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| {{SI}} | | {{Langerhans cell histiocytosis}} |
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| {{Editor Help}}
| | ==[[Langerhans cell histiocytosis overview|Overview]]== |
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| ==Overview== | | ==[[Langerhans cell histiocytosis historical perspective|Historical Perspective]]== |
| '''Langerhans Cell Histiocytosis (LCH)''' is a rare [[disease]] involving clonal proliferation of [[langerhans cell]]s, abnormal [[cell (biology)|cell]]s deriving from [[bone marrow]] and capable of migrating from skin to [[lymph nodes]]. Clinically, its manifestations range from isolated bone lesions to [[systemic disease|multisystemic disease]].
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| The disease is part of a group of clinical syndromes called [[Histiocytosis|histiocytoses]], which are characterized by an abnormal proliferation of [[histiocyte]]s (an archaic term for dendritic cells and macrophages). These diseases are related to other forms of abnormal proliferation of white blood cells, such as [[leukemia]]s and [[lymphomas]].
| | ==[[Langerhans cell histiocytosis pathophysiology|Pathophysiology]]== |
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| Langerhans Cell Histiocytosis used to be called histiocytosis X, until it was renamed in 1985 by the Histiocyte society<ref>{{cite journal |author= |title=Histiocytosis syndromes in children. Writing Group of the Histiocyte Society |journal=Lancet |volume=1 |issue=8526 |pages=208-9 |year=1987 |pmid=2880029}}</ref> | | ==[[Langerhans cell histiocytosis epidemiology and demographics|Epidemiology & Demographics]]== |
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| ==Pathophysiology== | | ==[[Langerhans cell histiocytosis risk factors|Risk Factors]]== |
| 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>.
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| There are three types of histiocytoses<ref name="robbins"/>1. malignant (true histiocytic lymphomas)
| | ==[[Langerhans cell histiocytosis screening|Screening]]== |
| 2. "reactive" (benign histiocytoses)
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| 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]]).
| | ==[[Langerhans cell histiocytosis causes|Causes of Langerhans cell histiocytosis]]== |
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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>
| | ==[[Langerhans cell histiocytosis differential diagnosis|Differentiating Langerhans cell histiocytosis from other Diseases]]== |
| * '''Unifocal''' (aka "Eosinophilic granuloma"): a slowly progressing disease, characterized by an expanding proliferation of Langerhans cells in various bones, skin, lungs or stomach.
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| * '''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.
| | ==[[Langerhans cell histiocytosis natural history|Natural History, Complications & Prognosis]]== |
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| == Prevalence == | |
| LCH histiocytosis usually affects children of between 1 and 15 years old and peak incidence is between age 5 and 10. Among children under the age of 10, yearly incidence is thought to be 1 in 200,000<ref>{{cite web |url=http://www.nlm.nih.gov/medlineplus/ency/article/000068.htm |title=MedlinePlus Medical Encyclopedia: Histiocytosis |accessdate=2007-05-10 |format= |work=}}</ref>; and in adults even more rare, in about 1 in 560,000.<ref>{{cite web|url=http://www.histiocytosis.ca/facts.html | title=Histiocytosis Association of Canada | accessdate=2007-05-16|format= |work=}}</ref> It has been reported in elderly but is vanishingly rare<ref>{{cite journal |author=Gerlach B, Stein A, Fischer R, Wozel G, Dittert D, Richter G |title=[Langerhans cell histiocytosis in the elderly] |language=German |journal=Der Hautarzt; Zeitschrift für Dermatologie, Venerologie, und verwandte Gebiete |volume=49 |issue=1 |pages=23-30 |year=1998 |pmid=9522189}}</ref>. commoner in white race and boys are effected twice as often as girls.
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| LCH is usually sporadic and non-[[hereditary]] condition but familial clustering has been noted in limited number of cases. Hashimoto-Pritzker disease, a variant of Hand-Schüller-Christian disease, is a congenital self-healing form<ref>{{cite journal |author=Kapur P, Erickson C, Rakheja D, Carder K, Hoang M |title=Congenital self-healing reticulohistiocytosis (Hashimoto-Pritzker disease): ten-year experience at Dallas Children's Medical Center |journal=J. Am. Acad. Dermatol. |volume=56 |issue=2 |pages=290-4 |year=2007 |pmid=17224372}}</ref>.
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| ==Clinical Feature==
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| [[Image:LCH1.jpg|left|thumb|LCH infiltrating peri-orbital tissue(arrowed)]]
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| Features can be non-specific from generalized [[inflammatory]] response e.g fever, [[lethargy]], weight loss or can be due to specific organ involvement.
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| [[Organs]] involved:
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| *Bone: Most frequent in both unifocal and disseminated disease presenting as painful swelling. Skull, long bone of upper extremity, flat bones are affected in descending order. Infiltration in hands and feet is unusual. Osteolytic lesions can lead to pathological fractures.
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| *Skin: Commonly present as rash which varies between scaly erythematous lesions to red papules pronounced in [[Intertrigo|intertriginous]] areas. Up to 80% of LS would have extensive eruptions on the scalp.
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| *Bone marrow : Pancytopenia with super added infection usually implies poor prognosis. Anemia can be due to number of factors and not necessarily implies bone marrow infiltration.
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| *Lymph node: cervical commonest. enlargement of the liver in 20%, spleen in 30% and lymph nodes in 50% of HSC.<ref>{{cite web |url=http://www.patient.co.uk/showdoc/40002781/#notes |title=Langerhans cell histiocytosis - Patient UK |accessdate=2007-05-10 |format= |work=}}</ref>
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| *[[Endocrine]] glands:[[Hypothalamic-pituitary-adrenal axis|Hypothalamic pituitary axis]] commonly involved. Diabetes insipidus most common. [[Anterior pituitary]] [[hormone]] deficiency is usually permanent.
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| *Lungs:
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| *Less frequently GIT, CNS.
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| ==Diagnosis== | | ==Diagnosis== |
| [[Image:Eosinophilic granuloma - X-ray of the skull.jpg|left|thumb|Xray of skull showing Eosinophilic Granuloma(arrow)]] | | [[Langerhans cell histiocytosis history and symptoms|History & Symptoms]] | [[Langerhans cell histiocytosis physical examination|Physical Examination]] | [[Langerhans cell histiocytosis laboratory tests|Lab Tests]] | [[Langerhans cell histiocytosis electrocardiogram|Electrocardiogram]] | [[Langerhans cell histiocytosis chest x ray|Chest X Ray]] | [[Langerhans cell histiocytosis CT|CT]] | [[Langerhans cell histiocytosis MRI|MRI]] | [[Langerhans cell histiocytosis echocardiography or ultrasound|Echocardiography or Ultrasound]] | [[Langerhans cell histiocytosis other imaging findings|Other Imaging Findings]] | [[Langerhans cell histiocytosis other diagnostic studies|Other Diagnostic Studies]] |
| Diagnosis is confirmed [[histologically]] by [[tissue]] [[biopsy]].
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| <br />Haemotoxilin-eosin stain of biopsy slide will show features of Langerhans cell e.g. distinct cell margin, pink granular [[cytoplasm]]. Presence of [[Birbeck granules]] on [[electron microscopy]] and [[immuno-cytochemistry|immuno-cytochemical]] features e. g. [[CD1]] positivity are more specific.
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| <br />Initially routine blood tests e.g. full blood count, liver function test, U&Es, bone profile are done to determine disease extent and rule out other causes.
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| <br />Radiology will show osteolytic bone lesions and damage to the lung. Latter may be evident in CXR 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]].
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| <br />Assessment of [[endocrine]] function and bonemarrow biopsy are also performed when indicated.
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| ==Treatment== | | ==Treatment== |
| Treatment is guided by extent of disease.
| | [[Langerhans cell histiocytosis medical therapy|Medical Therapy]] | [[Langerhans cell histiocytosis surgery|Surgery]] | [[Langerhans cell histiocytosis primary prevention|Primary Prevention]] | [[Langerhans cell histiocytosis secondary prevention|Secondary Prevention]] | [[Langerhans cell histiocytosis cost-effectiveness of therapy|Cost-Effectiveness of Therapy]] | [[Langerhans cell histiocytosis future or investigational therapies|Future or Investigational Therapies]] |
| <br />Solitary bone lesion may be amenable through excision or limited radiation. However systemic disease often require chemotherapy.
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| <br />Use of systemic steroid is common, singly or adjunct to chemotherapy. Local steroid cream is applied to skin lesions.
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| <br />Endocrine deficiency often require lifelong suppliment e.g. desmopressin for diabates insipidus which can be applied as nasal drop.
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| <br />[[Chemotherapeutic]] agents such as [[alkylating antineoplastic agent|alkylating agents]], [[antimetabolite]]s, [[vinca alkaloids]] either singly or in combination can lead to complete remission in diffuse disease.
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| ==Prognosis==
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| Excellent for single foci disease.
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| <br />With multi-focal disease 60% have a chronic course, 30% achieve remission and mortality is up to 10%<ref>{{cite journal |author=Komp D, El Mahdi A, Starling K, Easley J, Vietti T, Berry D, George S |title=Quality of survival in histiocytosis X: a Southwest Oncology Group study |journal=Med. Pediatr. Oncol. |volume=8 |issue=1 |pages=35-40 |year=1980 |pmid=6969347}}</ref>.
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| ==References== | | ==References== |
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| *[http://youtube.com/watch?v=KkBRqZS8nfM Seven Part Video Series] | | *[http://youtube.com/watch?v=KkBRqZS8nfM Seven Part Video Series] |
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| {{SIB}}
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| {{Hematology}} | | {{Hematology}} |
| {{Hematological malignancy histology}} | | {{Hematological malignancy histology}} |
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| [[Category:Oncology]] | | [[Category:Oncology]] |
| [[Category:Hematology]] | | [[Category:Hematology]] |
| [[Category:Mature chapter]]
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