Histiocitosis de células de Langerhans. Avances en la patogenia y práctica clínica

Astigarraga, Itziar; García-Obregón, Susana; Pérez‐Martínez, Antonio; Gutiérrez-Carrasco, Ignacio; Santa-María, Vicente; Iturrate, Carmen Rodríguez-Vigil; Reggiori, Mikael Lorite; Carrillo, Thais Murciano; Torrent, Montse

doi:10.1016/j.anpedi.2022.05.002

Cited by 4 publications

(5 citation statements)

References 39 publications

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“…The LCH patients with the BRAF V600E mutation had higher serum levels of CCL7. These cytokines can promote the recruitment and activation of immune cells, contributing to the formation of granulomas [12,15,23]. Overall, LCH is a complex disease with multiple potential factors contributing to its development.…”

Section: Discussionmentioning

confidence: 99%

“…participate in therapy and the potential for late cutaneous malignancy limit its use [10,15,25]. Oral treatment options include methotrexate and thalidomide, both of which have been used to treat severe or recalcitrant LCH [26,27].…”

Section: Discussionmentioning

confidence: 99%

“…Oral treatment options include methotrexate and thalidomide, both of which have been used to treat severe or recalcitrant LCH [26,27]. For more extensive or severe cases of LCH, chemotherapy agents like vinblastine may be used [15]. These drugs target and kill the abnormal cells responsible for the disease.…”

Section: Discussionmentioning

confidence: 99%

“…This involves replacing the patient's bone marrow with healthy stem cells from a donor. Medications that modulate the immune system, such as interferon-a, may be prescribed to control the disease and reduce its impact on affected organs [15,20]. In certain instances, surgical excision of isolated skin lesions may be considered, especially if they are causing significant symptoms or if there is concern about malignancy.…”

Section: Discussionmentioning

confidence: 99%

“…Skin lesions are common in LCH and affect about 40% of cases, although exclusive cutaneous involvement is rare (5%) [15]. It is reported that skin lesions are usually the first manifestation of LCH in 80% of patients [16].…”

mentioning

confidence: 99%

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Diagnostic and therapeutic difficulties in the cutaneous form of Langerhans cell histiocytosis in infant

Arasiewicz,

Dec,

Lesniak-Jakubiec

2023

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Introduction: Langerhans cell histiocytosis is an uncommon inflammatory and neoplastic condition of myeloid dendritic cells. Langerhans cell histiocytosis presents with a wide spectrum of clinical manifestations, ranging from single-system involvement to disseminated disease involving multiple organs. Diagnosis is based on a combination of clinical features, radiological findings, and histopathological examination. Positive immunohistochemical staining of lesional cells for CD1a and S100 is necessary for definitive diagnosis of Langerhans cell histiocytosis. Case report: Herein, we describe the case of a male infant who presented with multiple erythematous-papular lesions covered with yellow scales on the scalp, temples and face and oozing erythematous and erosive lesions mainly on the abdomen, upper chest, groins, gluteal cleft and armpits. Conclusions: Further research is needed to unravel the underlying pathogenesis, develop standardized diagnostic criteria, and improve treatment strategies. Collaboration among various specialties, including dermatology, pediatrics, oncology, radiology, and pathology, is essential for optimal management and long-term follow-up of this complex entity.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Diagnostic and therapeutic difficulties in the cutaneous form of Langerhans cell histiocytosis in infant

Arasiewicz,

Dec,

Lesniak-Jakubiec

2023

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An extremely rare case of Langerhans cell hyperplasia in the thymus

Xu,

Tian,

Feng

et al. 2024

Pulmonology

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A challenging diagnosis of Langerhans’ cell histiocytosis with hypothalamic-pituitary and mandibular involvement: case report and literature review

Ahmad,

Ktyman

et al. 2024

Annals of Medicine &Amp; Surgery

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Background: Langerhans cell histiocytosis (LCH) is a rare bone marrow derived neoplasm that mainly affects children. It is a multi-organ disorder and hypothalamic-pituitary involvement is uncommon. LCH reveals a wide spectrum of indications; thus, the diagnosis and treatment are usually challenging. Case report: A 22-year-old male presented with polydipsia, polyuria with non-specific radiological findings, later on, developed a mandibular lesion and a biopsy was conducted which led to LCH diagnosis. After many improper treatments due to unclear diagnosis, the patient was finally placed on chemotherapy and is now under surveillance. Discussion: Langerhans cell histiocytosis (LCH) is a rare disease with diverse clinical manifestations affecting various organs. Associated mutations, such as BRAF V600E, contribute to its complexity. In adults, initial symptoms include pain, weight loss, and fever, with potential pituitary involvement leading to Arginine vasopressin (AVP) deficiency. Commonly affected organs include bone, skin, and the pituitary gland. The disease can be categorized into single-system and multisystem. Pathological diagnosis involves electron microscopy or immunohistochemical staining. Treatment options vary; the presented case utilized Desmopressin acetate and prednisolone before transitioning to cyclophosphamide for multi-systemic LCH. Conclusion: AVP deficiency can suggest hypothalamic-pituitary LCH, and a biopsy, if possible, is recommended to confirm the diagnosis.

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Histiocitosis de células de Langerhans. Avances en la patogenia y práctica clínica

Cited by 4 publications

References 39 publications

Diagnostic and therapeutic difficulties in the cutaneous form of Langerhans cell histiocytosis in infant

Diagnostic and therapeutic difficulties in the cutaneous form of Langerhans cell histiocytosis in infant

An extremely rare case of Langerhans cell hyperplasia in the thymus

A challenging diagnosis of Langerhans’ cell histiocytosis with hypothalamic-pituitary and mandibular involvement: case report and literature review

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