Erdheim-Chester Disease in Childhood A Challenging Diagnosis and Treatment

被引：38

作者：

Tran, Tu-Anh ^{[1
]}

Fabre, Monique ^{[1
]}

Pariente, Daniele ^{[1
]}

Craiu, Irina ^{[1
]}

Haroche, Julien ^{[1
]}

Charlotte, Frederic ^{[1
]}

Eid, Pierre ^{[1
]}

Durrbach, Antoine ^{[1
]}

Taoufik, Yassine ^{[1
]}

Kone-Paut, Isabelle ^{[1
]}

机构：

[1] Bicetre Univ Hosp, Dept Pediat, F-94275 Le Kremlin Bicetre, France

来源：

JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY | 2009年 / 31卷 / 10期

关键词：

Erdheim-Chester disease; childhood; histiocytosis; interferon-alpha; INTERFERON-ALPHA; INVOLVEMENT; HISTIOCYTOSIS; BONE;

D O I：

10.1097/MPH.0b013e3181b76827

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Erdheim-Chester disease is a rare, non-Langerhans systemic histiocytosis characterized by bilateral sclerosis of the metaphyseal regions of the long bones and infiltration in other organs. The histopathologic hallmark is defined by a mononuclear infiltrate of foamy histiocytes and rare pathognomonic Touton giant cells with extensive fibrosis. This condition is exceptional in children. We report here a case of Erdheim-Chester disease in a 10-year-old girl with retroperitoneal infiltration and bone involvement, for whom the diagnosis was only established after a 3-year Course with multiple biopsies. It is also the first pediatric case successfully treated with interferon-a suggesting that interferon-a can be it safe and efficient first-line therapy for this disease in children.

引用

页码：782 / 786

页数：5

共 19 条

[1] Erdheim-Chester disease: radiographic findings in five patients [J].

Bancroft, LW ;

Berquist, TH .

SKELETAL RADIOLOGY, 1998, 27 (03) :127-132

[2] Successful treatment of Erdheim-Chester disease, a non-Langerhans-cell histiocytosis, with interferon-α [J].

Braiteh, F ;

Boxrud, C ;

Esmaeli, B ;

Kurzrock, R .

BLOOD, 2005, 106 (09) :2992-2994

[3] Erdheim-Chester disease with hemophagocytosis [J].

Busemann, Christoph ;

Kallinich, Bianca ;

Schwesinger, Guenther ;

Krueger, William ;

Schueler, Frank ;

Schmidt, Christian A. ;

Doelken, Gottfried .

ANNALS OF HEMATOLOGY, 2007, 86 (11) :847-849

[4] Erdheim-Chester disease in a child [J].

Clerico, A ;

Ragni, G ;

Cappelli, C ;

Schiavetti, A ;

Gonfiantini, M ;

Uccini, S .

MEDICAL AND PEDIATRIC ONCOLOGY, 2003, 41 (06) :575-577

[5] Association between Erdheim-Chester disease, Hashimoto thyroiditis, and familial thrombocytopenia [J].

Cruz, AAV ;

de Alencar, VM ;

Falcao, MF ;

Elias, J ;

Chahud, F .

OPHTHALMIC PLASTIC AND RECONSTRUCTIVE SURGERY, 2006, 22 (01) :60-62

[6] Bone involvement in Erdheirn-Chester disease: Imaging findings including periostitis and partial epiphyseal involvement [J].

Dion, E ;

Graef, C ;

Miquel, A ;

Haroche, J ;

Wechsler, B ;

Amoura, Z ;

Zeitoun, D ;

Grenier, PA ;

Piette, JC ;

Laredo, JD .

RADIOLOGY, 2006, 238 (02) :632-639

[7] Interferon-γ receptor deficiency mimicking Langerhans' cell histiocytosis [J].

Edgar, JDM ;

Smyth, AE ;

Pritchard, J ;

Lammas, D ;

Jouanguy, E ;

Hague, R ;

Novelli, V ;

Dempsey, S ;

Sweeney, L ;

Taggart, AJ ;

O'Hara, D ;

Casanova, JL ;

Kumararatne, DS .

JOURNAL OF PEDIATRICS, 2001, 139 (04) :600-603

[8]

GLOBERMAN H, 1991, AM J PEDIAT HEMATOL, V13, P42

[9] MR findings of Erdheim-Chester disease [J].

Gottlieb, R ;

Chen, A .

JOURNAL OF COMPUTER ASSISTED TOMOGRAPHY, 2002, 26 (02) :257-261

[10] Cardiovascular involvement, an overlooked feature of Erdheim-Chester disease -: Report of 6 new cases and a literature review [J].

Haroche, J ;

Amoura, Z ;

Dion, E ;

Wechsler, B ;

Costedoat-Chalumeau, N ;

Cacoub, P ;

Isnard, R ;

Généreau, T ;

Wechsler, J ;

Weber, N ;

Graef, C ;

Cluzel, P ;

Grenier, P ;

Piette, JC .

MEDICINE, 2004, 83 (06) :371-392

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