A common progenitor cell in LCH and ECD.
نویسنده
چکیده
In this issue of Blood, Hervier et al has identified that cooccurrence of Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) in the same patient is not a rare event. Mixed histiocytosis (MH) highlights existence of a link between distinct groups of histiocytic disorders and suggests presence of a common progenitor cell. Today, histiocytic disorders are classified into 3 groups based on clinical presentation and gross anatomic findings.
منابع مشابه
The “Rare” or “Non-LCH” Histiocytic Disorders in Childhood: A Brief Overview
Diseases of the monocyte, macrophage and dendritic cell system are referred to as histiocytoses. Based on improved understanding of their pathobiology and molecular background histiocytoses have been recently re-classified into five groups. Nevertheless, for practical reasons the histiocytoses are grouped into: Langerhans cell histiocytosis (the most common entity), hemophagocytic lymphohistioc...
متن کاملDiverse cutaneous manifestations of Erdheim-Chester disease in a woman with a history of Langerhans cell histiocytosis.
ECD: Erdheim-Chester disease LCH: Langerhans cell histiocytosis PET-CT: Positron emission tomographye computerized tomography INTRODUCTION Erdheim-Chester disease (ECD) is a rare, systemic, noneLangerhans cell histiocytosis (non-LCH). Diagnosis is based on a combination of specific radiologic, histologic, and clinical findings. Although there have been hundreds of prior reports of ECD, very few...
متن کاملEffective BRAF inhibitor vemurafenib therapy in a 2-year-old patient with sequentially diagnosed Langerhans cell histiocytosis and Erdheim–Chester disease
Erdheim-Chester disease (ECD) is a rare histiocytic disorder, characterized by the xanthomatous infiltration of tissues by CD68-positive and CD1a-/CD100-negative foamy histiocytes. In childhood, ECD is exceptionally rare, and only a dozen cases have been published so far. The cooccurence of Langerhans cell histiocytosis (LCH) and ECD is even rarer. Here, we report a 2-year-old boy, the youngest...
متن کاملDisseminated histiocytoses biomarkers beyond BRAFV600E: frequent expression of PD-L1
The histiocytoses are rare tumors characterized by the primary accumulation and tissue infiltration of histiocytes and dendritic cells. Identification of the activating BRAFV600E mutation in Erdheim-Chester disease (ECD) and Langerhans cell histiocytosis (LCH) cases provided the basis for the treatment with BRAF and/or MEK inhibitors, but additional treatment options are needed. Twenty-four cas...
متن کاملBRAF-mutant hematopoietic malignancies
Mutations activating the serine-threonine kinase BRAF have been identified in a variety of cancers since they were first described in 2002. Currently, it is estimated that BRAF is mutated in ~8% of all cancers [1]. Sequencing studies performed in a broad spectrum of hematologic malignancies have revealed that mutations in BRAF, although generally quite rare amongst leukemias and lymphomas, are ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید
ثبت ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Blood
دوره 124 7 شماره
صفحات -
تاریخ انتشار 2014