Histiocytosis

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Histiocytosis Classification and external resources
ICD-10	C96.1, D76.0
ICD-9	202.3, 277.89
eMedicine	ped/1997
MeSH	D015614

WikiDoc Resources for Histiocytosis
Articles
Most recent articles on Histiocytosis Most cited articles on Histiocytosis Review articles on Histiocytosis Articles on Histiocytosis in N Eng J Med, Lancet, BMJ
Media
Powerpoint slides on Histiocytosis Images of Histiocytosis Photos of Histiocytosis Podcasts & MP3s on Histiocytosis Videos on Histiocytosis
Evidence Based Medicine
Cochrane Collaboration on Histiocytosis Bandolier on Histiocytosis TRIP on Histiocytosis
Clinical Trials
Ongoing Trials on Histiocytosis at Clinical Trials.gov Trial results on Histiocytosis Clinical Trials on Histiocytosis at Google
Guidelines / Policies / Govt
US National Guidelines Clearinghouse on Histiocytosis NICE Guidance on Histiocytosis NHS PRODIGY Guidance FDA on Histiocytosis CDC on Histiocytosis
Books
Books on Histiocytosis
News
Histiocytosis in the news Be alerted to news on Histiocytosis News trends on Histiocytosis
Commentary
Blogs on Histiocytosis
Definitions
Definitions of Histiocytosis
Patient Resources / Community
Patient resources on Histiocytosis Discussion groups on Histiocytosis Patient Handouts on Histiocytosis Directions to Hospitals Treating Histiocytosis Risk calculators and risk factors for Histiocytosis
Healthcare Provider Resources
Symptoms of Histiocytosis Causes & Risk Factors for Histiocytosis Diagnostic studies for Histiocytosis Treatment of Histiocytosis
Continuing Medical Education (CME)
CME Programs on Histiocytosis
International
Histiocytosis en Espanol Histiocytosis en Francais
Businness
Histiocytosis in the Marketplace Patents on Histiocytosis
Experimental / Informatics
List of terms related to Histiocytosis

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In medicine, histiocytosis is an excessive number of histiocytes,^[1] that is an excessive number of tissue macrophages, and is typically used to refer to a group of rare diseases which share this as a characteristic. Occasionally and confusingly it is sometimes used to refer to individual diseases.

According to the Histiocytosis Association of America, 1 in 200,000 children in the United States are born with histiocytosis each year.^[1] HAA also states that most of the people diagnosed with histiocytosis are children under the age of 10. The University of California, San Francisco, states that the disease usually occurs from birth to age 15.^[1]

Histiocytosis (and malignant histiocytosis) are both important in veterinary as well as human pathology.

Presentation

Histiocytosis is frequently associated with diabetes insipidus, even after several years of diagnosis and successful therapy.

Classification, and relationships to other conditions

There are competing systems for classifying histiocytoses. According to the 1999 classification proposed by the World Health Organization, they can be divided into three categories.^[1][1]. However, the classifications in ICD10 and MeSH are slightly different, as shown below:

Name	WHO	ICD10	MeSH
Langerhans cell histiocytosis (LCH)	I	D76.0	Langerhans-cell histiocytosis
Juvenile xanthogranuloma (JXG)	II	D76.3	non-Langerhans-cell histiocytosis
Hemophagocytic lymphohistiocytosis (HLH)	II	D76.1	non-Langerhans-cell histiocytosis
Niemann-Pick disease	-	E75.2	non-Langerhans-cell histiocytosis
Sea-blue histiocyte syndrome	-	-	non-Langerhans-cell histiocytosis
Acute monocytic leukemia	III	C93.0	malignant histiocytic disorders
Malignant histiocytosis	III	C96.1	malignant histiocytic disorders
Erdheim-Chester disease	-	C96.1	malignant histiocytic disorders

Types of LCH have also been known as "Eosinophilic Granuloma", "Hand-Schuller-Christian Disease", "Letterer-Siwe Disease", and "Histiocytosis X". (See LCH history for details).

Common treatments

• Chemotherapy
  • Cladribine or 2CDA or leustatin
  • Etoposide
  • Vinblastine or Velban

Organizations

Patients and families can gain some support by contacting patient organisations such as the Histiocytosis Association of America ([3]) or the Histiocytosis Research Trust ([4]). The Histiocytosis Association of America has several stable and proven treatment protocols available only for physicians ([5]).

References

v • d • e Pathology: hematology, myeloid hematologic disease (primarily D50-D77, 280-289)
RBCs/ hemoglobinopathy	+ Polycythemia - Macrocytosis - Anemia Nutritional Iron deficiency anemia (Plummer-Vinson syndrome), Megaloblastic anemia (Pernicious anemia) Hemolytic Hereditary enzyme: G6PD Deficiency - Pyruvate kinase deficiency - Triosephosphate isomerase deficiency hemoglobin: Thalassemia - Sickle-cell disease/trait membrane: Hereditary spherocytosis - Hereditary elliptocytosis - Hereditary stomatocytosis Acquired Autoimmune (Warm, Cold), HUS, MAHA, PNH, PCH, Myelophthisic Aplastic Acquired PRCA, Diamond-Blackfan anemia, Fanconi anemia, Sideroblastic anemia Blood tests Normocytic - Microcytic - Macrocytic - Normochromic - Hypochromic Other Methemoglobinemia
Coagulation/platelets/ coagulopathy/ bleeding diathesis	+ Hypercoagulability primary: Antithrombin III deficiency - Protein C deficiency/Activated protein C resistance/Protein S deficiency/Factor V Leiden - Hyperprothrombinemia acquired: DIC (Congenital afibrinogenemia, Purpura fulminans) - autoimmune (Antiphospholipid) Other Essential thrombocytosis - clotting factor: Hemophilia (A/VIII, B/IX, C/XI) • Von Willebrand disease • Hypoprothrombinemia/II - XIII platelet function: Bernard-Soulier syndrome - Glanzmann's thrombasthenia - Gray platelet syndrome - May Hegglin anomaly - Pelger-Huet anomaly Purpura: Henoch-Schönlein, ITP (Evans syndrome), TTP Thrombocytopenia (Heparin-induced thrombocytopenia)
Monocytes/ macrophages	+ Histiocytosis WHO-I (Langerhans cell histiocytosis) WHO-II/non-Langerhans-cell (Juvenile xanthogranuloma, Hemophagocytic lymphohistiocytosis) WHO-III/malignant (Acute monocytic leukemia, Malignant histiocytosis, Erdheim-Chester disease) Other Chronic granulomatous disease -cytosis: Monocytosis - -penia: Monocytopenia
Granulocytes	+ -cytosis: granulocytosis (Neutrophilia, Eosinophilia, Basophilia) - -penia: Granulocytopenia/agranulocytosis (Neutropenia/Kostmann syndrome, Eosinopenia, Basopenia)
See also hematological malignancy and immune disorders

v • d • e WikiDoc Research Resources for Histiocytosis
Articles on Histiocytosis	Most recent articles on Histiocytosis • Most cited articles on Histiocytosis • Review articles on Histiocytosis • Articles on Histiocytosis in N Eng J Med, Lancet, BMJ
Media (Slides, Video, Images, MP3) on Histiocytosis	Powerpoint slides on Histiocytosis • Images of Histiocytosis • Photos of Histiocytosis • Podcasts & MP3s on Histiocytosis • Videos on Histiocytosis
Evidence Based Medicine Regarding Histiocytosis	Cochrane Collaboration on Histiocytosis • Bandolier on Histiocytosis • TRIP on Histiocytosis
Cost Effectiveness of Histiocytosis	Cost Effectiveness of Histiocytosis
Clinical Trials Involving Histiocytosis	Ongoing Trials on Histiocytosis at Clinical Trials.gov • Trial results on Histiocytosis • Clinical Trials on Histiocytosis at Google
Guidelines / Policies / Government Resources (FDA/CDC) Regarding Histiocytosis	US National Guidelines Clearinghouse on Histiocytosis • NICE Guidance on Histiocytosis • NHS PRODIGY Guidance • FDA on Histiocytosis • CDC on Histiocytosis
Textbook Information on Histiocytosis	Books and Textbook Information on Histiocytosis
Pharmacology Resources on Histiocytosis	Dosing of Histiocytosis • Drug interactions with Histiocytosis • Side effects of Histiocytosis • Allergic reactions to Histiocytosis • Overdose information on Histiocytosis • Carcinogenicity information on Histiocytosis • Histiocytosis in pregnancy • Pharmacokinetics of Histiocytosis •
Genetics, Pharmacogenomics, and Proteinomics of Histiocytosis	Genetics of Histiocytosis • Pharmacogenomics of Histiocytosis • Proteomics of Histiocytosis
Newstories on Histiocytosis	Histiocytosis in the news • Be alerted to news on Histiocytosis • News trends on Histiocytosis
Commentary on Histiocytosis	Blogs on Histiocytosis
Patient Resources on Histiocytosis	Patient resources on Histiocytosis • Discussion groups on Histiocytosis • Patient Handouts on Histiocytosis • Directions to Hospitals Treating Histiocytosis • Risk calculators and risk factors for Histiocytosis
Healthcare Provider Resources on Histiocytosis	Symptoms of Histiocytosis • Causes & Risk Factors for Histiocytosis • Diagnostic studies for Histiocytosis • Treatment of Histiocytosis
Continuing Medical Education (CME) Programs on Histiocytosis	CME Programs on Histiocytosis
International Resources on Histiocytosis	Histiocytosis en Espanol • Histiocytosis en Francais
Business Resources on Histiocytosis	Histiocytosis in the Marketplace • Patents on Histiocytosis
Informatics Resources on Histiocytosis	List of terms related to Histiocytosis

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Acknowledgement and Attribution Regarding Sources of Content

Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .