a Unidad Médico-Quirúrgica de Enfermedades Respiratorias. Área de Neumopatías Intersticiales y Ocupacionales. Hospital Universitario Virgen del Rocío. OSSES A, RODRIGO; GONZALEZ B, SERGIO; AGUIRRE Z, MARCIA SALDIAS P, FERNANDO. Histiocitosis de células de Langerhans pulmonar: Caso clínico. La histiocitosis de células de Langerhans es una enfermedad . Tras plantearse el caso como histiocitosis hepático y/o pulmonar).4 Su presentación.
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Langerhans cell histiocytosis LCH is a rare disease involving clonal proliferation of Langerhans cellsabnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Clinically, its manifestations range from isolated bone lesions to multisystem disease.
LCH is part of a group of clinical syndromes called histiocytoseswhich are histioditosis by an abnormal proliferation of histiocytes an archaic term for activated dendritic cells and macrophages.
These diseases are related to other forms of abnormal histiocitosls of white blood cellssuch as leukemias and lymphomas. The disease spectrum results from clonal accumulation and proliferation of cells resembling the epidermal dendritic cells called Langerhans cellssometimes called dendritic cell histiocytosis.
These cells in combination with lymphocyteseosinophilsand normal histiocytes form typical LCH lesions that can be found in almost any organ. LCH is clinically divided into three groups: Unifocal LCH, also called eosinophilic granuloma an older term which is now known to be a misnomeris a slowly progressing disease characterized by an expanding proliferation of Langerhans cells in various bones.
It can be a monostotic involving only one bone or polyostotic involving more than one bone disease. It typically has no extraskeletal involvement, but rarely an identical lesion can be found in the skin, lungs, or stomach. When found in the lungs, it should be distinguished from Pulmonary Langerhans cell hystiocytosis—a special category of disease most pulomnar seen in adult smokers.
Histiocitosis de células de Langerhans pulmonar: Caso clínico
Seen mostly in children, multifocal unisystem LCH is characterized by fever, bone lesions and diffuse eruptions, usually on the scalp and in the ear canals. Peak onset histiocitossi 2—10 years of age. Multifocal multisystem LCH, also called Letterer-Siwe diseaseis a rapidly progressing disease in which Langerhans Cell cells proliferate histiocirosis many tissues.
It is mostly seen in children under age 2, and the prognosis is poor: It is now considered a form of smoking-related interstitial lung disease. Some affected people recover completely after they stop smoking, but others develop long-term complications such as pulmonary fibrosis and histiocitosjs hypertension. This is a privacy protected site that provides up-to-date information for individuals interested in the latest scientific news, trials, and treatments related to rare lung diseases.
LCH provokes a non-specific inflammatory responsewhich includes fever, lethargyand weight loss.
Organ involvement can also cause more specific symptoms. The pathogenesis of Langerhans cell histiocytosis LCH is a matter of debate. There are ongoing investigations to determine whether LCH is a reactive non-cancerous or neoplastic cancerous process.
Arguments supporting the reactive nature of LCH include the occurrence of spontaneous remissions, the extensive secretion of multiple cytokines by dendritic cells and bystander-cells a phenomenon known as cytokine storm in the lesional tissue, favorable prognosis and relatively good survival rate in patients without organ dysfunction or risk organ involvement. On the other hand, the infiltration of organs by monoclonal population of pathologic cells, and the successful treatment of subset of disseminated disease using chemotherapeutic regimens are all consistent with a neoplastic process.
Recurrent cytogenetic or genomic abnormalities would also be required to demonstrate convincingly that LCH is a malignancy. Two independent studies have confirmed this finding.
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Diagnosis is confirmed histologically by tissue biopsy. Hematoxylin-eosin stain of biopsy slide will show features of Langerhans Cell e. Presence of Birbeck granules on electron microscopy and immuno-cytochemical features e. CD1 positivity are more specific. Initially routine blood tests e. Radiology will show osteolytic bone lesions and damage to the lung. The latter may be evident in chest X-rays with micronodular and interstitial infiltrate in the mid and lower zone of lung, with sparing of the Costophrenic angle or honeycomb appearance in older lesions.
MRI and CT may show infiltration in sella turcica. Assessment of endocrine function and bonemarrow biopsy are also performed when indicated. Guidelines for management of patients up to 18 years with Langerhans cell histiocytosis have been pumonar. Solitary bone lesion may be amenable through excision or limited radiation, dosage of Gy for children, Gy for adults. Hiatiocitosis systemic diseases often require chemotherapy.
Use of systemic steroid is common, singly or adjunct to chemotherapy. Local steroid cream is applied to skin lesions.
Endocrine deficiency often require lifelong supplement e. Chemotherapeutic agents such as alkylating agentsantimetabolitesvinca alkaloids either singly or in combination can lead to complete remission in diffuse disease.
Excellent for single-focus disease. LCH usually affects children between 1 and 15 years old, with a peak incidence between 5 and 10 years of age. Among children under the age of 10, yearly incidence is thought to be 1 in ,;  and in adults even rarer, in about 1 inLCH is usually a sporadic and non- hereditary condition but familial clustering has been noted in limited number of cases. In the 10th episode of season 3 of House entitled “Merry Little Christmas”the primary patient is a girl with dwarfism who has a variety of symptoms, who is ultimately diagnosed with Langerhans cell histiocytosis.
Also in the 5 series of the series Good doctor Dr. Murphy tried to diagnose Langerhans cell histiocytosis in a boy with a previously diagnosed osteosarcoma. Langerhans cell histiocytosis is occasionally misspelled as “Langerhan” or “Langerhan’s” cell histiocytosis, even in authoritative textbooks.
The name, however, originates back to its discoverer, Paul Langerhans. From Wikipedia, the free encyclopedia. Langerhans cell histiocytosis Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells accompanied by abundant eosinophils.
Specialty Hematology Langerhans cell histiocytosis LCH is a rare disease involving clonal proliferation of Langerhans cellsabnormal cells deriving from bone marrow and capable of migrating from skin to lymph nodes. Writing Group of the Histiocyte Society”. Robbins and Cotran pathologic basis of disease. Robbins and Cotran Pathologic Basis of Disease 9th ed.
In Kliegman, Robert M. Nelson Textbook of Pediatrics 19th ed. The Journal of Clinical Endocrinology and Metabolism. Medical and Pediatric Oncology. The American Journal of Surgical Pathology. Int J Clin Exp Pathol. British Journal of Haematology. British Journal of Dermatology. Gary 21 July New England Journal of Medicine. The Journal of Pathology. The British Journal of Dermatology. S protein, peanut agglutinin, and transmission electron microscopy study”.
American Journal of Clinical Pathology. Guidelines for diagnosis, clinical work-up, and treatment for patients till the age of 18 years”. Orphanet Journal of Rare Diseases. Archived from the original on Der Hautarzt in German. International Journal of Pediatric Otorhinolaryngology.
Langerhans cell histiocytosis – Wikipedia
Report from the International Registry of the Histiocyte Society”. European Journal of Cancer. Robin; Hoang, Mai P. Ten-year experience at Dallas Children’s Medical Center”. Journal uplmonar the American Academy of Dermatology. Journal of Clinical Pathology. Juvenile pulmonqr Hemophagocytic lymphohistiocytosis Erdheim-Chester disease Niemann—Pick disease Sea-blue histiocyte Benign cephalic histiocytosis Generalized eruptive histiocytoma Xanthoma disseminatum Progressive nodular histiocytosis Papular xanthoma Hereditary progressive mucinous histiocytosis Reticulohistiocytosis Multicentric reticulohistiocytosisReticulohistiocytoma Indeterminate cell histiocytosis.
Histiocytic sarcoma Langerhans cell sarcoma Interdigitating dendritic cell sarcoma Follicular dendritic cell sarcoma. Retrieved from ” https: Histiocytosis Monocyte- and macrophage-related cutaneous conditions Rare pulmonae.
Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells pullmonar by abundant eosinophils. D ICD – Wikimedia Commons has media related to Langerhans Cell Histiocytosis.