Marie Dominique Vignon-Pennamen

Localisation cutanée et épidurale d’une histiocytose de Rosai-Dorfman

Resume Introduction L’histiocytose de Rosai-Dorfman ou histiocytose sinusale avec lymphadenopathie massive est une maladie rare qui correspond a une proliferation histiocytaire benigne interessant les ganglions lymphatiques. Alors que l’association d’une atteinte ganglionnaire a une atteinte extra-ganglionnaire, notamment cutanee, est frequente, les localisations extra-ganglionnaires pures sont rares. Observation Un homme de 30 ans avait des lesions papulo-nodulaires erythemateuses, d’aspect lupoide a la vitropression, de la face et des jambes. Ces lesions precedaient l’apparition d’un deficit neurologique avec hypoesthesie des deux membres inferieurs. L’examen histologique d’un papulo-nodule de la face montrait un infiltrat dermique constitue majoritairement d’histiocytes de grande taille comportant pour certains un phenomene d’emperipolese, marque par la presence dans leur cytoplasme de noyaux de lymphocytes et de polynucleaires neutrophiles. L’immunohistochimie mettait en evidence l’expression par ces histiocytes de l’antigene CD68 et de la proteine S100, sans expression de l’antigene CD1a. Par ailleurs, l’imagerie par resonance magnetique rachidienne objectivait une masse epidurale posterieure etendue de D4 a D7. Le diagnostic retenu etait celui de localisations cutanee et epidurale d’une histiocytose de Rosai-Dorfman. Une corticotherapie intraveineuse permettait une regression rapide et partielle du deficit neurologique. Un relais par une corticotherapie orale et une chimiotherapie par etoposide entrainait une nette amelioration des lesions cutanees. Discussion L’originalite de cette observation d’histiocytose de Rosai-Dorfman reside d’une part dans l’absence d’atteinte ganglionnaire associee a l’atteinte cutanee et d’autre part, dans l’existence d’une atteinte neurologique, exceptionnellement decrite au cours de cette affection.

Angioimmunoblastic T-cell lymphoma revealed after treatment of B-cell lymphoproliferative diseases

Rituximab is an anti-CD20 monoclonal antibody that has demonstrated efficacy in patients with various lymphoid malignancies, including indolent and aggressive forms of B-cell non-Hodgkin lymphoma (NHL) and B-cell chronic lymphocytic leukemia (CLL) [1]. We report two cases of angioimmunoblastic T-cell lymphoma (AILD-TL) revealed after treatment of B-cell lymphoproliferative diseases. In the first case, a 62-year-old man presented in February 2003 with a diffuse large B-cell lymphoma stage III. Monoclonal TCR-gene rearrangement studies showed a monoclonal T-cell CD4þ, CD37 population in the initial lymph node. The patient was treated with six cycles of R-CHOP and three CHOP. Complete response was achieved. In April 2004 a monoclonal T-cell CD4þ, CD37 population was detected in peripheral mononuclear cells (PBMC) (33%). Six months later, he presented with a persistent febrile diffuse macular eruption with fatigue, cough, peripheral adenopathies, and hepatosplenomegaly. Cutaneous histology showed small to medium-sized T-cell infiltration with irregular nuclei CD3þ, CD5þ, CD107 with an associated histiocytic infiltration. Lymph node histology showed T-cell infiltration CD3þ (intracytoplasmic), CD2þ, CD5þ with clear CD10þ T-cells compatible with AILD-TL (Figure 1). Surface CD3 was negative in flow cytometry. Monoclonal TCR-gene rearrangement studies yielded a unique clonal T-cell population in the PBMC, skin, lymph node and a minor clone in the bone marrow. HTLV-1 and HIV serology were negative. EBV PCR was highly positive in peripheral blood mononuclear cells (PBMC; 9600 copies/150 000 cells), and in adenopathies (69 074 copies/150 000 cells), and negative in skin. Coomb’s test was positive. Initial adenopathy have been reviewed by a pathologist in the light of AILD-TL, but initial diagnosis of B cell lymphoma was confirmed. Thoracic computerised tomography disclosed multiple perivascular nodules in lung. Despite chemotherapy with high-dose aracytine, etoposide, cisplatine and dexamethasone, the patient rapidly deceased in November 2004. In the second case, a 73-year-old woman presented in November 2003 with low-grade B cell lymphoproliferative associated with a monoclonal immunoglobulin (IgM kappa). Before any treatment, PBMC analysis and monoclonal TCR-gene rearrangement studies showed the presence of 12% of CD4þ CD37 T-cell clonal population. She was treated in February 2004 with only one cycle of rituximab because of the development of acute polyradiculoneuritis. In July 2004 she presented with multiple peripheral and retroperitoneal adenopathies, fever, sweats, and hepatosplenomegaly, while cutaneous facial and truncal eruption appeared