{"id":106155,"date":"2020-02-05T09:53:34","date_gmt":"2020-02-05T08:53:34","guid":{"rendered":"https:\/\/sdk.mk\/?p=106155"},"modified":"2020-02-05T09:53:34","modified_gmt":"2020-02-05T08:53:34","slug":"ju-njoftojme-me-semundjet-e-rralla-limfohisticitoza-hemofagocitike","status":"publish","type":"post","link":"https:\/\/sdk.mk\/index.php\/histori-te-parrefyera\/ju-njoftojme-me-semundjet-e-rralla-limfohisticitoza-hemofagocitike\/","title":{"rendered":"JU NJOFTOJM\u00cb ME S\u00cbMUNDJET E RRALLA: LIMFOHISTICITOZA HEMOFAGOCITIKE"},"content":{"rendered":"<div class=\"f52428582a4b906b2d2e2a7d733c7d27\" data-index=\"7\" style=\"float: none; margin:10px 0 10px 0; text-align:center;\">\n<!-- MSP Ads Asynchronous JS Tag - Generated with Revive Adserver v5.4.1 -->\r\n<ins data-revive-zoneid=\"167\" data-revive-id=\"060fdb01f6430ec164d3519234aa4967\"><\/ins>\r\n<script async src=\"\/\/panel.ads.com.mk\/www\/delivery\/asyncjs.php\"><\/script>\n<\/div>\n<p><a href=\"https:\/\/www.youtube.com\/watch?v=oYEs-bCAlFM&amp;list=PLPGcigDEy-qj0I1um2ByEqy4a1IdVRjzf&amp;index=6&amp;t=0s\" target=\"_blank\" rel=\"noopener noreferrer\"><em><strong>Limfohisticitoza hemofagocitike<\/strong><\/em><\/a><br \/>\nMKB D76.1<br \/>\nHemophagocytic lymphohistiocytosis<\/p>\n<p>Limfohistiocitoza hemofagocitike \u00ebsht\u00eb nj\u00eb grup s\u00ebmundjesh me origjin\u00eb makrofagale q\u00eb<br \/>\nkarakterizohet nga nj\u00eb rrjedh\u00eb t\u00eb shpejt\u00eb, fatale; Simptomat kryesore klinike p\u00ebrfshijn\u00eb ethe,<br \/>\nsplenomegali masive, bi- ose pancitopeni, hipofibrinogenemia, hipertrigliceridemia, simptoma<br \/>\nt\u00eb SNQ. Limfohistiocitoza ndahet n\u00eb dy grupe &#8211; par\u00ebsore (familjare dhe sporadike) me<br \/>\ntrash\u00ebgimi autosomale recesive dhe sekondare q\u00eb lidhet me infeksione t\u00eb ndryshme, imunitet<br \/>\nt\u00eb dob\u00ebt, s\u00ebmundje autoimune dhe lloje t\u00eb tjera t\u00eb histiocitoz\u00ebs qelizore<br \/>\nEpidemiologjia<\/p>\n<p>Limfohistiocitoza hemofagocitike primare (familjare dhe sporadike) paraqitet te grupe t\u00eb<br \/>\nndryshme etnike dhe \u00ebsht\u00eb e p\u00ebrhapur n\u00eb mbar\u00eb bot\u00ebn. Incidenca e limfohistocitoz\u00ebs<br \/>\nhemofagocitike primare, sipas J. Henter, \u00ebsht\u00eb rreth 1.2 n\u00eb 1.000.000 f\u00ebmij\u00eb n\u00ebn mosh\u00ebn 15<br \/>\nose 1 n\u00eb 50,000 foshnje. K\u00ebta indikator\u00eb jan\u00eb t\u00eb krahasuesh\u00ebm me p\u00ebrhapjen e fenilketonuris\u00eb<br \/>\nose galaktozemis\u00eb te foshnjet.<br \/>\nLimfohistiocitoza hemofagocitike primare haset p\u00ebraf\u00ebrsisht\u00eb nj\u00ebsoj si te djemt\u00eb ashtu edhe te<br \/>\nvajzat. Te 56-80% t\u00eb f\u00ebmij\u00ebve s\u00ebmundja zhvillohet n\u00eb vitin e par\u00eb t\u00eb jet\u00ebs, me at\u00eb q\u00eb te disa prej<br \/>\ntyre s\u00ebmundja diagnostikohet gjat\u00eb lindjes, nd\u00ebrsa te rreth 20% t\u00eb f\u00ebmij\u00ebve shenjat e para<br \/>\nklinike t\u00eb s\u00ebmundjes shfaqen pas mosh\u00ebs 3 vje\u00e7are. Ekzistojn\u00eb gjithashtu t\u00eb dh\u00ebna p\u00ebr zhvillimin<br \/>\ne s\u00ebmundjes n\u00eb nj\u00eb mosh\u00eb t\u00eb m\u00ebvonshme: 6, 8, 12, 25 vje\u00e7are. \u00cbsht\u00eb e r\u00ebnd\u00ebsishme t\u00eb<br \/>\ntheksohet se mosha e v\u00ebllez\u00ebrve dhe motrave t\u00eb prekur shpesh \u00ebsht\u00eb e nj\u00ebjt\u00eb. Rreth gjysma e<br \/>\nrasteve kan\u00eb nj\u00eb histori pozitive familjare<br \/>\nKy lloj i histiocitoz\u00ebs mund t\u00eb jet\u00eb par\u00ebsor ose sekondar. N\u00eb rastin e par\u00eb, s\u00ebmundja<br \/>\ntransmetohet nga prind\u00ebrit (trash\u00ebgimi autosomale recesive) me nj\u00eb rrjedh\u00eb klinike m\u00eb t\u00eb<br \/>\nr\u00ebnd\u00eb (mungesa e trajtimit rezulton me vdekje). Shfaqja e limfohistiocitoz\u00ebs sekondare<br \/>\nshoq\u00ebrohet me nj\u00eb proces infektiv ose neoplastik, si dhe me s\u00ebmundje imune.<\/p>\n<p>Manifestimet klinike t\u00eb s\u00ebmundjes jan\u00eb nga m\u00eb t\u00eb ndryshmet:<br \/>\nEthet me koh\u00ebzgjatje t\u00eb zgjatur,<br \/>\nskuqje eritematoze-papulare n\u00eb pikun e temperatur\u00ebs s\u00eb trupit;<br \/>\nrritje e m\u00ebl\u00e7is\u00eb dhe shp\u00ebnetk\u00ebs;<br \/>\nd\u00ebmtimi i sistemit nervor (simptoma meningeale, rritje e presionit intrakranial, hemiplegji, etj.);<br \/>\nSindromi KISH; (koagulopatia intravaskulare e shp\u00ebrndar\u00eb);<br \/>\npancitopenia (anemi, neutropeni, trombocitopeni) n\u00eb gjak n\u00eb kombinim me limfocitoz\u00eb;<br \/>\nnivele t\u00eb rritura t\u00eb transaminazave n\u00eb serum, bilirubin\u00ebs dhe triglicerideve;<br \/>\nrritje e p\u00ebrqendrimit t\u00eb histiociteve t\u00eb pjekura n\u00eb palc\u00ebn e kuqe t\u00eb eshtrave (m\u00eb shum\u00eb se 3%);<br \/>\nhemofagocitoza e histiociteve n\u00eb palc\u00ebn e eshtrave, shp\u00ebnetk\u00ebs ose nyjeve limfatike<br \/>\nDijagnostifikimi<\/p>\n<p>Nj\u00eb most\u00ebr e palc\u00ebs kockore ose biopsia e indit t\u00eb prekur mundet t\u00eb ndihmoj\u00eb n\u00eb konfirmimin e<br \/>\ndiagnoz\u00ebs.<br \/>\nDiagnoza e histiocitoz\u00ebs X bazohet n\u00eb t\u00eb dh\u00ebna tipike klinike, laboratorike dhe radiologjike.<br \/>\nSidoqoft\u00eb, p\u00ebr ta konfirmuar at\u00eb, \u00ebsht\u00eb e nevojshme t\u00eb kryhet punkcion i palc\u00ebs s\u00eb eshtrave ose<br \/>\nbiopsi n\u00eb vendin e lezionit (eshtrave, nyjes limfatike), me at\u00eb q\u00eb m\u00eb pas duhet t\u00eb kryhet analiz\u00eb<br \/>\npatologjike.<br \/>\nMe q\u00ebllim t\u00eb evitimit t\u00eb gabimeve n\u00eb dijagnostifikim, duhet t\u00eb b\u00ebhet nj\u00eb dijagnostifikim<br \/>\ndiferencial i s\u00ebmnundjeve n\u00eb vijim:<br \/>\nTuberkuloza<br \/>\nTumore<br \/>\nOsteomielitis<br \/>\nLimfogranulomatoz\u00eb<br \/>\nOsteodistrofi fibroze etj.<\/p>\n<p>Parime n\u00eb trajtim<br \/>\nOpsione t\u00eb ndryshme p\u00ebr terapi konservative t\u00eb kombinuar p\u00ebrdoren p\u00ebr t\u00eb trajtuar njer\u00ebzit q\u00eb<br \/>\nvuajn\u00eb nga histiocitoza X. Efekte t\u00eb mira arrihen p\u00ebrmes kombinimit t\u00eb kortikosteroideve me<br \/>\nmedikamente citotoksike. P\u00ebrve\u00e7 k\u00ebsaj, gjat\u00eb trajtimit mund t\u00eb p\u00ebrdoret:<br \/>\nglobulin anti-limfocitar;<br \/>\nantitrupa monoklonal\u00eb ndaj antigjenit CD1;<br \/>\nnukleozide analoge<br \/>\npreparate timusore;<br \/>\ntrajtim me rrezatim (n\u00eb zon\u00ebn e gj\u00ebndrr\u00ebs s\u00eb hipofiz\u00ebs).<\/p>\n<p>N\u00eb raste t\u00eb manifestimeve t\u00eb kufizuara n\u00eb l\u00ebkur\u00eb s\u00ebmundja trajtohet me kortikosteroide ose me<br \/>\nTRUF terapi (terapi rrezatimi ultravjollc\u00eb n\u00eb kombinim me nj\u00eb fotosensibilizator).<br \/>\nKur b\u00ebhet fjal\u00eb p\u00ebr limfohistiocitoz\u00ebn par\u00ebsore hemofagocitike, si trajtim m\u00eb efektiv<br \/>\nkonsiderohet transplantimi alogjenik i palc\u00ebs s\u00eb eshtrave.<br \/>\nTrajtimi i limfohistiocitoz\u00ebs hemofagocitike p\u00ebrfshin kemoterapi me q\u00ebllim t\u00eb stabilizimit t\u00eb<br \/>\npacientit para kryerjes s\u00eb transpaltacionit t\u00eb palc\u00ebs s\u00eb eshtrave. P\u00ebrve\u00e7 k\u00ebsaj, administrohen<br \/>\nagjent\u00eb antifungal, imunoglobuline intravenoze. Te rastet e splenomegalin\u00eb s\u00eb r\u00ebnd\u00eb, kryhet<br \/>\nsplenektomia (largimi i shp\u00ebnetk\u00ebs).<br \/>\nPrognoza e s\u00ebmundjes \u00ebsht\u00eb serioze, madje edhe me trajtimin e duhur, vet\u00ebm te 25% t\u00eb<br \/>\npacient\u00ebve niveli i mbijetes\u00ebs \u00ebsht\u00eb 5 vjet. Remisionet jan\u00eb t\u00eb p\u00ebrkohshme. Transplantimi i<br \/>\npalc\u00ebs s\u00eb eshtrave \u00ebsht\u00eb m\u00ebnyra e vetme p\u00ebr t\u00eb trajtuar k\u00ebt\u00eb s\u00ebmundje t\u00eb rrall\u00eb.<\/p>\n<p>Trajtimi i f\u00ebmij\u00ebve me histiocitoz\u00eb k\u00ebrkon nj\u00eb qasje multidisiplinare, respektivisht\u00eb angazhim t\u00eb<br \/>\nmjek\u00ebve me specializime t\u00eb ndryshme si: imunolog, hematolog, kirurg, ortoped, stomatolog,<br \/>\nspecialist p\u00ebr vesh ,hyund\u00eb dhe fyt, dermatolog, endokrinolog dhe t\u00eb tjer\u00eb.<\/p>\n\n<div style=\"font-size: 0px; height: 0px; line-height: 0px; margin: 0; padding: 0; clear: both;\"><\/div>","protected":false},"excerpt":{"rendered":"<p>Limfohisticitoza hemofagocitike MKB D76.1 Hemophagocytic lymphohistiocytosis Limfohistiocitoza hemofagocitike \u00ebsht\u00eb nj\u00eb grup s\u00ebmundjesh me origjin\u00eb makrofagale q\u00eb karakterizohet nga nj\u00eb rrjedh\u00eb [&hellip;]<\/p>\n","protected":false},"author":24,"featured_media":106151,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"_mo_disable_npp":"","inline_featured_image":false,"footnotes":""},"categories":[3085],"tags":[],"class_list":["post-106155","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-histori-te-parrefyera"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v28.6 - 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