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Congenital aggressive variant of Langerhans cells histiocytosis with CD56+/E‐Cadherin− phenotype
Author(s) -
Lucioni Marco,
Beluffi Giampiero,
Bandiera Laura,
Zecca Marco,
Inzani Frediano,
Fiandrino Giacomo,
Viglio Alessandra,
Stronati Mauro,
Necchi Vittorio,
Riboni Roberta,
Locatelli Franco,
Paulli Marco
Publication year - 2009
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22196
Subject(s) - langerhans cell histiocytosis , medicine , pathology , histiocytosis , phenotype , immunohistochemistry , skin biopsy , variable expression , biopsy , chemotherapy , presentation (obstetrics) , disease , biology , biochemistry , gene , radiology
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. © 2009 Wiley‐Liss, Inc.