Journal of Clinical Pathology 2007;60:1168-1169
CASE REPORTS
Cytophagic histiocytic panniculitis with fatal haemophagocytic lymphohistiocytosis in a paediatric patient with perforin gene mutation
1 Division of Paediatric Hematology/Oncology, Cancer Centre, Chi-Mei Medical Centre, Liou Ying Campus, Tainan, Taiwan
2 Department of Pathology, Buddhist Tzu Chi Medical Centre and University, Hualien, Taiwan
3 Department of Paediatrics, Kyoto Prefectural University of Medicine, Kyoto, Japan
4 Department of Paediatrics, Takasago-Seibu Hospital, Takasago, Japan
5 Department of Pathology, Cancer Institute of Japanese Foundation for Cancer Research, Tokyo, Japan
6 Institute of Biomedical Sciences, Academia Sinica, Taipei, Taiwan
7 Department of Pathology, Chi-Mei Medical Centre, Tainan, and Taipei Medical University, Taipei, Taiwan
Dr Shih-Sung Chuang, Department of Pathology, Chi-Mei Medical Centre, 901 Chung-Hwa Road, Yung-Kang City, Tainan County 710, Taiwan; cmh5301@mail.chimei.org.tw
Accepted 13 June 2007
| The first 150 words of the full text of this article appear below. |
Cytophagic histiocytic panniculitis (CHP), or histiocytic cytophagic panniculitis, is a rare form of panniculitis associated with haemorrhagic diathesis and histiocytic lymphohistiocytosis (HLH), initially described in 1980 as a benign lymphoproliferative disease.1 In 1991 Gonzalez et al reported a unique entity of subcutaneous T-cell lymphoma with haemophagocytosis, later designated as subcutaneous panniculitis-like T-cell lymphoma (SPTL).2 Marzano et al suggested that CHP and SPTL might span a clinicopathological spectrum in which there is a natural progression from CHP to SPTL.3 HLH is a clinical syndrome of immune deregulation with hypercytokinaemia causing dysfunctions of various organs and a high mortality. Familial HLH (FHL) is associated with several hereditary defects. Stepp et al first showed that the mutation in perforin gene (PRF1) at chromosome 10q21 was responsible for 20–40% of FHL patients.4
An 11-year-old girl with CHP and HLH presented with spiking fever and indurated skin nodules over the left thigh (
CiteULike 
Complore 
Connotea 
Del.icio.us 
Digg 
Reddit 
Technorati What's this?
- Full Text
- Full Text (PDF)
-
All Versions of this Article:
jcp.2007.049551v1
60/10/1168 most recent - Submit a response
- Alert me when this article is cited
- Alert me when eLetters are posted
- Alert me if a correction is posted
- Citation Map
Services
- Email this link to a friend
- Similar articles in this journal
- Similar articles in PubMed
- Add article to my folders
- Download to citation manager
- Request Permissions
Citing Articles
Google Scholar
PubMed
Topic Collections
-
Dermatology
-
Connective tissue disease
-
Immunology (including allergy)
-
Paediatric oncology
-
Clinical diagnostic tests
Bookmark with
Register for free content
The full back archive is now available for all BMJ Journals. Institutional subscribers may access the entire archive as part of their subscription. Personal subscribers will also have access to all content when logged in. Non-subscribers who register have free access to all articles published before 2006 right back to volume 1 issue 1. Register here to access the free archive of all BMJ Journals.
Don't forget to sign up for content alerts so you keep up to date with all the articles as they are published.
