Adult Langerhans’ Cell Histiocitosis (LCH). A Single Institution Experience.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3890-3890
Author(s):  
Luis Fayad ◽  
Jimena Cannata
Keyword(s):  
Overall Survival ◽  
Clinical Presentation ◽  
Smoking Habit ◽  
Langerhans Cell ◽  
Risk Category ◽  
Single System ◽  
Single Institution ◽  
Limited Disease ◽  
Agressive Behaviour ◽  
Disseminated Disease

Abstract Langerhans’ cell histiocytosis (LCH) is a rare clonal disorder that affects mainly children. Clinical presentation as well as behaviour are heterogeneous and can range from solitary, relatively benign lesions to a fatal disseminated disease. Although the features have been well described in children, they remain poorly defined in adults. We report the clinical characteristics and outcome of 30 adults from a single institution. Clinical presentation: From 1957 to 2003, 30 patients were diagnosed with LCH at MDA. Median age at presentation was 37 years-old, with 60% of being male. Depending on the organs involved and according to the pediatric classification, 17 cases (57%) had a limited form of LCH (single-system) and 13 cases (43%) had disseminated disease (multisystemic). Patients with single-system LCH had mainly bone (76%) and skin (24%) involvement, which was localized to a single site in 10 cases (58%) and multifocal in the remaining. Patients with limited disease were younger (median age 30 yo). Patients with multisystemic LCH were subdivided into a “high risk” category (5 patients) if lung, liver, spleen, or bone marrow was involved, or into a “low risk” category (8 cases), if none of the risk organs was affected. Six patients had pulmonary involvement, typically as bilateral small nodules although diffuse progressive infiltrates were also present in 2 cases. Clinical course: Patients with limited disease were mainly treated with steroids, radiation, or immunomodulatory agents while those with multifocal or multi-systemic disease had a more agressive behaviour and were treated with systemic therapy. Patients with pulmonary nodules usually responded to systemic steroids and stop of smoking habit. Median of 3 treatments were given (range 1 to 7). Sixty-one percent of patients had a 30-year overall survival (OS). Single-system LCH had a more indolent course (79% OS at 30 years) than multi-systemic LCH (median OS of 16 years). Risk organ involvement was the most important adverse prognostic factor (median OS of 2.2 years vs. 65% OS at 30 years, p: 0.007). Figure 1. Global overall survival on LCH patients Figure 1. Global overall survival on LCH patients Figure 2. Overall survival according to the extent of the disease Figure 2. Overall survival according to the extent of the disease

scholarly journals Clinical characteristics and survival of children with Langerhans cell hystiocytosis

2008 ◽  
Vol 136 (9-10) ◽  
pp. 514-518
Author(s):  
Nada Krstovski ◽  
Dragana Janic ◽  
Lidija Dokmanovic ◽  
Radivoj Brdar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Characteristics ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Langerhans Cell ◽  
Single System ◽  
Multisystem Disease ◽  
System Disease ◽  
The Central Nervous System

INTRODUCTION Langerhans cell histiocytosis is a rare disease in children, initial presentation is variable, clinical course, prognosis and survival are mostly unpredictable. OBJECTIVE To summarise clinical characteristics and treatment results in children with Langerhans cell histiocytosis. METHOD Retrospectively there were analyzed patients with LCH diagnosed and treated at Hematology Department of University Children's Hospital in Belgrade from 1990 to 2006. Clinical presentation, therapy and survival according to Kaplan-Meier's statistical test was analysed. RESULTS 30 patients were treated, aged from 4 months to 14 years, mean 3.9 years, median 2.3 years, 18 (60%) males, 12 (40%) females. A single system disease was diagnosed in 16 (53%) patients, of whom 6 patients with multifocal bone disease. All patients were in complete remission averagely following162 and 82 months respectively. Multisystem disease was found in 14 (47%) patients. The lymph nodes and skin were more frequently involved organs than the central nervous system (diabetes insipidus), lung, liver and spleen. The number of involved organs ranged from 2 to 8, mean 4.2. Four patients died due to disease progression 3, 16, 36 and 66 months after diagnosis. Nine patents with multisystem disease were in remission with 117 months of follow-up. One patient was lost on follow-up. CONCLUSION The clinical course of patients with a single system disease is usually benign while a multisystem disease has to be aggressively treated with precise initial evaluation and staging before therapy.

scholarly journals CLINICAL FEATURES AND OUTCOME IN CHILDREN WITH LANGERHANS CELL HISTIOCYTOSIS. A SINGLE INSTITUTION EXPERIENCE FROM PAKISTAN

2022 ◽  
Vol 71 (6) ◽  
pp. 2194-2201
Author(s):  
Nida Noor ◽  
Rabia Muhammad Wali ◽  
Annis -Ur- Rehman ◽  
Muhammad Abu Bakar
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Manifestations ◽  
Langerhans Cell ◽  
Single System ◽  
Single Institution ◽  
Multisystem Disease ◽  
Disease Mortality ◽  
Multisystem Involvement ◽  
Related Mortality

Objective: To find out the clinical manifestations, treatment given and outcome of children with diagnosis of Langerhans cell histiocytosis. Study Design: Retrospective observational study. Place and Duration of Study: Shaukat Khanum Cancer Hospital, Lahore Pakistan, from Jan 2005 to Dec 2015. Methodology: Medical charts were reviewed in detail along with the available imaging for the patients. The data included age at the time of diagnosis, extent of the disease, involvement of risk organs, treatment given, response at 6th week of chemotherapy and at the end of the treatment, and outcome in terms of disease progression during the treatment, relapse of disease on follow up and cause of death either due to treatment related mortality or disease complications. Results: There were 29 patients, 12 patients (41%) had single system and 17 (58%) had multisystem involvement. 7 patients (41%) had risk organ involvement in the multisystem group. All the patients of multisystem and 6 patients of single system were treated according to the Langerhans cell histiocytosis III protocol. Commonest sites of involvement were bone in 22 (75%), followed by lymph nodes in 18 (62%) patients. Disease relapse was seen in 6 patients and all of them had multisystem disease. Mortality was observed only in multisystem Langerhans cell histiocytosis patients and more than 50% were risk organ positive. Conclusion: Langerhans cell histiocytosis is a highly heterogeneous disease. Some forms are curable without chemotherapy, while the multisystem disease requires aggressive treatment. However, despite intensive treatment, the multisystem disease and risk organs involved have poor...........

Long-term outcomes of lumbar microdiscectomy in the pediatric population: a large single-institution case series

2019 ◽  
Vol 24 (5) ◽  
pp. 549-557
Author(s):  
Malia McAvoy ◽  
Heather J. McCrea ◽  
Vamsidhar Chavakula ◽  
Hoon Choi ◽  
Wenya Linda Bi ◽  
...  
Keyword(s):  
Conservative Management ◽  
Functional Outcomes ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Csf Leak ◽  
Single Institution ◽  
The Mean

OBJECTIVEFew studies describe long-term functional outcomes of pediatric patients who have undergone lumbar microdiscectomy (LMD) because of the rarity of pediatric disc herniation and the short follow-up periods. The authors analyzed risk factors, clinical presentation, complications, and functional outcomes of a single-institution series of LMD patients over a 19-year period.METHODSA retrospective case series was conducted of pediatric LMD patients at a large pediatric academic hospital from 1998 to 2017. The authors examined premorbid risk factors, clinical presentation, physical examination findings, type and duration of conservative management, indications for surgical intervention, complications, and postoperative outcomes.RESULTSOver the 19-year study period, 199 patients underwent LMD at the authors’ institution. The mean age at presentation was 16.0 years (range 12–18 years), and 55.8% were female. Of these patients, 70.9% participated in competitive sports, and among those who did not play sports, 65.0% had a body mass index greater than 25 kg/m2. Prior to surgery, conservative management had failed in 98.0% of the patients. Only 3 patients (1.5%) presented with cauda equina syndrome requiring emergent microdiscectomy. Complications included 4 cases of postoperative CSF leak (2.0%), 1 case of a noted intraoperative CSF leak, and 3 cases of wound infection (1.5%). At the first postoperative follow-up appointment, minimal or no pain was reported by 93.3% of patients. The mean time to return to sports was 9.8 weeks. During a mean follow-up duration of 8.2 years, 72.9% of patients did not present again after routine postoperative appointments. The total risk of reoperation was a rate of 7.5% (3.5% of patients underwent reoperation for the same level; 4.5% underwent adjacent-level decompression, and one patient [0.5%] ultimately underwent a fusion).CONCLUSIONSMicrodiscectomy is a safe and effective treatment for long-term relief of pain and return to daily activities among pediatric patients with symptomatic lumbar disc disease in whom conservative management has failed.

scholarly journals Clinical presentation and management of malignant germ cell ovarian tumours in BPKMCH

2016 ◽  
Author(s):  
Jitendra Pariyar ◽  
Binuma Shrestha
Keyword(s):  
Neoadjuvant Chemotherapy ◽  
Germ Cell ◽  
Clinical Presentation ◽  
Early Stage ◽  
Germ Cell Tumour ◽  
Cystic Teratoma ◽  
Germ Cell Tumours ◽  
Early Stage Disease ◽  
Cancer Hospital ◽  
Disseminated Disease

Background: Germ cell malignancies account for about 5% of all ovarian cancers. These tumours grow rapidly and often produce symptoms quicker than the slow growing epithelial tumour. Commonly seen in the first two decades of life germ cell malignancies are highly chemosensitive and are potentially curable with surgery and chemotherapy. This study is the first of its kind regarding the epidemiology, management and outcome of patients with malignant germ cell tumour in Nepal. Objective: To analyze the clinical presentation and management outcomes of malignant germ cell tumours managed in B.P. Koirala Memorial Cancer Hospital, Nepal. Methodology: Descriptive study conducted in B.P. Koirala Memorial Cancer Hospital, Nepal. Case records of malignant germ cell tumours attending the hospital from January 1999 to December 2009 were analyzed regarding their illness history, clinical examination, investigations, treatment, follow-up and outcomes measured. Observations: Total 65 cases of malignant germ cell tumours with age range from 2 to 58 years (mean 21.7 years) were received. 42% cases were Tibeto-Burmese; 30% were Indo-Aryans. There were 15 cases (23%) of dysgeminoma, 21 endodermal sinus tumor (32%), 16 Immature Cystic Teratoma (24.5%), 9 (14%) Mixed Germ Cell, 2 unclassified GCT (3.5%) and 2 malignant transformation in teratoma (3.5%). 33 (49.5%) patients had early stage disease, 37 (57%) underwent fertility preserving surgery. 4 cases (9%) due to disseminated disease, underwent neoadjuvant chemotherapy followed by debulking surgery. 51 cases (78.5%) received adjuvant chemotherapy (BEP or EP regimen). The overall survival was 70%. Conclusion: Early stage germ cell malignancies can be safely managed by fertility preserving surgery followed by, chemotherapy if indicated. For advanced diseases, neoadjuvant chemotherapy followed by surgery can be undertaken with curable intent.

Five-Year Actual Overall Survival in Resected Pancreatic Cancer: A Contemporary Single-Institution Experience from a Multidisciplinary Perspective

2017 ◽  
Vol 24 (6) ◽  
pp. 1722-1730 ◽  
Author(s):  
Vincent J. Picozzi ◽  
Stephen Y. Oh ◽  
Alicia Edwards ◽  
Margaret T. Mandelson ◽  
Russell Dorer ◽  
...  
Keyword(s):  
Pancreatic Cancer ◽  
Overall Survival ◽  
Single Institution ◽  
Multidisciplinary Perspective

scholarly journals Chronic granulomatous ‘Aspergillus lung mass’

2020 ◽  
Vol 90 (3) ◽  
Author(s):  
Kavitha Venkatnarayan ◽  
Uma Devaraj ◽  
Uma Maheswari Krishnaswamy ◽  
Priya Ramachandran ◽  
Sreekar Balasundaram ◽  
...  
Keyword(s):  
Immune Response ◽  
Lung Disease ◽  
Respiratory Symptoms ◽  
Clinical Presentation ◽  
Wide Spectrum ◽  
Lung Mass ◽  
Pulmonary Aspergillosis ◽  
Histological Picture ◽  
Life Threatening ◽  
Disseminated Disease

The diverse clinicopathological spectrum of pulmonary aspergillosis is a consequence of varying levels of invasiveness of this ubiquitous fungus, which largely depends on the host immune response and pre-existing lung disease. The clinical presentation of pulmonary aspergillosis spans a wide spectrum from hypersensitivity to life threatening angio-invasive and disseminated disease. We report the case of a young immunocompetent male with no underlying lung disease, who presented with an incidentally detected ‘infective mass’ lesion in the lung associated with minimal respiratory symptoms. The diagnostic challenges posed by the unusual clinical, radiological and histological picture as well as the therapeutic dilemmas faced are discussed in this report.

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