Yakakura congenital neutropenia inotsanangura chimiro ichi zvakakwana. Iko kusangana (kureva kuti iwe unoberekwa nayo) mamiriro ane chirwere chakanyanya neutropenia (chinotsanangurwa se neutrophil count <500 masero per microliter, kazhinji <200). Imwe fomu chaiyo inonzi Kostmann Syndrome. Icho chinhu chisingawanzoiti chinokonzera 2 -3 vanhu kune mumwe nomumwe mamiriyoni evanhu.
Ndezvipi Zviratidzo?
Zviratidzo zvinowanzotanga pfupi kuberekwa.
Neutrophils inoratidziro yeropa rega rega rinorwisa mabhakitiriya zvirwere. Nhamba yakaoma yakanyanya neutrophil inowedzera mukana wako wehutachiona hwehutachiona. Fivhiri isiriwo chiratidzo chinowanzoonekwa, asi izvi zvinokonzerwa nehutachiona kwete neutropenia. Kukanganisa kwekuzvarwa kunowanzoonekwa.
- Otitis media (ear infections)
- Cellulitis (utachiona hweganda)
- Oral ulcers
- Gingivitis (gum inflammation)
Inozivikanwa Sei?
Zvichida kuti pane imwe yezvipembenene izvi, chiremba wako achawana ropa rose rakazara (CBC) . Yakakura neutropenia (<500 masero / microliter) inofanira kuva ndiyo yeropa rega rega rakabatwa. Masero matsvuku eropa uye maplatelets anofanira kuva akaenzana. Kazhinji ma monocytes (rumwe rudzi rwechichena chepajeri richawedzerwa). Kana kune rimwe boka rega rega rega rakagadziriswa, zvimwe zvirwere (semuShwachman Diamond Syndrome) zvinofanira kufungwa.
Kana imwe chete yeutroenia yakanyanya kuonekwa, chiremba wako angangokurukura achikutaurira kune hematologist, chiremba anonyanya kugadzirisa ropa.
Pakutanga iwe unogona kuva neCBC yakagadzirwa 2 - katatu vhiki nevhiki kuti uone kutenderera kweutroene (nehuwandu hwemamiriro ezvinhu) sechikonzero.
Nhanho inotevera ifupa remafupa. Uyu muedzo unosanganisira kubvisa chiduku chepfupa kubva muchiuno chako kuti uongorore marora emapfupa, nzvimbo iyo masero eropa eropa anoitwa.
Muchiremera chekurwa neutropenia, masero anoitwa kazhinji pakutanga asi ipapo pane imwe nguva vanofa vasati vasunungurwa muhuwandu.
Kana fupa rako repafu rakafanana nehutano hwakakura hweusutropenia, chiremba wako angangotumira kutengesa mazamu kuti aone kushanduka kwawakaita iwe. Izvi zvinokosha sezvo zvicharatidza kana kana iwe ungapfuura sei chirwere ichi kuvana vako.
Ndeapi Mishonga?
- Granulocyte Colony Inokurudzira Chiitiko (G-CSF kana filgrastim): G-SCF inopiwa sejasi (pasi peganda) majekiseni zuva rega rega. Iyi mishonga inokurudzira kushandiswa kwemafuta emapfupa uye kucheneswa kweeutrophils. Chinangwa ndechokuunza nhamba ye neutrophil kuti igare yakagadzirirwa kuchengetedza zvirwere.
- Bhuna marora kuiswa: Bone marrow kupera kunogona kuva kurapa. Izvi zvinowanzotarisirwa kune vanhu vane maitiro asina kunaka kuG-CSF. Kana kugamuchira kuvepa kwemapfupa kubva kumunin'ina wako, zvakakosha kuve nechokwadi kuti havana hutsika hwakaoma hwehutano hwakakura hweutachiona neutropenia.
- Mishonga inorwisa mabhakitiriya: Kana uine chirwere chakakomba neutropenia uye uchitanga fivhiri, unofanira kutsvaga kurapwa kwenguva pfupi. Fivhiri inogona kuva iyo chete chiratidzo chetachiona hutachiona. Basa reRopa rinofanira kutumirwa kuti rione chinokonzera chinokonzera utachiona. Munguva ino iwe uchaiswa ku IV (kuburikidza nemuviri) antibiotics kana iwe uine utachiona.
Kune Chero Murefu Wakare Nguva Yekutya?
Nekurapa kwakanaka, tarisiro yehupenyu yevanhu vane chirwere chakakura cheutachiona neutropenia yakavandudza zvikuru. Nezera rinokura, iwe uri pangozi yakawanda ye myelodysplastic syndrome (MDS) uye leukemia (inowanzove yakanyanya myeloid leukemia) inofananidzwa nehuwandu hwevanhu. Iyi njodzi yaifungidzirwa kuva yechipiri kune G-CSF kurapwa asi inoita sechinhu chakaoma chechiitiko chacho.
Kurapa neG-CSF kunogona kuita kuti splenomegaly (kuwedzera kwepleen). Pane dzimwe nguva kuwedzera uku kuwedzera kwepleen kunokonzera pasi platelet count (thrombocytopenia). Kana iyo thrombocytopenia yakaoma, iwe unogona kutora splenectomy.