scholarly journals Langerhans Cell Histiocytosis as Cause of Central Diabetes Insipidus: Case Report

2018 ◽  
Vol 37 (01) ◽  
pp. 76-79 ◽  
Author(s):  
Diana Nascimento ◽  
Manoel Teixeira ◽  
Eberval Figueiredo
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Vital Functions ◽  
Hypothalamic Pituitary Axis ◽  
The Individual ◽  
Single Bone ◽  
Made In

AbstractLangerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system, characterized by the aberrant proliferation of specific dendritic cells. The clinical presentation ranges from a single bone lesion to widespread multiorgan involvement. This disease is usually considered to be a disease of childhood; however, the diagnosis is frequently made in adulthood. The course of the disease is fairly unpredictable and varies from spontaneous resolution to progress into a debilitating form, which compromises the vital functions with occasional fatal consequences. Langerhans cell histiocytosis exhibits a predilection for the hypothalamic-pituitary-axis, with diabetes insipidus being the most common endocrine consequence related to the disease, which may be prior to diagnosis or develop at any time during the course of the disease. The diagnosis of LCH should be based on histologic and immunophenotypic examination of a lesional biopsy, although other testing may be done, depending on the symptoms. There is no established, widely agreed-upon treatment of LCH, in general. The treatment depends upon the individual patient and the extent and areas of involvement. The present article aims to describe the case of a 26-year-old male patient whose symptoms started with a headache and occipital bone lesion that progressed later with diabetes insipidus.

scholarly journals Treatment of Multifocal Multisystem BRAF Positive Langerhans Cell Histiocytosis with Cladribine, Surgery and Allogenic Stem Cell Transplantation

2017 ◽  
Vol 60 (4) ◽  
pp. 152-156 ◽  
Author(s):  
Filip Gabalec ◽  
Martin Šimkovič ◽  
Alžběta Zavřelová ◽  
Petra Kašparová ◽  
Paula Morávková ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplantation ◽  
Cell Transplantation ◽  
Allogeneic Stem Cell Transplantation ◽  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Allogenic Stem Cell Transplantation ◽  
Single Bone

Langerhans cell histiocytosis (LCH) is a very rare disease in adults and as well a very rare cause of sellar expansion. The clinical presentation can be heterogeneous, from a single bone lesion to potentially fatal, widespread disease. We describe the difficulties with the diagnosis and treatment of LCH as well as successful treatment with cladribine chemotherapy and allogeneic stem cell transplantation.

scholarly journals A Painful Shoulder Revealing Langerhans Cell Histiocytosis

2020 ◽  
pp. 24-27
Author(s):  
N. A. R. Ranaivo ◽  
M. L. Rakotomahefa Narison ◽  
M. Bemena ◽  
S. H. Raobijaona
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Clinical Presentation ◽  
Bone Lesion ◽  
Langerhans Cell ◽  
Irregular Boundary ◽  
X Ray ◽  
Multidisciplinary Consultation ◽  
Life Threatening ◽  
Spontaneous Improvement ◽  
The Right

Introduction: Langerhans cell histiocytosis is a systemic proliferative disease. It is a rare disease that can affects all tissues. Evolution can be spontaneously favorable. Multi-organ involvement may be life-threatening. We report the case of an toddler with bone lesion and issues. Case Report: It was a 33-month-old infant with right shoulder pain. Clinically, she had a swelling in the front side of the right shoulder with a limited abduction. X-ray of the right shoulder showed osteolysis with an irregular boundary of the right humeral head. The blood work was normal. In view of the painful swelling of the right shoulder, a biopsy was performed. Histological examination confirmed the diagnosis of Langerhans histiocytosis. A conservative treatment was decided after a multidisciplinary consultation meeting. After six months, spontaneous improvement was noted. Conclusion: Langerhans cell histiocytosis is a proliferative pathology that can affect one or more organs. The clinical presentation is polymorphic according to the affected organ. An extension assessment is fundamental in the management in order to determine the treatment.

scholarly journals How I treat Langerhans cell histiocytosis

Blood ◽  
2015 ◽  
Vol 126 (1) ◽  
pp. 26-35 ◽  
Author(s):  
Carl E. Allen ◽  
Stephan Ladisch ◽  
Kenneth L. McClain
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Randomized Clinical Trials ◽  
Bone Lesion ◽  
Disease Recurrence ◽  
Langerhans Cell ◽  
Low Risk ◽  
Clinical Severity ◽  
Mitogen Activated Protein Kinase ◽  
Risk Patients ◽  
Single Bone

Abstract “Langerhans cell histiocytosis” (LCH) describes a spectrum of clinical presentations ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease. Regardless of clinical severity, LCH lesions share the common histology of CD1a+/CD207+ dendritic cells with characteristic morphology among an inflammatory infiltrate. Despite historical uncertainty defining LCH as inflammatory vs neoplastic and incomplete understanding of mechanisms of pathogenesis, clinical outcomes have improved markedly over the past decades through cooperative randomized clinical trials based on empiric therapeutic strategies. Significant advances include recognition of high- and low-risk clinical groups defined by hematopoietic and/or hepatic involvement, and of the importance of optimal intensity and of duration of chemotherapy. Nevertheless, mortality of high-risk patients, disease recurrence, lack of robustly tested salvage strategies, and significant disease morbidity of both high- and low-risk patients remain challenges. Recent discovery of recurrent somatic mutations in mitogen-activated protein kinase pathway genes at critical stages of myeloid hematopoietic differentiation in LCH patients supports redefinition of the disease as a myeloproliferative disorder and provides opportunities to develop novel approaches to diagnosis and therapy.

Central diabetes insipidus revealing Langerhans cell histiocytosis

2019 ◽  
Author(s):  
Najoua Lassoued ◽  
Salmane Wannes ◽  
Asma Wardani ◽  
Abir Omrane ◽  
Raoudha Boussofara ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus

Isolated Langerhans Cell Histiocytosis Bone Lesion in Pediatric Patients

2015 ◽  
Vol 153 (5) ◽  
pp. 751-757 ◽  
Author(s):  
Aren Bezdjian ◽  
Abdullah A. Alarfaj ◽  
Namrata Varma ◽  
Sam J. Daniel
Keyword(s):  
Langerhans Cell Histiocytosis ◽  
Pediatric Patients ◽  
Bone Lesion ◽  
Langerhans Cell

scholarly journals Occult Langerhans Cell Histiocytosis Presenting with Papillary Thyroid Carcinoma, a Thickened Pituitary Stalk and Diabetes Insipidus

2016 ◽  
Vol 2016 ◽  
pp. 1-3 ◽  
Author(s):  
Michael S. Gordon ◽  
Murray B. Gordon
Keyword(s):  
Papillary Thyroid Carcinoma ◽  
Thyroid Carcinoma ◽  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Hypogonadotropic Hypogonadism ◽  
Langerhans Cell ◽  
Pituitary Stalk ◽  
Bright Spot ◽  
Molecular Testing ◽  
Papillary Thyroid

Etiologies of a thickened stalk include inflammatory, neoplastic, and idiopathic origins, and the underlying diagnosis may remain occult. We report a patient with a thickened pituitary stalk (TPS) and papillary thyroid carcinoma (PTC) whose diagnosis remained obscure until a skin lesion appeared. The patient presented with PTC, status postthyroidectomy, and I131therapy. PTC molecular testing revealed BRAF mutant (V600E, GTC>GAG). She had a 5-year history of polyuria/polydipsia. Overnight dehydration study confirmed diabetes insipidus (DI). MRI revealed TPS with loss of the posterior pituitary bright spot. Evaluation showed hypogonadotropic hypogonadism and low IGF-1. Chest X-ray and ACE levels were normal. Radiographs to evaluate for extrapituitary sites of Langerhans Cell Histiocytosis (LCH) were unremarkable. Germinoma studies were negative: normal serum and CSF beta-hCG, alpha-fetoprotein, and CEA. Three years later, the patient developed vulvar labial lesions followed by inguinal region skin lesions, biopsy of which revealed LCH. Reanalysis of thyroid pathology was consistent with concurrent LCH, PTC, and Hashimoto’s thyroiditis within the thyroid. This case illustrates that one must be vigilant for extrapituitary manifestations of systemic diseases to diagnose the etiology of TPS. An activating mutation of the protooncogene BRAF is a potential unifying etiology of both PTC and LCH.

Monostotic Langerhans' Cell Histiocytosis in a Child with Central Diabetes Insipidus

2012 ◽  
Vol 36 (4) ◽  
pp. 377-381 ◽  
Author(s):  
E Costa Studart Soares ◽  
ARP Quidute ◽  
FW Gurgel Costa ◽  
MH Costa Gurgel ◽  
AP Negreiros Nunes Alves ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Reticuloendothelial System ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Important Indicator ◽  
Presenting Symptoms ◽  
Dental Practitioners ◽  
Multi Agent ◽  
Underlying Diseases

Langerhans' cell histiocytosis (LCH) comprises a rare group of reticuloendothelial system disorders that can produce focal or systemic manifestations. Diabetes insipidus is considered to be an important indicator of serious underlying diseases in children, including LCH. We report the case of a young patient with monostotic LCH confined to the mandibular ramus, who was diagnosed with the disease after presenting symptoms of central diabetes insipidus and was satisfactorily treated with multi-agent chemotherapy. Additionally, we discuss the clinical, radiographic, histological and immunohistochemical findings, as well as the multidisciplinary approach of this important disease, which should receive attention by dental practitioners, especially when it occurs in children.

scholarly journals Topical Imiquimod for the Treatment of Relapsed Cutaneous Langerhans Cell Histiocytosis after Chemotherapy in an Elderly Patient

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Shinsaku Imashuku ◽  
Miyako Kobayashi ◽  
Yoichi Nishii ◽  
Keisuke Nishimura
Keyword(s):  
Elderly Patient ◽  
Elderly Patients ◽  
Diabetes Insipidus ◽  
Cytosine Arabinoside ◽  
Langerhans Cell Histiocytosis ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Diagnosis And Treatment ◽  
Topical Imiquimod ◽  
Multiagent Chemotherapy

Diagnosis and treatment of Langerhans cell histiocytosis (LCH) in elderly patients are often difficult. We report here a 61-year-old female suffering from a refractory axillary ulcer for nearly a year, whose biopsy revealed LCH. It was also noted that the patient had other cutaneous papulovesicular eruptions of LCH as well as central diabetes insipidus. The patient was first successfully treated with multiagent chemotherapy (cytosine arabinoside/vinblastine/prednisolone). DDAVP also well controlled diabetes insipidus; however, the axillary ulcer and cutaneous LCH relapsed. Thereafter, we found topical imiquimod to be effective in the treatment of relapsed cutaneous LCH lesions.

scholarly journals Langerhans Cell Histiocytosis with Isolated Central Diabetes Insipidus, Low Grade Fever and Sellar Erosion

2021 ◽  
Author(s):  
İclal Okur ◽  
Hasan Ari ◽  
Semra Çetinkaya ◽  
Betül Emine Derinkuyu ◽  
Gizem Çağlar ◽  
...  
Keyword(s):  
Diabetes Insipidus ◽  
Langerhans Cell Histiocytosis ◽  
Bone Structure ◽  
Sella Turcica ◽  
Langerhans Cell ◽  
Central Diabetes Insipidus ◽  
Bone Lesions ◽  
Warning Sign ◽  
Low Grade ◽  
First Case

Langerhans cell histiocytosis (LCH) is a rare disease of the monocyte-macrophage system. Although it is known that bone involvement is seen very frequently in cases with LCH, our case is the first case with a lytic-destructive lesion in the bone structure forming sella turcica. A 4-year-old, 5-month-old male patient who applied to our outpatient clinic was diagnosed with Langerhans cell histiocytosis in further examination after the diagnosis of central diabetes insipidus (CDI) was made. On cranial magnetic resonance imaging (MRI), widespread lytic-destructive bone lesions were observed in the bone structure forming the sella (sphenoid bone), sellar destruction not previously described in the literature. Sellar erosion has not been reported before in cases diagnosed with LCH in the literature. The presence of low-grade fever in a patient presenting with isolated CDI is a warning sign for the diagnosis of LCH.

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