A 54 y.o. woman with a history of lymphoma comes in with shortness of breath.
You notice an abnormality on her cardiac US.
The RV free wall appears thickened. What does this mean?
Our patient had diffuse lymphomatous involvement of the pericardium and myocardium( especially the septum and RV free wall) with filling limitations in the right atrium and right ventricle. She had a diagnosis of lymphoma made 7/22 but elected not to have treatment. She recently was visiting Arizona and developed shortness of breath. At that time she had cardiac tamponade and underwent a pericardial window with drain. She returned to St. Louis and presented to the ED with continued shortness of breath. While her pericardial effusion was reduced by the drain; large masses of lymphoma caused restriction to cardiac flow—a sort of “ tumor tamponade”.
While primary cardiac lymphomas are rare; secondary cardiac lymphoma occurs in 9-24% of patients with lymphoma in the course of the disease.
Autoimmune diseases are associated with lymphomas Lymphomas arising from B cells are associated with myasthenia gravis, RA, lupus and Sjogren’s syndrome. Lymphomas arising fom T cells occur in conjunction with dermatopolymyositis, inflammatory bowel diseases , multiple sclerosis, sarcoid and type I diabetes. Autoimmune diseases are thought to stimulate cytokines, leading to a proliferation of lymphocytes some of which can transform into malignant cells.
Almost the reverse of this overstimulation of the immune system in autoimmune disease can also lead to lymphoma. Patients on immune suppression for solid organ transplants are at higher risk for lymphoma.
Tumors of the heart
NONCANCEROUS-75-95%
Myxoma- 50% of all noncancerous heart tumors. It can present with just fever. It apprears most commonly in the L atrium and creates a risk for emboli.
Fibroelastoma- grows on a heart valve
Pediatric heart tumors- rhabdomyomas usually go away on their own., fibromas grow within the muscle and need to be removed, teratomas are oftern on the pericardium, and hamartomas can affect the conducting system.
CANCEROUS-5-25%
Sarcoma- Sarcomas account for 75% of cardiac malignancies. Angiosarcoma usually develops in the right atrium; rhabdomyosarcoma can occur in any chamber.
Malignant fibrous histoicytoma- can block the mitral valve
Lymphoma-is often associated with autoimmune diseases.
Our patient developed a fib and complete heart block. She was admitted to the ICU, given allopurinol, rasburicase, prednisone and colchicine. She received rituximab. She maintained a normal blood pressure and was discharged from the hospital.
Zhao Y, Huang S, Ma C, et al. Clinical features of cardiac lymphoma; a analysis of 37 cases. J Int Med Res 2021 Mar;49(3) doi.10.1177/0300060521999558
Masciopinto P, Dell’Olio, De Robertis, R. The role of autoimmune diseases in the prognosis of lymphoma. J Clin Med 2020 Nov;9(11):3403.
Hayter S, Cook M. Updated assessment of the prevalence, spectrum and case definition of autoimmune disease. Autoimmun Rev 2012;11:754-765.