Pablo Luis Ortiz Romero

Panobinostat activity in both bexarotene-exposed and -naïve patients with refractory cutaneous T-cell lymphoma: results of a phase II trial.

BACKGROUND Panobinostat is a potent, oral pan-deacetylase inhibitor (pan-DACi) that increases the acetylation of proteins involved in multiple oncogenic pathways. Here, panobinostat is studied in bexarotene-exposed and -naïve patients with refractory cutaneous T-cell lymphoma (CTCL). PATIENTS AND METHODS Patients with CTCL subtypes mycosis fungoides and Sézary syndrome who received ⩾2 prior systemic therapy regimens received panobinostat (20mg) three times every week. The primary objective was overall response rate (ORR) as determined by a combined evaluation of skin disease and involvement of lymph node and viscera. Disease progression was defined as an unconfirmed, ⩾25% increase in modified Severity Weighted Assessment Tool (mSWAT) compared with nadir. RESULTS Seventy-nine bexarotene-exposed and 60 bexarotene-naïve patients were enrolled. Reductions in baseline mSWAT scores were observed in 103 patients (74.1%). The ORR was 17.3% in all patients in the primary analysis (15.2% and 20.0% in the bexarotene-exposed and -naïve groups, respectively). The median progression-free survival was 4.2 and 3.7 months in the bexarotene-exposed and -naïve groups, respectively. The median duration of response was 5.6 months in the bexarotene-exposed patients and was not reached at data cutoff in the bexarotene-naïve patients. Additional responses were observed when less-stringent progression criteria were used. The most common adverse events were thrombocytopenia, diarrhoea, fatigue and nausea. Thrombocytopenia and neutropenia were the only grade 3/4 adverse events in >5% of patients and were manageable. CONCLUSION Despite a very conservative definition of disease progression, panobinostat demonstrated activity with a manageable safety profile in bexarotene-exposed and -naïve CTCL patients. ClinicalTrials.gov Identifier: NCT00425555.

Cutaneous plasmacytosis: Report of a case in a white man

We describe a 40-year-old white man with a peculiar skin eruption in association with polyclonal hypergammaglobulinemia. No underlying disease was detected. A skin biopsy specimen showed a proliferation of mature plasma cells intermingled with some lymphocytes and histiocytes, an appearance consistent with cutaneous plasmacytosis. This disease had been previously described only in Japanese patients. In our patient the disease progressed slowly. Lymph node infiltration by mature plasma cells was later noted.

Eosinophilic fasciitis as a manifestation of a cutaneous T-cell lymphoma not otherwise specified.

Eosinophilic fasciitis (EF) is a rare entity characterized by symmetrical and painful thickness and induration of the skin, especially localized on forearms and thorax and generally accompanied by eosinophilia. Although several reports indicate the relationship between EF and hematological disorders such as aplastic anemia, polycythemia vera, or myelomonocytic leukemia, the association with lymphomas is extremely rare. Only a few cases of EF have been previously described preceding or concomitant to the Hodgkin disease, peripheral T-cell lymphoma, B-cell lymphoma, and mycosis fungoides. We report for the first time a 76-year-old man with an EF associated with a peripheral T-cell lymphoma not otherwise specified. We review the relationship between both conditions. In conclusion, we present a unique case of EF as a manifestation of a T-cell lymphoma not otherwise specified. The present case demonstrates the importance of clinical and radiological studies in those cases of EF to rule out a visceral, lymph node, or cutaneous lymphoma.

Nail involvement can predict enthesopathy in patients with psoriasis

: Although subclinical enthesopathy is a well‐established diagnostic criterion for psoriatic arthritis (PsA), it is frequently overlooked, as many patients are asymptomatic. The possibility of finding a clinical clue predicting enthesopathy would help clinicians establish an early diagnosis of PsA.

Histiocitosis de células de Langerhans en un paciente anciano asociada a leucemia mielomonocítica crónica

Resumen —La histiocitosis de celulas de Langerhans (HCL) comprende un grupo polimorfo de enfermedades poco frecuentes que tienen en comun la proliferacion clonal de celulas con el fenotipo de las celulas de Langerhans. La HCL se presenta generalmente en ninos. La incidencia en ancianos es excepcional; en ellos es dificil de diagnosticar clinicamente, pero generalmente tiene mejor pronostico. Su etiologia es desconocida, existiendo en la actualidad gran controversia respecto a si se trata de un proceso reactivo o neoplasico. Ha sido descrita la asociacion de HCL y leucemias de la linea mielomonocitica, lo que sugiere un origen comun para ambos procesos, por alteracion de la celula madre pluripotencial. Presentamos un caso de HCL en un varon de 76 anos con afectacion cutanea extensa y ganglionar que posteriormente se asocio a una leucemia de la linea mielomonocitica (leucemia mielomonocitica cronica), sin existir previamente ningun tratamiento capaz de inducirla.

A phase III study of lenalidomide maintenance after debulking therapy in patients with advanced cutaneous T-cell lymphoma - EORTC 21081 (NCT01098656): results and lessons learned for future trial designs

BackgroundEORTC 21081 was a randomized phase III study of observation alone versus lenalidomide maintenance (25 mg po for 21 days) after debulking therapy in patients with advanced-stage cutaneous T-cell lymphomas (CTCLs).ObjectivesThe aim was to investigate whether maintenance treatment with lenalidomide prolonged response after debulking in patients who had not been previously treated with intravenous chemotherapy.Materials & methodsA total of 26 centres from 10 different European countries registered 30 patients with advanced CTCL. Twenty-one patients were randomized (20% of the 105 patients initially deemed necessary for the study; the study was terminated early following withdrawal of funding support from Celgene).ResultsOf 30 registered patients, nine failed to be randomized, 12 were randomized to observation alone, and nine to lenalidomide maintenance. Median progression-free survival was 5.3 months (95% CI: 1.87-22.54) in the maintenance lenalidomide group and two months (95% CI: 0.92- 7.82) in the observation alone group.ConclusionAlthough statistical comparison in the study was severely underpowered and would not be meaningful, this study provides useful information, revealing rapid disease progression within four weeks in a third of patients, highlighting the need for maintenance therapy.

Eruptive nevi in a patient with metastatic breast cancer

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Nagelbefall kann bei Patienten mit Psoriasis auf eine Enthesiopathie hinweisen: Enthesiopathie und Nagelbefall

Obwohl subklinische Enthesiopathie ein gut etabliertes diagnostisches Merkmal der Psoriasisarthritis (PsA) ist, wird sie häufig übersehen, da viele Patienten asymptomatisch sind. Gäbe es klinische Hinweise auf das Vorliegen einer Enthesiopathie, würde dies den Klinikern die Möglichkeit eröffnen, eine PsA frühzeitig zu diagnostizieren.

Pili annulati (pelos anillados)

Resumen —Presentamos un caso de pili annulati (pelos anillados), anomalia infrecuente del tallo del pelo no asociada a rotura o pelo indocil, en una nina de 9 anos. Es una alteracion autosomica dominante (ocasionalmente esporadica), que muestra al microscopio optico bandas alternantes claras y oscuras, estas ultimas producidas por la presencia de espacios de aire en la corteza.