Ramon Bordes

Distribution of human herpesvirus-8 sequences throughout the spectrum of AIDS-related neoplasia

Objective:AIDS frequently associates with certain malignancies, including Kaposis sarcoma, non-Hodgkins lymphoma (NHL), and anogenital neoplasia. In this study we aimed to define the frequency of infection by human herpesvirus (HHV)-8 throughout the spectrum of AIDS-related neoplasia in Europe. Design:A tumour panel representative of the distinct types of AIDS-related neoplasms was tested for the presence of HHV-8 DNA sequences. Autologous uninvolved tissues were also tested in selected cases. Methods:The presence of HHV-8 DNA sequences was assayed by a combination of polymerase chain reaction followed by oligohybridization and Southern blot hybridization of genomic DNA with an HHV-8-specific probe. Results:HHV-8 sequences were detected in 100% of AIDS-related Kaposis sarcoma (all 35 cases). Among AIDS-related NHL, HHV-8 sequences selectively clustered with body-cavity-based lymphomas (BCBL; all three cases), although they were consistently negative in small non-cleaved cell lymphomas (none in 18 cases), diffuse large cell lymphomas (none in seven), or anaplastic large cell lymphomas (none in three). No HHV-8 sequences were found in cases of anogenital neoplasia (out of 14) or Hodgkins disease (out of three). HHV-8 DNA sequences were also positive in the uninvolved skin of all six AIDS-related Kaposis sarcoma patients, but not in the circulating lymphocytes of a BCBL patient. Positivity for HHV-8 sequences occurred in patients belonging to all major AIDS risk categories. Conclusions:These data confirm that HHV-8 sequences associate at high frequency with selected types of AIDS-related neoplasia, namely Kaposis sarcoma and BCBL, although they are consistently absent in other types of AIDS-NHL and AIDS-related anogenital neoplasia.

Aggressive natural killer cell leukaemia/lymphoma in two patients with lethal midline granuloma

Summary. We report two patients with leukaemic proliferations of large granular lymphocytes. The immunophenotype study showed that the leukaemic cells were positive for CD2, CD38, CD56 and anti‐HLA‐DR monoclonal antibodies and negative for other T‐cell (CD3, CD4, CD8) and B‐cell markers (CD19, CD20 and surface immunoglobulins). The clinical course was acute and a diagnosis of aggressive natural killer cell leukaemia/lymphoma was made. No clonal rearrangements of either Cβ T‐cell receptor or JH immunoglobulin genes were found. Functional studies done in one patient demonstrated non‐restricted cytotoxic activity after activation with IL‐2. Lethal midline granuloma had been previously diagnosed in both patients. A possible relationship between this entity and the natural killer cell leukaemia is discussed.

Invisible mycosis fungoides: A diagnostic challenge

We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patients course. Skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautriers microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.

Clinical utility of bone marrow flow cytometry in B‐cell non‐Hodgkin lymphomas (B‐NHL)

Aim : To determine the efficacy of flow cytometry (FC) in the assessment of bone marrow (BM) in B‐cell non‐Hodgkin lymphoma (B‐NHL). FC is a common practice, but is far from being validated.

Variant t(2;18) translocation in a burkitt conversion of follicular lymphoma

We describe here a patient with a low-grade follicular lymphoma evolving to a Burkitt-like lymphoma which at the time of blastic conversion showed multiple chromosome aberrations, including a t(8;22) (q24;q11) typical Burckitt variant translocation associated with a translocation between chromosome 2p11 and chromosome 18q21 which may be an undescribed variant of the t(14;18) follicular lymphoma chromosomal rearrangement

Activation of the NF‐κB signalling pathway in diffuse large B‐cell lymphoma: clinical implications

Aim:  To characterize the activation of the nuclear factor (NF)‐κB pathway in diffuse large B‐cell lymphoma (DLBCL) by immunohistochemistry.

Oral Involvement in Lymphomatoid Papulosis

Oral involvement in cutaneous CD30+ T-cell lymphoid proliferations is rare and has received little attention in the dermatologic literature. The authors report 2 patients with self-healing, recurrent papulonodular eruptions with the classic clinical, histopathological and immunophenotypic features of lymphomatoid papulosis, which developed two ulcerated papules and an ulcerative nodule on the dorsum of the tongue, respectively. The lesions appeared coincident with a new cutaneous relapse of the disease. Histopathological and immunophenotypic features were similar to those of the cutaneous lesions. All lesions regressed spontaneously after several weeks. Since then, and after follow-up periods of 3 and 7 years, respectively, no evidence of extracutaneous involvement has been detected. Oral involvement in lymphomatoid papulosis is an uncommon event, probably without prognostic significance. Previously reported cases are reviewed. The differential diagnosis of atypical T-cell lymphoid infiltrates observed in the oral mucosa is discussed.

Case ReportsInvisible mycosis fungoides: A diagnostic challenge☆☆☆

We describe a 76-year-old woman who had persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patients course. Skin biopsy specimens obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautriers microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J8V2C2 TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed. (J Am Acad Dermatol 2002;47:S168-71.)

Bcl‐10 protein highly correlates with the expression of phosphorylated p65 NF‐κB in peripheral T‐cell lymphomas and is associated with clinical outcome

Aims:  In T cells, protein kinase C (PKC) θ plays a major role in T‐cell receptor (TCR)‐mediated activation of a novel nuclear factor (NF)‐κB pathway that involves phosphorylation of p65 at serine 536 (Pp65Ser536). Bcl‐10 acts along the same pathway downstream of PKC θ to activate NF‐κB. The aim was to investigate the relationship between the expression of PKC θ, Bcl‐10 and P‐p65Ser536 proteins and their prognostic significance in peripheral T‐cell lymphomas (PTCLs).

Translocation (3;21) in a patient with secondary hematological malignancy.

Abstract A case of secondary chronic myelomonocytic leukemia with a t(3;21)(q26;q22), identical to that described in Philadelphia-positive chronic myeloid leukemia, is reported.