Disease Information for Langerhans' cell histiocytosis disorder

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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

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
Google
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)
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