Nukawoe nye ƒunukpeƒetetedɔ siawo siwo le kɔ kple akɔta? Mina míasrɔ̃ nu tso Rosai-Dorfman Dɔlélea ŋu!

Enye nusi sɔ be míavɔ̃ vie ne míekpɔ tɔtrɔwo le míaƒe ŋutilã me, alo? Vevietɔ ne míekpɔ ƒuƒu suesuesuewo le míaƒe kɔ, abɔta, alo akɔta la, míesusuna be, ‘Nukae nye esia?’ Egbea míele nu ƒom tso nɔnɔme aɖe si mebɔ o si ate ŋu ahe ƒunukpeƒetetedɔ mawo tɔgbe vɛ ŋu, gake ame geɖe mesee kpɔ o. Woyɔa esia be Rosai-Dorfman Dɔléle.

Ènya nusi Ross-Dorffman ƒe dɔléle (RDD) nyea?

Kpuie ko la, ʋumenugbagbevi ɣi ƒomevi aɖe si woyɔna be histiocytes le míaƒe ŋutilã me. Amesiawo le abe kpovitɔ siwo le míaƒe ŋutilã me ene, wole avu wɔm kple dɔlékui siwo hea dɔléle vɛ, eye wonye míaƒe dɔlélenutsiŋutete ƒe akpa vevi aɖe. Gake ne lãmenugbagbevi siawo tsi akpa la, woyɔnɛ be Ross-Dorffmann dɔléle (RDD). Esia menye kansa (benign) ŋutɔŋutɔ o , si fia be menye dɔléle si me afɔku le si kaka tso ŋutilã ƒe akpa ɖeka yi bubu me o. Gake ne lãmenugbagbevi siawo ƒo ƒu wògbɔ eme la, míate ŋu akpɔ vevesese kple dzesiwo.

Zi geɖe la, histiocyte siawo siwo sɔ gbɔ la ƒoa ƒu ɖe ʋuka siwo le míaƒe kɔ me la me. Emegbe wovuvuna abe ƒuƒu le kɔ me ene. Ðɔktawo yɔa ƒunukpeƒewo ƒe vuvu sia ɣeaɖewoɣi be `(lymphadenopathy).` Gake menye kɔ koe ate ŋu agblẽ o, ke ate ŋu agblẽ nu le lãkusi siwo le ŋutilã ƒe akpa bubuwo hã ŋu. Ɣeaɖewoɣi la, tsɔ kpe ɖe lãmenugbagbevi siawo ŋu la, woate ŋu aƒo ƒu ɖe ŋutilã ƒe akpa bubuwo hã (extranodal sites).

Wogayɔa dɔléle sia be `sinus histiocytosis with massive lymphadenopathy`. Esia nye dɔléle si mebɔ o si le ƒuƒoƒo si nye `non-Langerhans cell histiocytosis` me.

Nukawoe nye Ross-Dorffmann dɔléle (RDD) ƒomevi veviwo?

Dɔléle sia medzea amesiame dzi sɔsɔe o. Ame aɖewo vuvu le teƒe ɖeka, evɔ ame bubuwo ya ate ŋu avuvu le teƒe geɖe. Azɔ hã, menye ƒunukpeƒetetedɔ siawo me koe lãmenugbagbevi siawo ate ŋu aƒo ƒu ɖo o, ke woate ŋu aƒo ƒu ɖe ŋutilã ƒe akpa bubuwo hã. Aleae wodae ɖe hatsotsowo me.

• Ross-Dorffmann Dɔléle si nye Classic (Nodal) (Classic/Nodal RDD): Esiae nye ƒomevi si bɔ wu. Nusiwo koŋ wòlɔna ɖe kɔa ƒe lãkusiwo ƒe vuvu ŋu. Gake dzesiawo ate ŋu ato vovo le lãkusi agbɔsɔsɔme si ŋu wògblẽ nu le nu.
• Extranodal RDD: Le ƒomevi sia me la, histiocyte siawo ƒoa ƒu ɖe lãkusi bubu siwo menye ʋuka siwo me ʋu sina tona o me. Ŋutilãa ŋue wògblẽa nu le wu. Woyɔa esia be ŋutigbalẽ ƒe RDD (CRDD) . Gakpe ɖe eŋu la, nɔnɔmea ateŋu akpɔ ŋusẽ ɖe míaƒe lãkusiwo, ŋkuwo kple ŋkume, ƒuwo, kple titina lãmekawo (CNS) – si nye ahɔhɔ̃ kple aklã dzi. Ɣeaɖewoɣi la, ate ŋu agblẽ nu le gbɔgbɔtsixe kple dɔgbo ƒe dɔwɔwɔ hã ŋu.

Ame siwo ŋu RDD le la dometɔ siwo ade 40% ate ŋu akpɔ histiocyte lãmenugbagbevi siawo siwo woƒo ƒu ɖe ŋutilã ƒe akpa bubuwo, tsɔ kpe ɖe ʋuʋudediwo ŋu, abe alesi nègblɔe ene.

Amekae ate ŋu axɔ dɔléle sia wu?

Ross-Dorffmann ƒe dɔlélea dzea ɖeviwo, ƒewuiviwo, kple sɔhɛ tsitsiwo koŋ dzi . Zi geɖe la, wokpɔnɛ le amesiwo xɔ ƒe 20-awo me. Ke hã, wogblɔ be dɔlélea le ame siwo xɔ ƒe 70 kple edzivɔ hã ŋu.

RDD, si gblẽa nu le vidzidɔ ŋu la bɔ ɖe ŋutsu siwo tso Afrika me wu. Gake ŋutigbalẽ ƒe nɔnɔme si míeyɔ va yi ``CRDD`` bɔ ɖe nyɔnu siwo tso Asia me wu. ``CRDD`` bɔ ɖe amesiwo le woƒe ƒe 20, 30, kple 40 me wu.

Aleke gbegbee Ross-Dorfman ƒe dɔlélea mebɔ o?

Esia nye dɔléle si mebɔ kura o . Edzea ame siwo ade ɖeka le akpe alafa eve ɖesiaɖe me dzi. Le kpɔɖeŋu me, le United States la, wokpɔa ame yeye siwo ade 100 ƒe sia ƒe. Ema fia be le Sri Lanka la, eɖiɖi wu gɔ̃ hã.

Nukae nye Rose-Dorfman dɔléle (RDD) ƒe dzesiwo?

Dzesi siwo nàkpɔ la anɔ te ɖe afisi histiocyte siwo sɔ gbɔ wu la ƒoa ƒu ɖo le wò ŋutilã me dzi. Ne ʋuka siwo le wò kɔ me koe wògblẽ la, wò dzesiwo ate ŋu anɔ bɔbɔe alo womanɔ anyi kura gɔ̃ hã o. Gake ne lãmenugbagbevi siawo ƒo ƒu hedo kplamatse ŋutinu aɖe ƒe dɔwɔwɔ la, wò dzesiwo ate ŋu asẽ wu.

Dzesi siwo wonya nyuie (nodal).

Zi geɖe la, histiocytes ƒoa ƒu ɖe kɔ ƒe lãkusiwo me. Eyata dzesi si bɔ wue nye ƒuƒu siwo me vevesese mele o siwo vuvu le kɔ ƒe akpa eveawo . Ðewohĩ àɖo ŋku alesi ɣeaɖewoɣi la, ʋuʋudedi sue aɖe va nɔa wò kɔ me ne dzamezã alo dzamezã le ŋuwò dzi, alo? Esɔ, gake ɖewohĩ mavee o. Gake le afisi lãkusi si ŋu dɔlélea le nu la, abi la ate ŋu anɔ teƒe bubuwo hã.

Nu vevi siwo me dɔdzẽ ate ŋu adzɔ le le histiocytes ƒe dzidziɖedzi tae nye:

• Wò kɔ
• Groin ƒe nuto
• Akɔtaƒuwo
• Akɔta ƒe titina (mediastinum) .

Ðewohĩ màde dzesi dzesi bubu aɖeke tsɔ wu abi, alo àde dzesi dzesi siwo le abe esiawo ene:

• Asrã
• Ŋutilã si ƒe ŋutigbalẽ le ɣie
• Ðeɖiteameŋu si gbɔ eme
• Zã me fifia
• No me si le sisim
• Kpekpeme dzi ɖeɖe kpɔtɔ si womeɖe o

Extranodal ƒe dzesiwo

Rose-Dorffmann dɔléle (`CRDD`) ateŋu adze ŋutigbalẽ dzi eye ateŋu adze le ŋutilã ƒe akpa sia akpa. Zi geɖe la, ƒunukpeƒe siawo siwo histiocyte lãmenugbagbeviwo ƒe ƒuƒoƒo wɔ la tsina blewu. Dzesi siwo woate ŋu akpɔ le ŋutigbalẽa ŋu dometɔ aɖewoe nye:

• Kpe gbadzɛwo alo esiwo kɔ ɖe dzi
• Kpe siwo me ʋuʋudedi yɔ fũ alo esiwo sesẽ
• Aŋɔ ɣi, aŋutiɖiɖi, dzĩ, alo aŋutiɖiɖi
• Ate ŋu akaka ɖe ŋutigbalẽa katã dzi alo wòakaka ɖe ŋutigbalẽa ƒe akpa ɖeka ko.

Ne histiocyte siawo siwo sɔ gbɔ la gblẽ nu le ŋutinu alo ŋutilã ƒe ɖoɖo aɖe koŋ ŋu la, dzesi vovovowo ate ŋu ado mo ɖa. Le kpɔɖeŋu me, ne RDD gblẽ nu le ŋkuwo ŋu la, ate ŋu ana woakpɔ nu zi eve. Ne egblẽ nu le lãmeka siwo le titina ŋu la, ate ŋu ana ame naxɔ alɔ̃. Ne edze lãkusiwo dzi la, ate ŋu ana ame natsi akɔta atraɖii. Nenema ke dzesiawo hã toa vovo le teƒe si dɔlélea le nu.

Nukae hea Ross-Dorffman ƒe dɔléle (RDD) vɛ?

Dzɔdzɔmeŋutinunyalawo menya nusi tututu gbɔ dɔléle sia tso haɖe o . Esi wònye be RDD gblẽa nu le ame ɖesiaɖe ŋu le mɔ vovovowo nu ta la, nu vovovowo ate ŋu atso eme. Le kpɔɖeŋu me, numekuku siwo wowɔ nyitsɔ laa ɖee fia be ŋutigbalẽ ƒe nɔnɔme ``CRDD`` tso nane si menye nu mawo ke siwo hea RDD edziedzi vɛ gbɔ o.

Numekulawo kpɔe nyitsɔ laa be domenyiŋusẽfianu ƒe tɔtrɔ aɖewo le RDD ƒomevi bubuwo me tsɔ kpe ɖe CRDD ŋu. Dzɔdzɔmeŋusẽ ƒe tɔtrɔ siawo ate ŋu ana lãmenugbagbeviwo natsi le mɔ si dzi womate ŋu aɖu o nu.

Dɔléle sia le ame geɖe siwo ŋu RDD le ŋu kpe ɖe atikewɔwɔ me dɔléle bubuwo ŋu. Eyata, kadodo ate ŋu anɔ nɔnɔme mawo kple RDD dome. Numekuku geɖe hiã le esia ŋu.

Wokpɔe be RDD do ƒome kple nɔnɔme siwo gbɔna:

• Dɔlékuiwo ƒe dɔlékuiwo: Kpɔɖeŋu aɖewoe nye herpes, Epstein-Barr dɔlékui, cytomegalovirus, kple HIV dɔlékui.
• Kansa: Hodgkin lymphoma, non-Hodgkin lymphoma, kple ŋutigbalẽ me kansa abe cutaneous clear-cell sarcoma ene. (Gake ɖo ŋku edzi be menye kansae RDD nye o.)
• Dɔlélenutsiŋutete ƒe nɔnɔmewo: Lupus, Ðeviwo ƒe Ƒunukpeƒetetedɔ, Ʋusɔgbɔdɔ si Dɔlélenutsiŋutete ƒe Ʋusɔgbɔdɔ.

Aleke wokpɔa Ross-Dorffman ƒe dɔléle (RDD) dzea sii?

Wò ɖɔkta awɔ wò ŋutilã dodokpɔ gbã eye wòabia wò dzesiwo. Woadi RDD ƒe dzesiwo, abe lãkusi siwo vuvu kple ŋutigbalẽ ƒe ƒuƒu ene. Woabia wò atikewɔwɔ ŋutinya hã be woakpɔe ɖa be dɔléle aɖewo le ŋuwò alo do ƒome kple RDD hã.

Tsɔ kpe ɖe eŋu la, ɖɔkta ate ŋu awɔ dodokpɔ siawo hã:

• Nɔnɔmetatawo ɖeɖe ƒe mɔnuwo: Le lãkusi ƒomevi si wosusu be histiocytes le nu la, ɖɔkta ate ŋu abia be woawɔ X-ray, ultrasound, MRI, CT, PET, PET/CT, alo ƒu ƒe fotoɖeɖe. Woate ŋu azã esiawo atsɔ akpɔ nusiwo le ŋutilãa me.
• Ʋudodokpɔ: Woate ŋu awɔ ʋudodokpɔ geɖe, abe ʋuxexlẽ blibo (CBC) kple lãmenugbagbeviwo ƒe dɔwɔwɔ ŋuti ɖoɖo si me kɔ nyuie ene, be woakpɔ tɔtrɔ siwo le edzi yim le ŋutilãa me.
• Biopsy: Esia nye dodokpɔ vevi aɖe ŋutɔ.Esia bia be woaɖe lãmenugbagbevi siwo ŋu dɔlélea le ƒe kpɔɖeŋu sue aɖe (e.g., ʋuka si vuvu) eye woado lãmenugbagbeviawo kpɔ le nusuekpɔmɔ̃ me akpɔe ɖa be RDD ƒe nɔnɔmewo le wo ŋu hã. Biopsy sia ate ŋu anya ne dɔléle bubu aɖee le lãmenugbagbeviwo ƒe tsitsi si mesɔ o hem vɛ hã.

Aleke wodaa Ross-Dorffmann ƒe dɔléle (RDD)?

Ɣeaɖewoɣi la, RDD ate ŋu ayi ɖe edzi le eɖokui si atike aɖeke mawɔmawɔe. Esia fia be dɔlélea anyo le eɖokui si. Ke hã, ɣeyiɣi si wòaxɔ la gbɔgblɔ ɖi sesẽ. Ate ŋu axɔ ɣleti geɖe alo ƒe geɖe gɔ̃ hã hafi eƒe lãme nasẽ. Ke hã, le go aɖewo me la, ate ŋu adzɔ be madzo le eɖokui si o, alo ate ŋu atrɔ ava. Ne womedae o la, dɔlélea ate ŋu agblẽ ɖe edzi.

Atike si nàxɔ anɔ te ɖe alesi RDD kpɔ ŋusẽ ɖe dziwòe dzi.

• Kpɔkpɔ: Ne dzesi siwo le ŋusẽ gã aɖe kpɔm ɖe wò agbe dzi mele ŋuwò o la, wò ɖɔkta ate ŋu atso nya me be yealé ŋku ɖe wò nɔnɔmea ŋu. Esia sɔ kple "ŋudzɔnɔnɔ ƒe lala" ƒe mɔnu.
• Amekoko: Woate ŋu aɖe ƒunukpeƒeawo ɖa to amekoko me. Ne `CRDD` (cutaneous RDD) le ŋuwò, alo ne ƒunukpeƒewo le wò yaƒoƒomɔ̃ xem alo le ŋusẽ kpɔm ɖe wò aklã dzi la, woate ŋu awɔ dɔ na wò.
• Kekeli ƒe atikewɔwɔ: Ne amekoko mete ŋu ɖe histiocytes la ɖa o la, woate ŋu awɔ keklẽŋusẽdɔdamɔnu. Esia bia be woazã mɔ̃ aɖe atsɔ aɖo keklẽŋusẽ ƒe keklẽ siwo woɖo taɖodzinu na ɖe lãmenugbagbeviawo ŋu, atsɔ atsrɔ̃ wo.
• Atikewo zazã: Ne dɔlélea nu sẽ, alo ne dɔdamɔnu bubuwo, abe amekoko ene, meɖe dzesiawo dzi kpɔtɔ o la, woate ŋu abu atikewɔmɔnu ŋu. Esia bia be woazã atike siwo wua lãmenugbagbevi siwo le tsitsim kabakaba, abe kansa lãmenugbagbeviwo ene.
• Corticosteroids: Corticosteroids, abe prednisone ene, ate ŋu aɖe ƒunukpeƒewo ƒe vuvu dzi akpɔtɔ eye wòaɖe dzesiawo dzi akpɔtɔ. Esiawo nye atike ƒomevi aɖe si ɖea dɔdzẽ dzi kpɔtɔna.
• Dɔlélenutsiŋutete ƒe atikewɔwɔ: Dɔdamɔnu siawo kpena ɖe wò ŋutɔ wò dɔlélenutsiŋutete ŋu wòkpɔa histiocyte lãmenugbagbevi siwo sɔ gbɔ wu eye wogblẽa wo dome nyuie wu.

Nukae nye etsɔme na amesi ŋu Ross-Dorffman dɔléle (RDD) le?

Wò ŋgɔdonya nɔ te ɖe nu geɖe dzi. Lãmenugbagbevi siwo ŋu wògblẽ nu le ƒe xexlẽme, afisi histiocyte siwo sɔ gbɔ le le wò ŋutilã me, kple alesi nèwɔa nu ɖe ​​atikewɔwɔ ŋu nyuie. Zi geɖe la, RDD kpɔa egbɔ le eɖokui si. Gake le go aɖewo me la, ehiã be woawɔ atike na ame, alo kuxi sesẽwo ate ŋu ado mo ɖa.

Le nyateƒe me la, zi alesi lãmenugbagbevi siwo ŋu dɔlélea le ƒe xexlẽme mesɔ gbɔ o la, zi nenemae emetsonua nyona ɖe edzii.. Ne extranodal RDD, si gblẽa nu le ŋutigbalẽ, akɔta, alo gbɔgbɔtsixe ƒe akpa si le etame ŋu la, anɔ eme be emetsonua anyo wu. Gake ne histiocytes la le gbɔgbɔtsixe ƒe akpa si le ete, ayiku, alo aklã me la, nɔnɔmea ate ŋu agblẽ ɖe edzi vie.

Nya kawoe wòle be mabia nye ɖɔkta?

Mègaŋlɔ be be yeaƒo nu tso nu siawo ŋu na wò ɖɔkta o, elabena ele vevie ŋutɔ be nàse wò nɔnɔmea gɔme nyuie:

• "Ðɔkta, nye ŋutilã ƒe akpa kawo tututue nye RDD nɔnɔmea gblẽa nu le?"
• "Ðe wòahiã be mawɔ nɔnɔmetatawo dodokpɔ bubuwo (abe scans ene) alo ʋudodokpɔwoa?"
• "Ðe mehiã atikewɔwɔa, alo ɖe wòate ŋu adzɔ be nye RDD ƒe nɔnɔmea nakpɔ gbɔ le eɖokui sia?"
• "Aleke wòle be matsi dzi ɖe RDD ƒe nɔnɔme sia ŋui?"
• "Atikewɔwɔ nyuitɔ kae ɖɔktaa kafu nam?"
• "Nu gbegblẽ kawoe mate ŋu akpɔ mɔ na le atikewɔwɔ vɔ megbe?"
• "Nukae nye mɔnukpɔkpɔ siwo li be nye RDD nɔnɔmea nagadzɔ le atikewɔwɔ vɔ megbe?"
• "Zi nenie wòhiã be mava do wo kpɔ be malé ŋku ɖe nye nɔnɔmea ŋu?"

Ðe Ross-Dorffman ƒe dɔléle (RDD) nye kansaa?

Ao, nya sia nye nya si ame geɖe biana. Rose-Dorffman ƒe dɔlélea menye kansa (si me dɔlékui aɖeke mele o) o . Eyae nye be mekaka tso ŋutilã ƒe akpa ɖeka yi bubu abe kansa (dɔléle vɔ̃ɖi) ene o. Gake ƒunukpeƒetetedɔ siwo dzɔna le RDD ta ate ŋu agblẽ nu le ŋutinuwo ŋu, ado kplamatse woƒe dɔwɔwɔ, eye wòahe kuxiwo vɛ. Wò ɖɔktae nye ame nyuitɔ kekeake si anya alesi wò RDD nɔnɔmea le ŋusẽ kpɔm ɖe wò ŋutilã dzi kple nenye be ele be nàtsi dzi ɖe eŋu.

Amekae ke ɖe Ross-Dorffman ƒe dɔléle (RDD) ŋu?

Enyo be nànya esia hã. Le ƒe 1965 me la, dzɔdzɔmeŋutinunyala aɖe si ŋkɔe nye Pierre Paul Louis Lucien Destombes ke ɖe RDD ƒe dzesiwo ŋu le dɔnɔ ene ŋu. Emegbe le ƒe 1969 me la, dzɔdzɔmeŋutinunyala eve siwo ŋkɔe nye Juan Rosai kple Ronald Dorfman srɔ̃ nu tso dɔnɔwo ƒe ƒuƒoƒo bubu si ƒe dzesiwo sɔ kple wo nɔewo ŋu. Fifia woyɔa dɔlélea be Rosai-Dorfman dɔléle, eye wotsɔ woƒe ŋkɔwo ƒo ƒui. Ƒã hafi woyɔnɛ hã be Rosai-Dorfman-Destombes dɔléle, tsɔ dea bubu dzɔdzɔmeŋutinunyala etɔ̃awo katã ŋu.

Mlɔeba la, nusiwo dzi wòle be woaɖo ŋkui (Take-Home Message) .

Ross-Dorffmann ƒe dɔléle (RDD) ate ŋu anye dɔléle didi sesẽ elabena ƒã ŋutɔ eye wòdzea ame ɖesiaɖe dzi le mɔ vovovowo nu . Mɔfiame ɖeka aɖeke meli si ku ɖe RDD ƒe atikewɔwɔ ŋu o. Esia tae wòle vevie be nàƒo nu kple wò ɖɔkta gaglãa, ase dɔléle si wokpɔ le ŋuwò gɔme, eye nànya atikewɔmɔnu siwo nàte ŋu azã.

Ðewohĩ RDD fafɛ si akpɔ egbɔ le eɖokui si le ŋuwò. Alo, àte ŋu ahiã atikewɔmɔnu vovovowo. Bia wò ɖɔkta alesi wò atikewɔwɔ ƒe ɖoɖoa kple emetsonu siwo nèle mɔ kpɔm na asɔ ɖe wò nɔnɔmea nu hã.

Ðo ŋku edzi be menye wò ɖeka koe o . Ne èle nɔnɔme si mebɔ o alea gbɔ kpɔm la, ele vevie ŋutɔ be nàwɔ ɖe atikewɔlawo ƒe aɖaŋuɖoɖowo dzi eye nànɔ susu me sẽm. Ne nyabiase alo ɖikeke aɖe le ŋuwò la, dzro eme kple wò ɖɔkta. Mègavɔ̃ o, elabena sidzedzee nye aʋawɔnu nyuitɔ kekeake si woatsɔ awɔ avu kple nu mawo tɔgbe.

` Rosai-Dorfman Dɔléle, RDD, histiocytes, lymphadenopathy, kɔ ƒe ƒuƒu, ŋutigbalẽdɔlélewo, CRDD, dɔléle siwo mebɔ o, dɔlélenutsiŋutete