Autoantibodies to lamins in rheumatoid arthritis

The presence of autoantibodies reacting with lamins A and C was demonstrated in sera from 2 patients with rheumatoid arthritis (RA). One patient developed antilamin antibodies several years after being diagnosed as having RA; she was also found to have chronic active hepatitis. The second patient had severe nodular RA. We describe the other serologic findings in these 2 patients and discuss the relationships between antilamin antibodies and RA.     

Démarche diagnostique devant une lymphopénie : quand penser à la lymphopénie CD4 idiopathique ?

Lymphocytopenia is defined by a lymphocyte count less than 1500/mm³ in adults and less than 4500/mm³ in children before the age of 8 months. Lymphocytopenia can be global or selectively affect a peculiar lymphocyte subpopulation. The patient's age, the context as well as the associated clinical manifestations and treatment prescribed must be taken into account in order to identify the etiology of lymphocytopenia. In adults, lymphocytopenia can be caused by: (1) insufficient thymic output (primary immune deficiencies, corticosteroid treatment, zinc deficiency, etc.), (2) increased lymphocyte catabolism (radiotherapy, chemotherapy, immunosuppressant, HIV infection, systemic lupus, etc.), (3) modified lymphocyte distribution (viral infections, septic shock, extensive burns, splenomegaly, granulomatosis, etc.), (4) multifactorial or unknown etiology (end-stage renal disease, lymphoid malignancies, solid tumor, ethnicity, etc.). In children, in addition to these etiologies, other immune deficiencies may be responsible for severe lymphocytopenia (thymocytes apoptosis, cytokine deficiencies, altered B-cell and T-cell receptor synthesis, signal transduction and cellular interactions deficiencies). Idiopathic CD4⁺ lymphocytopenia is a rare disorder. It is defined by a persisting lymphocyte CD4⁺ count less or equal to 300/mm³ or less or equal to 20% of total lymphocytes in the absence of alternative diagnosis. Clinical symptoms can be absent or include opportunistic infections, auto-immune manifestations, lymphoma or solid tumors. Treatment is similar to that of HIV-infected patients and sometimes relies on specific immunotherapy even though clinical benefit has not been evaluated.     

Synovial hemangioma of the knee joint. A case report

Synovial hemangioma of the knee joint was diagnosed in a young woman 15 years after the first signs. The principal clinical manifestation involved repeated episodes of hemorrhagic joint effusion. MRI is the exploration of choice for this vascular tumor of the synovial membrane, although a pathology study is needed to confirm the diagnosis. Cure is achieved with surgical resection.     

Esophageal involvement in Behcet's disease

Esophageal involvement in Behcet's disease is very rare, and normally is observed as aphtosis and esophagitis, but serious complications such as erosions, perforations, and stenosis may occur. We carried out this prospective study to evaluate the prevalence of esophageal involvement in Behcet's disease and to establish if routine endoscopy and/or manometry are necessary. Twenty-three patients who fulfilled the diagnostic criteria of the international study group for Behcet's disease were enrolled. None were taking drugs or had disease that might produce esophageal abnormalities or alter any existing changes due to the Behcet's disease itself. Twenty- three patients underwent esophagogastroduodenoscopy by a single observer. Esophageal biopsies were performed in thirteen patients and esophageal manometry in twenty. At the beginning of the study, the disease activity was defined by the presence of more than one symptom related to Behcet's disease, and upon the classification of Behcet's disease. Of the twenty three patients enrolled two were excluded from final analysis because of the presence of hiatus hernia. Thus, 13 men and 8 women, ranging in age from 20 to 63 years with a mean age of 36.2 years were included. Ten patients (47.6%) had active disease and four (19%) complained of upper gastrointestinal symptoms at the time of the study. Fourteen patients had endoscopic, manometric and/or microscopic abnormalities. Esophageal manometry was performed in twenty patients and was abnormal in 7 cases (35%). Esophageal biopsies were done in 13 patients and revealed histopathological abnormalities in 5 cases. Microscopic findings showed vasculities in one case, and non-specific inflammatory infiltration mainly consisting of neutrophils in 4 cases. Our results suggest that the prevalence of esophageal involvement in Behcet's disease is rather high and occur even in asymptomatic patients, but that this usually does not result in specific abnormalities.     

Effectiveness of platelet transfusions after plasma exchange in adult thrombotic thrombocytopenic purpura: a report of two cases.

Plasma infusion (PI) and plasma exchange (PE) are the most efficient treatment of thrombotic thrombocytopenic purpura (TTP), allowing achievement of complete remission in 60 to 90% of cases. Life-threatening bleeding, related to severe thrombocytopenia, is one of the main complications of the disease. Thrombocytopenia may also preclude invasive procedures such as splenectomy, which may be required during the management of TTP. Platelet concentrates transfusions are usually thought to worsen the disease, especially if not associated with the appropriate treatment of this latter, and thus should be avoided. We report hereon 2 patients with TTP who experienced a surgical procedure i.e., a cholecystectomy for a cholecystitis, and a splenectomy for a refractory TTP. In both patients, the surgical procedure was preceded by a 60 mL/kg plasma exchange with solvent/detergent treated plasma as replacement fluid, followed by platelet transfusion, with a corrected count increment of 57.1% (Patient 1) and 69.3% (Patient 2). Using this sequential treatment, the patients did not experience any deterioration of their status. Both patients had a favorable outcome after surgery. However, until such a procedure will be validated on a larger series of patients, it should be restricted to patients presenting with a refractory life-threatening thrombocytopenia and/or requiring surgery or any kind of invasive procedure. Am. J. Hematol. 68:198-201, 2001. Published 2001 Wiley-Liss, Inc.     

Cell-based quantification of molecular biomarkers in histopathology specimens

Al‐Kofahi Y, Lassoued W, Grama K, Nath S K, Zhu J, Oueslati R, Feldman M, Lee W M F & Roysam B
(2011) Histopathology 59 , 40–54 Cell‐based quantification of molecular biomarkers in histopathology specimens Aims: To investigate the use of a computer‐assisted technology for objective, cell‐based quantification of molecular biomarkers in specified cell types in histopathology specimens, with the aim of advancing current visual estimation and pixel‐level (rather than cell‐based) quantification methods. Methods and results: Tissue specimens were multiplex‐immunostained to reveal cell structures, cell type markers, and analytes, and imaged with multispectral microscopy. The image data were processed with novel software that automatically delineates and types each cell in the field, measures morphological features, and quantifies analytes in different subcellular compartments of specified cells.The methodology was validated with the use of cell blocks composed of differentially labelled cultured cells mixed in known proportions, and evaluated on human breast carcinoma specimens for quantifying human epidermal growth factor receptor 2, estrogen receptor, progesterone receptor, Ki67, phospho‐extracellular signal‐related kinase, and phospho‐S6. Automated cell‐level analyses closely matched human assessments, but, predictably, differed from pixel‐level analyses of the same images. Conclusions: Our method reveals the type, distribution, morphology and biomarker state of each cell in the field, and allows multiple biomarkers to be quantified over specified cell types, regardless of their abundance. It is ideal for studying specimens from patients in clinical trials of targeted therapeutic agents, for investigating minority stromal cell subpopulations, and for phenotypic characterization to personalize therapy and prognosis.