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Biomedical subjects

X Mariette

Publications and source records attributed to X Mariette.

At least 37 records · Page 2Linked to original sources
PubMed2002

[The phenol red thread first results for the assessment of the cut-off value in ocular sicca syndrome].

PURPOSE: To determine the cut-off value of the phenol red-impregnated thread test (Zone-Quick((R)), Menicon trade mark ) for the diagnosis of ocular sicca syndrome using the ROC (receiver operating characteristic) procedure and to estimate the agreement with the Schirmer I test (without anesthetics). MATERIAL: and methods: Fifty-four consecutive patients (including 50 females) with dry eyes, presumably related to an immune disorder, were recruited on the basis of subjective ocular symptoms and medical history (sicca syndrome). Both the phenol red thread (PRT) test and the Schirmer I test (testing periods, 15s and 5min, respectively) were performed in both eyes in random order. Only the lowest result for each test was used in statistical analyses. The same procedure was applied to 29 normal volunteers (no subjective symptoms). The patient and the control groups were matched for age and gender (mean age, 58.1 and 59.6, respectively). RESULTS: The ROC procedure showed that a cut-off value of 12mm in the PRT test provided the best ratio between sensitivity and specificity (56% and 69%, respectively) for the detection of dry eyes. Using this threshold, the agreement with the Schirmer I test was highly significant (kappa test; P<10(-3)). However, discordant results were observed in 32% of subjects. CONCLUSION: Giving a cut-off value at 12mm, the sensitivity and specificity of the PRT are 56% and 69%, respectively. Even if the agreement with the Schirmer I test is highly significant, 32% of patients have discordant results. These two methods of functional assessment of tear secretion are therefore complementary and further studies remain necessary to better understand the place of both tests in clinical practice.

Coloring Agents↗
PubMed2001

[Immunopathology of Gougerot-Sjogren syndrome].

Sjögren's syndrome presents as an autoimmune exocrinopathy, and the term autoimmune epitheliitis has recently been coined. The major lesion is a lymphocyte infiltrate affecting the salivary glands and consisting predominantly of activated CD4+ T cells. The remaining 20% B lymphocytes, first polyclonal, turn out to be monoclonal. Epithelial cells are endowed with a key-part in the play, inasmuch as they gather together most of the Sjögren's syndrome-specific target autoantigens within apoptotic bodies, and possess all the characteristics of antigen presenting cells. There appears to be every likelihood that the sequence is triggered off by a thus far unknown virus.

Apoptosis↗
PubMed2001

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.

Adult↗
PubMed2001

Lymphomas complicating Sjögren's syndrome and hepatitis C virus infection may share a common pathogenesis: chronic stimulation of rheumatoid factor B cells.

BACKGROUND: The occurrence of B cell non-Hodgkin's lymphoma is a complication of Sjögren's syndrome (SS) and, at least in some countries, of chronic hepatitis C virus (HCV) infection. Lymphomas occurring in both diseases share a number of characteristics: predominance of low grade, marginal zone histological type, frequency of mucosal localisation, possible transformation into a large B cell lymphoma, association with asymptomatic low level cryoglobulinaemia, absence of virus within lymphoma cells, but localisation of lymphomas in organs where the chronic viral infection is active in patients with HCV and where the autoimmune disease is active in patients with SS. HYPOTHESIS: It is proposed that in both diseases the first event of lymphomagenesis is the chronic stimulation at the site of the disease of polyclonal B cells secreting rheumatoid factor (RF). Then, that these RF B cells may become monoclonal and disseminate in other organs. The monoclonal secreted RF complexed with polyclonal IgG may cryoprecipitate. The following step would be a chromosomal abnormality (for example, trisomy 3 or bcl-2 translocation) which would confer to these cells a low grade B cell lymphoma comportment. A last event (for example, a mutation of p53) might transform this low grade B cell lymphoma into a high grade, large B cell lymphoma. The non-random utilisation of VH and VL by SS associated lymphoma B cells and the recent demonstration that these lymphoma B cells may display RF activity support the hypothesis that these lymphomas grow through an autoantigen driven process. CONCLUSION: The best preventive treatment of lymphoproliferations occurring in SS probably consists in decreasing the hyperactivation of autoreactive B cells when it is present, allowing the use of immunosuppressive drugs such as methotrexate or even tumour necrosis factor alpha antagonists, which in theory could favour other types of lymphoproliferation.

Antiviral Agents↗
PubMed2000

Image-guided core-needle biopsy in patients with suspected or recurrent lymphomas.

BACKGROUND: It is now commonly admitted that the diagnosis of recurrence of lymphoma can be assessed by image-guided needle biopsy (IGNB). However, the means of obtaining tissue for the original diagnosis of lymphoma is often surgery. The aim of this study was to compare the accuracy of IGNB at the time of diagnosis and at the time of recurrence or progression. METHODS: The authors performed 212 IGNBs on 194 patients who eventually had a diagnosis of lymphoma. One hundred three IGNBs were obtained at original diagnosis and 109 at recurrence or progression. Large-cutting core-biopsy needles, ranging in size from 20 gauge to 14 gauge, were used. Immunohistochemistry studies were performed in all lymphoma cases. RESULTS: A diagnosis of lymphoma with subtyping was obtained in 88% of all cases, in 85% at initial diagnosis, and in 89% at follow-up. Therapy was initiated on the basis of IGNB in 93% of all cases, in 91% at initial diagnosis, and in 94% at follow-up. Benign complications occurred in 7.5% of cases and did not require specific treatment. IGNB was equally effective for making a specific diagnosis of lymphoma and initiating therapy at the time of original diagnosis and at follow-up. CONCLUSIONS: The authors recommend that IGNB be performed as the initial procedure for the diagnosis of lymphoma in the absence of peripheral lymph nodes, either at presentation or at recurrence.

Adolescent↗
PubMed2000

[Polyclonal B lymphocytosis and hyper-IgM: immunodeficiency and/or benign lymphoid proliferation associated with tobacco?].

PURPOSE: To study the association of polyclonal B-cell lymphocytosis with binucleated lymphocytes with clinical manifestations suggesting the existence of an immunodeficiency, to evaluate the effect of cigarette smoking on this 'benign lymphoid proliferation', to analyze the clonality of lymphocytes, to determine the levels of immunoglobulins (Ig) G, A, M. METHODS: Description and analysis of the results obtained in four patients and literature review. RESULTS: Polyclonal B-cell lymphocytosis is associated with both a decrease in IgA and IgG and an increase in IgM. Recurrent infectious episodes (bronchitis) were observed in two patients. Transient smoking cessation allowed a decrease in lymphocytosis and IgM levels in two patients. No hematological malignancy occurred during the follow-up, while biological abnormalities persisted. CONCLUSION: Persistent polyclonal B-cell lymphocytosis may be associated with minor clinical features of immunodeficiency. Smoking cessation may sometimes lead to a decrease in lymphocytosis and IgM.

Adult↗
PubMed2000

Hematological malignancies and the bone (myeloma excluded).

Bone involvement is a rare event in lymphomas, except in patients with adult T-cell leukemia/lymphoma associated with HTLVI. It is usually characterised by lytic bone lesions located in the metaphysis of long bones or in the axial skeleton. The occurrence of bone lesions reflects a progression of the disease affecting the prognosis that is related to lymphoma histologic features and staging. Bone lesions may occur in some lymphoproliferative disorders such as LLC or Waldenstrom's disease, or in myeloproliferative disorders. They may reflect a progression to a more aggressive disorder with a worse prognosis. The treatment of hematologic malignancies presenting with bone lesions and/or hypercalcemia is similar to the treatment of the systemic disease. In primary lymphomas of bone presenting with an isolated bone lesion, local treatment with radiation therapy and/or surgical ablation is required, and adjuvant chemotherapy may improve the prognosis of these located lymphomas. Glucocorticoid therapy and bisphosphonates are effective in treating associated hypercalcemia. Except for myeloma and ATL, the underlying mechanisms responsible for bone involvement in hematologic malignancies remain poorly understood. The unusual occurrence of bone lesions in these diseases probably implies distinct pathogenic mechanisms, but one can speculate that an increased expression of RANK/RANKL, the common final pathway in bone resorption, may be involved.

Bone Neoplasms↗
PubMed1999

[Bone marrow transplantation in the treatment of autoimmune diseases. ISAMAIR Group].

EXPERIMENTAL BASIS AND CLINICAL OBSERVATIONS: Remission of an autoimmune disease has been observed in certain patients after bone marrow allograft from a healthy donor. Autoimmune disease in the donor can also be transmitted to an unaffected recipient. These phenomena would be hematopoietic-dependent. BONE MARROW ALLOGRAFTS: Indications for the treatment of refractory autoimmune diseases are exceptional due to the related mortality even in patients without malignant hematologic disease. A NEW THERAPEUTIC CONCEPT: Therapeutic intensification, followed with autologous peripheral stem cell grafts, a procedure with a mortality below 3% in 1997, constitutes a therapeutic alternative in these difficult indication concerning severe refractory autoimmune diseases including: sclerodermia, vasculitis, lupus, inflammatory myositis, autoimmune cyopenia.

Autoimmune Diseases↗
PubMed1999

[Gougerot-Sjögren syndrome. Risk of lymphoma].

LYMPHOMA RISK: Lymphoma is a very severe complication of primary Sjögren's syndrome: 5 to 10% of patients followed for more than 10 years will develop a lymphoma. Predictive factors include serum monoclonal immunoglobulins or cryoglobulins and a B clone population in accessory salivary glands. TYPICAL CLINICAL AND HISTOLOGICAL PRESENTATION: Mucosal involvement (parotid as well as gastric or pulmonary localizations) is frequent. According to the recent classification of lymphomas, most lymphomas developing in patients with Sjögren's syndrome are B lymphomas of the marginal zone: MALT lymphomas or low-grade nodal monocytoid lymphomas which are sometimes not identified until transformation to the giant cell stage. SIMILARITIES WITH HEPATITIS C LYMPHOMAS: The pathophysiology of lymphoma in Sjögrën's syndrome remains unknown. To date, there is no argument favoring a viral infection or a deregulation of a unique oncogene or anti-oncogene. Certain similarities between lymphomas in Sjögren's syndrome and lymphomas associated with hepatitic C virus would suggest a common pathogenisis: possibly a permanent stimulation of auto-reactive B cells carrying a surface immunoglobulin with rheumatoid factor activity in the target organs of the autoimmune disease. These B lymphocytes would then proliferate secondarily.

Humans↗
PubMed1999

Prognostic value of vertebral lesions detected by magnetic resonance imaging in patients with stage I multiple myeloma.

We assessed the role of spinal magnetic resonance imaging (MRI) and bone densitometry as prognostic factors in patients with asymptomatic stage I multiple myeloma (MM) and negative skeletal survey. 55 consecutive patients underwent spinal MRI and 41 of them underwent bone densitometry by dual-energy X-ray absorptiometry (DEXA). Spinal MRI studies showed evidence of bone marrow involvement in 17/55 patients (31%). A diffuse pattern was present in three patients and a focal pattern in 14 patients, nine of them with only one nodular lesion. During a median follow-up of 25 months, 10 patients had disease progression, 8/17 patients with abnormal MRI and 2/38 patients with normal MRI. Median time to disease progression was not reached in both groups but was significantly different for patients with normal and those with abnormal patterns on MRI (P < 0.0001). Lumbar BMD was only slightly decreased compared with normal people (median lumbar Z score -0.43) and was not of prognostic value. Using a multivariate analysis the only two independent significant prognostic parameters were abnormal MRI (P<0.001, HR 30.4, 95% CI 4.3-213) and bone marrow plasmacytosis >20% (P=0.004, HR 16.4, 95% Cl 2.6-104). Thus, spinal MRI but not bone densitometry, appeared to be justified in patients with stage I asymptomatic MM and negative skeletal survey.

Absorptiometry, Photon↗
PubMed1999

Neurolymphomatosis in Waldenström's macroglobulinaemia.

We report four patients with Waldenström's macroglobulinaemia with an unusual neurologic complication, neurolymphomatosis, characterized by meningeal and root nerve infiltration by lymphoplasmacytic cells. Patients presented with rapidly progressive leg proximal weakness. Examination of cerebrospinal fluid disclosed lymphoplasmacytic cells. Magnetic resonance imaging of the lumbar spine was suggestive of a tissular infiltration of leptomeninges and nerve roots. Chemotherapy and irradiation of involved tissues led to a remarkable and sustained neurological improvement.

Aged↗
PubMed1999

Nodular glomerulosclerosis with deposition of monoclonal immunoglobulin heavy chains lacking C(H)1.

The objective of this study was to further characterize the clinical and immunopathologic features of heavy chain deposition disease (HCDD), a recently described entity. Four patients were diagnosed as having HCDD on a kidney biopsy. All presented with nodular glomerulosclerosis with deposition of gamma1 heavy chains lacking CH1 epitopes, but without light chains. Two different patterns were observed in the serum. First, patients 1 and 2 had a circulating monoclonal IgGlambda containing a short gamma1 heavy chain lacking CH1 epitopes, with an apparent molecular weight of 40 kD consistent with a complete CH1 deletion. Biosynthetic experiments also showed that the deleted heavy chain was produced in excess compared with light chains, and was secreted in vitro together with half Ig molecules, although these abnormal components were not detected by Western blot analysis of whole serum. Second, patients 3 and 4 had a circulating monoclonal IgG1lambda with an apparently normal, nondeleted heavy chain subunit, but serum fractionation followed by immunoblotting revealed an isolated monoclonal gamma1 chain lacking CH1 epitopes. These data strongly suggest that renal deposition of a CH1-deleted heavy chain circulating in low amounts in the serum as a free unassembled subunit is a major feature of HCDD. The CH1 deletion is most likely responsible for the premature secretion in blood of the heavy chain by a clone of plasma cells.

Adult↗