Paroxysmal husiku hemoglobinuria (PNH)

Paroxysmal nocturnal hemoglobinuria chirwere chisingawanzoitika mukati umo masero matsvuku eropa anodonha pamberi pekare.

Vanhu vane chirwere ichi vane masero eropa ari kushaikwa geni rinonzi PIG-A. Iyi geni inobvumira chinhu chinonzi glycosyl-phosphatidylinositol (GPI) kubatsira mamwe mapuroteni kunamatira kumasero.

Pasina PIG-A, mapuroteni akakosha haakwanise kubatana nesero pamusoro uye kuchengetedza sero kubva kuzvinhu zviri muropa zvinonzi complement. Nekuda kweizvozvo, masero matsvuku eropa anoputsika nekukurumidza. Maseru matsvuku anodonhedza hemoglobin muropa, iyo inogona kupinda mumuti. Izvi zvinogona kuitika chero nguva, asi zvinowanzoitika panguva yehusiku kana mangwanani-ngwanani.

Chirwere ichi chinogona kubata vanhu vezera ripi zvaro. Inogona kunge ichibatanidzwa neaplastic anemia, myelodysplastic syndrome, kana acute myelogenous leukemia.

Njodzi zvinhu, kunze kwekutanga aplastic anemia, hazvizivikanwe.

Zviratidzo zvinogona kusanganisira:

• Marwadzo emudumbu
• Kutambudzika shure
• Ropa rakagwamba, rinogona kuumbika mune vamwe vanhu
• Weti yakasviba, inouya yoenda
• Kupwanya kuri nyore kana kubuda ropa
• Kutemwa nemusoro
• Kufema kwekufema
• Kushaya simba, kuneta
• Pallor
• Kurwadziwa pachifuva
• Zvakaoma kumedza

Tsvuku uye chena masero eropa kuverenga uye maplatelet kuverenga anogona kunge akaderera.

Weti tsvuku kana shava inoratidza kuparara kwemasero matsvuku eropa uye kuti hemoglobin iri kuburitswa mukutenderera kwemuviri uye pakupedzisira kupinda mune weti.

Miedzo inogona kuitwa kuti uongorore mamiriro aya inosanganisira:

• Yakazara kuverenga ropa (CBC)
• Coombs bvunzo
• Kuyerera cytometry kuyera mamwe mapuroteni
• Hamu (acid hemolysin) bvunzo
• Serum hemoglobin uye haptoglobin
• Sucrose hemolysis bvunzo
• Kuongorora urinal
• Weti hemosiderin, urobilinogen, hemoglobin
• LDH bvunzo
• Reticulocyte kuverenga

Steroid kana mimwe mishonga inodzvinyirira immune system inogona kubatsira kudzora kuparara kwemasero matsvuku eropa. Kuwedzerwa ropa kunogona kudiwa. Supplemental iron uye folic acid inopihwa. Ropa rakapfava rinogona zvakare kudikanwa kudzivirira kugwamba kubva pakuumba.

Soliris (eculizumab) mushonga unoshandiswa kurapa PNH. Inodzivirira kuparara kwemasero matsvuku eropa.

Bone marrow kuisirwa inogona kurapa chirwere ichi. Inogona zvakare kumisa njodzi yekuvandudza PNH mune vanhu vane aplastic anemia.

Vanhu vese vane PNH vanofanirwa kupihwa majekiseni ekudzivirira mamwe marudzi ebacteria kudzivirira hutachiona. Kumbira mubatsiri wako wehutano kuti ndeapi akakodzera iwe.

Mhedzisiro yacho inosiyana. Vanhu vazhinji vanorarama kweanopfuura makore gumi mushure mekuonekwa kwavo. Rufu runogona kukonzerwa nezvinetso zvakaita sekuumbika kweropa (thrombosis) kana kubuda ropa.

Muzviitiko zvisingawanzoitika, masero asina kujairika anogona kudzikira nekufamba kwenguva.

Zvinetso zvinogona kusanganisira:

• Acute myelogenous leukemia
• Aplastic kupererwa neropa
• Ropa rinovhara
• Rufu
• Hemolytic kupererwa neropa
• Iron kushomeka anemia
• Myelodysplasia

Fonera mupiki wako kana iwe ukawana ropa mumurini rako, kana zviratidzo zvikawedzera kana kusavandudza nemushonga, kana kana zviratidzo zvitsva zvichikura.

Iko hakuna inozivikanwa nzira yekudzivirira iyi kusagadzikana.

PNH

• Masero eropa

Brodsky RA. Paroxysmal husiku hemoglobinuria. Muna: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Basic Misimboti uye Maitiro. 7th ed. Philadelphia, PA: Elsevier; 2018: chits.

Michel M. Autoimmune uye intravascular hemolytic anemias. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chits. 151.