Research article summary (published 29 Nov 2009):

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

Full 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.

Author information

Author/s: Lucioni, Marco (M); Beluffi, Giampiero (G); Bandiera, Laura (L); Zecca, Marco (M); Inzani, Frediano (F); Fiandrino, Giacomo (G); Viglio, Alessandra (A); Stronati, Mauro (M); Necchi, Vittorio (V); Riboni, Roberta (R); Locatelli, Franco (F); Paulli, Marco (M);

Affiliation: Anatomic Pathology Section, Department of Human Pathology, University of Pavia, Foundation IRCCS Policlinico San Matteo, Pavia, Italy.

Journal and publication information

Publication Type: Case Reports; Journal Article

Journal: Pediatric blood & cancer (Pediatr Blood Cancer), published in United States. (Language: eng)

Reference: 2009-Dec; vol 53 (issue 6) : pp 1107-10

Dates: Created 2009/09/16; Completed 2009/10/13;

PMID: 19621455, status: MEDLINE (last retrieval date: 10/13/2009, IMS Date: )

Sourced from the National Library of Medicine. Abstract text and other information may be subject to copyright.

Comments and Corrections

CommentIn: Pediatr Blood Cancer. 2009 Dec;53(6):924-5. (PMID: 19672979)

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MeSH headings (categories)

This article was linked to the MESH Headings shown below.

Antigens, CD56 Cadherins Cell Shape Fatal Outcome Female

Histiocytosis, Langerhans-Cell - congenital; pathology Humans Infant, Newborn Phenotype Prognosis

Associated Chemicals: Antigens, CD56 (0) ; Cadherins (0)

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