© F e r r a t a S t o r t i F o u n d a t i o neral T-cell lymphomas (PTCL) but mainly in angioimmunoblastic T-cell lymphoma (AITL). [14][15][16][17][18] PTCLs have also been reported during CD3-CD4 + L-HES course. 6,9,[19][20][21][22][23][24] Two of 23 patients currently followed in the French Eosinophil Network, and one more patient recently reported by others, 25 developed well-defined AITL several years after L-HES diagnosis, which thus raised the problem of the diagnosis of well-defined T-cell lymphoma in patients who have clonal circulating T cells.In this study, we focused on the lymphoid infiltrates in lymph nodes, skin and other available biopsies of tissue involved in L-HES, to assess the presence of clonal T cells at diagnosis and during CD3 -
CD4+ L-HES course. We secondly aimed to distinguish L-HES from AITL by comparing histopathological and immunophenotypic characteristics between both entities
Methods
PatientsTwenty-three hypereosinophilic syndrome (HES) patients with a documented presence of CD3 -
CD4+ aberrant subset and a negative FIP1L1-PDGFRA fusion gene research are currently followed The clonal T-cell disease in CD3 -CD4 + L-HES haematologica | 2015; 100(8) 1087
© F e r r a t a S t o r t i F o u n d a t i o nSkin lesions in L-HES patients were pruritic papulo-nodular inpatients P1 and P2, pruritic papular lesions in patient P3, P5, P8, isolated pruritus in patients P7 and P9. Numbers in in the French Eosinophil Network. For the present study, 16 patients (P1-P16) were included, 12 of them had available tissue biopsies during L-HES course. All satisfied criteria for HES (n=15) or hypereosinophilia (HE) (n=1, P13, no organ damage or clinical manifestation) criteria in accordance with the latest up-dated consensus definitions.1 Main clinical characteristics are summarized in Table 1. For the 7 remaining patients, no complementary lymphocyte immunophenotyping was performed, no biopsy was performed (n=5) or biopsies were not available for analysis (n=2). The study was approved by the Lille Hospital Ethical Committee and carried out in accordance with the Declaration of Helsinki.Ten of these patients had bone marrow biopsies at CD3 -CD4 + L-HES diagnosis in order to exclude a T-cell lymphoma (Patients P2-4, P8, P9, P11, P12, P14-16) (data not shown). Four patients had lymph nodes biopsies for a suspicion of T-cell lymphoma during follow up (Patients P1, P3, P4 and P10). For this work, all their biopsies were retrieved for further investigation and for a centralized compared analysis to be made.Finally, Patients P4 and P16 developed a well-defined AITL during L-HES course (AITL/L-HES patients).Patient P4. Patient P4 was 18-years old when a CD3 -CD4 + L-HES diagnosis was made in 1999 and was previously reported by us.
26He presented with eczema-like lesions, rare episodes of angioedema and multiple adenopathy. Despite high circulating CD3 -CD4 + T-cell count (28 G/L), lymph node histological examination confirmed lymphoid reactive hyperplasia. As he was in really good G. Lefèvre ...