NOI KHOA - SO 2/2010 BENH ROSAI - DORFMAN
BAO CAO HAI TRUONG HOP VA HOI CU'U Y VAN Nguyen Phi Hung*, Ta Van To*
Nguyen Van Chu, Dang The Can*
TOM TAT
Benh md bao xoang gay hach to (benh Rosai - Dorfman) la mot benh lanh tinh hiem gap. Ton thuang thudng gap d hach co, tren lam sang thu'dng nham vdi u lympho hoac cac benh ly ac tinh khac d hach. Tdn thuong ngoai hach gap vdi ty le cao. Hinh anh vi the gom xoang bach huyet trong hach gian rpng, tang sinh md bao. Cac md bao nay cd nhan Idn, hoc hda sang, bao tuong rdng ua toan va thudng cd cac te bao mau trong bao tuong (lympho bao, tuang bao, bach cau da nhan). Xen ke giua cac xoang bach huyet la md lympho cdn lai vdi rat nhieu tuong bao trudng thanh.
Cac md bao bdc Id dau an S-100 va CD68, khong boc Id CD1a. Benh cd dien bien phUc tap, den nay chua cd phac dd dieu tri chuan. Chung tdi bao cao hai trudng hap va hdi cUu y van ve loai benh nay.
SUMMARY
Sinus histiocytosis with massive lymphadenopathy (Rosai - Dorfman disease) is a rare benign idiopathic condition that commonly involves the cervical lymph nodes but secondarity may involves other organs. Clinically, this disorder often confused with lymphoma and had variable courses.
Characteristic microscopic features are dilated sinuses with sheets of histiocytes. The were blood cells (lymphocytes, plasma cells or neutrophils) in the cytoplasm of the cells (emperipolesis). Scanty lyphoid tissue (mostly mature plasma cells) is seen in between sinuses. Immunohistochemical study showed the histiocytes are positive with CD68 and S-100. No form of therapy has been found to be of any benefit.
* Benh vien K - Ha Noi
I. OAT VAN OE
Benh mo bao xoang gay hach to la mot rdi loan tang sinh mo bao tu phat lanh tinh hiem gap. Ton thuong nguyen phat thuong d hach. Tuy nhien, nhung ton thuang thu phat hoac nguyen phat ngoai hach cung co the gap [1, 2]. Tren lam sang thuong bi chan doan nham la u lympho khong Hodgkin hoac u lympho Hodgkin, benh Castleman, viem hach do cac benh ly d da, hpi chuhg hach da - niem mac, benh hach to hoai tir mo bao, chuyen dang huyet quan cua hach hay cac ton thuang viem gia u [1]. Benh nhan thudng co bieu hien hach cd sung to thanh khdi Idn d hai ben, cac nhom hach khac nhu hach nach, hach ben, hach trung that va hach quanh dong mach chu cung co the gap. Cac vi tri ton thuang ngoai hach thudng gap la dudng ho hap tren, xuong, he thdng tiet nieu sinh due, dudng ho hap dudi khoang mieng, mo mem [3, 4]. Benh nhan thudng khong co trieu chirng phdi hpp, tuy nhien trong mot so truong hop co the co dau, tang cam. Khoang
1/4 so truong hop co sot, met moi, ra mo hoi trpm va sut can. 6 Viet Nam, theo chiing toi dupe biet cho den nay chua co trudng hop nao duoc bao cao. Do vay, chiing toi xin bao cao hai trudng hop va hoi ciiu y van ve loai benh hiem gap nay.
Benh nhan 1: Benh nhan nam, 53 tuoi, que d Binh Duong, thang 12/2008 benh nhan thay hach co ben phai sung to, dau, toan than sot. Di kham dupe bac sy cho thudc (khong ro thudc gi), sau khi udng het sot, het dau, 9
NOI KHOA - SO 2/2010 hach nho lai. Thang 1/2009 hach sung to tra
lai, den benh vien Gia Dinh kham, duac sinh thiet hach vai chan doan hach viem man tinh.
Benh nhan dugc cho thuoc ud'ng nhung khong da (dan thudc bi mat nen khong biet da dieu tri thud'c gi). Thang 3/2009 benh nhan den Benh vien Ung budu Thanh pho Ho Chi Minh kham va duac sinh thiet hai lan vdi chain doan giai phau benh la hach viem man tinh. Duoc dieu tri bang khang sinh va chdng viem nhung khong da. 4/2009 den Benh vien K kham thay the trang binh thuang, khong sot, khong co bieu hien thie'u mau. Tai chd thay hach goc ham va doc ca uc don chum hai ben sung to, ddi xung nhau. Co mot so hach d thuang don va duoi cam. Hach tuong ddi cung, chad, di dong han che. Xet nghiem te bao hpc chan doan u lympho Hodkin.
Chup Xquang tim phoi va sieu am 6 bung khong thay ton thuang. Benh nhan duoc sinh thiet hach ngay 7/4/09. Tren tieu ban mo benh hpc thay can true hach bi xoa, cac xoang trong hach gian rpng, trong chua nhieu dai thuc bao nhan tron, chat nhidm sac co keo lam nhan hoc hoa sang. Hat nhan to ro nam 6 trung tam, mang nhan mong deu. Bao tuong te bao rpng ua toan, trong bao tuong cd cac lympho bao va manh vun te bao. Mo lympho con lai giua cac xoang co nhieu tuong bao.
Nhupm hoa mo mien dich cac dai thuc bao duong tinh vdi S-100 va CD68.
Benh nhan tir chdi didu tri tai Benh vien K nen mat theo doi.
Benh nhan 2. Benh nhan nu, 41 tuoi, que 6 Ha Npi. Benh nhan co bieu hien noi hach cP each day khoang 3 nam. Benh tien trien tirng dot vdi cac chain doan hach viem phan ung, dupe dieu tri bang khang sinh va prednisolon. 4/2009 benh nhan xuat hien khdi u a goc ngoai mat trai vdi chan doan lam sang la u phan mdm dudi mat trai hoac u
tuyen le. Benh nhan dupe md cat bo u lam xet nghiem giai phau benh. Dai the' thay u co vo, dudng kinh Ion nhat khoang 1,3cm. Dien cat dai, chad, mau hong nhat. Vi the thay u co vo xa kha mong, mot goc con dinh mot so tuyen le lanh tinh. Nhu mo u thay cac khoang rpng trong chu'a nhieu mo bao xen ke lympho bao nho. Cac mo bao co hinh tuong tu nhu cac te bao d trudng hop thu nhat. Co rat nhieu tuang bao nam xen ke giua cac dam mo bao ndi tren. Nhupm HMMD cac te bao mo bao cung duong tinh vdi CD68 va S-100. Do khong cd trieu chung toan than khac nen benh nhan ve khong dieu tri gi. Hien tai benh nhan con nhieu hach nhd d hai ben co, duoi ham, nach hai ben. Trung that, ben va 6 bung khong thay hach.
II. BAN LUAN
Con co y kien khong thdng nhat ve tac gia va thdi diem phat hien benh lan dau [1,3, 4]. Mot so tac gia cho rang Robb - Smith md ta truong hop dau tien a tre em nam 1947 va dat ten la benh luoi xoang te bao khong Id.
Mot so tai lieu khac cho rang truong hop dau tien duoc ghi nhan nam 1960 d Nam Phi bdi cac bac sy Dorfman. Trong nhung nam 1969 va 1972. Rosai va Dorfman xac nhan day la mot bien thd benh dpc lap va dat ten la benh mo bao xoang gay hach to [4], Mac dii hau het cac truong hop co bieu hien nguyen phat tai hach va toi 90% cac trudng hop co hach cd to. Tuy nhien, ton thuong cac nhom hach khac cung thuong gap va co den 43% co it nhat mot vi tri ngoai hach bi ton thuang.
Trong mot so trudng hpp, tdn thuang ngoai hach la biSu hien dau tien va duy nhat cua benh va ten "benh Rosai - Dorfman" dupe sir dung chu yeu cho cac truong hop nay. Cac vi tri ngoai hach thudng gap la dudng ho hap tren, tuyen nude bpt, hoc mat, tinh hoan, da.
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NOI KHOA - SO 2/2010 Cac vi tri it gap hon nhu xuong, ket mac, vom
hong, tuyen uc, he thong sinh due va he thong than kinh [2, 3].
Do benh hiem gap nen chua xac dinh duoc tan suat mac benh chinh xac. Trong mot nghien ciru hoi curu 423 benh nhan duoc bao cao, nguoi ta thay hon mot nira so truong hop gap o Hoa Ky (38,7%). 19,5 6 Tay Au va 15,4% d Chau Phi. Ty le mac benh tuong duong nhau o hai nhom nguoi da den va da trdng (43,6% d mdi nhom) va ty le 6 nhom ngudi chau A va cac thap hem ro ret. Ty le mdc benh d nam gidi cao hon nu giai (58%
so vdi 42%). Tuoi mdc benh trung binh la 20, pham vi mdc tur khi mdi sinh den 74 tuoi.
HHV-6 va EBV duac cho la co lien quan den can nguyen gay benh [3]. C3 Viet Nam, cho den nay chiing toi chua thay co trudng hop nao duoc ghi nhan va bao cao trong y van tieng Viet. Hai benh nhan chiing toi gap a lua tuoi trung nien (41 va 53) vd mot benh nhan nam, mot benh nhan nu.
Hdu het cac trudng hop mdc benh co bieu hien hach to khong kem cac trieu chirng gi khac. Tuy nhien co mot so truong hop benh nhan co dau, sdt. Mot sd trudng hep co bieu hien viem dudng ho hap tren trudc khi ndi hach cd. Mot benh nhan co kem dau va sot khi ndi hach lan ddu. Mot truong hop hach cd to doi xiing nhau, trudng hop con lai hach to rai rac d cd, nach, dudi ham, benh sau do tien trien va tdn thuong ngoai hach (tuyen le).
Hinh anh mo benh hoc ciia tdn thuang tai hach va ngoai hach tuong tir nhau. Tai hach tha'y cac xoang bach huyet trong hach gian rpng, tang sinh mo bao. Cac te bao co nhan Ion, hoc hoa sang, bao tuong rpng ua toan. Cac te bao nay thuong cd cac te bao mau trong bao tuong (lympho bao, tuong bao, bach cau da nhan). Xen ke gitta cac
xoang bach huyet la mo lympho con lai vdi rat nhieu tuong bao trudng thanh. Cac te' bao mo bpc lp dau an S-100 va CD68, am tinh vdi CD la. Ca hai trudng hop ciia chiing toi tdn thuong deu co hinh anh dien hinh nhu da dupe mo ta va bpc lp cac dau ah mien dich neu tren [3,4].
Benh tien trien tir nhien bao gom cac dot thoai trien xen ke tai phat, cudi ciing benh thoai then hoan toan. Cho den nay khong co phac dp dieu tri dac hieu cho benh. Trong mot nghien cUu gom 34 benh nhan mdc benh Rosai - Dorfman ngudi ta thay mot benh nhan chet do thoai hoa tinh bpt than, mot benh nhan khac chet do dieu tri hda chat.
Dieu tri hda chat cho den nay dupe cho la chdng chi dinh voi benh nay. Trong hai benh nhan cua chiing toi, mot benh nhan khong dieu tri va mat theo doi, mot benh nhan duoc dieu tri chdng viem bdng khang sinh va corticoid, benh thoai trien tirng dot. Dilu tri phdu thuat cd the chi dinh cho nhirng trudng hop co da'u hieu chen ep [1, 3].
TAI LIEU THAM KHAO
1. Gull Mohd Baht. Surender Kumar, Atul Sharma: Rosai - Dorfman disease: a case report with review of literature. Indian Journal of Medical and Paediatric Oncology.
2004, 25: 39-41.
2. Rubenstein M.A. Fansworth N.N, Pielop J.A, Orengo I.F, Cutaneous Rosai - Dorfman disease. Dermatology Online Journal 12 (1): 8.
3. Strauhen J.A. Diagnostic Histopathology of the Lymph Node. 1998, Sinus Histiocytisis with Massive Lymphadenopathy, 334-337.
4. Warnke R.A, Weiss L.M, Chan JKC, Cleary ML, Dortman RF, 1994. Tumors of the Lymph Nodes and Spleen. Atlas of Tumor Pathology, third series Fascicle 14: 349-360.
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