Isifo sikaM Werlhof - thrombocytopenic purpura - isifo esibonakala ngesimo segazi elimnyama ngokumelene nesizinda sokwanda kwe-aggregation (gluing) yamaplatelets. Ukwakhiwa kwe-microthrombi okwakhiwe ngezimpawu zamathambo amancane. Ngaphezu kwalokho, kuncibilika kwamangqamuzana egazi futhi kunciphisa inani lamaplatelet.
Izimbangela zezifo zikaW Werlhof
Njengamanje, izimbangela eziqondile ze-thrombocytopenic purpura aziwa. Nikeza izinhlobo eziyinhloko nezesibili zesifo sikaVerff. Amafomu aphambili ayinzalo ngokwemvelo noma abonakaliswe ngenxa yesifo esithathelwanayo. Amafomu ajwayelekile anezibonakaliso zezifo eziningi.
Izimpawu zezifo zikaVerlhof
Lesi sifo siqala kahle, ngenxa yokuthi asikho isizathu esicacile, ngezinye izikhathi ngokumelene nesimo sangasese sokutheleleka emathunjini noma i-ARI. Esigulini esiteji sokuqala, izimpawu ezilandelayo zibonakala:
- umkhuhlane;
- isicanucanu (ukuhlanza okungenzeka);
- ikhanda;
- ubuthakathaka jikelele, ukukhathala;
- ubuhlungu esifubeni nasesisu.
Isibonakaliso esiyinhloko yiziphazamiso kanye nama-hemorrhages angaphansi, okuchaza igama lesibili le-purpura yesifo - thrombocytopenic purpura.
Ngemva kwesikhashana, i-hemorrhagic syndrome ibonakala ikakhulukazi ngendlela:
- ukuphuma kwegazi, ukuphuma kwamathumbu, kanye nakwabesifazane futhi uterine;
- izinsini eziphaphayo , izitho zangasese;
- ukulimaza e-retina iso;
- i-hemoptysis.
Ukubonakaliswa kwemvelo kuhambisane nezinkinga zezinzwa, njengokuthi:
- ukuphuza;
- ukulala;
- ukudideka kwesikhala nesikhathi;
- Umbono wehlile;
- unilateral ukukhubazeka.
Ezimweni ezimbi, kungenzeka ukuthuthukisa i-coma.
Ukushisa kwegazi ngaphansi kwesikhumba kuyaba kakhulu futhi kunendawo enkulu. Ngokuya komshini wokugaya, ama-effusions anombala ovela obomvu-obomvu kuya ophuzi (njengesihlungu esidala).
Ukubona ngesifo sikaVerff kuqala ngokuhlolwa kwesiguli kanye ne-anamnesis. Inkimbinkimbi yokuxilonga ihlanganisa ukuhlolwa okulandelayo:
- Ukuhlaziywa jikelele kwegazi (OAK). Lesi sifo sinqunywa ngokunciphisa izinga lama-erythrocyte ne-hemoglobin, ukunciphisa inani lamaplatelet futhi kutholakala ama-antiplatelet antibodies.
- I-sternary i-puncture - ithatha umnkantsa wamathambo ukuze uhlolwe ngokusebenzisa i-sternum puncture. Esikhathini sokucwaninga kokubunjwa kwamaseli, ukwanda kwenani lama-megakaryocytes, inombolo encane yamaplatelet, atholakala, kanti azikho ezinye izinguquko emangeni lomthambo, isibonelo, lezo izici zesimo sezinsalela.
- I-Trepanobiopsy - isifundo somnkantso wamathambo nge-periosteum ne-bone (esuka esifundeni se-pelvic), esithathwe ngosizo lwedivayisi yezokwelapha ye-trephine. Ngesifo sikaVerlhof, isilinganiso samarrow fatty and hematopoietic bone somnumzane sifana nokujwayelekile.
Ukwelashwa kwezifo zikaVerlhof
Izinyathelo zokwelapha zixhomeke esimweni sesifo. Ukwelashwa kwenziwa ngenye yezindlela ezilandelayo:
- Ukusetshenziswa kwe-corticosteroids ngenhloso yokubopha i-hemorrhagic syndrome nokwandisa izinga lamaplatelet egazini. I-Prednisalon inqunywe ngesilinganiso se-1 mg nge-1 kg yesisindo sesiguli ngosuku. Uma kwenzeka inkinga enkulu yesifo, umthamo uphindwe kabili.
- Uma umphumela omuhle ungafinyeleleki, isiguli sinconywa ukususa ipeni . Ngokwezibalo zezokwelapha, ezigulini ezingu-80% ngemuva kokungenelela kokuhlinzwa kutholakala ngokuphelele.
- Ezimweni ezingavamile, ngemuva kwe-spleen splenectomy, ama-pass pass, kanti lesi sifo sihlala, ama-immunosuppressants anqunywe (Azathioprine, Vincristine) ne-glucocosteroids.
Ukususa izimpawu zangaphandle ze-hemorrhagic syndrome, ama-haemostatic asetshenziswa:
- I-Adroxon;
- Ukudla;
- Isiponji se-Hemostatic;
- ama-estrogens.