On Jan 1, , Lina Parra and others published Sindrome de Histiocitosis } El diagnóstico hematológico y anatomopatológico fue histiocitosis maligna. Roa, I., Araya, J. C., Soza, D., & Thomas, K. (). Histiocitosis maligna en el niño. Revista Chilena de Pediatria, 60(2), Histiocitosis maligna en el niño. La Histiocitosis maligna (también conocida como “reticulosis medular histiocitica” ​) es una rara enfermedad genética encontrada en los boyeros de Berna.

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Langerhans cell histiocytosis LCH is a rare disease involving clonal proliferation of Langerhans cellsabnormal cells deriving maliggna 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 characterized by an abnormal proliferation of histiocytes an archaic term for activated dendritic cells and macrophages. These diseases mlaigna related to other forms of abnormal proliferation 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.

Msligna found in the lungs, it should be distinguished from Pulmonary Langerhans cell hystiocytosis—a special category of disease most commonly seen in adult smokers. Seen mostly in children, multifocal unisystem LCH is characterized by fever, bone lesions and diffuse histicitosis, usually on the scalp and in the ear canals. Peak onset is 2—10 years of age. Multifocal multisystem LCH, also called Letterer-Siwe diseaseis a rapidly progressing disease in which Langerhans Cell cells proliferate in 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 pulmonary 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 histiocitosie 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. 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 malignz 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 suggested. Solitary bone lesion may be amenable through excision or limited radiation, dosage of Gy for children, Gy for adults.

However systemic diseases often require chemotherapy. Use of systemic steroid is common, singly or adjunct to chemotherapy. Local steroid histioxitosis is applied to skin lesions.

Conectivas lógicas

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 hisriocitosis.

Excellent for single-focus disease. LCH usually affects children between 1 and 15 years old, with a hkstiocitosis incidence between 5 and 10 years of age. Among children under the age of 10, yearly incidence is thought maliyna be 1 in ,; [35] 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. Histiocutosis name, however, originates back to its discoverer, Paul Langerhans. From Wikipedia, the free encyclopedia.


Langerhans cell histiocytosis – Wikipedia

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.

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Histiocitosis maligna – Spanish-English Dictionary – Glosbe

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Archived from the original on Der Hautarzt in German. International Journal of Pediatric Otorhinolaryngology. 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 of the American Academy of Dermatology. Journal of Clinical Pathology. Juvenile xanthogranuloma 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 histikcitosis cell sarcoma Follicular dendritic cell sarcoma. Retrieved from ” https: Histiocytosis Monocyte- and macrophage-related cutaneous conditions Rare diseases. CS1 German-language sources de Infobox medical condition new All articles with unsourced statements Articles with unsourced statements from April Commons category link is locally defined.

Views Read Edit View history. In other projects Wikimedia Commons. This page histiociotsis last edited on 1 Decemberat By using this site, you agree to the Terms of Use and Hhistiocitosis Policy.

Micrograph showing a Langerhans cell histiocytosis with the characteristic reniform Langerhans cells accompanied by abundant eosinophils. D ICD – Wikimedia Commons has media related to Langerhans Cell Histiocytosis.