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J Wechsler

Publications and source records attributed to J Wechsler.

At least 19 recordsLinked to original sources

Cutaneous T-cell infiltrates: analysis of T-cell receptor gamma gene rearrangement by polymerase chain reaction and denaturing gradient gel electrophoresis.

In cutaneous T-cell infiltrates, the demonstration of a clonal T-cell receptor (TCR) gene rearrangement has been considered helpful to distinguish Cutaneous T-cell lymphomas from reactive lymphoproliferation. Hence, a polymerase chain reaction (PCR) method using GC-clamp primers and denaturing gradient gel electrophoresis has been developed in our laboratory to analyze the TCR gamma locus configuration. Two hundred eleven cutaneous samples from 155 patients were analyzed. A detectable clonal TCR gamma rearrangement was significantly associated with cutaneous T-cell lymphomas as defined by morphologic and immunologic criteria. A clonal TCR gamma rearrangement was also detected frequently in lymphomatoid papulosis, never in reactive lymphocytic infiltrates and B-cell lymphomas, and rarely in parapsoriasis en plaque and cutaneous lymphoid hyperplasia. Forty five patients had both a cutaneous and a peripheral blood sample. Fifteen had a detectable clonal rearrangement in the two samples and 22 were negative. Six patients had a positive skin sample and a negative blood sample, whereas two patients had a positive blood sample and a negative skin sample. Four lymph node samples were analyzed and the PCR results were the same as in the skin. Finally, 21 patients had sequential samples of recurrent skin lesions. The PCR results were concordant in all and, when detectable, the clonal TCR gamma rearrangement remained unchanged in a given patient. Because of its simplicity and accuracy, the newly designed PCR procedure improves the monitoring of diagnosis, staging, and follow-up in cutaneous T-cell infiltrates.

Base Sequence

Dermatomyositis without muscle weakness. Long-term follow-up of 12 patients without systemic corticosteroids.

BACKGROUND AND DESIGN: We retrospectively studied 12 patients with dermatomyositis (DM) with at least one laboratory criterion of muscle involvement but without muscle weakness. We reported their outcome without systemic corticosteroid treatment and searched for distinctive baseline data and associated neoplasia among a group of 29 patients with classic DM. RESULTS: After a mean follow-up of 5.3 years, complete or partial skin improvement occurred in nine patients with DM without muscle weakness, and muscle enzyme levels were normalized in all. When compared with the classic DM group, no statistical differences were found for associated neoplasia or for any baseline data studied except, as expected, for muscle parameters. CONCLUSIONS: The lack of distinctive baseline data between DM without muscle weakness and classic DM suggests that there is a continuum from amyopathic to classic DM. However, DM without muscle weakness is a clinical entity with respect to favorable long-term prognosis. Unless frank muscle weakness develops, treatment with systemic corticosteroids can be avoided.

Adolescent

Primary cutaneous pleomorphic small T-cell lymphoma. A review of 11 cases. The French Study Group on Cutaneous Lymphomas.

BACKGROUND AND DESIGN: Cutaneous pleomorphic small T-cell lymphoma is a rare, recently recognized lymphoma, different from mycosis fungoides and Sézary syndrome. Only a few cases have been reported and no treatment modalities have been defined. We reviewed the clinical, histologic, immunohistochemical, molecular biologic, and follow-up data of 11 primary cutaneous pleomorphic small T-cell lymphomas. RESULTS: The lesions presented as red purplish nodules, tumors, or plaques. The infiltrate consisted of small pleomorphic lymphoid cells without epidermotropism in nine patients and with a propensity to infiltrate the dermis and subcutaneous fat. Most cases were CD4+/CD8-. A T-cell clone was detected in the skin lesions of nine patients tested. The mean follow-up was 70.1 months and the median follow-up was 20 months. Ten patients are alive with three having persistent lesions. Interferon alfa-2a induced partial or complete remissions in five patients. Interferon alfa-2a combined with a regimen containing doxorubicin chlorhydrate induced a complete remission in a patient suffering a relapse after cyclophosphamide and interferon alone. CONCLUSIONS: Cutaneous pleomorphic small T-cell lymphoma is a well-defined type of low-grade cutaneous lymphoma with favorable prognosis. Interferon and/or chemotherapy are the treatment of choice in patients with large tumor burden.

Adult

Clinicopathologic correlation in erythema multiforme and Stevens-Johnson syndrome.

BACKGROUND AND DESIGN: To confirm the recent hypothesis that the spectrum of severe erythema multiforme (EM) is actually composed of two different disorders, we retrospectively studied 38 such cases, particularly in regard to their histopathologic features. Based on photographs and a recent proposal, the cases were classified as EM major when the eruption consisted of typical or raised atypical target lesions located on the extremities and/or the face or as Stevens-Johnson syndrome when the eruption consisted of flat atypical target lesions or purpuric macules that were widespread or distributed on the trunk. The cases were also assessed for causal agent. A biopsy specimen was obtained in each case. Several histologic parameters were analyzed (and scored) without clinical data and correlated to the clinical pattern. These parameters were first studied in a global assessment and then in a detailed evaluation. RESULTS: The global assessment showed two different histological patterns: (1) a predominantly inflammatory pattern characterized by a lichenoid infiltrate and epidermal necrosis that mainly affected the basal layer; and (2) a predominantly necrotic pattern in which major epidermal necrosis and minimal inflammatory infiltration were found. The former pattern was associated with EM major, the latter with Stevens-Johnson syndrome (P < .001) and with drug-related cause (P < .001). The detailed evaluation showed also less epidermal necrosis, and more dermal inflammation, and more exocytosis in EM major. Conversely, there was more epidermal necrosis, less dermal inflammation, and less exocytosis in Stevens-Johnson syndrome. The difference was statistically significant for the inflammation and exocytosis. CONCLUSIONS: This study suggests that the two different symptomatologies in the spectrum of severe EM correlate with two different patterns of histopathologic changes. A prospective multicentered study should be conducted to definitively characterize these entities.

Erythema Multiforme

Langerhans' cell histiocytosis. Definitive diagnosis with the use of monoclonal antibody O10 on routinely paraffin-embedded samples.

The histological and immunohistochemical findings of 34 biopsy specimens from patients with Langerhans' cell histiocytosis (LCH) are reported, with special emphasis on the findings with CD1a mouse monoclonal antibody (MAb) O10 using paraffin-embedded material. Eighteen patients were treated in an adult hospital (mean age, 26.3 years), and the 16 others were children (mean age, 3 years) from a pediatric center. Specimens included 17 bone, 14 skin, two lung, and one lymph node. Tissue was fixed in formalin or Bouin's, and most bone samples were decalcified in nitric acid. Frozen sections were available for 16 cases and electron microscopy for one. Light microscopy was suggestive of LCH in all cases, characterized by large mononucleated cells with abundant eosinophilic cytoplasm and "coffee bean" nucleus. In 33 of the 34 paraffin-embedded LCH samples, mononucleate cells were stained by MAb O10. As controls, we investigated seven tumors expressing S-100 protein (three nevi, two melanomas, two neurofibromas): all were negative with MAb O10. Five non-Langerhans' cell histiocytoses (three juvenile xanthogranulomas and two Rosai-Dorfman lymphadenopathies) were also negative with MAb O10. The results show that in most cases a definitive diagnosis of LCH can be assessed on paraffin-embedded tissue specimens with the help of immunohistochemistry using MAb O10.

Adult

Primary cutaneous medium and large cell lymphomas other than mycosis fungoides. An immunohistological and follow-up study on 54 cases. French Study Group for Cutaneous Lymphomas.

Primary cutaneous medium and large cell lymphomas (MLCL) other than mycosis fungoides (MF) are rare, and their prognosis and treatment are controversial. The clinical, immunohistological and follow-up data of 54 well-documented cases of primary cutaneous MLCL other than MF, seen in our institutions over a 14-year period, were retrospectively reviewed, in order to determine the prognostic factors related to these lymphomas, and to analyse the results obtained with different treatment regimens. Forty-six patients presented with a solitary tumour or with localized lesions, and eight had disseminated cutaneous lesions. According to the updated Kiel classification, 45 cases (83%) corresponded to B-cell lymphomas: centroblastic lymphomas, 32 cases; centroblastic-centrocytic lymphomas, 11 cases; immunoblastic lymphomas, two cases. Nine cases (17%) were classified as T-cell lymphomas: pleomorphic medium and large cell lymphomas, eight cases; anaplastic large cell lymphoma, one case. Four of eight patients with disseminated skin lesions had a T-cell lymphoma, whereas 41 of 46 patients with a solitary tumour had a B-cell lymphoma. Patients with disseminated skin lesions and elevated serum lactate dehydrogenase (LDH) levels had a poor prognosis. Comparison of patients' overall survival, depending on immunohistological subtype, showed that the median survival of patients with pleomorphic T-cell lymphoma was 2.5 years, whereas it was not reached at 12 years for patients with centroblastic-centrocytic and centroblastic lymphoma. The eight patients with disseminated skin lesions were treated with polychemotherapy.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult

Pachydermodactyly and atrophia maculosa varioliformis cutis.

Pachydermodactyly is a rare form of superficial digital fibromatosis characterized by progressive asymptomatic thickening of the back and sides of the proximal interphalangeal joints of the fingers. Atrophia maculosa varioliformis cutis is an acquired dermal atrophy, localized on the cheeks. Only a few cases of each pathology have been published. We find it interesting to report the case of a patient with both conditions as these two connective-tissue diseases are very rare. The association is probably fortuitous.

Adult

Gene analysis in 18 cases of cutaneous lymphoid infiltrates of uncertain significance.

BACKGROUND AND DESIGN: Patients with cutaneous lymphoid infiltrates that appear reactive histologically and immunophenotypically may develop clinically overt cutaneous lymphoma, suggesting the possibility of misdiagnosis by classical methods. We investigated DNA rearrangement in such cases of lymphoid infiltrates of uncertain significance to determine whether this more sensitive method could detect an occult monoclonal lymphoid proliferation. METHODS AND PATIENTS: Skin biopsy specimens were taken from 18 cutaneous lymphoid infiltrates diagnosed as reactive on the basis of clinical, histopathological, and immunohistochemical criteria. Specimens included 12 cases with mixed lymphoid infiltrates rich in polytypic B cells and inconstant follicle formation and 6 cases with exclusive T-lymphoid infiltrates. Southern blot analysis for immunoglobulin and T-cell-receptor beta-chain gene rearrangements was performed in all cases. RESULTS: No specimen showed T-cell-receptor beta-chain gene rearrangement. Clonal immunoglobulin gene rearrangement was demonstrated in one case with polytypic B cells, but no clinical malignancy has appeared 19 years after disease onset duration and 7 years after detection of the B-cell clone. CONCLUSIONS: In the present series, the results suggest that histological and immunohistological criteria are appropriate to establish the diagnosis of most cases of cutaneous lymphoid infiltrates. The detection of a B-cell clone is remarkable by absence of clinical malignancy, suggesting that such a discovery does not necessarily mean an aggressive evolution. Nevertheless, there is presently no way to predict the prognosis of a clonal lymphoid proliferation, indicating that a long-term follow-up is necessary.

Adult

Hyperplastic pacinian corpuscles: an uncommonly encountered lesion of the hand.

The occurrence of hyperplastic pacinian corpuscles in the hand is rare, with only 13 cases reported in the literature. We describe such a case in a 70-year-old male who had worked as a locksmith for many years. A grape-like cluster of firm, rice-sized nodules was discovered in the subcutaneous tissue of the finger following a glass-induced injury. Histopathological findings revealed pacinian corpuscles to be increased in size and number. Individual corpuscles consisted of a central nerve fiber surrounded by 35 to 60 concentric lamellae (normal controls from other specimens: 13-15 lamellae). The external corpuscular diameter ranged from 1.8 to 3.2 mm (normal controls from other specimens: 1.6 mm). Immunohistochemistry showed positive staining with Leu 7 antibody and antiglial fibrillary acidic protein in the small nerves situated in the vicinity of the pacinian corpuscles, but not in the corpuscles themselves. The lesion reported here clearly differed from both neurofibroma with occasional pacinian differentiation and the so-called pacinian neurofibroma. There was no evidence of neurofibromatosis.

Aged

[Non-bacterial thrombotic endocarditis complicating metastatic melanoma].

INTRODUCTION: Non-bacterial thrombotic endocarditis is defined by the presence of cardiac aseptic valvular vegetations made of fibrin and platelets. We report a case diagnosed ante mortem that occurred in a patient with metastatic melanoma. CASE REPORT: M. S., presented with a metastatic melanoma associated with an acute neurological deficit. Cerebral computerized tomographies and nuclear magnetic resonance showed occipital ischaemic lesions in the territory of the posterior cerebral artery. Cardiac echography showed large vegetations of the mitral valve. Bacterial endocarditis was eliminated. The diagnosis of non-bacterial thrombotic endocarditis was made, confirmed by post mortem examination. DISCUSSION: Non-bacterial thrombotic endocarditis is the second cause of neurological manifestations in patients with cancer. The presentation of our case was unusual because of an audible heart murmur and vegetations visible on echography.

Adult

[From Murphy's button to Valtrac].

The author gives an account of the history of methods of mechanical compression viscerosynthesis without using sewing material and presents a report of the first clinical application of Valtrac on the territory of former Czechoslovakia.

Aged