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J Wechsler

Publications and source records attributed to J Wechsler.

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

[Blue-red macules and pseudoatrophic macules in neurofibromatosis 1].

INTRODUCTION: Blue-red macules are exception during neurofibromatosis 1. Between October 1988 and March 2000 in our cohort of patients, we systematically looked for these lesions. PATIENTS AND METHODS: Presence and number of blue-red macules were evaluated. Biopsies and photographs were proposed to patients. RESULTS: Forty-four patients out of 583 (39 y; range: 20-66 y) (7.5 p. 100; CI 95 p. 100: 5.5-9.8 p. 100) had blue-red macules mainly on the trunk. Histologically, blue-red macules corresponded to neurofibromatous tissue infiltrating capillary blood vessels and venules. CONCLUSION: Blue-red macules are a peculiar type of neurofibroma and can therefore be considered as a criterion for the diagnosis of neurofibromatosis 1.

Adult↗
PubMed2002

[Intravascular large cell lymphoma revealed by diffuse telangiectasia and cauda equina syndrome].

BACKGROUND: Intravascular lymphoma is a rare disease with usually fatal outcome, characterized by the proliferation within the lumen of small blood vessels of neoplastic large lymphoid cells of B-cell origin. We report a case of intravascular lymphoma revealed by diffuse telangiectasia and cauda equina syndrome. CASE REPORT: A 64-year-old Vietnamese woman presented with unexpected fever and weight loss. Three months after the onset of the first symptoms, an oedema appeared on the lower limbs and the trunk, followed by the eruption of diffuse superficial telangiectasia. Neurologic examination revealed a cauda equina syndrome. The diagnosis of intravascular B cell lymphoma was established on cutaneous and muscular biopsy specimen. A moderate hemophagocytic syndrome was observed, confirmed by bone marrow biopsy. Corticosteroid therapy was started, followed by combination chemotherapy yielding complete response. Six months later death occurred, without evidence of relapse of intravascular lymphoma. DISCUSSION: Clinical presentation of intravascular lymphoma is often confusing, mimicking systemic disease, with a predilection for skin and nervous system involvement. Diagnosis is difficult and often an autopsy finding. Prognosis is generally poor, but favourable responses to chemotherapy have been observed after early diagnosis and treatment. The pathogenesis of intravascular lymphoma remains unknown. Dysfunction of cell-endothelial interaction affecting adhesion molecules has been suspected. The implication of Epstein-Barr virus in intravascular lymphoma remains controversial.

Female↗
PubMed2002

[Acute pancreatitis from the aspect of the surgeon].

The authors provide evidence of the extreme increase of the frequency of acute pancreatitis. At the First Surgical Clinic in Brno in 1934 the frequency of acute pancreatitis (AP) was 0.2 pro mille (in the course of 3 years 4 patients among 20 000 hospitalized patients), at present (65 years later) in 1999 - 2001 it is 0.7%--74 patients among 10 676 hospitalized patients. This is 35 times more, 48 men (65%) and 26 women (35%). The mean age was 48.6 years, range 26 - 83 years. The lethality of the whole group was 9.5%. From the total number of 74 patients seriously ill patients (Atlanta II classification) 25 patients--34%, Atlanta I--49 patients--66%. 7 patients with acute necrotizing infected pancreatitis died--28% of the whole group. Initial treatment of AP is based on adherence to three principles:(a) intensive treatment, (b) elimination of etiological factors and (c) control of complications. Contrary to other affectios in AP, there is no specific surgical treatment. Indications for surgical treatment of AP: I. early stage--explorative laparotomy if stabilization of patient by adequate treatment to resolve comorbidity (NPB etc.) is impossible, II. advanced stage--evacuation of infected necroses, abscesses, infected or bleeding pseudocyst, elective sanation of biliary tract. The optimal timing is between the 2nd and 3rd week of the disease. Premature surgery does not have a favourable effect on the subsequent course of the disease.

Acute Disease↗
PubMed2001

Prognostic factors in primary cutaneous large B-cell lymphomas: a European multicenter study.

PURPOSE: Most primary cutaneous B-cell lymphomas have an excellent prognosis. However, primary cutaneous large B-cell lymphomas (PCLBCLs) of the leg have been recognized as a distinct entity with a poorer prognosis in the European Organization for Research and Treatment of Cancer (EORTC) classification. This distinction on the basis of site has been debated. Our aim was to identify independent prognostic factors in a large European multicenter series of PCLBCL. PATIENTS AND METHODS: The clinical and histologic data of 145 patients with PCLBCL were evaluated. According to the EORTC classification, 48 patients had a PCLBCL of the leg and 97 had a primary cutaneous follicle center-cell lymphoma (PCFCCL). Data from both groups were compared. Univariate and multivariate analyses of specific survival were performed using a Cox proportional hazards model. RESULTS: Compared with PCFCCL, PCLBCL-leg were characterized by an older age of onset, a more recent history of skin lesions, a more frequent predominance of tumor cells with round nuclei and positive bcl-2 staining, and a poorer 5-year disease-specific survival rate (52% v 94%; P <.0001). Univariate survival analysis in the entire study group showed that older age, a more recent onset of skin lesions, the location on the leg, multiple skin lesions, and the round-cell morphology were significantly related to death. In multivariate analysis, the round-cell morphology (P <.0001), the location on the leg (P =.002), and multiple skin lesions (P =.01) remained independent prognostic factors. The round-cell morphology was an adverse prognostic factor both in PCLBCL-leg and in PCFCCL, whereas multiple skin lesions were associated with a poor prognosis only in patients with PCLBCL-leg. CONCLUSION: With site, morphology, and number of tumors taken into account, guidelines for the management of PCLBCL are presented.

Adult↗
PubMed2001

[Pulmonary hyalinising granuloma: report of 2 original cases with cervicofacial and orbital involvement].

INTRODUCTION: Pulmonary hyalinizing granuloma is a rare fibrosing nodular disease of the lung characterized by its histological appearance, which includes at the center of the lesion a dense network of concentric hyalinized collagen lamella surrounded by perivascular lymphoplasmacytic infiltrate that rarefies in the center of the nodule. EXEGESIS: We report two new cases: the first with laryngeal (endoluminal tumor-like), orbital (subeyelid nodule) and mesenteric (9 x 6 cm mass) location of hyalinizing granuloma; the second with cervical, facial (trismus), orbital (pseudotumor) and limb (ankylosing elbow) fibrosis. CONCLUSION: The extrapulmonary diffusion of the disease is extremely rare. In one of the cases, with corticosteroids and after a follow-up of 12 months, the pulmonary tumors vanished but the fibrosis resolved only partially.

Adult↗
PubMed2001

[Juvenile dermatomyositis and panniculitis-type subcutaneous T-cell lymphoma. A case report].

INTRODUCTION: Juvenile dermatomyositis does not have, unlike the adult form, a paraneoplastic character. EXEGESIS: We report the case of a 12-year-old girl who has a typical dermatomyositis complicated by the appearance of very aggressive lesions of lobular panniculitis predominating in the lower limbs. Their refractory character to different treatments (in particular corticosteroids, chloroquine, dapsone, methotrexate, azathioprine, intravenous immunoglobulins and ciclosporin A) will finally lead to the diagnosis of subcutaneous T-cell lymphoma. This will be complicated by a macrophagic activation syndrome leading to death despite chemotherapy. CONCLUSIONS: The occurrence of panniculitis during a dermatomyositis should lead to the search for a subcutaneous T-cell lymphoma, especially if the lesions are locally aggressive and refractory to usual treatments, which permits an early chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2001

Trichoepithelioma with "monster" stromal cells.

Trichoepithelioma is a benign tumor of trichogenic origin which appears predominantly in childhood or in young adults. Different forms have been described according to clinical and histological features. The authors report a unique variant of trichoepithelioma arising on the limb of a 27-year-old man. The tumor was characterized by the mixture of an atypical fibroxanthomatous proliferation and basaloid epithelial strands of trichoepithelioma. Such histological features have not been previously reported. It raises the question of an additional variant of hair follicle tumor with a mixed epithelial and mesenchymal proliferation.

Adult↗
PubMed2001

Erythrodermic cutaneous T-cell lymphoma with disseminated pustulosis. Production of high levels of interleukin-8 by tumour cells.

Interleukin (IL) -8 is a neutrophil chemoattractant cytokine with proinflammatory and growth-promoting activities, which is involved in the pathogenesis of several inflammatory diseases. It is found in high amounts in lesional biopsies of pustular diseases such as psoriasis and palmoplantar pustulosis. We report a 50-year-old woman with a 10-year history of erythroderma with disseminated pustulosis. Skin biopsies showed an epidermotropic infiltrate composed of atypical CD4+ CD8+ lymphocytes with numerous admixed neutrophils. Peripheral blood flow cytometric analysis revealed a major clonal subset of CD3+ CD4+ CD8+ T-cell receptor Vbeta22+ atypical lymphocytes. Bone marrow biopsy, lymph node biopsy and computed thoracoabdominal tomography were normal. Serologies for human T-cell lymphotropic virus type I and human immunodeficiency virus were negative. Our patient's status deteriorated despite topical (nitrogen mustard, psoralen plus ultraviolet A) and systemic (interferon, methotrexate, multiagent chemotherapy) treatments, and she finally died. We showed that our patient's peripheral blood lymphocytes (PBL) spontaneously produced high amounts of IL-8. In contrast, PBL of patients with classical Sézary syndrome produced lower amounts of IL-8. The production of IL-8 by tumour T cells could explain this unusual clinical and histopathological presentation of cutaneous T-cell lymphoma as disseminated pustulosis.

Dermatitis, Exfoliative↗
PubMed2001

Crosstalk between tumor T lymphocytes and reactive T lymphocytes in cutaneous T cell lymphomas.

We have established several tumor T cell lines, both from the skin and from the blood of a patient with an MHC class II-/class I+, CD4+ cutaneous T cell lymphoma (CTCL). These cell lines, like the initial tumor cells, had a CD3+CD4+CD8- phenotype. We also isolated two cytotoxic T lymphocyte clones from the tumor site of this CTCL patient. These clones displayed a CD4+CD8dim+ (TC5) and CD4+CD8- (TC7) phenotype and mediated a specific MHC class I-restricted cytotoxic activity toward noncultured tumor cells and autologous tumor cell lines. Despite surface expression of Fas on tumor cells and Fas-L induction on TC5 and TC7 cell membrane after coculture with autologous tumor cells, the CD4+ CTL clones did not use this cytotoxic mechanism to lyse their specific target. TC7 used a granzyme/perforin-dependent pathway, whereas TC5 used a TRAIL-dependent mechanism. Quantitative analysis of cytokine mRNA expression indicated that while the tumor cells displayed a Th2-type profile, the CTL clones expressed Th1-type cytokines. Preincubation of TIL clones with autologous tumor cells in a short-term culture induced their activation and subsequent amplification of the Th1-type response, which indicates a direct contribution of the malignant cells in the Th1/Th2 imbalance. However, we found that tumor cells produced high amounts of TGF-beta, which could explain the inhibition of a specific antitumor immune response. Another mechanism to avoid the host immune response was the expression of CD158a, CD158b, p70, and CD94/NKG2A inhibitory receptors by tumor-specific lymphocytes. Finally, we present recent data on new antigen structures expressed both by long-term CTCL lines and uncultured tumor cells.

Antigens, Neoplasm↗
PubMed2001

[Large hairy pigmented spots in neurofibromatosis type 1: an atypical form of neurofibromas].

INTRODUCTION: Large hairy pigmented spots have been observed in patients with neurofibromatosis type 1. In this study we tried to determine the nature and the frequency of these hairy pigmented spots in neurofibromatosis type 1. PATIENTS AND METHODS: In patients with neurofibromatosis type 1, hairy pigmented spots with a diameter more than 3 cm were systematically notified. Realisation of the biopsy of the spot was proposed to the patient. RESULTS: Among 614 patients with neurofibromatosis type 1, seven (1.1 p. 100) had a large hairy pigmented spot. Biopsy was realized in six cases. In five cases, diagnosis was superficial and plexiform neurofibroma, the 6(th) case was a Becker's nevus. CONCLUSION: Large hairy pigmented spot is a rare aspect of superficial and plexiform neurofibroma during neurofibromatosis type 1. A biopsy may be useful if it is necessary for the disorder diagnosis.

Adolescent↗
PubMed2001

Malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1: a clinicopathologic and molecular study of 17 patients.

OBJECTIVE: To identify potential prognostic factors and criteria for early detection of malignant peripheral nerve sheath tumors associated with neurofibromatosis type 1 (NF1). DESIGN: Retrospective study of malignant peripheral nerve sheath tumors in a cohort of 395 patients with NF1 followed up between October 1, 1988, and January 1, 1999; review of the clinical and histological characteristics of treatment and course; and analysis of p53 mutations and overexpression in tumors. SETTING: Teaching hospital referral neurofibromatosis center for adults. PATIENTS: Seventeen patients with NF1 (9 males and 8 females). Mean +/- SD patient age at diagnosis was 32 +/- 14 years. MAIN OUTCOME MEASURES: (1) Clinical symptoms, (2) comparison of p53 mutations and overexpression in benign vs malignant tumors; and (3) median survival. RESULTS: Twelve patients had high-grade tumors. All tumors except 1 developed on preexisting nodular or plexiform neurofibromas. Pain and enlarging mass were the first and predominant signs. None of the benign tumors displayed significant p53 staining or p53 mutations. Six of 12 malignant tumors significantly overexpressed p53, and 4 of 6 harbored p53 missense mutations. Median survival was 18 months overall, 53 months in peripheral locations, and 21 months in axial locations. CONCLUSIONS: Malignant peripheral nerve sheath tumors are highly aggressive in NF1. They mostly arise from plexiform or nodular neurofibromas. Investigations and deep biopsy of painful and enlarging nodular or plexiform neurofibromas should be considered in patients with NF1. Late appearance of p53 mutations and overexpression precludes their use as predictive markers of malignant transformation.

Adolescent↗
PubMed2001

[Catamenial pneumothorax--case report].

The objective of the work was to present summarized information on a very rare catamenial pneumothorax. Catamenial pneumothorax is one of the forms of spontaneous pneumothorax. An integral part of the paper is a review of the literature. The authors present the case-record of a young woman with catamenial pneumothorax who suffered in the course of almost 11 months five episodes of spontaneous pneumothorax.

Adult↗
PubMed2000

Diagnostic value of dominant T-cell clones in peripheral blood in 363 patients presenting consecutively with a clinical suspicion of cutaneous lymphoma.

It is now widely accepted that polymerase chain reaction (PCR) analysis of cutaneous T-cell clonality is of diagnostic value in cutaneous T-cell lymphomas (CTCLs) and most helpful in the diagnosis of mycosis fungoides (MF). However, the diagnostic and prognostic value of circulating clonal T cells remains unclear. We studied T-cell clonality in the peripheral blood (PB) and the cutaneous lesion, sampled at the same time, in 363 consecutively seen patients with a clinical suspicion of cutaneous lymphoma. Using a PCR technique providing a specific imprint of T-cell clones (PCRgamma-denaturing gradient gel electrophoresis), we found that detection of identical circulating and cutaneous T-cell clones was associated with the diagnosis of CTCL (P <.001). Detection of circulating tumor cells in patients with MF was infrequent (12.5%), except in those with erythrodermic MF (42%; P =.003). Moreover, among the 46 patients who had identical circulating and cutaneous T-cell clones, 25 (56%) had erythroderma. The finding of a dominant clone in the PB but not in the skin was frequent, regardless of the clinicohistologic classification; it occurred in 30% of patients with CTCL, 41% with non-CTCL malignant infiltrates, and 34% with benign infiltrates. This pattern was significantly more frequent in patients over 60 years of age (P <.002), even in the CTCL group (P <. 01). In conclusion, dominant T-cell clones detected in the PB of patients with MF by using a routine PCR technique are rarely tumoral and are more often related to age. A multicenter prospective study is under way to establish the prognostic value of circulating tumor cells.

Adult↗
PubMed2000

Transformation of mycosis fungoides: clinicopathological and prognostic features of 45 cases. French Study Group of Cutaneious Lymphomas.

The course of mycosis fungoides (MF) is indolent except when transformation to a large T-cell lymphoma occurs. The diagnosis of transformed MF (T-MF) relies on the presence of more than 25% of large cells on biopsy of an MF lesion. We analyzed 45 patients with T-MF recorded by the French Study Group on Cutaneous Lymphomas to better determine clinicopathological features of MF transformation and to analyze their impact on prognosis. Median time from diagnosis of MF to transformation was 6.5 years. Extracutaneous progression was present in 20 patients. Mean survival from transformation to death was 22 months. In univariate analysis, only an extracutaneous progression was associated with a worse prognosis (5-year actuarial survival: 7.8% versus 32%). Neither sex, age, clinical and skin disease stage at transformation, transformation speed, nor percentage of large cells or CD30 expression (14 of 45) had a prognostic value. When performing multivariate analysis, age (at least 60 years), and extracutaneous spreading were found to be associated with a poor prognosis. There was no difference between survival curves of patients with T-MF and with pleomorphic large T-cell CD30- lymphomas. The main diagnostic pitfall was "histiocytic-rich" MF, requiring CD68 staining for the diagnosis of T-MF. Out of 45 patients, 6 presented an histologic transformation before clinical progression, suggesting that an early histopathological diagnosis may be performed by histological follow-up. The prognostic value of such early histopathological diagnosis must be confirmed by prospective studies.

Adult↗
PubMed2000

Cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia: a clinical, immunohistological, and molecular analysis.

OBJECTIVE: To determine whether cutaneous involvement in patients with angioimmunoblastic lymphadenopathy with dysproteinemia (AILD) is related to a clonal T-cell proliferation. DESIGN: Retrospective study. SETTING: University hospitals. PATIENTS: Ten patients with AILD and cutaneous involvement. MAIN OUTCOME MEASURE: The T-cell receptor-gamma (TCRG)gene rearrangement was studied with the use of polymerase chain reaction and denaturing gradient gel electrophoresis in blood, nodal, and skin samples. Skin and nodal samples were investigated also for the presence of Epstein-Barr virus (EBV) RNA by in situ hybridization. RESULTS: A transient morbilliform eruption of the trunk was seen most often. Other cutaneous features were infiltrated plaques and purpuric or urticarial lesions. A clonal TCRG gene rearrangement was detected in 7 skin samples, corresponding to a maculopapular eruption with a histological pattern of nonspecific mild lymphoid dermal infiltrate in 6 patients, and to erythematous plaques with histological findings of typical cutaneous lymphoma in 1 patient. In the 5 patients in whom a TCRG gene rearrangement was evidenced in skin and lymph node samples, identical clones were detected in both. Five patients died by the end of the study, with a mean survival of 33.2 months. Four of these 5 patients had a clonal infiltrate in skin and lymph nodes. The EBV RNA was detected in only 1 of 10 skin biopsy specimens and in 5 of 8 lymph nodes tested. CONCLUSIONS: Cutaneous involvement is often related to a clonal T-cell proliferation in AILD, even when clinical and histological features are nonspecific. Cutaneous infiltrate seems to be clonally related to the nodal T-cell proliferation. The role of EBV infection in skin lesions was not evidenced.

Adult↗
PubMed2000

Primary cutaneous large B-cell lymphomas.

According to the European Organization for Research and Treatment of Cancer classification, primary cutaneous large B-cell lymphomas are subdivided into 2 groups: follicle center cell large B-cell lymphomas and large B-cell lymphomas of the leg. The first type predominantly affects middle-aged adults with an equal gender distribution and presents with lesions on the head and trunk. Histology shows proliferations of predominantly large cleaved cells. The prognosis is excellent, with a 5-year survival rate >90%. The second group predominantly affects elderly females. Histologically the lesions are composed of centroblasts and/or immunoblasts (large round cells). The prognosis is more unfavorable with a 5-year survival rate of 50%. Intravascular large B-cell lymphoma is still classified as a provisional entity.

Age Distribution↗