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| ==Gross Pathology== | | ==Gross Pathology== |
| ==Microscopic Pathology== | | ==Microscopic Pathology== |
| | | On microscopic histopathological analysis, findings of langerhans cell histiocytosis include: |
| The disease spectrum results from [[Clone (genetics)|clonal]] accumulation and [[proliferation]] of cells resembling the epidermal [[dendritic cells]] called [[langerhans cell]]s, hence sometimes called '''dendritic cell histiocytosis'''. These cells in combination with [[lymphocytes]], [[eosinophils]] and normal [[histiocytes]] form typical LCH lesion that can be found in almost any [[Organ (anatomy)|organ]]<ref>{{cite journal |author=Makras P, Papadogias D, Kontogeorgos G, Piaditis G, Kaltsas G |title=Spontaneous gonadotrophin deficiency recovery in an adult patient with Langerhans cell histiocytosis (LCH) |journal=Pituitary |volume=8 |issue=2 |pages=169-74 |year=2005 |pmid=16379033}}</ref>.
| | *Langerhans cells histiocytes |
| | | :*Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm. |
| There are three types of histiocytoses<ref name="robbins"/>1. malignant (true histiocytic lymphomas)
| | ::*Nucleus may look like a "coffee bean", that is have nuclear grooves, appearance dependent on the rotation of the nucleus. |
| 2. "reactive" (benign histiocytoses)
| | ::*May be called "buttock cells". |
| 3. Langerhans Cell Histiocytosis
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| "Reactive" in this context indicates that the abnormality may be due to a physiological reaction to infection. For example [[leukocytosis]] (proliferation of white blood cells) is a normal reaction to infection, and "histiocytes" are developmentally related to white blood cells (see article [[hematopoiesis]]).
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| Langerhans Cell Histiocytoses are traditionally divided into three groups:<ref name="robbins"> {{cite book |author=Cotran, Ramzi S.; Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Robbins, Stanley L. |title=Robbins and Cotran pathologic basis of disease |publisher=Elsevier Saunders |location=St. Louis, Mo |year=2005 |pages=701- |isbn=0-8089-2302-1 |oclc= |doi=}}</ref>
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| * '''Unifocal''' (aka "Eosinophilic granuloma"): a slowly progressing disease, characterized by an expanding proliferation of Langerhans cells in various bones, skin, lungs or stomach. | |
| * '''Multifocal unisystem''': characterized by fever and diffuse eruptions, usually on the scalp and in the ear canals, as well as bone lesions. Mostly seen in children. In 50% of cases the stalk of the [[pituitary]] gland is involved, leading to [[diabetes insipidus]]. The triad of [[diabetes insipidus]], [[proptosis]], and lytic bone lesions is known as ''Hand-Schuller-Christian triad''
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| *'''Multifocal multisystem''' ([[Letterer-Siwe disease]]): a rapidly progressing disease where Langerhans cells proliferate in many tissues. It is mostly seen in children under age 2, and the prognosis is poor: even with aggressive chemotherapy, the 5-year survival is only 50%.
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| Pulmonary Langerhans cell histiocytosis (PLCH) is a smoking-related interstitial lung disease, used to be considered a benign condition in adults, long term complications like [[pulmonary hypertension]] is becoming increasingly recognized.
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| ==References== | | ==References== |
| {{Reflist|2}} | | {{Reflist|2}} |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Nawal Muazam M.D.[2]
Overview
Pathophysiology
Pathogenesis
Genetics
Associated Conditions
Gross Pathology
Microscopic Pathology
On microscopic histopathological analysis, findings of langerhans cell histiocytosis include:
- Langerhans cells histiocytes
- Clusters of cells (histiocytes) with a reniform (kidney-shaped) nucleus and abundant foamy cytoplasm.
- Nucleus may look like a "coffee bean", that is have nuclear grooves, appearance dependent on the rotation of the nucleus.
- May be called "buttock cells".
References
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