Annals of Pediatric Cardiology
About us | Current Issue | Archives | Ahead of Print | Instructions | Submission | Subscribe | Advertise | Contact | Login 
     
     
 


 

 
     
    Advanced search
 

 
 
     
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Clinical Details
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed126    
    Printed0    
    Emailed0    
    PDF Downloaded21    
    Comments [Add]    

Recommend this journal

 


 
Table of Contents   
CASE REPORT  
Year : 2022  |  Volume : 15  |  Issue : 1  |  Page : 70-72
Restrictive physiology masked by cardiac tamponade: A rare cooccurrence in a child with lymphoblastic T-cell lymphoma


1 Department of Cardiology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Clinical Haematology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Click here for correspondence address and email

Date of Submission16-Mar-2021
Date of Acceptance27-May-2021
Date of Web Publication14-Jun-2022
 

   Abstract 


Cardiac involvement in T-cell lymphoma is not uncommon. Pericardial effusion is the most common manifestation of cardiac involvement with restrictive cardiomyopathy (RCM) due to tumor infiltration being extremely rare. The presence of paroxysmal nocturnal dyspnea and orthopnea in a patient presenting with pericardial effusion could be related to tamponade or underlying myocardial disease. Hence, reevaluation after pericardiocentesis is warranted. We describe a 14-year-old boy with advanced T-cell lymphoma presenting with cardiac tamponade. Repeat echocardiography after pericardiocentesis revealed mediastinal mass infiltrating cardiac chambers and great vessels along with features of RCM secondary to tumor infiltration.

Keywords: Cardiac lymphoma, restrictive cardiomyopathy, T-cell lymphoma, tamponade

How to cite this article:
Gawalkar AA, Ghosh S, Bootla D, Revaiah PC, Singh C, Otaal PS. Restrictive physiology masked by cardiac tamponade: A rare cooccurrence in a child with lymphoblastic T-cell lymphoma. Ann Pediatr Card 2022;15:70-2

How to cite this URL:
Gawalkar AA, Ghosh S, Bootla D, Revaiah PC, Singh C, Otaal PS. Restrictive physiology masked by cardiac tamponade: A rare cooccurrence in a child with lymphoblastic T-cell lymphoma. Ann Pediatr Card [serial online] 2022 [cited 2022 Jul 5];15:70-2. Available from: https://www.annalspc.com/text.asp?2022/15/1/70/347417





   Introduction Top


Cardiac involvement secondary to lymphoma has been reported in up to 20% of cases,[1] the majority of which are of B-cell origin.[2] T-cell lymphoma involves cardiac structures rarely and tends to be more aggressive.[2] The most common cardiac involvement detectable is pericardial effusion with rare reports of endomyocardial involvement.[3] Clinical presentation with restrictive cardiomyopathy (RCM) due to tumor infiltration is extremely rare in lymphoma.[4],[5],[6]


   Clinical Details Top


A 14-year-old boy presented to us with fever, weight loss, and progressive dyspnea for 2 months. At a peripheral clinic, he was found to have pleural effusion. After 1 month, he was referred to our hospital due to the lack of response, worsening dyspnea associated with new-onset orthopnea, and paroxysmal nocturnal dyspnea (PND). On arrival, he had tachypnea, tachycardia, hypotension, and pulsus paradoxus (16 mmHg). Jugular venous pressure was elevated with prominent x descent and absent y descent, the Kussmaul sign was absent, and the first and second heart sounds were muffled. There were few basal crepitations. These clinical features predominantly suggested cardiac tamponade.

Relevant blood investigations revealed elevated total leukocyte count (12,000/mm3) and N-terminal pro-brain natriuretic peptide (NT pro-BNP) level (1255 pg/ml). Chest X-ray revealed an enlarged cardiac silhouette with evidence of pulmonary venous hypertension and bilateral mild pleural effusion. Electrocardiography revealed sinus tachycardia with T inversions in leads V2-V6, II, III, and aVF. Transthoracic echocardiography (TTE) revealed a large pericardial effusion with more than 25% variation of mitral E velocity with respiration and the diastolic collapse of right ventricular outflow tract suggestive of cardiac tamponade. No features suggestive of restrictive physiology were detected. Immediate pericardiocentesis of 1.2 L hemorrhagic fluid improved his hemodynamics, but the PND persisted pointing toward possible myocardial disease.

The pericardial fluid analysis revealed an exudative effusion with adenosine deaminase level of 16.8 IU/L, while the smear for acid-fast bacilli and Gene Xpert for tuberculosis were negative. Pericardial fluid cytology showed a discrete population of atypical lymphoid cells, 1.5–2.5 times the size of mature lymphocytes with opened up chromatin, high nuclear: cytoplasmic ratio, and scanty cytoplasm. In immunohistochemistry, cluster of differentiation (CD) 45 positive cells were gated. Of these, 98% were CD 3, CD 5, CD 43, and CD 38 positive. These cells showed dual positivity for CD 4 and CD 8, negative for CD 19, CD 20, CD 34, CD 23, and no light chain reaction was noted. All these features suggested T-cell lymphoblastic lymphoma (T-LBL).

A repeat 2D TTE after complete pericardiocentesis showed bi-atrial enlargement, biventricular wall thickening [Figure 1]a, Grade III diastolic dysfunction (septal e' was 5.9 cm/s, E/A was 2.7 and E/e' was 15), and left ventricular ejection fraction of 55% suggestive of RCM [Figure 2]a, [Figure 2]b, [Figure 2]c, possibly due to infiltration by tumor cells. Inferior vena cava was dilated and noncollapsible. A visible echogenic ring-like encasement of the ascending aorta, descending thoracic aorta, main pulmonary artery, and proximal segment of its branches indicated tumor infiltration [Figure 1]b, [Figure 2]c, [Figure 2]d, [Figure 2]d and Supplementary Videos 1 [Additional file 1] and Videos 2 [Additional file 2]].
Figure 1: (a) Postpericardiocentesis echocardiography showing mild pericardial effusion (white asterisk), right ventricular and left ventricular (yellow arrow) thickening and thickening of the descending thoracic aorta (yellow asterisk). (b) Pleural effusion (white asterisk), pericardial effusion, and infiltration of aorta (yellow arrows). (c) Lymphoma encasing the aorta. (d) Lymphoma infiltrating aorta (yellow arrows) and the pulmonary artery (white arrows)

Click here to view
Figure 2: (a) Septal mitral tissue Doppler showing e' of 5.9 cm/s and a' of 3.5 cm/s. (b) Lateral mitral tissue Doppler showing e' of 4.25 cm/s and a' of 3.3 cm/s. (c) Mitral inflow Doppler showing E of 88.5 cm/s and A of 32.5 cm/s (E/A of 2.7). (d) Suprasternal view showing tumor mass between the aortic arch and pulmonary artery (asterisk)

Click here to view


Contrast-enhanced computed tomography (CT) scan of the chest [Figure 3] revealed ill-defined sheet-like multi-compartmental soft-tissue lesions in the anterior mediastinum, insinuating between the great vessels and middle mediastinum. There was a pericardial extension of the lesion on the right anterior aspect of the mediastinum with moderate pericardial effusion. The lesion was also seen encasing great vessels. Inferior vena cava and hepatic veins were dilated with reflux of contrast.
Figure 3: Computed tomography showing ill-defined sheet-like multi-compartmental soft-tissue lesions in the anterior mediastinum (white asterisks) and insinuating between the great vessels and middle mediastinum (yellow asterisk). MPA: Main pulmonary artery

Click here to view


Bone marrow examination revealed no involvement. After confirming the diagnosis of T-LBL, he was started on chemotherapy with a modified BFM-2000 (Berlin Frankfurt Munster) protocol with vincristine, daunorubicin, and L-asparaginase along with a corticosteroid. Within 1 week of beginning the chemotherapy, his pericardial effusion resolved, and the pigtail drain was removed. Four days after, the second cycle of L-asparaginase, he developed focal seizures along with right hemiparesis. CT scan of the brain revealed left frontal lobe infarct with hemorrhagic transformation and subarachnoid hemorrhage suggestive of possible cerebral venous thrombosis. CT venography and angiography of intracranial vessels were unremarkable. Magnetic resonance imaging brain and further digital subtraction angiography of intracranial vessels could not be done. His seizure resolved and limb weakness completely resolved over 2 weeks. Due to a deterioration in his performance status, chemotherapy was de-escalated after discussion with the family. Despite the initial improvement, he succumbed to his illness 8 months later to severe community-acquired pneumonia.


   Discussion Top


Cardiac involvement in T-cell lymphoma is not uncommon.[2] The direct extension can lead to pericardial effusion and compression of cardiac structures. In patients with lymphoma, dyspnea related to diastolic heart failure is scarcely reported in the literature.[4],[5],[6],[7] The presence of pulmonary venous hypertension in chest X-ray and elevated NT pro-BNP support the diagnosis of heart failure in the index case. Although his hemodynamics improved, the persistence of PND after pericardiocentesis in our patient supports its origin to diastolic dysfunction because of RCM rather than cardiac tamponade. Pericardial effusion is the commonest echocardiographic feature of cardiac involvement in patients of NHL.[8] Massive pericardial effusion and cardiac tamponade as a primary manifestation of lymphoma is rare and mainly occurs in B-cell lymphoma.[3] Literature review reveals only a few published reports of T-cell lymphoma with cardiac tamponade.[9] Although dyspnea is common to both pericardial and myocardial involvement, associated orthopnea or PND as in our case is not specific for either of these. As it is difficult to differentiate based on presenting symptoms alone, a detailed echocardiography reassessment is warranted after the pericardiocentesis to help differentiate between these entities. The occurrence of RCM with lymphomas is extremely rare, and its evidence is limited to a few case reports.[4],[5],[6] Earlier studies have indicated toward a higher incidence of cardiomyopathy in patients with lymphoma.[10] Our patient developed cerebrovascular accident likely related to L-asparaginase therapy which is known to significantly increase the risk of thrombotic events (5.2%). After recovering from the cerebrovascular accident, he continued to receive nonasparaginase-based chemotherapy.

The index case illustrates that T-LBL can have an atypical presentation with cardiac tamponade and RCM, requiring careful reassessment after pericardiocentesis. It is the first report of these rare coexisting cardiac pathologies in a patient with T-cell lymphoma to the best of our knowledge. Although the nonavailability of CMR limits our case, the pattern of myocardial involvement points toward tumor infiltration and warrants evaluation for the underlying cause.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
McDonnell PJ, Mann RB, Bulkley BH. Involvement of the heart by malignant lymphoma: A clinicopathologic study. Cancer 1982;49:944-51.  Back to cited text no. 1
    
2.
Gordon MJ, Danilova O, Spurgeon S, Danilov AV. Cardiac non-Hodgkin's lymphoma: Clinical characteristics and trends in survival. Eur J Haematol 2016;97:445-52.  Back to cited text no. 2
    
3.
Quiroz E, Hafeez A, Mando R, Yu Z, Momin F. A malignant squeeze: A rare cause of cardiac tamponade. Case Rep Oncol Med 2018;2018:5470981.  Back to cited text no. 3
    
4.
Mohan JC, Khan JA, Srivastava S, Malik VK. Non-Hodgkin's lymphoma presenting as restrictive cardiomyopathy in a patient with two previously unrelated malignancies. Indian Heart J 1993;45:497-9.  Back to cited text no. 4
    
5.
Ngab T, Nikhanj N, Thomas M, Wachsner R, Kamangar N. Restrictive cardiomyopathy due to endocardial invasion by advanced peripheral T-cell lymphoma. Chest 2011;140:Vol 140(4_MeetingAbstracts):60A.  Back to cited text no. 5
    
6.
Fujisaki J, Tanaka T, Kato J, Saito T, Yano K, Shimizu Y, et al. Primary cardiac lymphoma presenting clinically as restrictive cardiomyopathy. Circ J 2005;69:249-52.  Back to cited text no. 6
    
7.
Toko H, Terasaki F, Kawakami Y, Hayashi T, Suwa M, Hirota Y, et al. A case of malignant lymphoma with diastolic heart failure. Jpn Circ J 1998;62:863-7.  Back to cited text no. 7
    
8.
Jeudy J, Burke AP, Frazier AA. Cardiac lymphoma. Radiol Clin North Am 2016;54:689-710.  Back to cited text no. 8
    
9.
Kapur S, Levin MB. Precursor t-cell lymphoblastic lymphoma presenting as cardiac tamponade in a 25-year-old male: A case report and review of literature. World J Oncol 2014;5:129-34.  Back to cited text no. 9
    
10.
Assuncao BMBL, Handschumacher MD, Brunner AM, Yucel E, Bartko PE, Cheng KH, et al. Acute leukemia is associated with cardiac alterations before chemotherapy. J Am Soc Echocardiogr 2017;30:1111-8.  Back to cited text no. 10
    

Top
Correspondence Address:
Dr. Parminder Singh Otaal
Department of Cardiology, Post Graduate Institute of Medical Education and Research, Sector-12, Chandigarh - 160 012
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/apc.apc_52_21

Rights and Permissions


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

Top