Hypereosinophilic Syndrome: Clinical, Laboratory, and Imaging Manifestations in Patients with Hepatic Involvement
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대 한 방 사 선 의 학 회 지 1993 ; 29 (4) : 757~ 764 Journal of Korean Radiological Society, July, 1993 Hypereosinophilic Syndrome: Clinical, Laboratory, and Imaging Manifestations in Patients with Hepatic Involvement Gi Beom Kim, M.D., Ok Hwoa Kim, M.D.*, Jong Min Lee, M.D., Yeong Soon Sung, M.D., Duk Sik Kang, M.D. Department 01 Radiology, Kyungpook National Universi낀I College 01 Medicine - Abstract- The hypereosinophilic syndrome (HES) commonly involves liver and spleen but only a few literature has reported the imaging features. In this article, we present the imaging features of the liver and spleen in HES patients together with clinical and laboratoη features. This study included 5 HES patients with hepatic involvement. Extensive laboratory tests including multiple hematologic, serolo밍 c , parasitologic, and immunologic examinations were performed. Imaging studies includ ed CT, ultrasound (US) of upper abdomen and hepatosplenic scintigraphy. All patients were perio벼C 떠 lyexam ined by laboratory and imaging studies for 4 to 24 months. The common clinical presentations were weakness, rnild fever, and dry cough. All patients revealed leukocy tosis with eosinophilia of 40 to 80% and benign eosnophilic hyperplasia of the bone marrow. The percutane ous biopsy of the hepatic focal lesions performed in 2 patients showed numerous benign eosinophilic infIltrates and one of them revealed combined centrilobular necrosis of hepatocytes. All cases revealed hepatomegaly with m띠 tiple focallesions on at least one of CT, US, or scintigraphy. These findings completely disappeared in 2 to 6 months following medication of corticosteroid or antihistamines. The HES involved the liver and CT, US , or scintigraphic studies showed hepatic multifocal lesions with hepatomegaly. Differential diagnoses of these findings should include metastatic disease, lymphoma, leukemia, can버 diasis or other opportunistic infections. Index Words: Hypereosinophilia Liver, CT 76 1.1 211 Liver, Neoplasm 761.31 Occasionally, despite extensive investigation, INTRODUCTION significant eosinophilia may be present without any identifiable underlying disorder, and this Eosinophilia commonly appeared in with a constitutes the term HES applied by Hardy and variety of disorders including allergic and para Anderson(4). sitic diseases, collagen vascular diseases, chronic The HES is a spectrum of clinical disorders granulomatous diseases, and some malignancies characterized by leukocytosis with marked eo (1 -3). sinophilia without identifiable cause and organ * 울산동강병원 진단방사선과 * De.φartmeη t 01 Radiology, Doη:gkang Geη eral Hospital, Ulsan, Korea 이 논문은 1993 년 2 월 3 일 접수하여 1993 년 5 월 3 일에 채택되었음. Received February 3, Accepted May 3, 1993 …? Journa l of Korean Radio logical Society 1993; 29 (4) : 757~ 764 system dysfunction. The criteria for the diagno This study included five patients with manifes sis of HES as outlined by Chusid et al.(2) are: tation of hepatic involvement. The patients (a) a persistent eosinophilia of 1500 eosinophils ranged from 27 to 60 years of age; all were j mm3 for longer than 6 months, or death be male. The patients were presented with flu-like fore 6 months associated with the signs and symptoms or right upper quadrant abdorninal symptoms of hypereosinophilic disease; (b) lack dull pain and marked blood eosiniphilia. Three of evidence for parasitic, allergic, or other påtients (Cases 1, 2 and 5) with progressive known causes of eosinophilia; and (c) an evi organ system dysfunction were treated with dence of organ system involvement. steroid and other two patients showed stable We have encountered several HES patients laboratoη data received antihistarnine treat in whom CT, US, or scintigraphy of liver and ment only. spleen has demonstrated hepatic multifocal le Laboratory exarnination included serial sions and hepatosplenomegaly. Although there complete blood counts, urinalysis, multiple ser have been many reports dealing with clinical 이0멍c tests for infectious and connective tissue and laboratory findings(1 ,2, 4 - 7) of HES or diseases, bone marrow aspiration and biopsy, chest radiographic findings in patient with car ECG, functional tests for liver and kidney, and diopulmonary involvement(8-1 0), only a few lit allergic skin tests. Multiple stool samples were erature(ll), to our knowledge, have reported exarnined for ova and parasites of Clonorchis the imaging features of involved liver in HES sinensis, Entamoeba histolytica, and Para patient. This article presents imaging features gonimus westermani. Multiple cultures and of the liver and spleen together with clinical smears of blood and sputum for bacteria and 없ld laboratory features in our 5 HES cases. fungi and extensive immunologic evaluations (e. g., serum IgE level, T4jT8 ratio) were done. MATERIALS AND METHODS Markers for certain leukemias such as serum vi tarnin B 12 levels and the chromosomes were We experienced six patients who ful analysed. Two patients under-went US-guided filled the criteria of HES over a 2-year period. needle biopsy of the liver. Table 1. Summary of Clinical and Laboratory Findings A α 않 pePL C pi Hematologic Data Electro α 찌 κ 뼈 m 따 m ” E VA Biopsy of Liver WBC, xl03/ mm3 Eosinophils, % cardiogram 1/ 27/ M Mild fever, dry 34.9 78 Numerous eosinophilic Sinus cough, weight loss (36.6-9.5) (82-39) infiltrates aπhythrnia 2/ 42/ M Fatigue, rnild fever, 25.8 50 Centrilobular necrosis Normal pruritic rash (29.5-8.1) (68-8) with eosinophilic infiltration 3/ 41 / M Cough, chest discomfort, 17.5 60 Normal weight loss, paresthesia (26.3-8.7) (65-16) 4/60/M RUQ abdorninal p떠 n , 23 .2 75 Sinus pruritic rashes (23.2-9.6) (75-10) bradycardia 5/34/M Left lower chest and 30 .1 48 LUQ abdorninal pain (4 1.1 -13.8) (70-9) Hematologic data represent values at the time of initial exarnination; figures in parentheses represent subse quent extremes. 얘ω Gi Beom Kim , et al : Hypereosinophilic Syndrome Imaging studies included CT and US scans (Table 1). of upper abdomen and hepatosplenic scintigra The patients presented with leukocytes be phy. All patients were periodic떠 ly examined by tween 10,000 and 40,000/mm3 with 40 to 80 laboratory and imaging studies for 4 to 24 % peripheral eosinophilia. Bone marrow aspira months. tion 뻐d biopsy revealed benign eosinophilic hyperplasia in all cases. US-guided needle biop RESULTS sy of hepatic focal lesions was performed in 2 patients (Cases 1, 2). Case 1 showed numerous All the 5 patients were male and the mean eosinophilic infùtrates and Case 2 showed age of onset was 41 . lnitial clinical presenta centrilobular necrosis of hepatocytes with eo tions of the patients were varied, weakness, sinophilic infiltrates (Fig. 2d). All patients had mild fever, dry cough with chest discomfort, mild liver function abnormalities with slightly and weight loss were frequent complaints. No elevated SGOT, SGPT or alkaline phosphatase remarkable allergy or drug history was presented level. Serologic and immunologic tests were un- a b c d Fig. 1. Case 1, A 27-year-old man with flu-like symptoms for 1 month. a. CT shows enlarged liver and spleen without focallesion. b. US of liver shows a diffuse coarse parenchym꾀 echogenicity without focal mass. c. Hepatosplenic scintigram shows marke버y enlarged liver with large photon defects in both lobes and prorni nent spleen. d. Follow up scintigraphy of the liver 6 months after corticosteroid treatment shows normal liver and spleen 쩌때 Journal of Korean Radiological Society 1993; 29 (4 ) : 757~ 764 a b c d Fig. 2. Case 2, A 42-year-old man with fatigue and pruritic rashes for 2 months. a. CT shows multiple ill-defined hypodense nodules in enlarged liver (arrows). b. US of liver shows multiple ill-defined hypoechoic nodules. c. Hepatic scintigram shows slightly enlarged liver with m버 tiple photon defects. d. Photomicrograph of liver shows centrilobular necrosis of hepatocytes and nurnerous eosinophlic infiltrates. remarkable except elevated serurn IgE level in sions were noted in four out of the 5 patients 4 patients. Other studies including and appeared as variable sized, hypodense le bacteriologic, parasitologic, and allergic skin sions with ill defined margins (Fig. 2a, 3a). The tests were normal. lesions of Case 4 disappeared on follow-up CT Table 2 summarized the findings of liver scan taken after 2 months. On US examination and spleen on CT, US, and scintigraphy in the the focal nodular lesions were noted in three. 5 patients. All patients showed mild to marked They were ill defined, hypoechoic (Case 2, 5) or hepatomegaly with multiple focal lesions. The hyperj isoechoic (Case 3) nodules. Our impres splenomegaly of mild to moderate degree with sion at the time of initial examination was mul out focal lesion was noted in four. Although tiple hemangioma due to the hyper and multiple focal lesions were visualized on at least isoechogenicity of the nodules showed in Case one of the three modalities, it was unusual that 3, but six months later these nodules were com these focal lesions appeared on all three pletely disappeared. Hypoechoic lesions of modalites (Case 2). On CT scans, the focal le- Cases 2 and 5 were also disappeared six and - 760- Gi Beom Kim , et al : Hypereosinoph ilic Syndrome a b Fig. 3. Case 5, A 34-year-old man with LUQ abdominal dull pain for 1 month. a. CT shows a 3cm ill-defined hypodense lesion (arrows) in anterior aspect of the right lobe of the liver. b. US of liver shows a 3cm hypoechoic mass (large arrows) and several smaller daughter nodules in adjacent area (small arrows).