scholarly journals LANGERHANS CELL HISTIOCYTOSIS OF THE SCAPULA - DIAGNOSIS & TREATMENT OPTIONS

2017 ◽  
Vol 16 (3) ◽  
pp. 240-243 ◽  
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.

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.

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

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

Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

2001 ◽  
Vol 5 (6) ◽  
pp. 486-489 ◽  
Author(s):  
Kelli W. Morgan ◽  
Jeffrey P. Callen
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Rare Condition ◽  
Signs And Symptoms ◽  
Male Infant ◽  
Skin Lesions ◽  
Langerhans Cell ◽  
Self Healing ◽  
Systemic Involvement ◽  
S 100 ◽  
Simplex Virus

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.

scholarly journals Langerhans Cell Histiocytosis: A Diagnostic Challenge in the Oral Cavity

2017 ◽  
Vol 2017 ◽  
pp. 1-6 ◽  
Author(s):  
Mehmet Ali Altay ◽  
Alper Sindel ◽  
Öznur Özalp ◽  
Burak Kocabalkan ◽  
İrem Hicran Özbudak ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Langerhans Cell Histiocytosis ◽  
Alveolar Bone ◽  
Reticuloendothelial System ◽  
Langerhans Cell ◽  
Unknown Etiology ◽  
Gingival Hyperplasia ◽  
Diagnostic Challenge ◽  
S 100

Background. Langerhans cell histiocytosis (LCH) is a rare disorder of the reticuloendothelial system with unknown etiology. This report aims to present a case of LCH with diffuse involvement of the oral cavity and to raise awareness of the distinguishing features of this diagnostically challenging entity. Case Report. A 26-year-old male patient presented with complaints of teeth mobility, intense pain, and difficulty in chewing. Intraoral and radiological examinations revealed generalized gingival hyperplasia and severe teeth mobility with widespread alveolar bone loss. Periodontal therapy was performed with no significant improvement. An incisional biopsy revealed Langerhans cells and positive reaction to S-100 and CD1, and the patient was diagnosed with LCH. The patient underwent systemic chemotherapy with vinca alkaloids and corticosteroids. Regression of gingival lesions, as well as significant decrease in mobility of the remaining teeth and severity of pain, was achieved during 12 months of follow-up. Conclusion. The rarity and variable system involvement of LCH necessitate a multidisciplinary approach be carried out for accurate diagnosis, effective treatment, and an uneventful follow-up. Awareness of oral manifestations of LCH may aid clinicians greatly in reducing morbidity and mortality associated with this debilitating condition.

scholarly journals Case series of cutaneous Langerhans cell histiocytosis in Indonesian children; The clinicopathological spectrum

2020 ◽  
Vol 12 (2) ◽  
Author(s):  
Irianiwati Widodo ◽  
Nita Sahara ◽  
Ery Kus Dwianingsih ◽  
Paranita Ferronika
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Case Series ◽  
Langerhans Cell ◽  
Multisystem Disease ◽  
Systemic Involvement ◽  
Clonal Proliferation ◽  
S 100 ◽  
Microscopic Features ◽  
Skin Biopsies ◽  
Histological Reactions

Langerhans Cell Histiocytosis (LCH) is a rare disease characterized by the clonal proliferation of Langerhans cells, which are immunoreactive to S-100 and CD-1a/ CD207 (Langerin). Cutaneous involvement is the most common presentation of LCH in children. It is suggested that the patients with single-system LCH limited to the skin have a better prognosis than those with systemic involvement. Three histologic reactions of cutaneous LCH have been reported and are associated with the clinical types of LCH. These histological reactions include: proliferative, granulomatous, and xanthomatous. This study presents the clinicopathological features of ten cutaneous LCH cases collected from Dr. Sardjito General Hospital Yogyakarta Indonesia between 2014-2018. The ten cases showed various clinical features, in which some features mimic other diseases. The microscopic features of skin biopsies showed granulomatous reaction in 80% of cases and proliferative reaction in the other 20%. Five patients (50% of cases) who died had systemic manifestation of thrombocytopenia, anemia, icterus, hepatosplenomegaly, and revealed the granulomatous type from their skin biopsy specimens. The clinical recognition of LCH and subsequent histological reaction determination are important since some cases may develop multisystem disease and have a poor prognosis.

scholarly journals An unusual case of adult-onset multi-systemic Langerhans cell histiocytosis with perianal and incident thyroid involvement

2017 ◽  
Vol 2017 ◽  
Author(s):  
Ozen Oz Gul ◽  
Pinar Sisman ◽  
Soner Cander ◽  
Erdem Gozden ◽  
Meral Kurt ◽  
...  
Keyword(s):  
Thyroid Gland ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
List Type ◽  
Adult Onset ◽  
System Disease ◽  
Multifocal Lesions ◽  
Mucosal Involvement ◽  
Euthyroid Status

Summary Langerhans cell histiocytosis (LCH) is a rare sporadic disease characterized by histiocytic neoplastic infiltration of various organ systems and a wide spectrum of clinical manifestations, ranging from benign and self-limiting to lethal. Herein, we report a rare case of adult-onset multi-systemic LCH in a 36-year-old male patient with an initial perianal presentation and incidental finding of subsequent thyroid gland involvement in the follow-up period. The patient with a history of perianal LCH treated with surgical excision and local radiotherapy was referred to our Endocrinology Department upon detection of hypermetabolic nodular lesions in the left lateral lobe of thyroid gland on positron emission tomography–computed tomography (PET/CT) scan in the nineth month of follow-up. Current evaluation revealed euthyroid status, a hypoechoic solid lesion of 13 × 9 mm in size with irregular borders in the left thyroid lobe on thyroid USG and cytologic assessment of thyroid nodule. The patient was diagnosed with suspected, oncocytic lesion, Hashimoto thyroiditis or LCH. The patient underwent total thyroidectomy and pathological assessment confirmed the diagnosis of Langerhans cell histiocytosis. Assessments in the sixth month of postoperative follow-up revealed euthyroid status with no thyroid tissue remnants or pathological lymph node on thyroid USG. In view of the multifocal lesions indicating multi-system disease, a systemic chemotherapy protocol with combination of prednisone (PRED) and vinblastine (VBL) has been planned by the hematology department. Learning points: Langerhans cell histiocytosis (LCH) shows a wide clinical spectrum and prognosis that ranges from benign and self-limiting single-system disease (with single or multifocal lesions) to a potentially lethal multi-system disease with severe organ dysfunction and death in some cases. It has been stated that the diagnosis is often delayed in perianal LCH unless LCH is specifically considered in the etiology, despite the fact that mucosal involvement may precede systemic involvement. Our findings support the statement that most of patients with LCH were PET positive at the time of initial diagnosis, while also emphasize the inclusion of this imaging modality as a part of the diagnostic workflow as well as in the setting of treatment response evaluation among adult LCH patients.

scholarly journals Langerhans Cell Histiocytosis - A Case Report

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

Pattern and course of single-system disease in Langerhans cell histiocytosis data from the DAL-HX 83- and 90-study

2001 ◽  
Vol 37 (2) ◽  
pp. 108-114 ◽  
Author(s):  
Carola Titgemeyer ◽  
Nicole Grois ◽  
Milen Minkov ◽  
Burgi Flucher-Wolfram ◽  
Irmgard Gatterer-Menz ◽  
...  
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Single System ◽  
System Disease
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