Acute Ischemia Hepatitis as a Major Clinical Presentation of the Primary Cardiac Lymphoma

118 J Emerg Crit Care Med. Vol. 23, No. 3, 2012 Acute Ischemia Hepatitis as a Major Clinical Presentation of the Primary Cardiac Lymphoma Yuan-Jhen ...
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J Emerg Crit Care Med. Vol. 23, No. 3, 2012

Acute Ischemia Hepatitis as a Major Clinical Presentation of the Primary Cardiac Lymphoma Yuan-Jhen Syue1,2, Yu-Chen Cheng3,4, Pai-Chun Yen2,5, Chin-Chen Chang6, Chao-Jui Li2,5 Primary cardiac lymphoma is extremely rare and antemortem diagnosis is difficult. Although this malignancy is not uncommonly reported, most patients in the literature typically presented with cardiac signs and symptoms. We described a 21-year-old man who presented initially with ischemic hepatitis but was finally diagnosed as having the tumor. Literature search from 1997 to 2009 revealed 27 cases of the tumor of which the clinical presentations were reviewed. Key words: acute ischemia hepatitis, primary cardiac lymphoma, B-cell lymphoma, congestive heart failure

Introduction Primary malignant lymphoma of the heart is

extremely rare with poor prognosis. Most primary cardiac lymphomas (PCLs) occur in the right atrium

or right ventricle(1). Because of its rarity and variety of clinical manifestations, antemortem diagnosis

remains a challenge to physicians. Although many reports of primary cardiac malignant lymphomas

have been published, most of them described patients with typical cardiac presentations. This article is the first to depict the manifestation of this tumor in a 21-year-old man with initial clinical manifestation of ischemic hepatitis. Literature search on the PubMed database from 1997 to 2009 was performed using the key words “primary

malignant cardiac lymphoma”. The retrieved literature was reviewed in terms of the patients’ clinical characteristics and presentations.

Case Report A 2 1 - y e a r- o l d , p r e v i o u s l y h e a l t h y m a n initially presented to a local clinic with intermittent abdominal discomfort for several days, including intermittent nausea, vomiting, and abdominal fullness sensation. Mild dyspnea on exertion was also noted a bout 2 weeks ago. He offered no history of cardiac, pulmonary, or gastrointestinal disease. Due to elevated serum levels of alanine transferase (ALT) and aspartate transferase (AST) at the clinic, the patient was transferred to our hospital

Received: February 3, 2012 Accepted for publication: September 15, 2012 From the 1Department of Anesthesiology, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan 2 Chang Gung University College of Medicine, Kaohsiung, Taiwan 3 Department of Diagnostic Radiology, E-Da Hospital, Kaohsiung, Taiwan 4 I-Shou University, Kaohsiung, Taiwan 5 Department of Emergency Medicine, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan 6 Department of Medical Imaging, National Taiwan University Hospital Yun-Lin branch Address reprint requests and correspondence: Dr. Yu-Chen Cheng Department of Diagnostic Radiology, E-Da Hospital 1 Yida Road, Jiaosu Village, Yanchao District, Kaohsiung City 82445, Taiwan (R.O.C.) Tel: (07)6150011 ext 2228 Fax: (07)6150915 E-mail: [email protected]

Acute ischemia hepatitis due to PCL 119

and was admitted with the tentative diagnosis of acute hepatitis. On arrival at our emergency

hepatic necro-inflammatory activities were noted. Anti-DS-DNA and anti-nuclear antibody (ANA)

minute, respiratory rate of 16 per minute, and a body temperature of 36.7 ºC with the absence of

deteriorate the next day with sudden onset of dyspnea that rapidly progressed to respiratory

department, his vital signs were stable with a blood pressure of 120/70 mmHg, heart rate of 75 per

metabolic acidosis on arterial blood gas analysis. On admission, initial laboratory investigations

were normal except for abnormal activities of liver enzymes with an ALT level of 119 U/L and AST level of 181 U/L. Viral screening tests for HBsAg, anti-HCV, and anti-HIV were negative. A chest radiograph showed a bulging contour at the right cardiac border and right pleural effusion (Fig. 1). Abdominal sonography showed no specific finding. On the sixth day of hospitalization, his vital signs

were stable. On the other hand, drastic elevations in levels of liver enzymes (ALT level: 1403 U/L, AST level: 1329 U/L) suggestive of rapidly progressive

Fig. 1

suggestive of autoimmune hepatitis were both negative. The condition of the patient began to

failure and cardiogenic shock. Echocardiography demonstrated a huge right atrial mass, the presence

of which was further confirmed by transesophageal echocardiography that also revealed its extension

to superior vena cava (SVC) (Fig. 2). During operation, tumor invasion of the SVC was noted with nearly complete luminal obstruction. The tumor was removed through right atriotomy. Pathological analysis of the tumor demonstrated large, transformed B-cells with prominent nucleoli

and basophilic cytoplasm, a diffuse growth pattern, and a high (>40 percent) proliferation fraction typical of a B-cell diffuse lymphocytic lymphoma

Chest radiograph showing a bulging contour at the right cardiac border and right pleural effusion


J Emerg Crit Care Med. Vol. 23, No. 3, 2012

(Fig. 3). Despite surgical removal of the tumor, the patient expired after surgery because of severe heart failure.

PCL is classically defined as an extranodal non-Hodgkin’s lymphoma, which is exclusively


ventricle and less often in left atrium and left ventricle(7). On the other hand, it can also extend

Primary cardiac neoplasms are rare with a reported prevalence of 0.001%-0.03% in autopsy series(1). Primary lymphoma is also an uncommon malignancy of the heart, accounting for only 1.3% of all primary cardiac neoplasms(1). The disease is most frequent in immunocompromised patients with a slight male predominance(2-5).

Fig. 2

Fig. 3

situated in the heart and/or pericardium (1,6) . It is usually located in the right atrium and right

to the inferior vena cava, superior vena cava, and jugular veins. Pathologically, it can involve all three layers of the heart including the epicardium, myocardium, and endocardium(8-10).

There are no pathognomonic symptoms or signs characteristic of a primary tumor of the heart(2). PCLs and other cardiac tumors present in a variety

Echocardiography demonstrating a huge right atrial mass

Pathological section of the tumor demonstrating large, transformed B-cells with prominent nucleoli and basophilic cytoplasm, a diffuse growth pattern, and a high proliferation fraction typical of a diffuse large cell lymphoma, B cell type

Acute ischemia hepatitis due to PCL 121

of ways and are rarely diagnosed on an antemortem basis(11-14). Most PCLs are discovered by postmortem examination without notable clinical presentations. The location of PCL reflects its pathophysiology

3. Exclusion of other putative causes of liver cell necrosis, such as viral or drug-induced hepatitis. Although laboratory studies of our patient showed high necro-inflammatory activities of

hemopericardium or pericardial effusion, the superior vena cava syndrome, cardiac arrhythmias, and

hepatitis B virus (HBV) and hepatitis C virus (HCV), and had not taken any hepatotoxic

and clinical symptoms and signs. More common presentations include congestive heart failure,

nonspecific electrocardiographic abnormalities(15). In many cases, the only clinical indication of a cardiac tumor is intractable congestive heart failure without an obvious cause(16-18). Demographic characteristics and clinical presentations of previously reported cases are summarized in Table 1.

In contrast to most published cases that presented with cardiac symptoms and signs, the

manifestation of ischemic hepatitis as in our patient has not been reported. Ischemic hepatitis, also known as hypoxic hepatitis or shock liver, is characterized by centrilobular liver cell necrosis

and sharply increasing serum aminotransferase levels in a clinical setting of cardiac, circulatory,

or respiratory failure (19) . The main underlying conditions contributing to ischemic hepatitis are

low cardiac output or septic shock. Although the pathological finding of

centrilobular liver cell necrosis is an essential element in the diagnosis of ischemic hepatitis, liver biopsy is usually not required. Furthermore, biopsy is often inadvisable in many patients of ischemic hepatitis who frequently exhibit abnormal coagulation profiles or receive anticoagulants due to their underlying cardiac diseases(20,21). The diagnosis of ischemic hepatitis is usually established using three simple criteria illustrated below(19): 1. Clinical setting of acute cardiac, circulatory, or respiratory failure. 2. Dramatic but transient increase in serum aminotransferase activity reaching at least 20 times the upper limit of normal.

the liver with dramatically elevated serum levels of ALT and AST, he was seronegative for both

m e d i c a t i o n s . C o n s i s t e n t l y, u l t r a s o n o g r a p h y demonstrated no abnormal finding in the abdominal organs. Laboratory data and physical findings after admission also showed no evidence of overt cirrhosis. Moreover, to date, there has been no report of lymphoma-related paraneoplastic effects

of hepatitis. Therefore, after exclusion of viral, drug-induced, and autoimmune hepatitis, we

speculated that the picture of ischemic hepatitis may result from hepatic outflow obstruction

possibly due to a cardiac lesion for which echocardiography was arranged that eventually identified the right atrial tumor. After the first report of PCL presenting with

gastrointestinal symptoms by Nakchbandi et al. in 1997 (22) , clinical manifestation of the tumor as hepatomegaly has also been documented by other authors (23,24) . Our case is the first report

of the rare presentation of PCL as acute-onset ischemic hepatitis. More importantly, our report is also the first to describe the occurrence of this unusual tumor in a young adult, thereby arousing the alertness of the possibility of this diagnosis in seemingly unlikely patients presenting with hepatic symptoms. Correct diagnosis in our case was further complicated by the fact that Taiwan is an hepatitis-endemic region in which viral cause is usually the first to consider.

Conclusions Our report is the first to describe the atypical


J Emerg Crit Care Med. Vol. 23, No. 3, 2012

Table 1


Summary of selected cases of primary cardiac lymphoma reported in the recent literature

Ceresoli et al.(3) Ceresoli et al.(3) Anghel et al.(4) Anghel et al.(4) Begueret et al.(5) Chim et al.(6)

Age Sex Location of tumor 64 M P, RA 55 M RA 52 M RA 70 F RA 62 F RA 69 F RA

Initial presentation Dyspnea Dyspnea Dyspnea Pericardial tamponade Dyspnea Dyspnea

Chim et al.(6)





Ikeda et al.(7)




Hsueh et al.(8) Zakja et al.(9)

58 61



Peripheral edema Dyspnea Chest pain Dyspnea

Nakayama et al.(10) Rockwell et al.(11) Rockwell et al.(11) Chalabreysse et al.(12) Chalabreysse et al.(12) Chalabreysse et al.(12) Saotome et al.(13)

61 41 63 72 65 9 69



Tai et al.(14) Carfagna et al.(15)

70 78



Catherine et al.(16)




Enomoto et al.(17) Enomoto et al.(17) Mejhert et al.(18) Nakchbandi et al.(22) Beckwith et al.(23) Beckwith et al.(23) Cordel et al.(24) present case

73 66 59 77 61 62 83 21



Liver function Heart failure ND N ND N WNL N WNL Y ND Y AST (36 U/l) Y ALT (73 U/l) AST (75 U/l) N ALT (27 U/l) WNL Y

WNL AST (73 U/l) ALT (106 U/l) Dyspnea ND Chest pain ND Brady arrhythmia ND SVCS ND Myalgia ND Fever, weight loss ND Pericardial effusion AST (147 U/l) ALT (395 U/l) Palpitation WNL Dyspnea positive for antibody to HCV Left hemiparesis AST (406 U/l) ALT (197 U/l) Dyspnea ND SVCS ND Dyspnea WNL Epigastric discomfort WNL Dyspnea WNL Left hemiparesis ND Dyspnea WNL Abdominal discomfort AST (181 U/l) ALT (119 U/l)

prognosis Died (7 months) CR (12 months) CR (24 months) Died (2 weeks) Died (1 month) Died (1 day) Died (10 weeks) Died (8 months)


Died (12 months) Died (20th day)


CR (ND) CR (ND) CR (ND) Died (12 months) CR (33 months) CR (12 months) Died (18th day)


CR (2 years) Died (2nd day)


Died (9th day)


CR (34 months) CR (21 months) Died (10 months) Died (2 weeks) Died (2 months) Died (12 months) CR (12 months) Died (7th day)

P: pericardium; RA: right atrium; RV: right ventricle; LA: left atrium; SVCS: superior vena cava syndrome; ND: not describe; WNL: within normal limits; AST: aspartate aminotransferase; ALT: alanine transaminase; CR: complete remission

Acute ischemia hepatitis due to PCL 123

presentation of ischemic hepatitis in a young adult with PCL, highlighting the wide variety of clinical manifestations of this rare tumor and also the possibility of young adults being involved.

Moreover, PCL should be included on the list of differential diagnosis for patients presenting with unaccountable impairment in hepatocyte integrity, especially in hepatitis-endemic areas, for early diagnosis and timely treatment of this highly lethal malignancy.

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8. Hsueh SC, Chung MT, Fang R, et al. Primary c a r d i a c l y m p h o m a. J C h i n M e d A s s o c 2006;69:169-74. 9. Z a k j a F, B a d a n o L P, S b r o j a v a c c a R, e t al. Unusual extension of an intracardiac primary lymphoma to the right jugular vein. J Cardiovasc Med (Hagerstown) 2007;8:652-5. 10. Nakayama Y, Uchimoto S, Tsumura K, et al. Primary cardiac lymphoma with infiltration of the atrioventricular node: remission with reversal of the atrioventricular block induced by chemotherapy. Cardiology 1997;88:613-6. 11. Rockwell L, Hetzel P, Freeman JK, Fereshetian A. Cardiac involvement in malignancies. Case 3. Primary cardiac lymphoma. J Clin Oncol 2004;22:2744-5. 12. C h a l a b r e y s s e L , B e r g e r F, L o i r e R , e t a l. P r i m a r y c a r d i a c l y m p h o m a i n immunocompetent patients: a report of three cases and review of the literature. Virchows Archiv 2002;441:456-61. 13. Saotome M, Yoshitomi Y, Kojima S, et al. Primary cardiac lymphoma: a case report. Angiology 2002;53:239-41. 14. Ta i C J, Wa n g W S, C h u n g M T, e t a l. Complete atrio-ventricular block as a major clinical presentation of the primary cardiac lymphoma: a case report. Jpn J Clin Oncol 2001;31:217-20. 15. Carfagna P, Redondi A, Taglietti F, et al. Difficulties in the diagnosis of primary cardiac lymphomas. Haematologica 2000;85:770-2. 16. Catherine R, Curtsinger BS, Michael J. et al. Primary cardiac lymphoma. Cancer 1989;64:521-5. 17. E n o m o t o S, A b o T, S u g a w a r a T, e t a l. Successful treatment of two patients with primary cardiac malignant lymphoma. Int J Hematol 1999;70:174-7. 18. Mejhert M, Muller-Suur R. Primary lymphoma of the heart. Scand Cardiovasc J 2000;34:606-8.


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19. Fuhrmann1 V, Jäger B, Zubkova A, Drolz A . H y p o x i c h e p a t i t i s - e p i d e m i o l o g y, pathophysiology and clinical management. Wien Klin Wochenschr 2010;122:129-39. 20. Fuhrmann V, Kneidinger N, Herkner H, et al. Hypoxic hepatitis: underlying conditions and risk factors for mortality in critically ill patients. Inten Care Med 2009;35:1397-405. 21. Gitlin N, Serio K. Ischemic hepatitis: widening horizons. Am J Gastroenterol 1992;87:831-6. 22. Nakchbandi IA, Day HJ. Primary cardiac

lymphoma: initial symptoms suggestive of gastrointestinal disease. South Med J 1997;90:539-43. 23. Beckwith G, Butera J, Sadaniantz A, et al. Diagnosis in oncology: non-Hodgkin’s lymphoma involving the heart. J Clin Oncol 2000;18:1996-9. 24. C o r d e l N, G e ff r o y C E, C a p e t C, e t a l. Cardiac malignant lymphoma successfully treated with chemotherapy. Eur J Intern Med 2001;12:130-3.

原發性心臟淋巴瘤引起急性缺血性肝炎 125

原發性心臟淋巴瘤引起急性缺血性肝炎 薛媛真1,2 鄭玉貞3,4 顏百駿2,5 張晉誠6 李肇瑞2,5 原發性心臟淋巴瘤是極其罕見且診斷困難。儘管此惡性腫瘤很少被報導,大多數病人在文獻中通常 仍以心臟病徵來表現。我們描述了一名最初以缺血性肝炎為表徵,但最後被查出患有此腫瘤的21歲男 子。 關鍵詞: 急性缺血性肝炎,心肌淋巴瘤,B細胞淋巴瘤,充血性心衰竭

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