| Autor(en) |
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| Lakatos K, Herbrüggen H, Pötschger U, Prosch H, Minkov M |
Radiological features of thymic langerhans cell histiocytosis. |
Pediatric blood & cancer 2013, 60:E143 |
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| TI was reported in 18/1,264 (1.4%) LCH patients. All nine patients with TI at initial LCH presentation were below 2 years of age and had multisystem LCH (9/242, 4%). Images (sonography, CT, MRI) for central review were available in 15 cases. Characteristic findings of TI were thymus enlargement (67%), few to many cysts (80%), and few to many calcifications (100%). Sonographic and MRI findings were in excellent agreement. We recommend adding sonography of the thymus to the standard for initial clinical evaluation of LCH patients below the age of 2 years. |
| Lau SK, Chu PG, Weiss LM |
Immunohistochemical expression of Langerin in Langerhans cell histiocytosis and non-Langerhans cell histiocytic disorders. |
The American journal of surgical pathology 2008, 32: 615 |
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| Langerin is a type II transmembrane C-type lectin associated with the formation of Birbeck granules in Langerhans cells. Langerin is a highly selective marker for Langerhans cells and the lesional cells of Langerhans cell histiocytosis. Although Langerin protein expression in Langerhans cell histiocytosis has been previously documented, the specificity of Langerin expression as determined by immunohistochemistry in the context of other histiocytic disorders has not been well established. In the present study, Langerin immunoreactivity was examined in a series of histiocytic disorders of monocyte/macrophage and dendritic cell derivation to assess the specificity and utility of Langerin as a diagnostic marker for Langerhans cell histiocytosis. Immunohistochemical expression of CD1a was also evaluated for comparison. Seventeen cases of Langerhans cell histiocytosis and 64 cases of non-Langerhans cell histiocytic disorders were examined. Langerin and CD1a were uniformly expressed in all cases of Langerhans cell histiocytosis, with the exception of one case that was positive for Langerin and negative for CD1a. Among the non-Langerhans cell histiocytic disorders evaluated, focal Langerin immunoreactivity was observed only in 2 of 10 cases of histiocytic sarcoma. All non-Langerhans cell histiocytic disorders showed no expression of CD1a. Langerin expression seems to be a highly sensitive and relatively specific marker of Langerhans cell histiocytosis. Immunohistochemical evaluation of Langerin expression may have utility in substantiating a diagnosis of Langerhans cell histiocytosis and separating this disorder from other non-Langerhans cell histiocytic proliferations. |
| Lazor R, Etienne-Mastroianni B, Khouatra C, Tazi A, Cottin V, Cordier JF |
Progressive diffuse pulmonary Langerhans cell histiocytosis improved by cladribine chemotherapy. |
Thorax 2009, 64: 274 |
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| Le Pavec J, Lorillon G, Jaïs X, Tcherakian C, Feuillet S, Dorfmüller P, Simonneau G, Humbert M, Tazi A |
Pulmonary Langerhans Cell Histiocytosis associated pulmonary hypertension: clinical characteristics and impact of pulmonary arterial hypertension therapies. |
Chest 2012 Mar 29; |
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| Abstract BACKGROUND:Precapillary pulmonary hypertension (PH) is a complication of pulmonary Langerhans cell histiocytosis (PLCH) associated with increased mortality. However, outcomes and efficacy of pulmonary arterial hypertension (PAH) therapies in patients with PH complicating PLCH (PLCH-PH) remain unknown. METHODS:Consecutive PLCH patients with PH confirmed by right heart catheterization were included in the study. Characteristics at baseline and during follow-up as well as survival were analyzed. RESULTS:29 patients were studied. Baseline characteristics of PLCH-PH patients were: 83% of patients in World Health Organization (WHO) functional class III-IV, mean 6-min walk distance of 355 ± 95 m, mean pulmonary arterial pressure (mPAP) of 45 ± 14 mm Hg, cardiac index of 3.2 ± 0.9 L.min(-1).m(-2) and pulmonary vascular resistance (PVR) of 555 ± 253 dyn.s.cm(-5). Use of PAH therapy in 12 patients was followed by improvement in mPAP (56 ± 14 and 45 ± 12 mm Hg, P = 0.03) and PVR (701 ± 239 and 469 ± 210 dyn.s.cm(-5), P = 0.01) between baseline and follow up evaluations. No significant oxygen worsening was observed in the treated group. The 1-, 3- and 5-year survival estimates of the 29 patients were 96%, 92% and 73%, respectively. Except a trend toward a better survival associated with the use of PAH therapy, WHO functional class was the only variable significantly associated with death. CONCLUSIONS:In this group of patients, PAH therapies improved hemodynamic without oxygen worsening or pulmonary edema. WHO functional class was the only prognostic factor identified. Prospective clinical trials focusing on this population of patients are warranted. |
| Lee JS, Ko GH, Kim HC, Jang IS, Jeon KN, Lee JH |
Langerhans cell sarcoma arising from Langerhans cell histiocytosis: a case report. |
Journal of Korean medical science 2006, 21: 577 |
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| Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells that have overtly malignant cytologic features. It is a very rare disease and theoretically, it can present de novo or progress from an antecedent Langerhans cell histiocytosis (LCH). However, to our knowledge, LCS arising from an antecedent LCH has not been reported on. We present here a case of LCS arising from a pulmonary LCH. A 34 yr-old man who was a smoker, had a fever and a chronic cough. Computed tomographic (CT) scan revealed multiple tiny nodules in both lungs. The thoracoscopic lung biopsy revealed LCH. The patient quit smoking, but he received no other specific treatment. One year later, the follow up chest CT scan showed a 4 cm-sized mass in the left lower lobe of the lung. A lobectomy was then performed. Microscopic examination of the mass revealed an infiltrative proliferation of large cells that had malignant cytologic features. Immunohistochemical stains showed a strong reactivity for S-100 and CD68, and a focal reactivity for CD1a. We think this is the first case of LCS arising from LCH. |
| Lefaucheur R, Maltête D, Haroche J, Borden A, Wallon D, Bourre B |
Teaching neuroimages: ataxia and diabetes insipidus. |
Neurology 2013 Jul 16; 81:e19 |
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| Lehmberg K, Pink I, Eulenburg C, Beutel K, Maul-Pavicic A, Janka G |
Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis. |
The Journal of pediatrics 2013, 162: 1245 |
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| To identify measures distinguishing macrophage activation syndrome (MAS) in systemic juvenile idiopathic arthritis (sJIA) from familial hemophagocytic lymphohistiocytosis (FHL) and virus-associated hemophagocytic lymphohistiocytosis (VA-HLH) and to define appropriate cutoff values. To evaluate suggested dynamic measures differentiating MAS in patients with sJIA from sJIA flares. |
| Lehmberg K, Albert MH, Beier R, Beutel K, Gruhn B, Kröger N, Meisel R, Schulz A, Stachel D, Woessmann W, Janka G, Müller I |
Treosulfan-based conditioning regimen for children and adolescents with hemophagocytic lymphohistiocytosis. |
Haematologica 2014, 99: 180 |
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| In hematopoietic stem cell transplantation for hemophagocytic lymphohistiocytosis, high transplant-related mortality after busulfan-based myeloablative regimens has been observed. Conditioning regimens with reduced toxicity based on melphalan or treosulfan are promising alternatives. We retrospectively analyzed hematopoietic stem cell transplantations in 19 hemophagocytic lymphohistiocytosis patients after conditioning with fludarabine, treosulfan, alemtuzumab, with or without thiotepa. Overall and disease-free survivals were 100% (follow up 7-31 months). Two patients required second transplant (1 after haploidentical transplantation). In 6 patients, overall donor chimerism dropped below 75% and prompted donor lymphocyte infusions. Administration of donor lymphocytes or second transplantation were significantly more frequent after transplantation from a human leukocyte antigen mismatched (9/10) versus matched (10/10) donor (P=0.018). The toxicity profile was favorable, with one veno-occlusive disease, one grade 3 graft-versus-host disease after donor lymphocyte infusion, and 2 severe viral infections (1 influenza, 1 Epstein Barr virus). In conclusion, the treosulfan-based regimen in hemophagocytic lymphohistiocytosis is effective with low toxicity and gives excellent overall and disease-free survival rates. In the future, the incidence of mixed chimerism, particularly after human leukocyte antigen mismatched donor transplants, needs to be addressed. |
| Lehmberg K, McClain KL, Janka GE, Allen CE |
Determination of an appropriate cut-off value for ferritin in the diagnosis of hemophagocytic lymphohistiocytosis. |
Pediatric blood & cancer 2014 Apr 21; |
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| Hyperferritinemia is a hallmark of hemophagocytic lymphohistiocytosis (HLH). In the HLH-2004 criteria, a ferritin value >500 µg/L is considered positive. However, this level was not determined based on evidence. We compared 123 patients with HLH and 320 patients with other hyperferritinemic conditions, calculated a receiver-operating characteristic, and determined sensitivity and specificity for different values. At 2,000 µg/L a trade-off is reached with sensitivity at 70% and specificity at 68%. If familial HLH and virus-associated acquired HLH are analyzed separately, sensitivity and specificity are similar for this level. The results may guide a potential modification of the current HLH criteria. Pediatr Blood Cancer © 2014 Wiley Periodicals, Inc. |
| Lo WC, Chen CC, Tsai CC, Cheng PW |
Isolated adult Langerhans' cell histiocytosis in cervical lymph nodes: should it be treated? |
The Journal of laryngology and otology 2009, 123: 1055 |
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| We report an extremely rare case of Langerhans' cell histiocytosis involving isolated cervical lymph nodes, and we discuss the diagnosis and treatment of this infrequent disease. |
| Lorillon G, Bergeron A, Detourmignies L, Jouneau S, Wallaert B, Frija J, Tazi A |
Cladribine is effective against cystic pulmonary Langerhans cell histiocytosis. |
American journal of respiratory and critical care medicine 2012 Nov 1; 186: 930 |
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| Lourda M, Olsson-Åkefeldt S, Gavhed D, Björnfot S, Clausen N, Hjalmars U, Sabel M, Tazi A, Aricò M, Delprat C, Henter JI, Svensson M |
Detection of IL-17A-producing peripheral blood monocytes in Langerhans cell histiocytosis patients. |
Clinical immunology (Orlando, Fla.) 2014 Apr 15; 153: 112 |
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| Langerhans cell histiocytosis (LCH) is a rare disease of unknown cause with manifestations ranging from isolated granulomatous lesions to life-threatening multi-system organ involvement. This disorder is further characterized by infiltration of immune cells in affected tissues and an association with interleukin (IL)-17A has been reported. Here, we investigated the presence of IL-17A-producing cells among peripheral blood mononuclear cells isolated from LCH patients and observed a high percentage of IL-17A(+) monocytes in peripheral blood of LCH patients compared to controls. The IL-17A(+) monocytes were also positive for the transcription factor retinoic acid orphan receptor (ROR) γt and showed increased mRNA levels for both IL-17A and RORγt. Notably, IL-17A was produced by all monocyte subsets and the expression level was positively associated with LCH disease activity. These data support a role for monocytes in the pathogenesis of LCH. Future therapeutic approaches may consider identification of patients who may benefit from IL-17A-targeted interventions. |
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