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Biomedical subjects

B Flageul

Publications and source records attributed to B Flageul.

At least 55 records · Page 3Linked to original sources
PubMed1995

Hyperkeratotic spicules and monoclonal gammopathy.

Hyperkeratotic spicules are a rare disorder that has been associated with different types of malignancies. An association with multiple myeloma raises the question of a relation between the monoclonal component and skin manifestations. We describe hyperkeratotic spicules in a 61-year-old man with a monoclonal gammopathy of undetermined significance. Immunofluorescence and immunoelectron microscopic findings demonstrated the deposition of the monoclonal immunoglobulin at the dermoepidermal junction. Our findings support previous reports that the association between hyperkeratotic spicules and monoclonal gammopathy is not fortuitous and may be related to particular properties of the monoclonal immunoglobulin.

Humans↗
PubMed1995

Detection of clonal T-cell receptor gamma gene rearrangements with the use of the polymerase chain reaction in cutaneous lesions of mycosis fungoides and Sézary syndrome.

BACKGROUND AND DESIGN: We used the amplification of junctional V (variable)-J joining sequences of the rearranged T-cell receptor gamma (TCR gamma) genes by polymerase chain reaction for rapid and sensitive detection of a clonal T-cell population in a total of 51 skin specimens obtained from 45 patients with mycosis fungoides, five patients with Sézary syndrome, and 29 patients with chronic inflammatory dermatoses. RESULTS: A clonal TCR gamma gene rearrangement was present in all tumors (3/3, 100%) and in most infiltrated plaques (16/22, 73%) and erythrodermas (10/12, 83%). In the patch stage, a clonal subset was found in more than half of the cases (8/14, 57%), whereas no clonality was observed in the controls. We also amplified the V-J sequences of the Igh locus coding for the heavy chain of immunoglobulins, without evidence of clonal rearrangement. These data were compared with those from in situ immunophenotypic analysis. Moreover, by using the same assay with successive dilutions of standard clonal T-cell DNA, a semiquantitative study of the T-cell clone was carried out in some cases. The highest ratios of clonal DNA were observed in advanced stages. CONCLUSIONS: These data validate polymerase chain reaction V gamma-J gamma as a rapid, sensitive tool that can be used in the routine analysis of clonality in cutaneous lesions of mycosis fungoides and in the early diagnosis of mycosis fungoides and Sézary syndrome. Semiquantitative studies suggest that the malignant T-cell clone follows a selective process during the course of the progressive form of mycosis fungoides.

Clone Cells↗
PubMed1995

High-dose recombinant interleukin-2 in advanced cutaneous T-cell lymphoma.

BACKGROUND AND DESIGN: Treatment of cutaneous T-cell lymphoma is still a difficult challenge, once the usual therapies (topical chemotherapy, phototherapy, radiation therapy, and chemotherapy) have proved to be unsuccessful. New therapies, mostly immunotherapies, are currently under investigation. The use of recombinant interleukin-2 has already been evaluated in hematopoietic malignancies. We decided to treat patients with advanced cutaneous T-cell lymphoma relapsing or progressing in spite of the usual treatments with high-dose recombinant interleukin-2. Seven patients (three with mycosis fungoides, three with Sézary syndrome, and one with nonepidermotropic large-cell cutaneous lymphoma) were included in this open study. They were scheduled to receive recombinant interleukin-2 at a dose of 20 x 10(6) IU/m2 per day, administered by continuous infusion during three fortnightly induction cycles and five monthly consolidation cycles. RESULTS: Three complete responses (two responses to mycosis fungoides; one response to large-cell lymphoma) and two partial responses were obtained. The clinical response appeared after the first cycle of treatment in the good responders. The complete responses are still ongoing 33, 28, and 6 months after completion of recombinant interleukin-2 therapy and without any further treatment. Sequential immunophenotypic studies showed an increase of the CD1+ cells in the dermal infiltrates. No significant modification of natural killer or cytotoxic T cells could be seen. CONCLUSIONS: Despite our low number of cases, our results clearly show that some advanced cutaneous T-cell lymphomas can benefit from high-dose recombinant interleukin-2 therapy. Further studies are necessary to determine the exact place of recombinant interleukin-2 in the therapeutic arsenal of cutaneous T-cell lymphoma.

Adult↗
PubMed1995

Lack of evidence of HTLV-I/II infection in T CD8 malignant or reactive lymphoproliferative disorders in France: a serological and/or molecular study of 169 cases.

Human T lymphotropic virus type II (HTLV-II), originally isolated in 1982 from a patient with a "T hairy cell leukemia", has not yet been proven to be the causative agent of any specific hematological disease. In order to screen for such an event, and because HTLV-II has a preferential tropism for OKT8 (CD8) T cells (both in vivo and in vitro), we searched for the presence of HTLV-II in lymphoproliferative diseases (LP) of CD8+ T cells. We report a serological and/or molecular study of 169 patients with a T CD8 LP, including 76 patients with malignant or reactive T CD8 LP (34 lymphomas, 27 large granular leukemias, three prolymphocytic leukemias, one hairy cell leukemia, 11 reactive T CD8 LP) and 93 HIV-1+ patients with a T CD8 peripheral lymphocytosis ( > 1500/mm3) from a prospective HIV cohort involving 1264 individuals. In the first series, the 40 sera available were all HTLV-I/II negative, except a 67-year-old French Guyanan man, with a cutaneous large T CD8 cell lymphoma, HTLV-I+. Furthermore, the molecular analysis of the 69 available DNA samples by PCR failed to detect any proviral HTLV-I/II sequences, except for the HTLV-I+ patient. The serological study of the 93 HIV-1+ individuals with CD8 lymphocytosis, showed that three patients were HTLV-I+, but none was HTLV-II+. Thus, in contrast to HTLV-I, whose etiological role in adult T cell leukemia is now well established, there is neither epidemiological nor molecular evidence that prototypic HTLV-II may be etiologically associated specifically with any of the CD8+ T cell LP investigated in this report.

Base Sequence↗
PubMed1995

[Intra-epidermal pustulosis in a child. Demonstration of a target antigen similar to foliaceus pemphigus antigen].

INTRODUCTION: Intraepidermal IgA pustulosis is a vesiculopustular dermatosis defined by pemphigus type intercellular deposit exclusively of IgA. It is a member of the pemphigus group and may be related to neurtrophilic dermatoses. CASE REPORT: A child had vesiculopustular lesions of the limbs since the age of 11 years. Biopsy showed the subcorneal intraepidermal nature of the pustules and exclusive IgA deposit throughout the epiderma. Indirect immunofluorescence and protein immunoelectrophoreses were negative. Immunotransfer to beef tongue extract evidenced a 150-160 kDa band corresponding to IgA and IgG desmoglein. Treatment with general corticosteroids followed by pristinamycin was successful. DISCUSSION: This is the first case report showing evidence of antiepidermal antibodies directed against the superficial pemphigus antigen.

Age Factors↗
PubMed1995

[Hypopigmented mycosis fungoides].

INTRODUCTION: Isolated areas of hypopigmented skin can be a manifestation of mycosis fungoides. This is a rare clinical form with a particular clinical course and histology which has been described only in black subjects. CASE REPORT: Over a 10-year period, a 28-year-old woman from Mali developed non-infiltrated hypopigmented and discretely squamous macules involving the entire body. The diagnosis of mycosis fungoides was made on pathology evidence of epidermotropic lymphocyte infiltration with a few small nests. PUVA led to rapid regression of the lesions. DISCUSSION: Hypopigmented mycosis fungoides has been described in young black subjects (mean age 19 years). The diagnosis is usually made late because benign skin affection is usually the first diagnosis (pityriasis alba, pityriasis versicolor). The pathogenesis of these hypopigmented lesions is not known. PUVA appears to be effective in most cases.

Adult↗
PubMed1994

Erythema gyratum repens. A case studied with immunofluorescence, immunoelectron microscopy and immunohistochemistry.

We report a patient with erythema gyratum repens (EGR), in whom a bronchial carcinoma was found. Direct immunofluorescence revealed granular deposits of immunoglobulins at the basement membrane zone (BMZ) in the skin, and in the lung tumour. Direct immunoelectron microscopy showed that the immune deposits were localized just beneath the lamina densa. Indirect immunofluorescence revealed circulating anti-BMZ antibodies. Immunohistochemical staining, using anti-transforming growth factor-beta, anti-epidermal growth factor receptor, anti-vimentin and anti-alpha-actin, was found to be more intense in the lesional skin and the lung tumour than in normal tissues. Possible mechanisms in the pathogenesis of EGR are discussed.

Autoantibodies↗
PubMed1994

Plasma thrombomodulin level in malignancy varies according to the tumor type.

Plasma thrombomodulin (TM) level was measured by ELISA in patients with either digestive tract cancers or malignant melanomas. A striking difference was seen between tumor types with an increase in the TM level of the former and a decrease in the latter. This finding is in agreement with the classification of tumors previously proposed by Zacharski et al.

Digestive System Neoplasms↗
PubMed1993

Paraneoplastic pemphigus in a child with a T-cell lymphoblastic lymphoma.

We report a child with a T-cell lymphoblastic lymphoma, and a widespread erosive mucocutaneous eruption, whose clinical and immunopathological features were consistent with a diagnosis of paraneoplastic pemphigus. Direct immunofluorescence showed deposition of C3 in the epidermal intercellular spaces, and in a linear pattern along the dermo-epidermal junction. Circulating autoantibodies binding in a pemphigus-like pattern to mouse urinary bladder epithelium were found. By Western immunoblotting, the patient's serum recognized two epidermal polypeptides with molecular weights of 210 and 190 kDa. No reactivity with the 230-kDa bullous pemphigoid antigen and the 250-kDa desmoplakin I antigen was found.

Autoantibodies↗
PubMed1993

Leprosy reversal reaction in HIV-positive patients.

We report two cases of leprosy in HIV-infected patients who, by their clinical, histological and immunological features, enhance the evidence that HIV-positive leprosy does not differ from nonHIV-positive leprosy. Moreover, extensive studies of reversal reactions in HIV-positive patients might be of great interest in determining the exact pathogenesis of this leprosy reactional state.

Adult↗
PubMed1992

TCR gamma delta bearing T lymphocytes infiltrating human primary cutaneous melanomas.

In situ immunophenotypic studies show that tumor infiltrating T lymphocytes from six of 11 fresh cutaneous primary melanomas tested contain T-cell receptor gamma delta expressing cells whereas those from 8 metastatic melanomas did not. In three cases these cells represent an unexpectedly high proportion of the lymphocytic infiltrate, ranging from 15 to 25%. This elevated percentage is maintained when tumor infiltrating lymphocytes are propagated in vitro for 2 or 3 weeks in the presence of recombinant interleukin 2 and autologous tumor cells. Functional studies reveal that gamma delta lines or clones developed from lymphocytes infiltrating primary melanoma display, in addition to their natural killer-like activity, a potent autologous tumor cell cytotoxic activity that does not seem to be restricted by major histocompatibility complex gene products.

Humans↗