Langerhans cell histiocytosis pathophysiology: Difference between revisions

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Revision as of 19:26, 21 December 2015

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

Template:WikiDoc Sources

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 ==Gross 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.
-. "reactive" (benign histiocytoses)
+::*May be called "buttock cells".
-. Langerhans Cell Histiocytosis
-"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]]).
-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>
-* '''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''
-*'''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%.
-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.
 ==References==
 {{Reflist|2}}