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1.
Medicine (Baltimore) ; 99(3): e18794, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32011480

RESUMO

RATIONALE: Spinal involvement in adult Langerhans cell histiocytosis (LCH) is rare, and epidural involvement is unusual. LCH is mostly indistinguishable from other spinal lesions such as infection, lymphoma, and metastasis. So, it could be easily misdiagnosed without suspicion. PATIENT CONCERNS: We report a case of a 33-year-old man who complained of gait disturbance with weakness in both legs and severe back pain. DIAGNOSES: A continuous enhancing epidural lesion with cord compression from the T7 to L1 level was detected in magnetic resonance imaging. Laboratory analysis indicated the possibility of spinal infectious disease. We assumed that the lesion could be tuberculous spondylitis. INTERVENTIONS AND OUTCOMES: The patient underwent posterior laminectomy with marginal excision of the epidural mass to relieve cord compression. Pathological examination confirmed the diagnosis of LCH. The 12-month follow-up evaluation revealed that the patient was neurologically intact and had no gait disturbance. LESSONS: This case report presents a patient with epidural LCH of the thoracic spinal cord, which can mimic spinal infections such as tuberculous spondylitis with abscess formation. Therefore, LCH could be considered as a possible diagnosis when a patient presents with features of infectious spondylitis with vertebral involvement.


Assuntos
Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/diagnóstico , Compressão da Medula Espinal/diagnóstico , Compressão da Medula Espinal/etiologia , Adulto , Diagnóstico Diferencial , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/cirurgia , Humanos , Masculino , Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/patologia , Compressão da Medula Espinal/cirurgia , Vértebras Torácicas
2.
J Pediatr Hematol Oncol ; 42(3): 222-227, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-30855315

RESUMO

We report 11 children with vertebral lesion of Langerhans cell histiocytosis (LCH) diagnosed and treated between 2000 and 2015. Vertebral lesions were usually present at LCH diagnosis. No child developed neurologic symptoms. Among 29 vertebral lesions, only 2 were unstable. Chemotherapy was used in all children but 3. A LCH recurrence was observed in 6 patients, involving vertebrae in 4 cases. All children were disease-free at their last follow-up. Sequelae were more often radiologic than clinical. Since potential recurrences and incomplete bone regeneration exist, discussion about optimal treatment and long-term follow-up of vertebral lesions are essential.


Assuntos
Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/patologia , Doenças da Coluna Vertebral/etiologia , Doenças da Coluna Vertebral/patologia , Adolescente , Canadá , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos
4.
BMC Endocr Disord ; 19(1): 143, 2019 Dec 19.
Artigo em Inglês | MEDLINE | ID: mdl-31856773

RESUMO

BACKGROUND: Langerhans cell histiocytosis (LCH) is a rare disease that mainly affects children, but this disease is significantly rarer in patients who are older than 15 years. In this disease, any organ can be involved. The skeleton, skin and lung are commonly affected, and isolated hypothalamic-pituitary (HP) involvement is relatively rare. Here we report a 17-year-old adolescent with isolated HP-LCH of enlarged pituitary stalk presented with central diabetes insipidus (CDI). CASE PRESENTATION: A 17-year-old male adolescent with polydipsia and polyuria accompanied with elevated serum sodium level and low urine osmolality for 3 weeks was referred to our hospital. After admission, hormonal evaluation showed that his growth hormone (GH) was slightly elevated, and serum osmolality and glucose were normal. The fluid deprivation-vasopressin test demonstrated CDI. Imaging examination showed an obvious thickening of the pituitary stalk. Lymphocytic hypophysitis, sarcoidosis and granulation tissue lesions were suspected. After oral 1-deamino-8-Darginine vasopressin (DDAVP) and prednisone were administered for 2 months, symptoms were relieved, and he discontinued taking the drugs by himself. On reexamination, imaging revealed changes in the size and shape of the pituitary stalk, with thickened nodules. Then, a diagnostic biopsy of the pituitary stalk lesion was performed. Immunohistochemistry confirmed the definitive diagnosis of LCH. The clinical symptoms subsided with oral hormone replacements. CONCLUSION: CDI is a rare symptom in children and adolescents. Most of the causes are idiopathic, while others are caused by central nervous system (CNS) disorders. Meanwhile, lymphocytic hypophysitis, germinoma, LCH and other CNS disorders can all present as thickening of the pituitary stalk, diffuse enlargement of the pituitary gland, and weakening of high signal intensity in the neurohypophysis on magnetic resonance imaging (MRI). The differential diagnosis among these diseases depends on immunohistochemistry evidence.


Assuntos
Diabetes Insípido Neurogênico/etiologia , Histiocitose de Células de Langerhans/complicações , Doenças Hipotalâmicas/complicações , Doenças da Hipófise/complicações , Adolescente , Hipofisite Autoimune/complicações , Hipofisite Autoimune/tratamento farmacológico , Hipofisite Autoimune/patologia , Desamino Arginina Vasopressina/uso terapêutico , Diabetes Insípido Neurogênico/tratamento farmacológico , Diabetes Insípido Neurogênico/patologia , Histiocitose de Células de Langerhans/tratamento farmacológico , Histiocitose de Células de Langerhans/patologia , Humanos , Doenças Hipotalâmicas/tratamento farmacológico , Doenças Hipotalâmicas/patologia , Masculino , Doenças da Hipófise/tratamento farmacológico , Doenças da Hipófise/patologia , Prednisona/uso terapêutico
7.
Orphanet J Rare Dis ; 14(1): 229, 2019 10 21.
Artigo em Inglês | MEDLINE | ID: mdl-31639032

RESUMO

BACKGROUND: Pneumothorax may recur during pulmonary Langerhans cell histiocytosis (PLCH) patients' follow-up and its management is not standardised. The factors associated with pneumothorax recurrence are unknown. METHODS: In this retrospective study, PLCH patients who experienced a pneumothorax and were followed for at least 6 months after the first episode were eligible. The objectives were to describe the treatment of the initial episode and pneumothorax recurrences during follow-up. We also searched for factors associated with pneumothorax recurrence and evaluated the effect on lung function outcome. Time to recurrence was estimated by the Kaplan Meier method and the cumulative hazard of recurrence handling all recurrent events was estimated. Univariate Cox models and Andersen-Gill counting process were used for statistical analyses. RESULTS: Fourty-three patients (median age 26.5 years [interquartile range (IQR), 22.9-35.4]; 26 men, 39 current smokers) were included and followed for median time of 49 months. Chest tube drainage was the main management of the initial pneumothorax, which resolved in 70% of cases. Pneumothorax recurred in 23 (53%) patients, and overall 96 pneumothoraces were observed during the study period. In the subgroup of patients who experienced pneumothorax recurrence, the median number of episodes per patient was 3 [IQR, 2-4]. All but one recurrence occurred within 2 years after the first episode. Thoracic surgery neither delayed the time of occurrence of the first ipsilateral recurrence nor reduced the overall number of recurrences during the study period, although the rate of recurrence was lower after thoracotomy than following video-assisted thoracic surgery (p = 0.03). At the time of the first pneumothorax, the presence of air trapping on lung function testing was associated with increased risk of recurrence (hazard ratio = 5.08; 95% confidence interval [1.18, 21.8]; p = 0.03). Pneumothorax recurrence did not predict subsequent lung function decline (p = 0.058). CONCLUSIONS: Our results show that pneumothorax recurrences occur during an "active" phase of PLCH. In this observational study, the time of occurrence of the first ipsilateral recurrence and the overall number of pneumothorax recurrences were similar after conservative and thoracic surgical treatments. Further studies are needed to determine the best management to reduce the risk of pneumothorax recurrence in PLCH patients.


Assuntos
Histiocitose de Células de Langerhans/complicações , Pneumotórax/etiologia , Adulto , Feminino , Humanos , Masculino , Pneumotórax/cirurgia , Recidiva , Estudos Retrospectivos , Adulto Jovem
8.
Gulf J Oncolog ; 1(31): 72-77, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31591994

RESUMO

Langerhans cell histiocytosis (LCH) is a rare neoplasm that is caused by an uncontrolled proliferation of Langerhans cells. The clinical presentation of LCH is heterogeneous and can manifest as single or multiple osteolytic lesions, skin ulcerations, and involvement of single or multiple systems. Central nervous system (CNS) involvement is reported in 3.4-57% of patients with multisystem disease. In this article, we present the case of a young man with single system involvement (bone) of LCH who presented with seizures, headache, papilledema, and tinnitus. His magnetic resonance imaging (MRI) of the brain findings were reported as a normal study. The subtle signs of CNS involvement were missed by the radiologist. However, the high index of suspicion resulted in early diagnosis and treatment. The presence of empty sella turcica in neuroimaging could be the first sign of intracranial disease with chronic intracranial hypertension associated with LCH. This is especially correct if previous computed tomography (CT) scan of the brain was normal with normal appearance of the pituitary gland and the sella. Neuroimaging films should be reviewed by an expert neuroradiologist. In patients with new neurological symptoms who were diagnosed previously with LCH, intracranial disease has to be excluded. The workup in such case should include an MRI of the brain, CT of the brain and temporal bones, bone scan, cerebrospinal fluid analysis, ophthalmological assessment, and measurement of intracranial pressure. In patients with LCH who present with symptoms and signs of raised intracranial pressure, the term idiopathic intracranial hypertension should not be applied until an intracranial disease has been excluded totally. Keywords: Langerhans Cell Histiocytosis; Central Nervous System Involvement; Neuroimaging; Intracranial Hypertension.


Assuntos
Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/radioterapia , Neuroimagem/métodos , Adulto , Histiocitose de Células de Langerhans/patologia , Humanos , Masculino
10.
World J Pediatr ; 15(6): 536-545, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31456157

RESUMO

BACKGROUND: Langerhans cell histiocytosis (LCH) is a group of diseases characterized by the proliferation and accumulation of Langerhans cells. Clinical presentations of LCH vary widely. DATA SOURCES: A PubMed search was conducted using Clinical Queries with the key term "Langerhans cell histiocytosis". The search strategy included meta-analyses, randomized controlled trials, clinical trials, observational studies, and reviews. This paper is based on, but not limited to, the search results. RESULTS: Generally, patients with LCH can be divided into two groups based on the extent of involvement at diagnosis, namely, single-system LCH and multisystem LCH. The involvement may be unifocal or multifocal. Patients with isolated bone lesions typically present between 5 and 15 years of age, whereas those with multisystem LCH tend to present before 5 years of age. The clinical spectrum is broad, ranging from an asymptomatic isolated skin or bone lesion to a life-threatening multisystem condition. Clinical manifestations include, among others, "punched out" lytic bone lesion, seborrheic dermatitis-like eruption, erythematous/reddish-brown crusted/scaly papules/maculopapules/plaques/patches, and eczematous lesions, diabetes insipidus, hepatosplenomegaly, cytopenias, lymphadenopathy, and an acute fulminant disseminated multisystem condition presenting with fever, skin rash, anemia, thrombocytopenia, lymphadenopathy, and hepatosplenomegaly. The diagnosis is clinicopathologic, based on typical clinical findings and histologic/immunohistochemical examination of a biopsy of lesional tissue. Positive CD1a, S100, and/or CD207 (Langerin) immunohistochemical staining of lesional cells is required for a definitive diagnosis. Watchful waiting is recommended for patients with skin-only LCH. Patients with symptomatic or refractory skin-only LCH may be treated with topical tacrolimus/corticosteroids, topical nitrogen mustard, oral methotrexate, or oral hydroxyurea. The current recommended first-line therapy for patients with multisystem LCH is 12 months therapy with prednisone and vinblastine. Mercaptopurine is added for patients with risk organ involvements. CONCLUSIONS: Because of the broad spectrum of clinical manifestations and the extreme diversity of disease, LCH remains a diagnostic dilemma. Morphological identification of LCH cells and positive immunochemical staining with CD1a, S100, and/or CD207 (Langerin) of lesional cells are necessary for a definitive diagnosis.


Assuntos
Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/diagnóstico , Adolescente , Criança , Pré-Escolar , Humanos
11.
JBJS Case Connect ; 9(3): e0159, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31390335

RESUMO

CASE: This report describes a 7-year-old girl with Langerhans cell histiocytosis (LCH) of the thoracic spine who developed neurological deterioration during nonsurgical management. She was treated with decompression and instrumented fusion followed by chemotherapy, recovered completely after surgery, and was doing well at 6-year follow-up. CONCLUSIONS: The best treatment of LCH of the spine is not clear, but in the setting of neurologic compromise after failing conservative management, surgical decompression with adjuvant chemotherapy should be considered.


Assuntos
Histiocitose de Células de Langerhans/complicações , Doenças da Coluna Vertebral/complicações , Fusão Vertebral , Vértebras Torácicas/cirurgia , Quimioterapia Adjuvante , Criança , Descompressão Cirúrgica , Feminino , Histiocitose de Células de Langerhans/diagnóstico por imagem , Histiocitose de Células de Langerhans/tratamento farmacológico , Histiocitose de Células de Langerhans/cirurgia , Humanos , Doenças da Coluna Vertebral/diagnóstico por imagem , Doenças da Coluna Vertebral/tratamento farmacológico , Doenças da Coluna Vertebral/cirurgia , Vértebras Torácicas/diagnóstico por imagem
13.
Int J Hematol ; 110(6): 756-762, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31350665

RESUMO

Gastrointestinal (GI) tract involvement in Langerhans cell histiocytosis (LCH) is extremely rare. Langerhans cell histiocytosis with GI tract involvement (GI-LCH) is frequently associated with multi-system disease, and usually presents with severe systemic symptoms, such as protein-losing enteropathy (PLE). Although the GI tract is not included among the organs at risk, the prognosis of GI-LCH is poor, and no effective chemotherapeutic regimen has been identified. Here, we report an infant case of primary refractory GI-LCH with PLE that showed marked improvement in response to 2-chlorodeoxyadenosine (2-CdA) therapy with no severe adverse events, even under conditions of deteriorating general health. The present findings indicate that 2-CdA may be effective for refractory GI-LCH with PLE. Further studies are warranted to determine the optimal therapeutic strategies for GI-LCH with PLE.


Assuntos
Cladribina/uso terapêutico , Trato Gastrointestinal/patologia , Histiocitose de Células de Langerhans/tratamento farmacológico , Terapia de Salvação/métodos , Histiocitose de Células de Langerhans/complicações , Histiocitose de Células de Langerhans/patologia , Humanos , Lactente , Pediatria , Enteropatias Perdedoras de Proteínas/tratamento farmacológico , Enteropatias Perdedoras de Proteínas/etiologia , Resultado do Tratamento
14.
Ophthalmic Plast Reconstr Surg ; 35(4): e92-e94, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31219941

RESUMO

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type cells. Orbital LCH is infrequent, typically manifesting as an isolated lytic bony lesion with an adjacent soft tissue mass in a child. Isolated lacrimal gland involvement by LCH is extremely rare, with only 2 previously reported cases. The authors describe a 37-year-old woman with a 6-month history of painless right upper eyelid swelling and diffuse right lacrimal gland enlargement without bony changes on computed tomography scan. Excisional biopsy of the lacrimal gland demonstrated concurrent LCH, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, and increased IgG4-expressing plasma cells. Work-up was negative for systemic hematolymphoid malignancy and IgG4-related disease. This case illustrates the association between LCH, mucosa-associated lymphoid tissue lymphoma, and elevated IgG4 plasma cells in the lacrimal gland, and we review the emerging theories proposed to explain this phenomenon.


Assuntos
Histiocitose de Células de Langerhans/complicações , Imunoglobulina G/imunologia , Doenças do Aparelho Lacrimal/complicações , Aparelho Lacrimal/diagnóstico por imagem , Linfoma de Zona Marginal Tipo Células B/complicações , Plasmócitos/imunologia , Adulto , Biópsia , Feminino , Histiocitose de Células de Langerhans/diagnóstico , Histiocitose de Células de Langerhans/imunologia , Humanos , Imunoglobulina G/metabolismo , Doenças do Aparelho Lacrimal/diagnóstico , Doenças do Aparelho Lacrimal/imunologia , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/imunologia , Plasmócitos/patologia , Tomografia Computadorizada por Raios X
20.
An. pediatr. (2003. Ed. impr.) ; 90(5): 293-300, mayo 2019. tab, ilus
Artigo em Espanhol | IBECS | ID: ibc-186661

RESUMO

Introducción: La diabetes insípida central (DIC) es una entidad poco frecuente en la edad pediátrica, siendo su etiología heterogénea. El objetivo de nuestro trabajo es demostrar que el seguimiento clínico y neurorradiológico de la región hipotálamo-hipofisaria, puede ayudar a establecer el diagnóstico etiológico de DIC y la presencia de otros déficits hormonales. Métodos: Se revisaron de forma retrospectiva 15 pacientes diagnosticados de DIC en un hospital pediátrico. Se analizaron las características clínicas y auxológicas; así como la valoración de la función adenohipofisaria junto con RM craneal de manera periódica. Resultados: La mediana de edad al diagnóstico fue de 9,6 años (rango: 1,3-15,9). El diagnóstico etiológico pudo establecerse en 9 de los 15 pacientes (germinomas: 7 e histiocitosis: 2). Tras una mediana de seguimiento de 5,5 años (rango: 1,6-11,8), los casos idiopáticos se redujeron a la mitad. Finalmente, los diagnósticos etiológicos fueron: germinoma 9 (60%), histiocitosis 3 (20%) y DIC idiopática 3 (20%). Existe una asociación estadísticamente significativa entre el engrosamiento del tallo y la etiología tumoral. El 67% desarrolló, al menos, una deficiencia hormonal adenohipofisaria, la mayoría en los dos primeros años de seguimiento. El déficit más prevalente fue el de hormona de crecimiento (60%). Conclusiones: En todos los pacientes con DIC se deberá realizar un control auxológico y hormonal, con especial atención, por su frecuencia, a la deficiencia de GH, y en aquellos con DIC idiopática se debería incluir una RM semestral, al menos durante los 2-3 primeros años después del diagnóstico, pues en nuestro estudio el 50% fueron diagnosticados de germinomas o histiocitosis en este periodo


Background: Central diabetes insipidus (CDI) is a rare disorder in children. The aetiology of CDI in childhood is heterogeneous. The aim of this study is to illustrate the importance of a careful clinical and neuro-radiological follow-up of the pituitary and hypothalamus region in order to identify the aetiology and the development of associated hormonal deficiencies. Methods: Clinical and auxological variables of 15 children diagnosed with CDI were retrospectively analysed in a paediatric hospital. Evaluations of adenohypophyseal function and cranial MRI were performed periodically. Results: The mean age at diagnosis of CDI was 9.6 years (range: 1.32-15.9). The aetiological diagnosis could be established initially in 9 of the 15 patients, as 7 with a germinoma and 2 with a histiocytosis. After a mean follow-up of 5.5 years (range: 1.6-11.8), the number of idiopathic cases was reduced by half. At the end of the follow-up, the aetiological diagnoses were: 9 germinoma (60%), 3 histiocytosis (20%), and 3 idiopathic CDI (20%). There is a statistically significant association between stalk thickening and tumour aetiology. At least one adenohypophyseal hormonal deficiency was found in 67% of cases, with the majority developing in the first two years of follow-up. Growth hormone deficiency (60%) was the most prevalent. Conclusion: The follow-up of CDI should include hormone evaluation with special attention, due to its frequency, to GH deficiency. In addition, a biannual MRI in an idiopathic CDI should be performed, at least during the first 2-3 years after diagnosis, as 50% of them were diagnosed with a germinoma or histiocytosis during this period


Assuntos
Humanos , Masculino , Feminino , Lactente , Pré-Escolar , Criança , Adolescente , Diabetes Insípido Neurogênico/fisiopatologia , Germinoma/complicações , Histiocitose de Células de Langerhans/complicações , Hipófise/patologia , Seguimentos , Germinoma/epidemiologia , Histiocitose de Células de Langerhans/epidemiologia , Hormônio do Crescimento Humano/deficiência , Imagem por Ressonância Magnética , Estudos Retrospectivos
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