I-hemophagocytic lymphohistiocytosis (HLH)

I-Hemophagocytic lymphohistiocytosis (HLH) yisimo esingavamile, esisongela ukuphila esibangelwa ukusebenza ngokweqile, ukusabela okungavamile kwamasosha omzimba. Amasosha omzimba ayisimiso somzimba sokuzivikela ezifweni zangaphandle noma ezihlaselayo noma izinto. Amasosha omzimba ayinethiwekhi eyinkimbinkimbi yamangqamuzana, izicubu, izitho zomzimba, namaprotheni asebenza ndawonye ukuze agcine umzimba unempilo. Ku-hemophagocytic lymphohistiocytosis, amasosha omzimba asabela esivuselelweni noma 'i-trigger', ngokuvamile ukutheleleka, kodwa impendulo ayisebenzi futhi ayijwayelekile. Le mpendulo engasebenzi, engavamile, idala izimpawu nezimpawu ezihlukahlukene, okuthi, uma zingelashwa, zibe sengozini yokuphila. Abanye abantu abathintekile bangase babe ne-genetic predisposition ekuthuthukiseni i-hemophagocytic lymphohistiocytosis. Lokhu kwaziwa njengefomu lokuqala noma lomndeni. Kwabanye abantu, lesi sifo siyenzeka ngezikhathi ezithile lapho kunesimo esicashile noma ukuphazamiseka okuthile. Lokhu kwaziwa njengefomu lesibili. Amafomu esibili avame kakhulu kunamafomu omndeni. I-Hemophagocytic lymphohistiocytosis ivamise ukuphatha izinsana kusukela ekuzalweni kuya ezinyangeni eziyi-18, kodwa ingathinta abantu banoma ibuphi ubudala. Ukuxilongwa kusenesikhathi nokwelashwa ngokushesha kubalulekile.

I-Hemophagocytic lymphohistiocytosis (HLH) yisimo esinezimbangela ezihlukene. Kunamagama amaningana asetshenziswa ukuchaza lesi simo. I-familial hemophagocytic lymphohistiocytosis (FHL) ibhekisela ezinhlotsheni zofuzo ezibangelwa ukwehluka okungavamile kofuzo. Kusukela ngo-Ephreli 2018, ukungahambi kahle ezakhini zofuzo eziningi kukhonjwe njengezimbangela. I-Macrophage activation syndrome (MAS) yigama elisetshenziselwa i-hemophagocytic lymphohistiocytosis eyenzeka kubantu abane-autoimmune noma isifo sokuvuvukala. Lolu wuhlobo lwe-HLH ​​yesibili. Izifo ezivame ukuhlotshaniswa ne-MAS i-juvenile systemic arthritis, i-adult-start Still's disease, kanye ne-systemic lupus erythematosus.

Izimpawu nezimpawu

Ukuqala kwe-hemophagocytic lymphohistiocytosis kanye nobukhulu besimo kungahluka kancane ukusuka komuntu oyedwa kuya komunye. Izimpawu ezithile ezizibonakalisayo nazo zingahluka kakhulu, nakuba isimo ngokuvamile siholela ekubandakanyekeni kwezitho eziningi. Ezimweni eziningi, labo abangenwe yileli gciwane bazoba nemfiva, ukuqubuka, i-hepatomegaly (isibindi esikhule ngendlela engavamile), kanye ne-splenomegaly (ubende olukhuliswe ngendlela engavamile) (splenomegaly). Umkhuhlane omude futhi ophikelelayo, ezimweni eziningi, ongaphenduli ekwelashweni kwama-antibiotic kungenzeka. Kukhona futhi izimo lapho ama-lymph nodes amakhulu ngokungavamile (i-lymphadenopathy). I-lymphatic system iyinethiwekhi yokujikeleza eyakhiwe yimikhumbi, imigudu, namanodi ahlunga futhi asabalalise amaprotheni athile (i-lymph) namangqamuzana egazi ezingxenyeni ezihlukahlukene zomzimba. Ama-lymph nodes ayingxenye yesistimu ye-lymphatic. Ama-lymph nodes ayizakhiwo ezincane ezingatholakala ngamaqoqo kuwo wonke umzimba. Badlala indima enqubweni yokuhlunga noma ukukhipha izinto eziyingozi emzimbeni.

Lezi zimpawu zokuqala nezimpawu zibizwa ngokuthi aziqondile. Lokhu kusho ukuthi lezi zimpawu nezimpawu zivamile ezinhlobonhlobo zezinye izifo noma izimo, ezingenza kube inselele ukuthola ukuxilongwa okunembile.

Labo abanalesi sifo bangase futhi baphathwe amazinga aphansi ajikelezayo amangqamuzana abomvu egazi (i-anemia), kanye namazinga aphansi e- platelet ajikelezayo ( thrombocytopenia ). Ama-platelet anesibopho sokuvala igazi nokumisa ukopha, kuyilapho amangqamuzana egazi abomvu anesibopho sokuthwala umoya-mpilo kuwo wonke umzimba. I-anemia ingabangela umuntu ukuba azizwe ekhathele, abe nesidingo esandayo sokulala, ukuba buthakathaka, ikhanda elilula, isiyezi, ukucasuka, ikhanda elibuhlungu, ibala lesikhumba eliphaphathekile, ukuphefumula kanzima (dyspnea), kanye nezimpawu zenhliziyo. I-anemia ingabangela ukushaya kwenhliziyo yomuntu ngendlela engavamile. Abantu abane-thrombocytopenia kungenzeka ukuthi bathole imihuzuko eyeqile ngemva kokulimala okuncane nokopha okuzenzakalelayo olwelwesini lwamafinyila, ikakhulukazi lelo lezinsini nekhala.

Izimpawu eziningi zemizwa, njengokudlikizela, ukuguqulwa kwesimo sengqondo nokucasuka, ukukhubazeka (ukukhubazeka) kwezinzwa ezithile ze-cranial, nobunzima bokuxhumanisa ukunyakaza kokuzithandela, kungenzeka ku-subset yalabo abathintekayo isimo (i-ataxia). Abantu abathintekayo yilesi simo basengozini yokuthola i-posterior reversible encephalopathy syndrome, ebonakala ngokuphathwa yikhanda kungazelelwe, ukushintsha kokwazi, ukuquleka, nokuphazamiseka kokubona. Ezigulini ezinomndeni we-hemophagocytic lymphohistiocytosis, izinkinga zemizwa cishe zihlala zikhona.

Kuye ngokuthi yisiphi isimiso sezitho ezithintekayo kumuntu othile, umuntu angase futhi abe nalezi zimpawu ezengeziwe ezengeziwe: Lezi zimpawu zingabandakanya ubunzima obukhulu bokuphefumula (ukungasebenzi kahle kwamaphaphu), umfutho wegazi ophansi kakhulu (hypotension), ukuvuvukala kwesibindi (isibindi), ukungasebenzi kahle kwezinso. , isikhumba sibe phuzi nokumhlophe kwamehlo (i-jaundice), ukuvuvukala ngenxa yokunqwabelana koketshezi (i-edeema), ukuvuvukala kwesisu ngenxa yokunqwabelana koketshezi (ama-ascites), nezinkinga ezihlukahlukene zesikhumba ezihlanganisa ukunwebeka okubomvu kwesikhumba ngenxa yokuvuvukala ( erythroderma ), ukuqubuka, amabala egazi (i-purpura), i-alopecia, ne-alopecia (petechiae).

Izimbangela

Izinhlobo eziyinhloko nezesibili (ezitholiwe) ze-hemophagocytic lymphohistiocytosis ziyizigaba ezimbili eziyinhloko zalesi simo. Lesi simo siwumphumela wokugcina wokusabela okungavamile kwesimiso somzimba sokuzivikela ezifweni kanye nokungasebenzi kahle kwesisusa, esaziwa nangokuthi “i-trigger.” Izindlela eziyisisekelo eziholela ekukhuleni kwezimpawu nezimpawu ziyinkimbinkimbi kakhulu. Kukhona ukwanda kukho kokubili ukukhiqizwa nokusebenza kwamangqamuzana amasosha omzimba aziwa ngokuthi ama-hitiocyte nama-T cell. Lezi yizinhlobo ezahlukene zamangqamuzana amhlophe egazi atholakala emzimbeni womuntu. Amangqamuzana amhlophe egazi angamangqamuzana ayinhloko akha amasosha omzimba futhi asize umzimba ekulweni nezifo.

Ama-Histiocyte, abuye abizwe ngokuthi ama-macrophages, amangqamuzana amakhulu e-phagocytic avame ukubamba iqhaza ekuphenduleni kwamasosha omzimba ekulimaleni nasekuthelelekeni. Noma yiliphi iseli elisebenza “njengomkhohlisi” ngokungenisa ama-microorganisms angaphandle noma udoti wamaselula bese liwabhubhisa laziwa ngokuthi i-phagocytic. Ngaphezu kwalokhu, ama-macrophage anesibopho sokukhishwa kwama-cytokines, okungamaprotheni angashukumisa noma avimbele ukusebenza kwamanye amangqamuzana amasosha omzimba futhi akhuthaze ukuvuvukala ekuphenduleni ukugula. Ukukhiqizwa ngokweqile kwama-cytokines, ngokuhamba kwesikhathi, kuzoholela ekulimaleni okukhulu kwezicubu. Esimeni esaziwa nge-hemophagocytosis, ama-macrophage angaba nephutha eliwenza adle futhi acekele phansi izicubu ezinempilo, okuhlanganisa namaseli egazi anempilo. Ama-lymphocyte e-cytotoxic, akhiwe ngama-T cell namaseli abulalayo emvelo, awasebenzi ngokugcwele. Lawa maseli aqeda noma yimaphi amanye amangqamuzana angenampilo, acindezelekile, noma angenwe yigciwane. I-HLH ibonakala ngokungakwazi kwama-cytotoxic lymphocyte ukuqeda ama-macrophages acushiwe, okuholela ekuqoqweni okungavamile kwalawa maseli ezithweni nasezicutshini zomzimba. Lokhu kunqwabelana okungavamile kuqhubezela phambili le mpendulo engasebenzi kahle yamasosha omzimba. Lokhu kungahambi kahle kwamasosha omzimba kuyimbangela yezimpawu eziphawulekayo zalesi simo, okuwukuvuvukala okudlulele kanye nokonakala kwezicubu.

I-Hemophagocytic Lymphohistiocytosis Eyisisekelo

Ifomu eliyinhloko lixhunyaniswe nokwahluka okungavamile ezakhini zofuzo eziningana. Iziqondiso zokwenza amaprotheni, abandakanyeka ezinhlobonhlobo zezinqubo emzimbeni wonke, aqukethwe ngaphakathi kwezakhi zofuzo. Lapho isakhi sofuzo sishintsha, umkhiqizo wamaprotheni owumphumela ungase untule, ungabi khona, ukhiqizwe ngokweqile, noma uguqulwe ngandlela thize. Lokhu kungaba nomthelela ezingxenyeni eziningi zomzimba nezinhlelo, kuye ngemisebenzi eyenziwa yiprotheni okukhulunywa ngayo.

Okungenani izakhi zofuzo ezine ezihlukene ziye zatholakala zibangela i-genetic predisposition ukuze kuthuthukiswe i-hemophagocytic lymphohistiocytosis. Lokhu kuthambekela kofuzo kungadluliselwa kusuka kubazali kuya ezinganeni. Umuntu unezakhi zofuzo zesifo esithile uma ephethe isakhi sofuzo esisodwa noma ngaphezulu salokho kuphazamiseka. Kodwa-ke, lesi sifo ngeke sizibonakalise kulowo muntu ngaphandle uma kunezinye izici ezisiza ukuvusa lesi sifo. Izakhi zofuzo ezine i-PRF1 (yohlobo 2 lwe-hemophagocytic lymphocytosis), i-UNC13D (yohlobo lwe-3 ye-hemophagocytic lymphocytosis), i-STX11 (yohlobo 4 lwe-hemophagocytic lymphocytosis) kanye ne-STXBP2 (yohlobo 2 lwe-hemophagocytic lymphocytosis). (uhlobo 5 lwe-hemophagocytic lymphocytosis). Okwamanje akukanqunywa ukuthi yisiphi isakhi sofuzo esinesibopho sohlobo loku-1 lwe-hemophagocytic lymphocytosis.

Lezi zakhi zofuzo zinesibopho sokukhiqiza amaprotheni adlala indima ebalulekile ohlelweni lokuzivikela komzimba. Banengxenye okufanele bayidlale ekuvaleni noma ekucekeleni phansi amaseli omzimba asebenze uma amasevisi abo engasadingeki. Lokhu kuhlukahluka (ukuguqulwa) kulezi zakhi zofuzo kubangela ukuthi izakhi zofuzo zikhiqize amanani anganele ephrotheni noma zikhiqize izinguqulo zephrotheni ezingasebenzi kahle. Ngenxa yalokhu, amangqamuzana amasosha omzimba acushiwe okufanele acishwe noma abhujiswe ngesikhathi esijwayelekile aqhubeka ekhona futhi enze imisebenzi yawo, agcine edala umonakalo kumaseli nezicubu ezinempilo.

Inhlanganisela yezakhi zofuzo zesici esithile esiqukethwe kuma-chromosome atholakala kunina nakubaba yikho okunquma ukuthi umuntu uzoba nesifo sofuzo noma cha. Uma umuntu ezuza isakhi sofuzo esifanayo sesici esifanayo kubazali bakhe bobabili, lokhu kungaholela ekuthuthukisweni kokuphazamiseka okuphindaphindekayo kulowo muntu. Uma umuntu ethola isakhi sofuzo esisodwa esivamile kanye nofuzo olulodwa olubangela lesi sifo, khona-ke lowo muntu uzoba umthwali walesi sifo kodwa ngokuvamile ngeke abonise noma yiziphi izimpawu zokuba nalesi sifo. Ekukhulelweni ngakunye, kunamathuba angamaphesenti angu-25 okuthi abathwali ababili bazodlulisela isakhi sofuzo esinesici enzalweni yabo futhi, ngenxa yalokho, babe nengane ethintekayo kulesi simo. Ngokukhulelwa ngakunye, kunamathuba amaphesenti angamashumi amahlanu okuba nengane enesimo esifanayo nesabazali. Kukhona ithuba elilodwa kwamane lokuthi ingane izozuza izakhi zofuzo ezivamile kubo bobabili abazali bayo futhi ibe evamile ngokokwakheka kwayo kofuzo kuleso sici. Kokubili abesilisa nabesifazane bachayeka ezingeni elifanayo lengozi.

Ama-heterozygote ayinhlanganisela abantu abanezinhlobonhlobo ezihlukene ezithinta ikhophi ngayinye yezakhi zofuzo zesifo, kanti ifa le-digenic libhekisela kubantu abathola lesi sifo kubazali babo bobabili. Banokwehluka okungavamile ezakhini zofuzo ezimbili ezihlukene ezaziwa ngokuthi zihlotshaniswa ne-hemophagocytic lymphohistiocytosis, okuyilokho okushiwo igama elithi “digenic inheritance.”

Abantu abathintwe yilesi simo bangase kutholakale ukuthi bane-hemophagocytic lymphohistiocytosis njengophawu lokuphazamiseka okubanzi kofuzo. Lezi zimo zihlanganisa i-Hermansky-Pudlak syndrome, ukungabekezelelani kwe-lysinuric protein, i-Griscelli syndrome uhlobo 2, i-Chediak-Higashi syndrome, i-X-linked lymphoproliferative disorder, isifo se-XMEN, ukuntuleka kwe-interleukin-2-inducible T cell kinase, ukuntuleka kwe-CD27, kanye ne-interleukin-2-inducible Ukuntuleka kwe-T cell kinase. Ezinye izimo zihlanganisa Kungenzeka ukuthi i-hemophagocytic lymphohistiocytosis ukuphela kwenkinga yomtholampilo abanye abantu abanalezi zinkinga ababhekana nayo.

I-Lymphohistiocytosis yesibili ye-Hemophagocytic

Abantu abane-hemophagocytic lymphohistiocytosis yesibili (noma abathole) bahlakulela lesi sifo ngenxa yempendulo eyihaba nengavamile evela kumasosha omzimba wabo, okwenzeka ngezizathu ezingaqondakali ngokugcwele. Awukho umlando walesi simo emndenini wesiguli, futhi azikho izici zofuzo ezihlonzayo ezitholakele. Izimo ezifana nezifo ezibangelwa amagciwane, ikakhulukazi igciwane le-Epstein-Barr, ezinye izifo ezihlanganisa amagciwane, amagciwane kanye nesikhunta, amasosha omzimba abuthakathaka noma acindezelekile, izifo ezizimele, izifo ezizimele, izifo ze-rheumatological ezifana ne-juvenile idiopathic arthritis, ukuphazamiseka kwe-metabolic, kanye nomdlavuza onjalo. njenge i-non-Hodgkin lymphoma konke kungaholela ku-hemophagocytic lymphohistiocytosis yesibili.

Ku-hemophagocytic lymphohistiocytosis, indlela enembayo lapho lezi zimo ezicatshangelwayo zibangela ngayo izimpawu nezimpawu, futhi ikakhulukazi ukuthi zibangela kanjani ukuphendula kokuzivikela komzimba okungasebenzi nokungajwayelekile, akuqondakali ngokugcwele. Lesi ngesinye sezizathu ezenza kube nzima ukuselapha lesi sifo.

Sifo 

Ukunqunywa kokuxilongwa kudinga ukubonwa kwezimpawu ezihlukile, ukuhlanganiswa komlando wesiguli ophelele, ukuhlolwa komtholampilo okuphelele, kanye nokuphathwa kokuhlolwa okukhethekile okuningi. Indlela yokuxilonga ye-hemophagocytic lymphohistiocytosis ibekwe ngemininingwane egcwele kusethi yemihlahlandlela eshicilelwe. Uma izimpawu ezinhlanu kweziyisishiyagalombili ezilandelayo zikhona, ukuxilongwa komtholampilo kungenziwa. Uma zonke izimpawu eziyisishiyagalombili zikhona, ukuxilongwa komtholampilo ngeke kwenziwe. Lezi zimpawu eziyisishiyagalombili umkhuhlane, i-spleen enkulu ngokungavamile (splenomegaly), i-red cell ephansi, i-white cell, noma amazinga e-platelet (cytopenias), amazinga aphezulu ngokungavamile ohlobo lwamafutha egazini okuthiwa i-triglyceride (hypertriglyceridemia), noma amazinga aphansi ngokungavamile. iphrotheni ethile yokujiya kwegazi (hypofibrinogenemia); Ukubhujiswa kwamangqamuzana egazi ngama-macrophages (hemophagocytosis).

Ngenxa yokuthi izimpawu ze-hemophagocytic lymphohistiocytosis azicacisi ikakhulukazi, kuvamile ukuthi abantu abathintekile bahlaselwe ukugula isikhathi eside futhi balaliswe esibhedlela ngaphambi kokuba bathole ukuxilongwa.

Amazinga amangqamuzana abomvu, amangqamuzana amhlophe, namaplatelet konke kungalinganiswa ngenani eliphelele lamangqamuzana egazi, elingalinganiswa ngokuhlolwa kwegazi okuyalwe udokotela wakho. Izinga eliphezulu ngokungavamile le-ferritin, kanye nezinga le-triglyceride eliphezulu ngokungavamile, kungatholwa kokubili ngokuhlolwa kwegazi. Ngaphezu kwalokho, odokotela bangasebenzisa ukuhlolwa kwegazi ukuze bafune izinkomba zokutheleleka egazini futhi bangase benze izivivinyo ukuze bahlole ukusebenza kahle kwegazi elihlangana ngakho (izifundo ze-coagulation). Ukuhlola okuhlola impilo nokusebenza kwesibindi kungolunye uhlobo lokuhlola odokotela abangaluyala.

Ama-biopsies omnkantsha wamathambo, ahlanganisa ukukhishwa ngokuhlinzwa kwesampula yethishu kanye nokuhlolwa kwawo okulandelayo ngaphansi kwesibonakhulu, ngezinye izikhathi kwenziwa ukuze kuphenywe ubukhona be-hemophagocytosis, izimpawu zokutheleleka noma izinto eziphilayo ezithathelwanayo, kanye nokunqwabelana kwama-macrophages.

Kubantu abathile, ukuxilongwa kwe-hemophagocytic lymphohistiocytosis kungaqinisekiswa ngokusebenzisa ukuhlolwa kofuzo lwamangqamuzana. Ukuhlolwa kofuzo lwamangqamuzana okukwazi ukubona ukuguquka kwezakhi zofuzo eyodwa kwezine ezaziwa ngokubangela izinhlobo zomndeni zalesi sifo kunikezwa kuphela njengesevisi yokuxilonga kumalabhorethri akhethekile. Nokho, lokhu kuhlola kungathola ukuguqulwa kofuzo olulodwa.

Ukwelashwa 

Izimpawu eziqondile ezibonakala kumuntu ngamunye othintwa i-hemophagocytic lymphohistiocytosis yilokho ukwelashwa kwalesi simo okugxile kukho. Kungenzeka ukuthi ukwelashwa kuzodinga imizamo ehlangene yeqembu lochwepheshe. Ukwelashwa kungase kudingeke ukuthi kuhlelwe ngendlela ehlelekile futhi ehlanganisa yonke into ngodokotela bezingane, odokotela begazi, odokotela bezifo ezibangelwa amagciwane, odokotela bamasosha omzimba, izazi zofuzo (ngokwezinhlobo zemindeni), osonhlalakahle, futhi mhlawumbe nabanye ochwepheshe bezokwelapha. Ukwengeza okudingekayo ukuhlinzekwa kokusekelwa ngokwengqondo komndeni wonke. Ukwelulekwa ngokofuzo kungase kube usizo kubantu abathintekile kanye nasemindenini yalabo bantu.

Kungenzeka ukuthi izinqubo ezithile zokwelapha kanye nokungenelela kuhluke, kuye ngezinhlobonhlobo zezakhi, njengembangela eyinhloko; ukuba khona noma ukungabi khona kwezimpawu ezithile; ubunzima obuphelele bezimpawu kanye nokuphazamiseka; iminyaka yomuntu nempilo jikelele; kanye/noma ezinye izici. Izinqumo eziphathelene nokusetshenziswa kwemithi ethile kanye/noma ezinye izindlela zokwelapha kufanele zenziwe odokotela namanye amalungu ethimba lokunakekelwa kwezempilo ngokubonisana ngokucophelela nesiguli. Lezi zinqumo kufanele zisekelwe emininingwaneni yecala lesiguli, kanye nengxoxo ebanzi yezinzuzo ezingase zibe khona kanye nezingozi, kuhlanganise nokwenzeka kwemiphumela emibi kanye nemiphumela yesikhathi eside. Ezinye izici ezifanele nazo kufanele zicatshangelwe.

Abantu abahlukunyezwe yilesi simo futhi impilo yabo ejwayelekile yanele bangakwazi ukuthola ukwelashwa ngesimo esikhona. Lokhu kungase kuhlanganise ukuphuza ama-antibiotics ukwelapha isifo esiwumsuka, noma ukuthola ukwelashwa okufanele kwezifo ezizimele noma umdlavuza. Uma isimo esiyisisekelo selashwa, kungenzeka ukuthi "i-trigger" ebangele ukusabela okungavamile kwamasosha omzimba nayo izosuswa.

Abantu abathintekile futhi impilo yabo iwohloka ngokushesha badinga ukwelashwa ngemithi eqondile ye-hemophagocytic lymphohistiocytosis. Izincomo zokwelashwa zalesi sifo zanyatheliswa okokuqala yi-Histiocyte Society ngonyaka we-1994. (HLA-94). Kuphinde kwaba khona izifundo ezashicilelwa ngo-2004 (HLA-2004) ezaveza imiphumela ehluke kancane.

Lezi zinhlelo zokwelapha zibandakanya ukusetshenziswa kwe-chemotherapy kanye nemithi edambisa ukusebenza kwamasosha omzimba (izidakamizwa ezivimbela ukuzivikela komzimba). Aqondisa ngokuqondile futhi abhubhise amangqamuzana amasosha omzimba asebenza ngokweqile, nawo asiza ekwehliseni ukuvuvukala okungaba yingozi okuwuphawu lwe-hemophagocytic lymphohistiocytosis.

Ngemuva kwenkambo yokuqala yokwelashwa evamise ukuthatha phakathi kwamaviki ayisi-6 nayisi-8, iziguli zibe sezishintshelwa kancane kancane zisuke emithini ebeziyidla ziyiswe kweminye imithi. Uma labo abaphethwe yilesi simo bengabonisanga ukusabela okuhle kulokhu kwelashwa, kungase kuphakanyiswe ukufakelwa kwe-stem cell allogeneic. Abantu abanokwehluka okungajwayelekile kufuzo olwaziwayo lwe-HLH, ukubandakanyeka kwesistimu yezinzwa emaphakathi, noma okungalapheki umdlavuza wegazi (i-hematologic malignancy) nabo bangamakhandidethi alokhu kwelashwa, okunconyelwe bona.

I-allogeneic stem cell transplant iyinqubo lapho ama-stem cells esigulini esiphethwe isifo athathelwa indawo ama-stem cells avela kumnikeli ophile kahle futhi onezakhi zofuzo ezifanayo nesiguli. Ama-stem cell angamaseli akhethekile angatholakala emnkantsheni futhi anesibopho sokukhiqiza izinhlobo ezahlukene zamaseli egazini (isb. amangqamuzana egazi abomvu, amangqamuzana egazi amhlophe, ama-platelet).

Iziguli ezinalesi simo zinikezwa imithamo ephezulu kakhulu ye-chemotherapy noma ngemisebe emzamweni wokuqeda ama-stem cells. Okulandelayo, ama-stem cells avela kumnikeli azothatha indawo yawo emzimbeni wesiguli. Inqubo yokufakelwa kwe-stem cell allogeneic yileyo ethwala izinga eliphezulu lengozi futhi inamandla okubangela imiphumela emibi.

Ngenxa yokuthi banamazinga aphansi amangqamuzana abomvu egazi ajikelezayo noma ama-platelet, abanye babantu abathintekile bangase badinge ukumpontshelwa igazi. Imithi elwa namagciwane ingenye yezindlela odokotela abangase beluleke iziguli zabo ukuba ziyisebenzise ukuvimbela ukuqala kokutheleleka (ukwelashwa kwe-prophylactic).

Ukwelashwa kwezingane kanye neziguli zabantu abadala ezine-HLH ​​eyinhloko enesifo esiphikisayo, esiphindaphindayo, noma esiqhubekayo noma ezingakwazi ukubekezelela ukwelashwa okuvamile kwe-HLH ​​kuvunyiwe ukuze kusetshenziswe i-Gamifant (emapalumab) ngo-2018. USobi uzophatha i-Gamifant yokumaketha.