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Tuesday, May 19, 2009

Juvenile onset of primary low-grade cutaneous B-cell lymphoma

Juvenile onset of primary low-grade cutaneous B-cell lymphoma
Br J Dermatol. 2009 Mar 26

Amitay-Laish I, Feinmesser M, Ben-Amitai D, David M, Manor Y, Kidron D, Barzilai A, Hodak E.
Department of Dermatology, Rabin Medical Center, Beilinson Hospital, Petah Tikva 49100, Israel.

Summary Background: Cutaneous lymphomas rarely occur in children and adolescents, and are mostly of the T-cell lineage. Low-grade primary cutaneous B-cell lymphoma (CBCL) is extremely rare in individuals under 18 years old. Only 11 patients under 20 years old have been reported in the literature.

Objectives: To evaluate the number of patients younger than 18 years with primary CBCL diagnosed at our centre and to investigate its clinicopathological features, treatment and course in this age group.

Methods: We reviewed the files of all 90 patients with primary CBCL who attended the Department of Dermatology of our tertiary care university-affiliated centre from 1992 to 2007.

Results: Four patients who met study criteria were identified: three girls and one boy. Mean age at diagnosis was 16.6 years (range 16-17). Three patients had cutaneous marginal zone lymphoma (CMZL), and one had a spindle-cell (sarcomatoid) lymphoma, most probably follicular centre cell type. All were treated with the standard regimen used in adults. The mean duration of follow up was 45 months. No extracutaneous progression was noted. At present two of the four patients are in complete clinical remission.

Conclusions: In Israel, primary CBCL apparently occurs more often in young patients than reported in the literature. CMZL is the most frequent type. Long follow up is mandatory to assess the biological behaviour of CBCL in the paediatric/adolescent age group.

Wiley InterScience

Gemcitabine treatment in cutaneous T-cell lymphoma: a multicentre study of 23 cases

Gemcitabine treatment in cutaneous T-cell lymphoma: a multicentre study of 23 cases
Br J Dermatol. 2009 May

Jidar K, Ingen-Housz-Oro S, Beylot-Barry M, Paul C, Chaoui D, Sigal-Grinberg M, Morel P, Dubertret L, Bachelez H.

Service de Dermatologie, Centre Hospitalier Victor Dupuy, 95100 Argenteuil, France.

Background: Primary cutaneous T-cell lymphomas (CTCLs) are malignancies characterized by a clonal T-cell infiltrate involving the skin. CTCLs often show resistance to conventional antineoplastic chemotherapy. Gemcitabine is a pyrimidine analogue which has shown efficacy and a favourable safety profile in solid tumours and haematological malignancies.

Objectives: We report a multicentre retrospective study of 23 patients who received gemcitabine for advanced-stage CTCL and emphasize the high incidence of serious unusual adverse events. Methods We collected data from 23 patients with refractory CTCL (14 mycosis fungoides, six Sézary syndrome and three other CTCL). Gemcitabine was given weekly within a 21- or 28-day schedule. Response was evaluated after three and six cycles of chemotherapy. For each patient, all adverse events were recorded.

Results: Of the 16 patients who received at least three cycles of gemcitabine, 10 achieved a response (62.5%). Only five patients reached the sixth cycle of treatment and four still had a favourable response. Haematological toxicity was recorded in 15 cases with severe grade 3 or 4 neutropenia in seven patients (30%) and six serious infections (26%). Other serious adverse events were observed in six cases (26%): one haemolytic-uraemic syndrome, one severe capillary leak syndrome, one acute heart failure related to cardiac arrhythmia, two bullous and erosive dermatitis, and one recurrent influenza-like syndrome with altered general condition.

Conclusions: Our study confirms the early efficacy of gemcitabine in advanced-stage CTCL. However, our results contradict the safety profile of gemcitabine previously reported and underline the high incidence of severe complications including visceral and cutaneous involvement.