In children <2 years of age, cutaneous involvement is the most frequent presentation of Langerhans cell histiocytosis (LCH). Cutaneous LCH can be localized or associated with dissemination and organ dysfunction. The clinical Course is variable, ranging from spontaneous regression to a fatal Outcome. We describe a female newborn presenting with congenital Cutaneous lesions who rapidly developed pulmonary infiltrates and Multiple osteolytic lesions. Skin biopsy showed a dermal infiltrate of medium to large cells morphologically and phenotypically consistent with LCH. The clinical Course was rapidly fatal in spite of chemotherapy. No strict correlation between morphology and prognosis has been documented in LCH, but, in our case, distinct morphological and immunohistochemical features (CD56 expression and no E-Cadherin expression) may have contributed to an aggressive clinical Course. Pediatr Blood Cancer 2009;53:1107-1110. (C) 2009 Wiley-Liss, Inc.

Lucioni, M., Beluffi, G., Bandiera, L., Zecca, M., Inzani, F., Fiandrino, G., Viglio, A., Stronati, M., Necchi, V., Riboni, R., Locatelli, F., Paulli, M., Congenital aggressive variant of Langerhans cells histiocytosis with CD56+/E-Cadherin- phenotype, <<PEDIATRIC BLOOD & CANCER>>, 2009; 53 (6): 1107-1110. [doi:10.1002/pbc.22196] [https://hdl.handle.net/10807/257654]

Congenital aggressive variant of Langerhans cells histiocytosis with CD56+/E-Cadherin- phenotype

Inzani, Frediano;Locatelli, Franco;
2009

Abstract

In children <2 years of age, cutaneous involvement is the most frequent presentation of Langerhans cell histiocytosis (LCH). Cutaneous LCH can be localized or associated with dissemination and organ dysfunction. The clinical Course is variable, ranging from spontaneous regression to a fatal Outcome. We describe a female newborn presenting with congenital Cutaneous lesions who rapidly developed pulmonary infiltrates and Multiple osteolytic lesions. Skin biopsy showed a dermal infiltrate of medium to large cells morphologically and phenotypically consistent with LCH. The clinical Course was rapidly fatal in spite of chemotherapy. No strict correlation between morphology and prognosis has been documented in LCH, but, in our case, distinct morphological and immunohistochemical features (CD56 expression and no E-Cadherin expression) may have contributed to an aggressive clinical Course. Pediatr Blood Cancer 2009;53:1107-1110. (C) 2009 Wiley-Liss, Inc.
2009
Inglese
Lucioni, M., Beluffi, G., Bandiera, L., Zecca, M., Inzani, F., Fiandrino, G., Viglio, A., Stronati, M., Necchi, V., Riboni, R., Locatelli, F., Paulli, M., Congenital aggressive variant of Langerhans cells histiocytosis with CD56+/E-Cadherin- phenotype, <<PEDIATRIC BLOOD & CANCER>>, 2009; 53 (6): 1107-1110. [doi:10.1002/pbc.22196] [https://hdl.handle.net/10807/257654]
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/10807/257654
Citazioni
  • ???jsp.display-item.citation.pmc??? 2
  • Scopus 6
  • ???jsp.display-item.citation.isi??? 6
social impact