La histiocitosis de células de Langerhans (HCL) es una enfermedad poco frecuente M.A. TossSurvey of Hand-Schuller- Christian’disease in otolaryngology. Xantomas en paciente con histiocitosis de células de Langerhans y cirrosis de formas crónicas progresivas, como la enfermedad de Hand-Schüller-Christian. Hashimoto-Pritzker disease, a congenital self-healing form; Letterer-Siwe disease, a severe, acute and disseminate form; Hand-Schüller-Christian disease, .

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Resolution of cutaneous lesions of histiocytosis X by intralesion injections of interferon-beta. CT scan showed a 3. Oral Langerhans cell histiocytosis.

Langerhans cell histiocytosis may also affect the pituitary gland which makes hormones that control other glands and many body functions, especially growth.

Hematology and Oncology Chapters.

enfermedad de Hand-Schüller-Christian

Por esta razon, a la forma multisistemica diseminada y aguda de HCL se le denomino enfermedad de Hand-Schuller-Christian, y a la forma multifocal, progresiva y cronica, enfermedad de Letterer-Siwe.

Bone scintigraphy is of limited usefulness. Biopsy revealed the diagnosis of LCH.

D ICD – Definition CSP group of disorders of histiocyte proliferation which includes Letterer-Siwe disease; Hand-Schueller-Christian syndrome; and eosinophilic granuloma; Langerhans cells are components of the lesions. Hematology and Oncology – Histiocytosis Pages. CT of the pelvis soft tissue window: Langerhans cell histiocytosis LCH is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs.


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A year-old female was referred to the Dept. Introduction Langerhans cell histiocytosis LCH is a disease of unknown etiology, characterized by proliferation of pathological Langerhans cells within different organs Revision de la literatura. Neoplasm of the immune system.

Schyller 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 Schuuller Multicentric reticulohistiocytosisReticulohistiocytoma Indeterminate cell histiocytosis.

Letterer-Siwe Disease is an association of cutaneous, systemic and bone lesions. Langerhans cells are oval or rounded in shape, pale, and have a predominantly eosinophilic cytoplasm 2,8. TI-weighted image with contrast: For patients with monostotic disease, some authors recommend therapeutic abstention, since spontaneous regression takes place in the majority of these cases. Intraorbital involvement can produce proptosis 3,8as occurred in Case 2 of this report.

There are no controlled studies establishing optimal treatment for LCH 2,7,9, This page was last edited on 6 Julyat The overall 5-year survival rate for LCH is approximately Smoking cessation may lead to reversal of changes or evolution to pulmonary fibrosis and pulmonary hypertension.


A year-old male was referred to the Dept.

enfermedad de Hand-Schüller-Christian

Schhuller Engl J Med ; Symptoms Signs Labs Diagnosis Extra: CT of the femur osseous window. Search other sites for ‘Hand-Schuller-Christian Syndrome’.

Histological appearance does not correlate with clinical behaviour There is involvement of multiple sites in one organ system, most frequently the bone. CT may be useful to define the extent of the process.

Bone scintigraphy showed no other lesions. It may involve cjristian bone, but preferred sites are the skull, mandible, spine and long bones.

Ann Otol Laryngol ; Content is updated monthly with systematic literature reviews and conferences. Nine years post-surgery, there are no signs of recurrence of the lesion. Scintigraphy is useful for evaluating extent of the disease and for monitoring its evolution 7.