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What is Hemophagocytic Lymphohistiocytosis (HLH)?

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Hugh Luettgen

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Q: What is Hemophagocytic Lymphohistiocytosis (HLH)?
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What words start with the letter H?

Hat, hate, have, high, higher, hats, hates, huge, hug, hugs, ham, hams, hale, hope, hop, hot, hott, hotter, hawk, hawks, hog, how, hole, hard, heart, harm, has, his, her, he, heck, hell.HagletHornHonorHomeHouseHenHandh-dnaH-Y antigenhabitabilityhabitathabituationhaemoglobin (hb)haemophiliahair cellshairpinhairpin-mediated polymerase slippage modelhalf-chromatid conversionhalf-lifehallucinosishalogenhaloperoxidasehalophilehalophilichalotoleranthamstringhaplo-haplodiploidyhaploidhaploidizationhaplotypehaptenhaptichapto-haptoglobinHarada-Mori culturehard acidhard baseHardy-Weinberg equilibriumharlequin chromosomeHAT mediumHClHD allelehead mesenchymehealthyheart rateheatheat of fusionheat of reactionheat of vaporizationheat shock factor 1 (HSF-1)heat shock proteinheat shock responsehelicaseheliopausehelix-turn-helix (HLH) proteinhelix-turn-helix motifHelminthhematocrithematologyhematopoiesishemehemerythrinhemispherehemizygoushemizygous genehemochromatosishemocyaninhemoglobinHemolysisHemophiliahemorrhageHenderson-Hasselbalch equationHensen's nodeheparinhepathepaticHepatitisHepatitis B surface antigen (HBsAg)hept-Herbicideherbivorehereditaryheredityheritabilityheritability in the narrow sensehermaphroditeherpesvirushetero-multimeric enzymeheterochromatinheterochronyheteroduplexheteroduplex analysisheteroduplex DNAheteroduplex DNA modelheteroduplex mappingheterogametic sexheterogamyheterogeneous nuclear RNAheterogenous matterheterokaryonheterokaryon testheteromorphic chromosomesheteroplasmonheteroplasmyheterosisheterothallicheterotrophheterozygosityheterozygoteheterozygote advantageheterozygous gene pairhex-hexadentate ligandhexaploidhexokinaseHGTHigh density lipoprotein cholesterol (HDLC)High frequency of recombinationHILIChindbrainhinge pointhippocampusHistidinehistocompatibility antigenhistocompatibility genehistogramhistologicalhistologyhistonehistone acetyltransferaseshistone deacetylase (HDAC)hnRNAholandric traitholoblastic cleavageholoenzymehome rangehomeo-boxhomeo-domainhomeodomainhomeologous chromosomeshomeosishomeostasishomeostatichomeothermalhomeotic mutations (genes)hominghomo-Homocysteinehomogametic sexhomogeneityhomogeneoushomogenous matterhomologhomologoushomologous chromosomeshomologous recombinationHomologyhomology modellinghomomorphic chromosomeshomoplasmyhomoplasyhomothallichomotrimerhomozygotehomozygous gene pairhorizontal gene transferhormonehormone response element (HRE)Horseradish PeroxidaseHosthost rangehot spotHPLCHsp 90Human artificial chromosome (HAC)Human Genome InitiativeHuman Genome Project (HGP)Human growth hormone (HGH)humectanthumerushumidityhumoralhumoral immunityhuntingtin-associated protein-1 (HAP-1)Huntington's diseasehyalinhyaluronidasehybridhybrid DNAhybrid dysgenesishybrid plasmidhybrid vigorhybridization in situHybridization Protection Assay (HPA)hybridizehybridomahydration shellhydrocarbonhydrogenhydrogen bondhydrogen bondinghydrogen chloridehydrogen peroxidehydrogenasehydrogenatehydrolasehydrologyhydrolysishydrolyzehydronhydronium ionhydrophilichydrophilic interaction chromatographyHydrophobichydrophobic effecthydrophobic interactionhydrostatichydroxyapatitehydroxyl radicalhygienehyper-hyperglycemiahypermorphhyperoxiahyperplasiahyperploidHyperpolarisationhyperpolarizationhypertensionHyperthyroidismhypertonic solutionhypertrophyhypervariable locushypervariable regionhyperventilationhypha (plural hyphae)hypo-hypodermic syringeHypoglycemiahypomorphhypoploidhypostatic genehypotensionHypothalamushypothesisHypothyroidismhypotonic solutionhypoventilationHypovolemiahypsometry


Histiocytosis?

DefinitionHistiocytosis is a general name for a group of syndromes that involve an abnormal increase in the number of immune cells called histiocytes.There are three major classes of histiocytoses:Langerhans cell histiocytosis, which is also called histiocytosis XMalignant histiocytosis syndrome (now known as T-cell lymphoma)Non-Langerhans cell histiocytosis (also known as hemophagocytic syndrome)This article focuses only on Langerhans cell histiocytosis (histiocytosis X).Alternative NamesHistiocytosis X; Langerhans cell histiocytosis; Eosinophilic granuloma; Pulmonary histiocytosis X; Nonlipid reticuloendotheliosis; Pulmonary Langerhans cell granulomatosis; Hand-Schuller-Christian disease; Letterer-Siwe diseaseCauses, incidence, and risk factorsHistiocytosis X has typically been thought of as a cancer-like condition. More recently researchers have begun to suspect that it is actually an autoimmune phenomenon, in which immune cells mistakenly attack the body, rather than fight infections. Extra immune cells may form tumors, which can affect various parts of the body including the bones, skull, and other areas.Some forms of the disorder are genetic.Histiocytosis X is thought to affect roughly 1 in 200,000 people each year. It is most often seen in children ages 1 to 15. The rate peaks among children ages 5 to10.Pulmonary histiocytosis X is a specific type of this disorder that involves swelling of the small airways (bronchioles) and small blood vessels in the lungs. It is most common in adults. The inflammation leads to lung stiffening and damage. The cause is unknown. It most often affects those ages 30 to 40, usually cigarette smokers.SymptomsHistiocytosis X often affects the whole body. A disease that affects the whole body is called a systemic disorder.Symptoms can vary between children and adults, although there can be some overlap. Tumors in weight-bearing bones, such as the legs or spine, may cause the bones to fracture without apparent reason.Symptoms in children may include:Abdominal painBone pain (possibly)Delayed pubertyDizzinessEar drainage that continues long-termEyes that appear to stick out (protrude) more and moreIrritabilityFailure to thriveFeverFrequent urinationHeadacheJaundiceLimpingMental deteriorationRash (petechiaeor purpura)Seborrheic dermatitis of the scalpSeizuresShort statureSwollen lymph glandsThirstVomitingWeight lossNote: Children over 5 years old often have only bone involvement.Symptoms in adults may include:Bone painChest painCoughFeverGeneral discomfort, uneasiness, or ill feeling (malaise)Increased amount of urineRashShortness of breathThirst and increased drinking of fluidsWeight lossSigns and testsThe tumors produce a "punched-out" look on a bone x-ray. Specific tests vary depending on the age of the patient.Tests in children may also include:Biopsy of skin to check for the presence of Langerhans cellsBone marrow biopsy to check for the presence of Langerhans cellsComplete blood count (CBC)X-rays of all the bones in the body (skeletal survey) to find out how many bones are affectedTests in adults may include:Bronchoscopywith biopsyChest x-rayPulmonary function testsHistiocytosis X is sometimes associated with cancer. CT scans and biopsy should be done to rule out possible cancer.TreatmentThis disorder is treated with corticosteroids, which suppress immune function (including the dangerous cells). Smoking may worsen the response to treatment and should be stopped.Children may be given other medications depending on their estimated outlook. Such medications may include:CyclophosphamideEtoposideMethotrexateVinblastineRadiation therapy or surgery may also be used to treat bone lesions.Other treatments may include:Antibiotics to fight infectionsBreathing support (with a breathing machine)Hormone replacement therapyPhysical therapySpecial shampoos for scalp problemsSupportive care to relieve symptomsSupport GroupsHistiocytosis Association of America -- www.histio.orgExpectations (prognosis)Histiocytosis X affects many organs and can lead to death.About half of those with pulmonary histiocytosis see improvement, while others eventually have permanent loss of lung function.In very young patients, the outlook depends on the specific histiocytosis and severity of the disease. Some children can live a normal life with minimal disease involvement, while others may have a poor outcome. Young children, especially infants, are more likely to have body-wide symptoms that lead to death.ComplicationsComplications may include:Diffuse interstitial pulmonary fibrosisSpontaneous pneumothoraxChildren may also develop:Anemia caused by spreading of the tumors to the bone marrowDiabetes insipidusLung problems that lead to lung failureProblems with the pituitary gland that lead to growth failureCalling your health care providerCall your health care provider if you or your child have symptoms of this disorder. Go to the emergency room if shortness of breath or chest pain develop.PreventionAvoid smoking. Quitting smoking can improve the outcome in people with histiocytosis that affects the lungs.There is no known prevention for the childhood forms of the disease.ReferencesLadisch S. Histiocytosis syndromes of childhood. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 507.Raghu G. Interstitial lung disease. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 92.Lipton JM, Arceci RJ. Histiocytic Disorders. In: Hoffman R, Benz EJ, Shattil SS, et al, eds. Hematology: Basic Principles and Practice. 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; 2008:chap 52.


Word beginning with the letter h?

Officially, yes. Most teachers/professors will accept it either way. ANSWER In American English, it depends on whether or not the "h" is sounded. In "hour," it is not sounded; therefore, we say, "an hour." In "hostage," it is sounded; therefore, we say, "a hostage." [Some U.S.A. dialects use "a" in some words even when the "h" is not sounded. In the South, for example, one hears, "I'll be back in a (h)our."] That view rests on the general rule that if a word begins with a vowel we use "an," if it begins with a consonant, we use "a." Some h-words have alternate pronunciations, with the h and without. For example, the word "homage" is homage or omage -- or, in modern film lingo "ohMAGE." In America, we would say, "a homage"; "an omage"; "an ohMAGE." [spelvin]