Journal ofthe Korean Radiological Society 1996 : 35 (3) : 343 - 345

Pulmonary Involvement of Hypereosinophilic Syndrome : High-Resolution CT Findings in Three Patients1

Eun-Young Kang, M.D., Mee Ran Lee, M.D., Jae Jeong Shim, M.D.2

Hypereosinophilic syndrome is a rare entity of eosinophilic lung disease characterized by idiopathic prolonged eosinophilia of marked degree and vari­ able organ involvement. Pulmonary involvement of hypereosinophilic syndrome occurs in up to 40% of patients. We report HRCT findings of three patients with p 비 monary involvement of hypereosinophilic syndrome diagnosed by clinical manifestation, bronchoalveolar lavage and transbronchiallung biopsy. On H RCT, several small nodules were seen in both lungs, especially in peripherallung areas of the three patients. One had nodules with ground-glass attenuation halo and also focal areas of ground-glass attenuation in this area.

Index Words : Lung , diseases Lung , CT

Hypereosinophilic syndrome (HES) is an infiltrative ated leukocyte count of 15000 per cubic mm with mar­ disease of eosinophils affecting multiple organs, in­ ked eosinophilia(total eosinophil count, 8880). Focal cluding the lung (1). The criteria for this diagnosis in­ lesions were found in her liver. Ultrasound guided bi­ clude persistent eosinophilia of 1500 eosinophils per opsy of the focallesion revealed eosinophilic abscess. cubic mm for longer than six months or death before six A bone marrow biopsy was performed ; it revealed 42 months, the absence of parasitic, allergic or other know­ % of mature eosinophils and no evidence of leukemia n causes of eosinophilia, and evidence of organ in­ She did not complain any neurologic symptoms or car­ volvement (1 , 2). Pulmonary involvement occurs in 40 diac problems. Chest radiograph was normal , though percent of HES, mainly presenting with cough or dysp­ HRCT showed several small nodules in the subpleural nea (3). Patients with HES and pulmonary infiltrates areas of both lungs(Fig. 1). The eosinophil content of may pose certain diagnostic problems such as infec­ bronchoalveolar lavage fluid was 26 % and transbron­ tion , infarction , congestive heartfailure or HES itselt(1, chiallung biopsy on the right lower lobe lesion showed 3 , 4) . The authors report three HES patients with pul­ focal pneumonic infiltration with eosinophils and fi­ monary involvement in whom high-resol ution CT show­ brous exudate. Steroid treatment was started and the ed small nodules in peripherallung areas. liver lesions and eosinophilia improved within two mon­ ths CAS E R EPO RTS Case2 Case1 A 44-year-old man was admitted for evaluation of a A 51-year-old woman was admitted to hospital be­ cough. His medical history was insignificant. Labora­ cause for six months she had felt discomfort in the right tory studies yielded leukocytosis(16000 per cubic mm) upper quadrant of the abdomen. She had no specific with marked eosinophilia(total eosinophil count, 7136) . medical history. Laboratory studies revealed an elev- A chest radiograph showed no abnormalities of the lun­ gs and heart; HRCT showed several small nodules with a ground-glass attenuation halo in both lungs(Fig. 2a) 'Oepartmento fD iag nostic Radio logy , Col lege of Medicine , Korea Universi ty and peripheral focal areas of ground-glass attenuation 20epartment of lnternal Medicine , College of Me di cine , Korea Un ive rsity in the lower lungs(Fig. 2b). Bronchoalveolar lavage Rece ived March23 , 1996 : Accepted June 18, 1996 Address repr in t reques ts to' Eun-Young Kang , M.D. , Oepartm ent of Radio logy , (BAL) revealed 4 % eosinophilia. Microbiologic cultur­ Korea University Guro Hospi tal , # 80 Guro-dong , Guro-ku , Seou l, Korea , es on BAL fluid for tuberculosis, bacteria and fungi 152-050 TE L. (82-2)818-6786 FAX. (82-2)863-9282 were negative. Focal hepatic lesions were found and 쇄% Journal ofthe Korean Radiological Society 1996 : 35 (3) : 343 - 345

ultrasound-guided liver biopsy showed periportal eos­ study, however still revealed eosinophil ia of 8.6 % inophilic infiltation with bridging hepatic necrosis. Gas­ troscopic biopsy revealed eosinophilic mucosal infil­ Case3 trations in the stomach ; A bone marrow biopsy reveal­ A 58-year-old woman was admitted for the evalu­ ed 39.5 % of matured eosinophils. Ster 이 d and busulfan ation of generalized myalgia. Laboratory study reveal­ treatment was started and ten months later the patient ed eosinophilia(total count, 4100). Bone marrow biospy was doing well while receiving therapy; laboratory revealed 16 % of eosinophils ; a chest radiograph show­ ed no abnormalities but HRCT showed several small nodules in both lungs(Fig. 3). Bronchoalveolar lavage revealed 18 % of eosinophils. Despite steroid and hydroxyurea treatment peripheral eosinophilia persis­ ted. Eighteen months later, laboratory study still reveal­ ed eosinophil ia of 21.6 %

DISCUSSION

The eosinophilic lung diseases are a diverse group of P 비 monary disorders linked by common findings of an increase in circulating or tissue eosinophils Although numerous classifications of these disorders have been proposed , there is no optimal way to classify these disorders (5). Hypereosinophilic syndrome is one characteristic entity of eosinophilic lung diseases and must be differentiated from all other causes of per­ ipheral eosinophilia. The term “ hypereosinophilic syndrome" was first proposed by Hardy and Anderson (6) in 1968. They de­ scribed three patients characterized by severe hyper­ eosinophilia, cardiac or pulmonary symptoms, hepatos-

Fig ‘ 1.HRCTshowstwosmallnodules(arrows)inthesubpleural plenomegaly, constitutional symptoms, anemia, and area 01 right lower lobe. diffuse organ infi Itration by matu re eosi noph ils. Hyper

2a 2b 3 Fig. 2. a. HRCT shows two small nodules with ground-glass attenuation halo (arrows) in left upper lobe and one in I eft lower lobe b. HRCT shows.local areas 01 ground-glass attenuation (arrows) in peripheral area 01 left lower lobe Fig. 3. HRCT shows small nodules (arrows) in peripheral areas 01 right middle and lower lobes t ‘ …않 Eun -Youn g Ka ng, etal : Pul mona ry lnvolvementof Hypereos in ophilic Syndrome eosinophilic syndrome represents a heterogeneous fluid was elevated in all three patients, and TBLB that group of disorders characterized by a idiopathic pro­ was performed i~ 0ne pattent showed focal pneumonic longed eosinophilia of marked degree and, further­ infiltrations with eosinophils. These results strongly more, by variable organ dysfunction. It may involve vir­ suggest HES-related pulmon'ary involvement. Other or­ tually any organ system , though bone marrow hyper­ gan systems involved in our patients included the stom­ eosinophilia is common to all patients. The most sev­ ach, liver and bone marrow. Early recognition of the ere clinicopathologic involvement is of the heart and syndrome is important becäuse there is strong evi­ nervous system (4). dence that prognosis may.be much improved if treat­ Pulmonary involvement in HES occurs in up to 40 % ment is instituted promptly (9) . of patients, who typically present with cough or dysp­ nea (3 , 5). In patients with HES and pulmonary infiltrat­ REFERENCES es, the condition may be attributed to infecton , infarc­ tion , congestive heart failure or HES-related pulmon­ 1. Winn RE , Kollel MH , Meyer JI. Pulmonary in volve ment in the ary involvement (1 -4). One half of these patients have hypereosi noph ili c syndrome. Chest 1994 ; 105 : 656-660 significant pleural effusion, probably due to congestive 2. Chusid MJ , Dale DC , West BC , Wolff SM . Th e hype r eos inop 에 lic heart fail ure or embol ic phenomena (1 -3). A chest rad syn drome: analysis 01 lourteen cases with review 01 the litera­ iograph shows focal or diffuse, interstitial or alveolar, ture. Medicine 1975 ; 54 : 1-27 and nonlobar infiltrates (1 -3, 5-8). Winn et al (1) rep­ 3. Slabbynck h, Impens N‘ Naegels S, Dewaele M; Schandevyl W orted a patient who presented with ARDS that was Idi opathic hypereosinophili c synd ro me-related pulmonary in­ thought to be a complication of HES. Hilar Iymphad­ volvement diagnosed by bro nchoal veolar lavage. Chest 1992 ; enopathy was noted, but the prevalence of intrathor­ 101 :1178-1180 acic Iymphadenopathy had not been assessed. Histop­ 4. Fauci AS , Har ley JB , Rob ers WC , Ferrans VJ, Gralnick'HR , Bj or­ athology demonstrates striking infiltration of involved nson BH. The idiopathic hypereosinophilic syndrome. Clinical , pathoph ys iologic , and therapeutic considerati ons ..Ann Inter Med organs by eosinophils, is associated with disruption of 1982 ; 97 : 78-92 the architecture, and there are areas of necrosis. BAL 5. Allen JN, Davis WB. Eosin ophilic lung disease. Am J Respir Crit fluid eosinophilia may suggest HES-related p 비 monary Care Med 1994; 150 : 1423-1438 involvem ent (3 , 5). 6. Hardy WR, Anderson RE. The hypereosinophili c synd rome. Ann To our knowledge, this is the first report of HRCT fin­ Int Med 1968 ; 68 : 1220-1229 dings in patients with lung involvement of HES. In spite 7. Shannon JJ , Lynch JP. Eosinophi li c pulmonary syndromes. Clin ofno specfific abnormalities on chest radiographs, sev­ Pulm Med 1995 ; 2: 19-38 eral small nodules were seen in both lungs, especially 8. Epstein DM , Taormina V, Gefter WB , Mi ll er WT. The hyperesos­ peripheral lung areas on HRCT. One of them showed in ophil ic syndrome. Radiology 1981 ; 140 : 59-62 focal areas of ground-glass attenuation located sub­ 9. Fraser RG , Pare JAP. Diagnosis of diseases ofthe chest. 3rd ed pleurally and ground-glass attenuation halo around Ph iladelph ia: Saunders , 1989 : 1298-1299 nodules. In our patients, the eosinophil count in BAL

대 한 방 사 선 의 학 회 지 1996 ; 35( 3) : 343 -345

과호산구증후군의 폐침범 : 3 예의 고해상전산화단층촬영 소견 1

1 고려대학교의과대학진단방사선과학교실

2 고려대학교의과대학 내과학교실

강은영·이미란 · 심재정 2

과호산구증후군은여러장기를침범하는 드문 특발성호산구성폐질환의하 나로써 그중폐침범 은 40 %에서보고되 고 있 다.저

자들은임상적으로 그리고 기관지 폐포세척액과 기관지내시겸하 패생검으로진단된 3예의 특발성 과호산구증후군의 폐 침범의

고해상전산화 단층촬영 소견 을보고하고자 한다 .3예 모두에서 수개의 작은결절들이 앙폐야특히 주 변부 폐야에 분포되었고，그

중 1 여 | 에서는결절주위의 간유리음영의 환과국소간유 리 음영을보였 다.

…x 10th European Congress

OFFlCE : of Radiology (ECR’97)

EUROPEAN CONGRESS OF RADIOLOGY - ECR’ 97 NEUTORGASSE 9/2A A-1010 VIENNA/AuSTRIA z 맘얘펴며야야야상매략Conference Oates : March 2-7, 1997 ”며￠ Vienna/ Austria PHONE: (+43/ 1) 533 4064, 5334065, 5334066 FAX: (+43/ 1) 533 40649 Administrative and Scientific Secretariat : PZφ EMAIL: [email protected] ECR ’97 WWW: WWW.ECR.TELECOM.AT'\ECR F{)φι Neutorgasse 9/2a 〕 A-1010 Vienna/Austria Z걷따({φν{{]니 φ{({Telephone ( +43/1) 533 40 64 Fax # : ( +43/1) 533 40 649 벼 Abstract Oeadline: September 20, 1996 ν{ President: Prof. Dr. Hans Ringertz -ιφ 며 (Stockhol m/Sweden) kγ Estimated Attendance : 12 500 @ Technical Exhibition : 200 Exhibitors on 8000m2 f γ{ Scientific Exhibition : about 1000 F) scientific exhibits 뉘써

DEADLlNES:

SUBMISSION OF ABSTRACTS : SEPT, 20, 1996 REDUCED REGISTRATlON FEE: DEC. 2 , 1996 AOVANCE REGISTRATION JAN. 3 1, 1997

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