70 http://jsms.sch.ac.kr

후천성면역결핍증 환자에서 발생한 위장관과 경부 림프절을 침범한 카포시육종 1예조윤희1, 한재필1, 허순미1, 김민진1, 정희재1, 명유식1, 김희경2, 이은정1, 전민혁1, 김태형1, 추은주1

순천향대학교 부천병원 1내과, 2병리과

A Case of Gastrointestinal and Cervical Lymph Nodal Kaposi’s Sarcoma in a Patient with Acquired Immunodeficiency SyndromeYoun Hee Cho1, Jae Pil Han1, Sun Mi Hur1, Min Jin Kim1, Hee Jae Jung1, Yu Sik Myung1, Hee Kyung Kim2, Eun Jung Lee1, Min Huok Jeon1, Tae Hyong Kim1, Eun Ju Choo1

Departments of 1Internal Medicine and 2Pathology, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine, Bucheon, Korea

Kaposi’s sarcoma was the first malignancy to be recognized as a cancer defining acquired immunodeficiency syndrome (AIDS). Ka-posi’s sarcoma, a rare tumor, usually presents itself with skin lesions but it may be widely disseminated internally such as digestive, respiratory organ, spleen, or lymph node. The incidence of Kaposi’s sarcoma in patients with AIDS has declined in the era of effective antiretroviral therapy (ART), and cases of disseminated Kaposi’s sarcomas have rarely been reported in Korea. Chemotherapy is usu-ally used in symptomatic or rapidly progressive disease, and interferon-α can be applied as a treatment option. We report a success-fully treated case of gastrointestinal and cervical lymph nodal Kaposi’s sarcoma in a patient with AIDS who had combined treatment with ART, interferon-α, and paclitaxel.

Keywords: Acquired immunodeficiency syndrome; Gastrointestinal Kaposi’s sarcoma

서 론

1981년에후천성면역결핍증(acquiredimmunodeficiencysyn-drome,AIDS)이처음보고된이래로,인간면역결핍바이러스(hu-manimmunodeficiencyvirus,HIV)감염환자에서카포시육종

(Kaposi’ssarcoma),비호지킨림프종(non-Hodgkin’slymphoma)과같은특정종양의발병위험이증가한다는것이잘알려져있다

[1].카포시육종은피부,점막,내부장기에발생하는다발성혈관성결절들로이루어진종양으로,1872년헝가리피부과의사인MoritzKaposi에의해처음으로보고되었고[2],AIDS관련카포시육종은

피부뿐만아니라,환자의10%에서는위장관,간,비장,림프절,폐실질등에서발생하는것으로알려져있다[3].미국에서는1981년에는AIDS환자의40%에서관찰되는높은유병률을보였으나,항레

트로바이러스제가사용되면서1992년에는20%로감소되었고[4],국내에서는AIDS환자의약1.1%에서나타나는것으로보고되었

다[5].본저자들은피부병변을동반하지않은위장관과경부림프

절의카포시육종이진단된AIDS환자에서항레트로바이러스치료및인터페론알파와Taxol계열항암제(paclitaxel)로치료하여호전된증례를경험하였기에문헌고찰과함께보고하는바이다.

Soonchunhyang Medical Science 18(1):70-73, June 2012 pISSN: 2233-4289 I eISSN: 2233-4297

CASE REPORT

Correspondence to:  Eun Ju ChooDepartment of Internal Medicine, Soonchunhyang University Bucheon Hospital, Soonchunhyang University College of Medicine,  170 Jomaru-ro, Wonmi-gu, Bucheon 420-767, KoreaTel: +82-32-621-5202, Fax: +82-32-621-5016, E-mail: mdchoo@schmc.ac.krReceived:  Apr. 26, 2012 / Accepted after revision:  Jun. 20, 2012

© 2012 Soonchunhyang Medical Research InstituteThis is an Open Access article distributed under the terms of the 

Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/).

AIDS 환자에서 발생한 위장관과 경부 림프절을 침범한 카포시육종 • 조윤희 외

Soonchunhyang Medical Science 18(1):70-73 http://jsms.sch.ac.kr 71

증 례

42세남자환자로내원5개월전전신쇠약감,미열,기침증상있어보존적치료만하던중내원일주일전부터전신쇠약감,열감이심해져타병원응급실을방문하여시행한검사에서혈색소5.0g/dL,HIV항체양성으로본원으로전원되었다.3년전부터동성연

애과거력이있었으며웨스턴블롯검사양성으로HIV감염으로확진되었다.내원당시혈압은110/70mmHg,맥박84회/분,호흡수20회/분,체온38°C이었고만성병색소견을보였으며의식은명료

하였고결막은창백하였다.양쪽경부에작은림프절이여러개촉지되었으며양쪽귀뒤에서사마귀(verruca)가발견되었으나그외피부병변은없었다.흉부청진에서심잡음및양측폐의수포음이들리지않았고복부진찰에서복부팽만과장음감소가관찰되었

다.말초혈액검사에서백혈구4,780/mm3(호중구55.2%,림프구

22.4%,단핵구22.0%,호산구0.2%),혈색소9.3g/dL,혈소판102,000/mm3이었고,적혈구침강속도23mm/hr,C반응단백11.33mg/dL이었다.생화학검사에서총빌리루빈0.54mg/dL,ala-ninetransaminase6IU/L,aspartatetransaminase12IU/L,알부

민1.6g/dL,혈중요소질소10.1mg/dL,크레아티닌0.8mg/dL이었다.CD4림프구수는25/mm3,HIV-RNA정량검사결과는1.23×105copies/mL이었다.복부전산화단층촬영에서위벽의비후와위주변의여러개의

작은림프절이관찰되었고(Fig.1),경부전산화단층촬영에서는양측경부와액와부의림프절비대가관찰되었다(Fig.2).식도위십이

지장내시경검사와대장내시경검사를시행하였고하부식도에서발적을동반한용종이관찰되었다(Fig.3A).위및십이지장전체에

서다발성의흑갈색궤양과삼출액을동반한결절및용종들이발견되었고(Fig.3B–D),대장에서도유사한병변들이관찰되었다.용종성병변에서조직검사를시행하였으나출혈을동반한만성염증

소견만관찰되었고,골수검사는정상이었다.입원9일째식도위십

이지장내시경검사를다시시행하였다.위체부의병변에서조직검

사를시행한결과저배율병리소견에서는점막층아래의고유판

을팽창시키는병변이관찰되었고(Fig.4A),고배율에서는고유판내에결절형태로다염색성핵을가진내피세포로싸여진열공모양(slit-like)의작은혈관증식과현저하게진한핵을갖고다형성을가진방추형(spindle-shaped)세포의증식이관찰되었다.또한혈관내에출혈소견및림프구와형질세포의침윤이있었다(Fig.4B).면역조직화학염색에서종양세포는혈관표지자인CD34에양성이었

고(Fig.4C),humanherpesvirus8(HHV-8)에서도핵에강하게염색되어(Fig.4D)카포시육종에합당한소견이었다.경부림프절에

서절제생검으로얻은조직에서도위체부의병리소견과같은결과를보였다.입원9일째항레트로바이러스치료(abacavir,lamivudine,ata-

zanavir/ritonavir)를시작하였고카포시육종에대한전신항암제투여를시작하였다.카포시육종에대한1차치료로인터페론알파

(interferon-α)2b85mIU(50Miu/m2)를매일피하주사하였다.2주마다주5회투여예정이었으나심한구역,구토,범혈구감소증,위장관출혈등의부작용이나타나인터페론알파2b치료3주후

Fig. 1. Abdominal computed tomography findings. Diffuse gastric wall thickening (A, B) and multiple small sized lymph nodes (C) were noted.

A B C

Fig. 2. Neck computed tomography findings. Multiple lymph nodes enlarge-ment was noted at both sides of neck (A, B).

A B

Cho YH, et al. • Gastrointestinal and Cervical Lymph Nodal Kaposi’s Sarcoma in AIDS

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투여중지하였다.이후paclitaxel로변경하여2주간격으로160mg(100mg/m2)의용량으로6차례항암치료를시행하였다.3개월후시행한추적내시경검사에서기존에있었던위카포시육종은많이호전되고,식도와십이지장의병변은거의사라진양상을보여paclitaxel항암치료를유지하였고,총12차례paclitaxel항암치료후시행한식도위십이지장내시경검사에서카포시육종은거의호전되었다(Fig.5).

고 찰

카포시육종은크게임상적으로4가지로분류하는데고전형(the

classicorEuropeanform),AIDS와관련된유행형(epidemicKa-posi’ssarcomaassociatedwithacquiredimmunedeficiencysyn-drome),장기이식과관련된면역억제관련형(Kaposi’ssarcomainpatientswithiatrogenicimmunosuppressionassociatedwithor-gantransplant),아프리카지방유행형(theendemicAfricanform)으로분류한다[6].미국질병관리센터는카포시육종을AIDS가발병한근거로삼을수있다고하여“AIDS정의암(AIDS-definingcancer)”으로분류하였다[7].AIDS관련카포시육종은피부가가장흔한침범부위지만다른부위에도침범가능한질환으로림프

절이두번째로흔한침범부위이며,위장관침범과폐침범순으로흔한것으로알려져있고항레트로바이러스치료가도입되면서빈도는이전과비교하여현저하게감소하였다[3].1994년Chang등[8]이AIDS환자의피부병변에서헤르페스바이러스의DNA분절

Fig. 5. Follow-up findings after 12th systemic chemotherapy with paclitaxel. The endoscopy showed improvement of Gastrointestinal lesions.

A B C

Fig. 3. Endoscopic findings. (A) A polypoid lesion was noted at the distal esoph-agus. (B, C, D) Multiple reddish elevated lesions covered with exudate material were found in stomach and duodenum.

A

C

B

DFig. 4. Microscopic findings. (A) Low power showed the lesion occupied the layer of lamina propria in the stomach (H&E,  × 100). (B) Close-up view showed proliferation of spindle-shaped cells, formation of slit-like space and extravasa-tion of red blood cells in the lamina propria of the stomach (H&E, × 400). (C) The tumor cells display immunoreactivity for the CD34 (× 200). (D) The tumor cells reveal strong nuclear immunoreactivity for human herpesvirus 8 (× 200).

A B

C D

AIDS 환자에서 발생한 위장관과 경부 림프절을 침범한 카포시육종 • 조윤희 외

Soonchunhyang Medical Science 18(1):70-73 http://jsms.sch.ac.kr 73

을분석하여카포시육종과관련된헤르페스바이러스(Kaposi’ssarcomaassociatedherpesvirus,KSHVorHHV-8)가카포시육종

의중요한병인임을발견하였으나카포시육종병소에성호르몬수용체가없음에도남성에서주로호발하는등발병에관여된많은요소들이아직잘밝혀져있지않다[9].카포시육종은내부장기의침범이있거나,광범위한점막피부

병변,림프부종이발생하는등의진행된상태가아니면항레트로바이러스치료만으로도60%이상에서호전을보인다[10].이는HIV-Tat단백질의생산감소,KSHV에대한세포독성T림프구에의한면역반응향상,치료약제인단백분해효소억제제(proteaseinhibitor)의항혈관활성도(antiangiogenicactivity)등의효과로추정된다[9,11].위장관을포함한내부장기의침범이있거나광범

위한점막피부병변,림프부종이발생한카포시육종의경우항암

화학요법으로치료를시도한다.AIDS관련카포시육종에대해미국식품의약국(FoodandDrugAdministration)에서승인된약으로는alitretinoingel,liposomaldaunorubicin,pegylatedliposomaldoxorubicin,인터페론알파2b와paclitaxel이있다[12].

AIDS환자에서위장관을침범한카포시육종에대한국내의증례보고는지금까지4예가있었다[13-16].이중피부와위장관카포

시육종에대해paclitaxel로치료한2예[14,15]와인터페론알파치료를시도한1예가있었으며[16],이중인터페론알파2b로치료를시도하였던1예의경우,치료도중심부전의진행으로환자가사망

하였다.본증례에서는항레트로바이러스약물치료와국내의보험급여

현실상먼저인터페론알파2b로치료를시작하였는데인터페론은항바이러스및항증식성,신생혈관생성의억제,면역을조절하는특성과HHV-8의재활성화를억제하는역할등으로AIDS카포시

육종에서최초로승인된치료제이다.그러나인터페론알파2b투여후환자는심한범혈구감소증및위장관출혈을보여2차치료시행후인터페론알파2b투여를중단하였고이후paclitaxel을투여하였다.Paclitaxel은미세소관을안정화시키고,신생혈관형성을억제하는효과로사용후에좋은치료반응도를보였음이보고된바있으며[14,17]본증례에서도paclitaxel투여3개월후시행한추적내시경검사에서카포시육종의크기가감소하였고증상의호전

도보였다.카포시육종의치료후예후는항레트로바이러스치료도입후

에는과거와달리치료전의CD4T림프구의수에영향을받지않는다[9].현재까지는카포시육종의치료에전통적인항암화학요법

이나인터페론알파투여가우선적으로고려되나,그외치료제로서HHV-8이카포시육종의원인물질임을근거로하여cidofovir,gan-ciclovir등의항바이러스제및항혈관생성(anti-angiogenesis)효과

를이용한thalidomide와카포시육종에대한항증식효과(anti-proliferativeeffect)를보인retinoid에대한연구가진행중이다[3].

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