Clinical course of the bony lesion of single-system single-site Langerhans cell histiocytosis – Is appropriate follow-up sufficient treatment?

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.

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A longitudinal study of single system langerhans cell histiocytosis from a centre in the UK: implications for follow-up

Archives of Disease in Childhood ◽

10.1136/adc.2011.212563.193 ◽

2011 ◽

Vol 96 (Supplement 1) ◽

pp. A82-A83 ◽

Cited By ~ 1

Author(s):

S. L. George ◽

V. Nanduri ◽

P. Brock

Keyword(s):

Longitudinal Study ◽

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Single System ◽

The Uk

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Congenital, Single System, Single Site, Langerhans Cell Histiocytosis: A New Case, Observations from the Literature, and Management Considerations

Pediatric Dermatology ◽

10.1111/j.1525-1470.2008.00852.x ◽

2009 ◽

Vol 26 (1) ◽

pp. 121-126 ◽

Cited By ~ 6

Author(s):

Theodore Zwerdling ◽

Thomas Konia ◽

Marc Silverstein

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Single Site ◽

Single System

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PP – 10-YEAR FOLLOW- UP OF A SINGLE-SYSTEM LANGERHANS CELL HISTIOCYTOSIS - MULTIFOCAL BONE INVOLVEMENT

Oral Surgery Oral Medicine Oral Pathology and Oral Radiology ◽

10.1016/j.oooo.2016.09.149 ◽

2017 ◽

Vol 123 (2) ◽

pp. e66

Author(s):

RENATA MENDONÇA MORAES ◽

JOYCE GIMENEZ MENON ◽

JULIANA ROCHA VERRONE ◽

JOSÉ DIVALDO PRADO ◽

FERNANDO AUGUSTO SOARES ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Bone Involvement ◽

Single System

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Langerhans Cell Histiocytosis - A Case Report

Journal of Nepal Paediatric Society ◽

10.3126/jnps.v27i2.1587 ◽

1970 ◽

Vol 27 (2) ◽

pp. 87-89

Author(s):

SM Gurubacharya ◽

RL Gurubacharya

Keyword(s):

Organ Dysfunction ◽

Langerhans Cell Histiocytosis ◽

Clinical Course ◽

Eosinophilic Granuloma ◽

Langerhans Cell ◽

Class I ◽

Unknown Etiology ◽

Single System ◽

Multisystem Disease ◽

System Disease

Histiocytosis is a heterogenous group of disorders that are characterized by proliferation and activation of mononuclear phagocyte system. Langerhans Cell Histiocytosis (LCH) or Class I histiocytosis is a rare disorder of unknown etiology with proliferation of Langerhan cells which may infiltrate a single or multiple organs. This disease is more common in infants and children. It is usually sporadic but a familial pattern is known. The term embraces the whole clinical spectrum of the disorder from single bone lesions (eosinophilic granuloma) to an aggressive widespread multisystem disease in very sick child (Letterer-Siwe disease) with a wide variety of intermediate forms including the Hand-shuller Christian triad. The cause of LCH is not firmly established and most investigators in the field have long suspected that LCH is immunologic disorder either in its etiology or in its pathophysiology. Recent evidence suggests that LCH is a clonal disorder rather than reactive disease. LCH is classified according to sites of involvement into single system disease and multisystem disease. Single system disease can be either unifocal or multifocal. Multisystem disease can be either without organ dysfunction or with organ dysfunction. Clinical course of LCH with single system disease is usually benign with high chance of survival. However, its clinical course is often unpredictable and patients can experience spontaneous remission and exacerbations. Histiocytic diseases are currently classified by the writing group of the Histiocyte Society in the three classes, namely;Class I: Langerhans Cell Histiocytosis (LCH) Class II: Histiocytosis of mononuclear phagocytes other than Langerhans Cells Class III: Malignant Histiocytic disorders Key words: Langerhans Cell Histiocytosis, Eosinophilic granuloma, Skull, lytic lesion doi:10.3126/jnps.v27i2.1587 J. Nepal Paediatr. Soc. Vol.27(2) p.87-89

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

Pakistan Armed Forces Medical Journal ◽

10.51253/pafmj.v71i6.4022 ◽

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...........

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Retrospective analysis of clinical manifestations and treatment outcomes of patients diagnosed with langerhans cell histiocytosis from a tertiary cancer hospital in South India

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20202255 ◽

2020 ◽

Vol 8 (6) ◽

pp. 2128

Author(s):

Roshan Koshy Jacob ◽

Shashidhar V. Karpurmath ◽

Manjunath Nandennavar ◽

Veerendra Angadi

Keyword(s):

Treatment Outcomes ◽

Retrospective Analysis ◽

Langerhans Cell Histiocytosis ◽

Clinical Manifestations ◽

Langerhans Cell ◽

Single System ◽

Multisystem Disease ◽

Cancer Hospital ◽

Mucosal Involvement

Background: Langerhans cell histiocytosis (LCH) comprises a diverse group of disorders where pathologic Langerhans cells accumulate in a variety of organs. Aims and objectives of the study is to analyse the clinical manifestations and treatment outcomes of patients diagnosed with LCH in a tertiary cancer hospital in South India.Methods: Retrospective analysis of the case records of patients presenting with histological proven case of LCH over a period of 7 years from 2011 to 2018, being treated at Vydehi Institute of Medical Sciences and Research Centre.Results: 10 patients with biopsy proven LCH were included. The median age of diagnosis was 8 years (range 1 to 73 years) and 3 patients aged 18 years or older at the time of diagnosis. The male: female ratio was 3:2. Multisystem involvement was found in 4 patients (40%) and Single system Involvement in remaining 6 patients. Isolated bone lesions were found in 4 patients (40%), 1 patient had isolated Lymph node involvement; 1 patient had oral cavity lesion. None of the 4 patients with multisystem diseases had skin/mucosal involvement; 3 had bony involvement, 2 patients had lung involvement. One patients with multisystem disease expired while 5 patients were lost to follow-up. 4 out of the 10 patients are on regular follow-up and are in remission.Conclusions: Despite limitation by the retrospective nature, this descriptive study was done to provide further disease information regarding Indian population. Data from this study clearly confirms the known fact that most of the patients with Single System LCH have a very good response rate. Patients with multisystem disease have the highest risk of disease related mortality and morbidity as one among the 4 patients with multisystem disease died just after initiating treatment.

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LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

Coluna/Columna ◽

10.1590/s1808-185120171603173214 ◽

2017 ◽

Vol 16 (3) ◽

pp. 240-243 ◽

Cited By ~ 1

Author(s):

ROHIT PANDEY ◽

HIMANSHU BHAYANA ◽

RAJESH KUMAR RAJNEESH ◽

ISH KUMAR DHAMMI ◽

REHAN UL-HAQ ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Treatment Options ◽

Langerhans Cell ◽

Single System ◽

Diagnostic Dilemma ◽

Systemic Involvement ◽

System Disease ◽

S 100 ◽

Specific Finding

ABSTRACT Langerhans cell histiocytosis (LCH) is characterised by an abnormal histiocytic accumulation in tissues such as the lung, spleen, bone marrow, skin, central nervous system, liver and lymph nodes, causing focal or systemic effects. No specific clinical & radiographic presentation of LCH is described in literature. This poses a diagnostic dilemma for surgeons. The scapula is the site of 3% of bone tumours, while for LCH it is the least common site. In a 10-year-old boy with isolated lesion of the scapula with no other systemic involvement, and no specific finding in MRI or CT scan of scapula, diagnosis was confirmed on biopsy. Division into single and multi-system disease is paramount in treatment, given that it is a single system disease. The patient improved clinically on follow-up of 2 years. The scapula is one of the rarest site of LCH, and because various lesions mimic each other, a biopsy is always required, with immunohistochemistry for CD68 & S-100. This was only a single system disease, so conservative management was performed, and the patent improved clinically.

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Langerhans Cell Histiocytosis of the Rib in an Adult: A Case Report

Case Reports in Oncology ◽

10.1159/000443722 ◽

2016 ◽

Vol 9 (1) ◽

pp. 83-88 ◽

Cited By ~ 4

Author(s):

Sung Hyun Kim ◽

Moon Young Choi

Keyword(s):

Back Pain ◽

Case Report ◽

Local Recurrence ◽

Bone Tumor ◽

Langerhans Cell Histiocytosis ◽

Osteolytic Lesion ◽

Langerhans Cell ◽

Pathologic Finding ◽

Single System

Single-site, single-system Langerhans cell histiocytosis (LCH) of the rib is one of the rarest causes of bone tumor in adults. Herein, we report a case of a healthy 35-year-old male who presented with upper back pain that was attributed to a solitary osteolytic lesion at the posterolateral aspect of his sixth rib. For diagnostic confirmation and treatment, partial resection of the sixth rib was performed and pathologic finding was consistent with LCH. At the final follow-up after 2 years, no local recurrence or metastasis was observed.

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