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- Disease Information
- Disease Comparison
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Disease Processes ▼
- Auto Immune
- Vascular-Arteriosclerosis
- Biochemical
- Congenital-developmental
- Deficiency
- Degenrative-Necrosis
- Electromagnetic-Physics
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Major Organs-Systems ▼
- Systemic
- Pediatric
- Nervous & Sensory System (Neurology)
- Cardiovascular System
- Respiratory (Pulmonary) System
- Gastro-Intestinal (Digestive) System
- Urinary System
- Dermatologic System
- Endocrine System
- Immune System
- Musculoskeletal System
- Genital Reproductive System
- Hematopoietic System (Hematology)
- Lymphatic System
- Tissue/Cells/Organelles
Disease Information for Langerhans' cell histiocytosis disorder
- Clinical Manifestations
- Signs & Symptoms
- Dandruff
- Scalp rash
- Scalp rash/Oily scales/pruritic
- Truncal distribution/rash
- Lymphadenopathy Systemic
- Tobacco Habit/Excess
- Bone Pains
- Splenomegaly
- Disease Progression
- Course/Chronic disorder
- Course/Chronic only
- Demographics & Risk Factors
- Population Group
- Child
- Population/Pediatrics population
- Sex & Age Groups
- Population/Child
- Population/Children/all
- Population/Male
- Laboratory Tests
- Abnormal Lab Findings - Decreased
- Platelet count (Lab)
- Diagnostic Test Results
- Pathology
- BX/Skin biopsy/Abnormal
- BX/Gingival biopsy/Abnormal
- BX/Liver biopsy/Abnormal
- PATH/Histiocyte/HLA-DR on surface
- PATH/Histiocytes Surface Nucleoproteins
- PATH/Langerhans' histiocyte/Birbeck granules/electronmicr
- PATH/Langerhans' histiocyte/CD1 cell surface
- PATH/Langerhans' histiocyte/Immune S100 protein
- PATH/Langerhans' histiocyte/Pale distinct/abundant cytoplasm
- PATH/Langerhans' histiocytes present
- X-RAY
- Xray/Upper lobe lesion/Chest
- Xray/Epiphysis Lesions/Site
- Xray/Skull single lytic lesion
- Associated Diseases & Rule outs
- Rule Outs
- Silicosis
- Associated Disease & Complications
- Bullous Emphysema Syndrome
- Eosinophilic granuloma
- Eosinophilic granuloma/hypophysis
- Hand-Schuller-Christian disease
- Histiocytosis X syndrome
- Hypernatremia
- Langerhans' cell histiocytosis disorder
- Letterer-Siwi disease
- Panhypopituitarism
- Pneumothorax
- Thrombocytopenia
- Thrombocytopenia in Children
- Upper respiratory infection/cold/URI
- Disease Mechanism & Classification
- Class
- CLASS/Langerhan Cell/Tissue Macrophages involvement/disorder (ex)
- CLASS/Epiphyses Lesion/Involvement
- CLASS/Reticuloendothelial system (RES) disorder (ex)
- CLASS/Multisystem/organ involvement/pathology (ex)
- CLASS/Systemic/no comment (category)
- Pathophysiology
- Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
- Pathophysiology/Multisystem disease
- Pathophysiology/Sporadic/hereditary/process
- Pathophysiology/Maternal Chromosome mutation
- Pathophysiology/Immunoregulatory defect/causal
- Process
- PROCESS/Congenital/developmental (category)
- PROCESS/Genetic disorder/Spontaneous mutations/sporadic
- PROCESS/Immunoproliferative disease (ex)
- PROCESS/Inflammatory/Granulomatous disorder (category)
- PROCESS/Metabolic/storage disorder (category)
- PROCESS/Storage disorder (ex)
- PROCESS/Proliferative/infiltrative disorder (ex)
- Synonyms
- Synonym
- Langerhans' cell histiocyte disorder, Synonym/Class l Histiocytosis syndrome, Synonym/Congenital Langerhans/histiocytosis, Synonym/Histiocytosis of childhood
- Treatment
- Drug Therapy - Indication
- RX/Cladribine (Leustatin)
- RX/Cytarabine (ARA-C)
- RX/Etoposide (Vepesid)
- SX/Stem cell transplant/allogenic
- SX/Stem-cell transplant
- Definition
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Langerhans Cell Histiocytosis; Histiocytosis X; LCH - Langerhans-Cell Granulomatosis; Non-Lipid Reticuloendotheliosis; Systemic Aleukemic Reticuloendotheliosis; Type II Histiocytosis;Abt-Letterer-Siwe Disease; Eosinophilic Granuloma; Hand-Schueller-Christian Syndrome; Hashimoto-Pritzker Syndrome;
Letterer-Siwe Disease; Pure Cutaneous Histiocytosis; Self-Healing Histiocytosis
Langerhans cell histiocytosis (LCH) is a rare spectrum of disorders characterized by overproduction (proliferation) and accumulation of a specific type of white blood cell (histiocyte) in the various tissues and organs of the body (lesions); The lesions may include certain distinctive granule-containing cells (Langerhans cells) involved in certain immune responses, as well as other white blood cells (monocytes, eosinophils); Associated symptoms and findings may vary from case to case, depending upon the specific tissues and organs affected and the extent of involvement; Most affected individuals have single or multiple bone lesions characterized by degenerative changes and loss of the calcium of bone (osteolysis); Although the skull is most commonly affected, there may also be involvement of other bones, such as those of the spine (vertebrae) and the long bones of the arms and legs; Affected individuals may have no apparent symptoms (asymptomatic), and may experience associated pain and swelling, and/or develop certain complications, such as fractures or secondary compression of the spinal cord; In some cases, other tissues and organs may also be affected, including the skin, lungs, or other areas; In some individuals, LCH may be associated with involvement of the pituitary gland leading to diabetes insipidus; The exact cause of Langerhans cell histiocytosis is unknown;
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- External Links Related to Langerhans' cell histiocytosis disorder
- Wikipedia
- Merck
- Images
- PubMed (National Library of Medicine)
- NGC (National Guideline Clearinghouse)
- Medscape (eMedicine)
- Harrison's Online (accessmedicine)
- NEJM (The New England Journal of Medicine)