Cardiac granulocytic sarcoma (chloroma): In vivo diagnosis with transesophageal echocardiography
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Cardiac Granulocytic Sarcoma (Chloroma): In Vivo Diagnosis with Transesophageal Echocardiography Pedro Marcos-Alberca, MD, Borja Iba ´n ˜ez, MD, Manuel Rey, MD, Alejandro Roma ´n, MD, Rosa Ra ´bago, MD, Miguel Orejas, MD, Jose ´ F. Toma ´s, MD, PhD, and Jero ´ nimo Farre ´, MD, PhD, Madrid, Spain Granulocytic sarcoma, or chloroma, is an uncom- mon presentation of acute leukemia. Cardiac in- volvement is very rare and usually diagnosed at autopsy. We present a case that discloses the essen- tial role of transesophageal echocardiography for its in vivo diagnosis. The principal features with this imaging technique are finely described. (J Am Soc Echocardiogr 2004;17:1000-2.) Granulocytic sarcoma is a local infiltrative mass formed by immature granulocytic cells that can complicate acute or chronic myeloid leukemia. 1 Cardiac granulocytic sarcoma is very rare, 2-6 and even more exceptional is its in vivo diagnosis on the basis of imaging techniques, mainly echocardiogra- phy. 7,8 The transesophageal echocardiographic (TEE) features of granulocytic sarcoma have been exceptionally described. CASE REPORT A 39-year-old man was admitted into our hospital with progressive fatigue, dyspnea, and edema in both legs during the last 2 weeks. Five years before this admission he had been given a diagnosis of M1 acute myeloid leukemia and treated with standard chemotherapy. A relapse of his hematologic disorder developed 1 year after the initial treatment, requiring an allogenic bone-marrow transplantation. He remained thereafter in complete clin- ical remission. On physical examination, he was tachypneic, his blood pressure was 85/60 mm Hg, and he had pulmonary auscultatory findings consistent with a right pleural effu- sion, hepatomegaly, and severe bilateral edema in the lower extremities. Moreover, neck veins were evidently distended to the angle of the jaw, with a tall A wave and a diastolic murmur heard along the lower parasternal border, increasing with inspiration. A chest radiograph confirmed the presence of severe right pleural effusion and cardiomegaly. A transthoracic echocardiogram re- vealed mild pericardial effusion, severe thickening of the right ventricular free wall (suggesting infiltration of the myocardium), and a well-defined right atrial (RA) mass extending through the tricuspid valve toward the right ventricle and producing a severe obstruction of the tricus- pid diastolic flow. In addition, the substernal view showed an enlarged liver with evident dilation of suprahepatic veins, together with a dilated inferior cava vein, not collapsing with inspiration. TEE confirmed the presence of a huge RA mass (29 35 mm) occluding almost completely the tricuspid orifice (Figure 1). Multiplane examination allowed us to show that the primary attach- ment of the mass was located at the junction between the superior cava vein and the RA (Figure 2). From this site the mass extended toward the tricuspid ring across the an- terolateral part of the RA. The peripheral blood count and the bone-marrow biopsy specimen were unremarkable, but the analysis of the pleural effusion revealed the presence of large quan- tities of granulocytic cells with an immunophenotype equal to that of the primary myeloid leukemic process. With the clinical suggestion of cardiac granulocytic sar- coma, the patient underwent intensive chemotherapy and thoracic radiotherapy for 8 consecutive weeks. At the end of this treatment, signs of right-sided heart failure had disappeared. TEE was not carried out because of the severe mucositis, but a transthoracic echocardiogram revealed no pericardial effusion, and the absence of any intracavitary mass or infiltration of the right ventricular myocardium. Doppler examination exhibited the dramatic improvement of the diastolic tricuspid flow after the resolution of the mass, according to the alleviation of the symptoms and signs of right-sided heart failure (Figure 3). The latter evolution supports the diagnosis of cardiac granulocytic sarcoma. 6 A second allogenic bone-marrow transplantation was subsequently performed. From the Departments of Cardiology and Hematology (A.R., J.F.T.), Fundacio ´ n Jime ´nez Di ´az, Universidad Auto ´ noma. Reprint requests: Pedro Marcos-Alberca, MD, Laboratorio de Ecocardiografi ´a, Servicio de Cardiologi ´a, Fundacio ´n Jime ´nez Di ´az, Avenida Reyes Cato ´ licos 2, 28040 Madrid, Spain (E-mail: [email protected]). 0894-7317/$30.00 Copyright 2004 by the American Society of Echocardiography. doi:10.1016/j.echo.2004.04.030 1000
Transcript of Cardiac granulocytic sarcoma (chloroma): In vivo diagnosis with transesophageal echocardiography
Cardiac Granulocytic Sarcoma (Chloroma): InVivo Diagnosis with Transesophageal
EchocardiographyPedro Marcos-Alberca, MD, Borja Ibanez, MD, Manuel Rey, MD, Alejandro Roman, MD,
Rosa Rabago, MD, Miguel Orejas, MD, Jose F. Tomas, MD, PhD, and Jeronimo Farre, MD, PhD,Madrid, Spain
Granulocytic sarcoma, or chloroma, is an uncom-mon presentation of acute leukemia. Cardiac in-volvement is very rare and usually diagnosed atautopsy. We present a case that discloses the essen-
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tial role of transesophageal echocardiography for itsin vivo diagnosis. The principal features with thisimaging technique are finely described. (J Am SocEchocardiogr 2004;17:1000-2.)
Granulocytic sarcoma is a local infiltrative massformed by immature granulocytic cells that cancomplicate acute or chronic myeloid leukemia.1
Cardiac granulocytic sarcoma is very rare,2-6 andeven more exceptional is its in vivo diagnosis on thebasis of imaging techniques, mainly echocardiogra-phy.7,8 The transesophageal echocardiographic(TEE) features of granulocytic sarcoma have beenexceptionally described.
CASE REPORT
A 39-year-old man was admitted into our hospital withprogressive fatigue, dyspnea, and edema in both legsduring the last 2 weeks. Five years before this admissionhe had been given a diagnosis of M1 acute myeloidleukemia and treated with standard chemotherapy. Arelapse of his hematologic disorder developed 1 year afterthe initial treatment, requiring an allogenic bone-marrowtransplantation. He remained thereafter in complete clin-ical remission.
On physical examination, he was tachypneic, his bloodpressure was 85/60 mm Hg, and he had pulmonaryauscultatory findings consistent with a right pleural effu-sion, hepatomegaly, and severe bilateral edema in thelower extremities. Moreover, neck veins were evidentlydistended to the angle of the jaw, with a tall A wave anda diastolic murmur heard along the lower parasternal
From the Departments of Cardiology and Hematology (A.R.,J.F.T.), Fundacion Jimenez Diaz, Universidad Autonoma.Reprint requests: Pedro Marcos-Alberca, MD, Laboratorio deEcocardiografia, Servicio de Cardiologia, Fundacion JimenezDiaz, Avenida Reyes Catolicos 2, 28040 Madrid, Spain (E-mail:[email protected]).0894-7317/$30.00Copyright 2004 by the American Society of Echocardiography.doi:10.1016/j.echo.2004.04.030
border, increasing with inspiration. A chest radiographconfirmed the presence of severe right pleural effusionand cardiomegaly. A transthoracic echocardiogram re-vealed mild pericardial effusion, severe thickening of theright ventricular free wall (suggesting infiltration of themyocardium), and a well-defined right atrial (RA) massextending through the tricuspid valve toward the rightventricle and producing a severe obstruction of the tricus-pid diastolic flow. In addition, the substernal view showedan enlarged liver with evident dilation of suprahepaticveins, together with a dilated inferior cava vein, notcollapsing with inspiration. TEE confirmed the presenceof a huge RA mass (29 � 35 mm) occluding almostcompletely the tricuspid orifice (Figure 1). Multiplaneexamination allowed us to show that the primary attach-ment of the mass was located at the junction between thesuperior cava vein and the RA (Figure 2). From this site themass extended toward the tricuspid ring across the an-terolateral part of the RA.
The peripheral blood count and the bone-marrowbiopsy specimen were unremarkable, but the analysis ofthe pleural effusion revealed the presence of large quan-tities of granulocytic cells with an immunophenotypeequal to that of the primary myeloid leukemic process.With the clinical suggestion of cardiac granulocytic sar-coma, the patient underwent intensive chemotherapy andthoracic radiotherapy for 8 consecutive weeks. At the endof this treatment, signs of right-sided heart failure haddisappeared. TEE was not carried out because of thesevere mucositis, but a transthoracic echocardiogramrevealed no pericardial effusion, and the absence of anyintracavitary mass or infiltration of the right ventricularmyocardium. Doppler examination exhibited the dramaticimprovement of the diastolic tricuspid flow after theresolution of the mass, according to the alleviation of thesymptoms and signs of right-sided heart failure (Figure 3).The latter evolution supports the diagnosis of cardiacgranulocytic sarcoma.6 A second allogenic bone-marrowtransplantation was subsequently performed.
Journal of the American Society of EchocardiographyVolume 17 Number 9 Marcos-Alberca et al 1001
DISCUSSION
Granulocytic sarcoma, or chloroma, is an uncom-mon presentation of acute leukemia and, as in ourcase, can exist in the absence of leukemic cells inperipheral blood or bone-marrow biopsy speci-mens.9 Chloromas are most frequently found in theskin at the sites of venopunctures.1,9 Cardiac in-volvement has been reported, generally consistingof a massive myocardial infiltration.4,5,8 The presen-tation as an isolated and locally invasive intracavitarymass has been rarely reported.3,6,7 Most cardiacchloromas so far reported have been diagnosed atautopsy.2 Our case is rare in that it was diagnosedwhile the patient was still alive and the TEE featuresof this extremely unusual tumoral cardiac pathology
Figure 1 Transesophageal long-axis horizontal view at 0degrees. A, Huge mass (29 � 35 mm) is at tricuspid valvelevel (arrow). There is unusual increase in thickness of rightventricular (RV) free wall and mild pericardial effusion(arrowheads) suggesting presence of infiltrative process. B,Intravenous contrast (Optison, 0.5 mL) was administeredto better delineate mass and establish relationships withsurrounding cardiac structures. Mass had well-definedboundaries and polylobulated morphology. Mild contrastreplenishment denotes some degree of perfusion. LA, Leftatrium; LV, left ventricle; RA, right atrium.
are precisely described.7,8 It is noteworthy to sup-port our clinical suggestion in the result of thecytometric analysis of the samples obtained from thepleural effusion, considering the negativity of theresults in peripheral blood sample and bone-marrowbiopsy specimen, thus, avoiding the risks of a trans-jugular biopsy of the mass. The early diagnosispermitted in our case an aggressive treatment of thislife-threatening condition that resulted in a completeechocardiographic resolution of the cardiac involve-
Figure 2 Transesophageal long-axis vertical view at 90degrees showing left atrium (LA), right atrium (RA), andopening of superior cava vein (SCV). Mass, in anterolateralRA, attaches just at joint of SCV (arrow). There are alsopericardial and pleural effusions (arrowheads).
Figure 3 Comparison of 2-dimensional transthoracic echo-cardiogram (top) and Doppler analysis of diastolic tricuspidflow (bottom) at admission (A) and after 8 consecutiveweeks of chemotherapy and radiotherapy (B). Note disap-pearance of huge right atrial (RA) mass (arrow) and dra-matic improvement of diastolic flow: at inspiration, maxi-mal diastolic velocity decreased from 2.2 m/s at admissionto 1.1 m/s after end of treatment. Chart velocity in A is100 mm/s and in B is 25 mm/s. LA, Left atrium; LV, leftventricle; RV, right ventricle.
Journal of the American Society of Echocardiography1002 Marcos-Alberca et al September 2004
ment and the disappearance of the clinical manifes-tations of tricuspid obstruction.
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