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| Makras P, Samara C, Antoniou M, Zetos A, Papadogias D, Nikolakopoulou Z, Andreakos E, Toloumis G, Kontogeorgos G, Piaditis G, Kaltsas GA |
Evolving radiological features of hypothalamo-pituitary lesions in adult patients with Langerhans cell histiocytosis (LCH). |
Neuroradiology 2006, 48: 37 |
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| Langerhans cell histiocytosis (LCH) is a rare, systemic disease caused by monoclonal expansion of dendritic cells that shows a particular predilection for the hypothalamic-pituitary system (HPS). We studied the function (anterior and posterior pituitary hormonal secretion) and morphology using magnetic resonance imaging (MRI) of the HPS in 17 adult patients (seven males, median age 35 years, range 18-59 years) with multisystem LCH. We also evaluated the evolution of structural HPS abnormalities in relation to pituitary function and response to treatment in 12 of these patients during a median follow-up period of 3.75 years (range 1.5-10 years). Of the 17 patients, 14 (82%) had abnormal HPS imaging, and 12 (70%) had more than one area involved. Lack of the bright spot of the posterior pituitary lobe was typically found in all patients with the diagnosis of diabetes insipidus (DI). Eight patients (47%) had infundibular enlargement, six (35%) pituitary infiltration, four (24%) partially or completely empty sella, three (18%) hypothalamic involvement, and two (12%) infundibular atrophy. DI was found in 16 patients (94%) and anterior pituitary hormonal deficiency (APHD) in 10 patients (59%); two patients had single (12%) and 8 (47%) multiple APHD. During the follow-up period there was improvement of the initially demonstrated HPS pathology in seven (47%) patients, and five (33%) of them had received at least one form of treatment. APHD and DI persisted in all patients except in one in whom established gonadotrophin deficiency recovered. In summary, DI and APHD are very common in patients with multisystem LCH and are almost always associated with abnormal HPS imaging. |
| Makras P, Alexandraki KI, Chrousos GP, Grossman AB, Kaltsas GA |
Endocrine manifestations in Langerhans cell histiocytosis. |
Trends in endocrinology and metabolism: TEM 2007, 18: 252 |
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| Langerhans cell histiocytosis is a rare, multisystem disease that shows a particular predilection for hypothalamo-pituitary axis involvement. Diabetes insipidus is the most frequent permanent consequence of Langerhans cell histiocytosis, developing in around a quarter of patients. Although the exact prevalence of anterior pituitary hormone deficiencies is not known, it is probably high and is almost always associated with diabetes insipidus. Established pituitary hormone deficiencies are mostly permanent and require prompt diagnosis and treatment, whereas continuous follow-up is needed to detect deficiencies that might evolve later during the course of the disease. Involvement of endocrine tissues other than the pituitary has also been described but is relatively rare. Further studies are needed to evaluate the effect that endocrine deficiencies exert on the overall prognosis of patients with Langerhans cell histiocytosis. |
| Makras P, Terpos E, Kanakis G, Papatheodorou A, Anastasilakis AD, Kokkoris P, Kaltsas GA |
Reduced bone mineral density in adult patients with Langerhans cell histiocytosis. |
Pediatric blood & cancer 2012, 58: 819 |
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| This retrospective study evaluated bone mineral density (BMD) and bone turnover in adults with LCH. Twenty-five adult patients and 25 matched controls were evaluated with BMD measurement and indices of bone metabolism. A BMD value below the expected range for age (Z-score ≤ - 2.0) was found in 20% of patients; in particular, all postmenopausal women and men over 50-years had either osteoporosis or osteopenia. Patients with active disease had significantly lower Z-scores compared to patients with inactive disease and controls. Reduced bone turnover was found in all 14 patients treated with chemotherapy. No fractures due to osteoporosis were identified during 305.15 patient-years of follow-up. |
| Marsh RA, Allen CE, McClain KL, Weinstein JL, Kanter J, Skiles J, Lee ND, Khan SP, Lawrence J, Mo JQ, Bleesing JJ, Filipovich AH, Jordan MB |
Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab. |
Pediatric blood & cancer 2013, 60: 101 |
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| Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome that remains difficult to treat. Even with current standard HLH therapy, only approximately half of patients will experience complete resolution of disease, and early mortality remains a significant problem. Salvage therapies have been described only in limited case reports, and there are no large studies of second-line therapies. |
| McClain KL |
Drug therapy for the treatment of Langerhans cell histiocytosis. |
Expert opinion on pharmacotherapy 2005, 6: 2435 |
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| Langerhans cell histiocytosis results from the abnormal accumulation of a class of dendritic cells normally found in the skin, which proliferate in many organ systems along with lymphocytes, macrophages and eosinophils. Standard therapy for Langerhans cell histiocytosis includes vinblastine and prednisone with or without methotrexate and mercaptopurine, depending on the extent of disease. Effective therapies for patients unresponsive to the above include cytosine arabinoside and cladribine. Thalidomide has proven useful for patients with Langerhans cell histiocytosis of the skin and/or bone. Emerging therapies include the use of monoclonal antibodies against the CD1a or CD52 epitopes found on Langerhans cells. Specific therapies directed against the cytokines that are apparently critical to the abnormal proliferation have not yet been defined. |
| McClain K |
Bone and Soft Tissue Involvement - Oral Presentation at the Annual Meeting of the Histiocyte Society, Vienna. 2011. |
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| McClain K, Ramsay NK, Robison L, Sundberg RD, Nesbit M Jr |
Bone marrow involvement in histiocytosis X. |
Medical and pediatric oncology 1983, 11: 167 |
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| Twenty-eight patients with histiocytosis X (HX) who had bone marrow aspirates and/or trephine biopsies taken at diagnosis were studied. Five of the 28 patients (18%) had bone marrow specimens with clusters of benign histiocytes characteristic of histiocytosis X. Several statistically significant clinical and laboratory differences were found between those patients with positive marrows versus those with uninvolved bone marrows. Presence of HX in the bone marrow was more frequently associated with fever (P less than 0.01), splenomegaly (P less than 0.01), rash (P = 0.04), diabetes insipidus (P = 0.09), and platelet counts less than 175 X 10(9)/L(P = 0.01). Two of the five patients with bone marrow involvement died, whereas there were no deaths due to HX in the other 23 patients. From this review, it is suggested that bone marrow involvement at diagnosis may be associated with more extensive and potentially fatal disease. HX of the marrow was more easily diagnosed from trephine biopsy sections than aspiration smears. |
| McClain KL, Kozinetz CA |
A phase II trial using thalidomide for Langerhans cell histiocytosis. |
Pediatric blood & cancer 2007, 48: 44 |
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| Few new drugs for treatment of Langerhans cell histiocytosis (LCH) have been studied. Tumor necrosis factor-alpha (TNF-alpha) is a prime therapeutic target since it appears to be present in elevated amounts in LCH lesions. Thalidomide inhibits TNF-alpha production by affecting the gene promoter as well as other anti-cytokine effects. |
| McCormack FX |
Lymphangioleiomyomatosis: a clinical update. |
Chest 2008, 133: 507 |
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| Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutations in tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread, and remarkable female gender restriction. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax, and chylous fluid collections. Lung function declines at approximately twofold to threefold times the rate of healthy subjects, based on an annual drop in FEV1 of 75 to 120 mL in reported series. The diagnosis of pulmonary LAM can be made on high-resolution CT (HRCT) scan with reasonable certainty by expert radiologists, but generally requires a lung biopsy in cases in which tuberous sclerosis complex, angiomyolipomata, or chylous effusions are absent. The currently available treatment strategies are based on the antagonism of estrogen action, and are empiric and unproven. A trial of bronchodilators is warranted in patients with reversible airflow obstruction seen on pulmonary function testing. Pleurodesis should be performed with the initial pneumothorax, because the rate of recurrence is high. Angiomyolipomas that exceed 4 cm in size are more likely to bleed and should be evaluated for embolization. Air travel is well-tolerated by most patients with LAM. Lung transplantation is an important option for LAM patients, and can be safely performed by experienced surgeons despite prior unilateral or bilateral pleurodesis in most patients. Women with unexplained recurrent pneumothorax, tuberous sclerosis, or a diagnosis of primary spontaneous pneumothorax or emphysema in the setting of limited or absent tobacco use should undergo HRCT scan screening for LAM. Multicenter clinical trials based on several well-defined molecular targets are currently underway in the United States and Europe. |
| Mendez JL, Nadrous HF, Vassallo R, Decker PA, Ryu JH |
Pneumothorax in pulmonary Langerhans cell histiocytosis. |
Chest 2004, 125: 1028 |
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| Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease characterized by development of cystic changes that predispose to occurrence of pneumothorax. |
| Micke O, Seegenschmiedt MH, German Working Group on Radiotherapy in Germany |
Consensus guidelines for radiation therapy of benign diseases: a multicenter approach in Germany. |
International journal of radiation oncology, biology, physics 2002 Feb 1; 52: 496 |
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| To overcome the lack of written guidelines for radiation therapy (RT) of benign diseases, the German Working Group on Radiotherapy of Benign Diseases initiated a consensus process in 1999 to warrant continuous quality assurance and outcome research in this field. |
| Minkov M, Grois N, Heitger A, Pötschger U, Westermeier T, Gadner H, DAL-HX Study Group |
Response to initial treatment of multisystem Langerhans cell histiocytosis: an important prognostic indicator. |
Medical and pediatric oncology 2002, 39: 581 |
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| Reliable prediction of prognosis allowing risk-adapted therapy remains a major issue in the management of multisystem Langerhans cell histiocytosis (LCH). In a recent publication of the International LCH Study Group, response to initial therapy appears to be a reliable outcome predictor. The aim of this study is to test this observation in a cohort of patients treated with more intensive initial therapy. Furthermore, we compare the predictive value of response to initial therapy to some other well-established stratification systems. |
| Minkov M, Pötschger U, Grois N, Gadner H, Dworzak MN |
Bone marrow assessment in Langerhans cell histiocytosis. |
Pediatric blood & cancer 2007 Oct 15; 49: 694 |
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| Bone marrow changes and their relation to blood cytopenia in patients with Langerhans cell histiocytosis (LCH) have not been extensively studied to date. The aim of the present study was to characterize the bone marrow changes in LCH patients and to ascertain their relation to disease severity. |
| Minkov M, Grois N, McClain K, Nanduri V, Rodriguez-Galindo C, Simonitsch-Klupp I, Visser J, Weitzmann S, Whitlock J, Windebank K |
Langerhans Cell Histiocytosis - Histiocyte Society Evaluation and Treatment Guidelines. 2009. cited. |
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| Available from: www.histiocytesociety.org/document.doc?id=290 |
| Montella L, Merola C, Merola G, Petillo L, Palmieri G |
Zoledronic acid in treatment of bone lesions by Langerhans cell histiocytosis. |
Journal of bone and mineral metabolism 2009, 27: 110 |
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| Langerhans cell histiocytosis (LCH) is a rare disease, probably an atypical myeloproliferative syndrome, with variable clinical presentation and behavior. In this report, we focus on bone involvement by LCH and treatment with zoledronic acid in six patients as they progressed after chemotherapy and radiotherapy. Zoledronic acid appeared safe and produced significant relief in pain. |
| Montella L, Insabato L, Palmieri G |
Imatinib mesylate for cerebral Langerhans'-cell histiocytosis. |
The New England journal of medicine 2004 Sep 2; 351: 1034 |
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