- Posted On 08.02.2018
Abstract. Abstract: Langerhans cell histiocytosis (LCH) is a proliferative histiocytic disorder of unknown cause originating from dendritic cells. The clinical presentation of LCH is highly variable. Although the features of this disease have been well described in children, they remain poorly defined in adults. The rarity of adult LCH makes assessment of its incidence difficult. Some of the best studies on incidence have been done in children. Carstensen and Ornvold 4 x 4 Carstensen, H. and Ornvold, K. The epidemiology of Langerhans cell histiocytosis in children in Denmark – Because adult pulmonary Langerhans cell histiocytosis (PLCH) is often confused with LAM, physicians at the 26 LAM clinics have taken an interest in PLCH. Relatively few studies have explored the natural history of PLCH, and the care of patients is often fragmented between hematology and oncology experts, pulmonologists, and other . Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAPK pathway genes. BRAF VE mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is.
Cladribine Activity in Adult Langerhans-Cell Histiocytosis
Diamond , Benjamin H. Thus, cladribine has major activity in adult LCH and warrants further investigation in both pediatric and adult LCH as a single agent and in combination with other drugs.
Of the 13 patients treated, seven were men and six were women. Because cladribine is potently toxic to monocytes, we conducted a phase II trial of cladribine. Carstensen and Ornvold 4 x 4 Carstensen, H. Graphs were plotted with Prism Version 5.
Characterization of human blood dendritic cell subsets. In vitro differentiation potential of monocytes and myeloid DCs. Bone marrow cytogenetics showed a normal male karyotype.
Langerhans cell histiocytosis - Wikipedia
An important caveat of the in vitro differentiation experiment is that healthy control cells without BRAF mutation were used. NT, not tested owing to insufficient number of cells. Classical monocytes appear able to contribute to histiocytes of both disorders, depending on the signals they receive. During therapy and subsequent follow-up, patients underwent a complete history and physical examination before each course of cladribine and monthly thereafter. Summary of adult patients included in the study.
It is not known if adult LCH or ECD arises from hematopoietic stem cells HSCs , nor which potential blood borne precursors lead to the formation of histiocytic lesions. Langerhans cell histiocytosis (LCH) is a dendritic cell (DC) neoplasm defined by the presence in the lesion of pathologic Langerhans cells (LCH cells) that are positive for CD1a, CD (Langerin), and S Because adult pulmonary Langerhans cell histiocytosis (PLCH) is often confused with LAM, physicians at the 26 LAM clinics have taken an interest in PLCH.
and Ornvold, K. J Dermatol Surg Oncol ; Local steroid cream is applied to skin lesions. Presence of Birbeck granules on electron microscopy and immuno-cytochemical features e. Diagnosis is confirmed histologically by tissue biopsy. There is significant interest in cell-free DNA as a marker of neoplasia, and this is also detectable in histiocytosis.
However systemic diseases often require chemotherapy.
A review of the etiology, pathology, staging, and therapy. Unifocal LCH, also called eosinophilic granuloma an older term which is now known to be a misnomer , is a slowly progressing disease characterized by an expanding proliferation of Langerhans cells in various bones.
Some overspill into adjacent quadrants was observed, owing to the close gating. In this report of cladribine use in adults with LCH, one patient developed chronic myelomonocytic leukemia and one patient had recurrence of papillary carcinoma of the thyroid gland. Alan Saven , Carol Burian.
J Clin Oncol ; Langerhans-cell histiocytosis LCH results from the accumulation of tissue histiocytes derived from the same progenitor cells as monocytes. Fifty-two adults with histiocytosis and 4 with HCL were recruited from a local clinic and collaborating centers Figure 1. Ten-year experience at Dallas Children's Medical Center".
Plasma and PBMCs were isolated by density centrifugation. Some of the best studies on incidence have been done in children. Responses and Response Duration Of the 13 patients treated, 12 were evaluable for response and all for toxicity.
Notably, recent gene expression studies described enrichment for myeloid progenitor signatures in ECD lesions. Antibodies were from BD unless stated otherwise and are denoted as follows: Juvenile xanthogranuloma Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann—Pick disease Sea-blue histiocyte Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis Multicentric reticulohistiocytosis , Reticulohistiocytoma Indeterminate cell histiocytosis.
As expected, BRAF VE was localized to hairy cells with a low level of alleles detectable in phenotypically normal B and NK cells but none in the remaining monocytes and myeloid cells. Among children under the age of 10, yearly incidence is thought to be 1 in ,;  and in adults even rarer, in about 1 in , After cladribine, there was complete resolution with the absence of asymmetry on CT scan.
Disease activity was assessed by radiological examination including magnetic resonance imaging and positron emission tomography.
Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma. Relatively few studies have explored the natural history of PLCH, and the care of patients is often fragmented between hematology and oncology experts, pulmonologists, and other . Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease.
Potent toxicity of 2-chlorodeoxyadeno sine towards human monocytes in vitro and in vivo: The ageing haematopoietic stem cell compartment. The rarity of adult LCH makes assessment of its incidence difficult.
The healthy counterparts of myeloid cells affected by BRAF mutation had a range of differentiation potentials depending on exogenous signals. In both patients, cultures remained negative.
Blood Jun , 93 12 ;. Med Pediatr Oncol ; The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cells , sometimes called dendritic cell histiocytosis.
Asterisk indicates the threshold of detection for a negative result where cell numbers were limiting. This page was last edited on 5 March , at Histiocytosis, also referred to as Langerhans Cell Histiocytosis (LCH), and formally called Histiocytosis X, represents a group of rare disorders involving specific cells that normally have important roles as part of the immune system.
The epidemiology of Langerhans cell histiocytosis in children in Denmark – In two of these seven patients, neutropenia was complicated by fever necessitating hospital admission for intravenous antibiotics.