Recurrent pericardial effusion with pericardial amyloid deposition: a case report and literature review

Our plain-English summary of Recurrent pericardial effusion with pericardial amyloid deposition: a case report and literature review, by H. Itagaki, T. Yamamoto, K. Uto, et al (Cardiovascular Pathology, 2020).

The study

• Authors: H. Itagaki, T. Yamamoto, K. Uto, A. Hiroi, H. Onizuka, H. Arashi, E. Shibahashi, S. Isomura, H. Oda, T. Yamashita, Y. Nagashima
• Journal: Cardiovascular Pathology
• Published: 2020;46:107191
• Type of article: Case report, Review
• Cited by: 4 articles, as counted by NIH iCite
• Read it: PubMed · Publisher

Abstract

Pericardial amyloidosis is a rare cause of pericardial effusion. Here, we report a case of recurrent pericardial effusion because of pericardial amyloid deposition. The patient was a man in his 40s admitted for pulmonary embolism. During hospitalization, arterial fibrillation and cardiac tamponade were observed, and an initial pericardial puncture was performed. Thereafter, pericardial puncture was repeated nine times over the next two years. Cytological examination of the pericardial effusion suggested malignant mesothelioma. Afterward, pericardial fenestration and partial resection were performed. Intraoperatively, a thickened pericardium and hemorrhagic pericardial effusion were noted. Histologically, the surface of the pericardium was covered by an eosinophilic amorphous material. Congo red and DYLON stains, electron microscopy, and immunohistochemical findings revealed localized amyloidosis composed of an immunoglobulin lambda light chain. Although the patient did not receive further treatment for 5 years postoperatively, his renal and cardiac functions remained within normal limits. Based on these findings, the patient was diagnosed with localized amyloidosis. So far, hemorrhagic pericardial effusion has been reported in few cases with systemic amyloidosis. Because localized immunoglobulin light-chain-derived (AL) amyloidosis may progress to systemic disease (although it is a very rare occurrence), long-term follow-up is necessary to detect recurrence or progression to a systemic form.

Copyright © 2019 Elsevier Inc. All rights reserved.

In plain English

This article is a case report, with a short look at earlier published cases, about one man in his 40s treated at Tokyo Women's Medical University in Japan for fluid that kept building up around his heart (recurrent pericardial effusion). It does not discuss asbestos. The fluid was drained repeatedly. When cells in one fluid sample were examined, the authors identified them as mesothelial cells, and malignant mesothelioma was suggested as a possible diagnosis. Surgeons then removed part of the pericardium, the sac around the heart, and found bloody fluid. No tumor was visible to the naked eye. Under the microscope, the pericardium's surface was covered with material that special stains, electron microscopy and antibody tests identified as amyloid, an abnormal protein deposit. The man received no further treatment, and his kidney and heart function stayed normal for 5 years after surgery. He was diagnosed with localized AL amyloidosis, with a significant possibility of the lambda type. No bone marrow test was done. The authors concluded that localized and systemic amyloidosis are hard to tell apart by appearance alone and that regular follow-up is essential.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report with a brief discussion of previously published cases
• Who was studied: One man in his 40s with a history of epilepsy and cerebral palsy, treated at Tokyo Women's Medical University, Tokyo, Japan (year given only as 201X)
• Main results:
  • Cells in the pericardial fluid from the third drainage were identified as mesothelial cells. About 20% of them were positive for Ki-67. Testing did not show homozygous p16 loss. Malignant mesothelioma was suggested as a possible (differential) diagnosis.
  • At surgery the fluid around the heart was bloody (hemorrhagic) and was classed as an exudate.
  • The removed pericardium was 3 mm thick and showed no visible tumor.
  • Congo red staining viewed under polarized light showed green birefringence, a typical sign of amyloid.
  • In antibody (immunohistochemical) testing, the deposited material was positive for amyloid P and partially positive for IGLL5. It was negative for amyloid A, transthyretin, beta-2-microglobulin, and the kappa and lambda light-chain antibodies.
  • Over 5 years after surgery without further treatment, kidney and heart function remained normal and no M protein was found in serum or urine.
  • Final diagnosis: localized AL amyloidosis, with a significant possibility of AL lambda amyloidosis.
• The authors' conclusion: The authors concluded that the case showed how hard it is to tell localized amyloidosis from systemic forms by visible appearance alone. They said clinical and tissue findings help, and regular follow-up is essential because localized disease can progress to the systemic form many years later.
• Limitations the authors state:
  • No bone marrow aspiration or biopsy was performed.
  • Because the amyloid was spread diffusely over the pericardium, systemic amyloidosis remained a possible alternative diagnosis.
  • A heart muscle (myocardial) biopsy was not performed.

Topics

Medical terms in this study, explained in our Lexicon: Pericardial Effusion, Pericardial, Deposition, Cardiac Tamponade, Malignant Mesothelioma, Pericardium, Eosinophilic.

Related studies

• Primary pericardial mesothelioma and asbestos exposure: a rare fatal disease — D.M.M. Freitas, R.L. Ramos, et al. (2017)
• Pericardial Mesothelioma in a 35-Year-Old Male With Ulcerative Colitis — J. Iliff, N.K. Bart, et al. (2019)
• Recurrence of Pericardial Mesothelioma Affecting the Myocardium After Pericardial Resection — M. Rodríguez, H.R. Mallidi, et al. (2018)
• Pericardial mesothelioma presenting as left atrial thrombus in a patient with mitral stenosis — Y. Miyamoto, S. Nakano, et al. (1996)
• New nonsurgical technique for multiple pericardial biopsies — J. Endrys, M. Simo, et al. (1988)
• Tetracycline sclerosis in the management of malignant pericardial effusion — F.A. Shepherd, J.S. Ginsberg, et al. (1985)

Browse return to medical research.