient été administrées avec une excellente efficacité. En 2011, ’envahissement médullaire lymphoplasmocytaire se majorait et ’importance du pic monoclonal conduisait à l’administration e quatre nouvelles cures de rituximab en association avec du yclophosphamide oral. Trois dernières cures de rituximab étaient