Facial Swelling and Dyspnea as Presenting Symptoms for Non-Hodgkin’s Lymphoma

B cell lymphoma comprises a group of malignancies originating from B lymphocytes. These malignancies can be broadly classified into Hodgkin lymphoma (HL) and non-Hodgkin lymphoma (NHL). NHL constitutes most cases, and diffuse large B cell lymphoma (DLBCL) is the most common subtype, accounting for approximately 25% of adult NHL cases 1. DLBCL is characterized by the rapid proliferation of large B cells with nuclear abnormalities. The exact causes of B cell lymphoma remain incompletely understood. However, potential risk factors include increasing age, EBV, Helicobacter pylori and Hepatitis C infections, and prolonged exposure to pesticides/herbicides/industrial chemicals 2.

Our patient is a 58-year-old female who presented to the emergency department with progressively worsening shortness of breath. Her past medical history includes sick sinus syndrome status post dual-chamber pacemaker placement, asthma, hypertension, and seasonal allergies. She reported experiencing increasing dyspnea on exertion over the past three months, along with orthopnea that required the use of two pillows each night for several days before presentation. The patient denied experiencing chest pain, palpitations, cough, fever, chills, syncope, dizziness, lightheadedness, nausea, or vomiting.

In the two weeks prior to her visit, she had been using her mother's supplemental oxygen at 2 liters via nasal cannula without any improvement in her symptoms. She also reported using two albuterol inhalers within a week, which provided minimal relief. Additionally, she noted symmetrical facial swelling for several weeks, for which she received a cortisone injection at Urgent Care. The patient had her dual-chamber pacemaker placed three months ago and was awaiting outpatient cardiac stress testing and follow-up transthoracic echocardiogram, which had been delayed due to a recent respiratory syncytial virus (RSV) infection.

She denied any recent illnesses, hospitalizations, surgeries, sick contacts, or travel. There were no reports of new rashes, bug bites, joint pains, or changes in detergents or soaps. The patient has a 40-pack year smoking history but quit two years ago and denies alcohol or recreational drug use. She works as a caregiver for her mother and has a family history notable for lung cancer in her maternal grandmother and leukemia in her aunt.

In the emergency department, vital signs were stable. A complete blood count showed a white blood cell count of 4.5 cells/µL, hemoglobin of 11.5 g/dL, hematocrit of 33.7 L/L, and platelets at 316,000/mcL. Coagulation studies revealed an INR of 1.0, PT of 12.1 seconds, and PTT of 29.6 seconds. The blood chemistry panel indicated normal levels of sodium, potassium, BUN, creatinine, magnesium, total bilirubin, alanine aminotransferase, aspartate aminotransferase, and alkaline phosphatase. B-type natriuretic peptide was elevated at 288.88 pg/mL, while troponin was at 0.000 ng/mL, and D-dimer was 334 mg/L. The respiratory viral panel was negative. An electrocardiogram (EKG) showed a regular heart rhythm at 81 beats per minute and chronic right bundle branch block, consistent with previous EKG findings from three months prior. A chest X-ray revealed mild vascular congestion, bilateral airspace opacities, a small right-sided pleural effusion, and a left chest wall dual-lead pacer. A CT angiogram, performed to rule out pulmonary embolism, showed no evidence of embolism but identified abnormal thickening and soft tissue density in the right atrium, a lobulated soft tissue density in the surrounding mediastinum extending into the right hilum, a soft tissue density/complex fluid collection surrounding the SVC, several enlarged lymph nodes measuring up to 16 mm, multiple bilateral pulmonary nodules measuring up to 1 cm, and bilateral ground-glass opacifications with nonspecific thickening of the right bronchovascular bundle and atelectasis versus infiltrate in the right middle and lower lobes.

The patient was admitted for further evaluation and management of a suspected cardiac mass and exacerbation of heart failure. Intravenous diuretics were initiated, and a cardiology consult was obtained for additional assessment of the right atrial and superior vena cava (SVC) mass. A cardiac MRI was performed, revealing a large right atrial mass primarily located in the atrium, with slight extension into the lower SVC, causing narrowing of the SVC, along with a right pleural effusion that exhibited a hemorrhagic component. Concerns regarding potential malignancy prompted an oncology consultation, which recommended a CT scan of the chest, abdomen, and pelvis. This imaging revealed metastatic disease affecting the lungs, kidneys, mesenteric root, right atrium, and right hilum. A transthoracic echocardiogram demonstrated a left ventricular ejection fraction of 60-65%, grade III diastolic dysfunction, mildly dilated right and left atria, and a small pericardial effusion.

The patient was continued on gentle diuresis to address a mild component of pulmonary edema and showed some symptomatic improvement. A consultation with cardiothoracic surgery was obtained, and a lymph node biopsy was performed by them via a right anterior mediastinotomy approach, which proceeded without complications.

The patient continued to show symptomatic improvement, and preliminary pathology results received prior to discharge indicated B cell lymphoma. After assessment, the patient was deemed stable for discharge from the perspectives of cardiology, cardiothoracic surgery, and oncology. The patient was discharged with recommendations for outpatient oncology follow-up, including a PET scan and the initiation of chemotherapy. Final pathology results confirmed a diagnosis of diffuse large B cell lymphoma.

This case report highlights the ambiguous clinical presentation and diagnostics in B cell lymphoma. The patient's presentation with painless, enlarged lymph nodes and elevated LDH levels is consistent with DLBCL, which is the most common subtype of non-Hodgkin lymphoma (NHL). It should be noted that B cell lymphoma may have contributed to this patient's medical history of sick sinus syndrome such that the mass infiltrated the sinus node. This case underscores the importance of early diagnosis of B cell lymphoma. The timely identification of lymphoma subtype and risk stratification are crucial for optimizing treatment outcomes and patient survival. Furthermore, this case emphasizes the need for a multidisciplinary approach involving hematologists, oncologists, radiologists, cardiothoracic, cardiologists and pathologists to ensure comprehensive care.

Despite advancements in diagnostic techniques, diagnosing B cell lymphoma remains complex, often requiring excisional biopsy and histopathological examination for confirmation3. Immunophenotyping and molecular studies assist with subtype classification and prognosis, while increasing awareness among healthcare providers can expedite diagnosis and treatment. Areas of improvement include enhancing early recognition by primary care physicians, identifying novel biomarkers for personalized therapies, and expanding access to innovative treatments like CAR-T cell therapy. Continued research into molecular mechanisms, along with strategies for early detection and prevention, will drive better management and outcomes for patients with B cell lymphoma.

BCL: B cell lymphoma

DLBCL: Diffuse large B cell lymphoma

NHL: Non-Hodgkin lymphoma

HL: Hodgkin lymphoma

SVC: Superior vena cava

RSV: Respiratory syncytial virus

PET: Positron emission tomography

MRI: Magnetic resonance imaging

CT: Computed tomography

LDH: Lactate dehydrogenase

CAR-T: Chimeric antigen receptor T-cell

1. Van Leeuwen MT, Turner JJ, Joske DJ, et al. Lymphoid neoplasm incidence by WHO subtype in Australia 1982-2006. Int J Cancer 2014; 135:2146.

2. Boyle J, Ward MH, Cerhan JR, Rothman N, Wheeler DC. Modeling historic neighborhood deprivation and non-Hodgkin lymphoma risk. Environ Res. 2023:116361. https://doi.org/10.1016/j.envres.2023.116361

3. Swerdlow, S. H., Campo, E., Pileri, S. A., Harris, N. L., Stein, H., Siebert, R., Advani, R., Ghielmini, M., Salles, G. A., Zelenetz, A. D., & Jaffe, E. S. (2016). The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood, 127(20), 2375–2390. https://doi.org/10.1182/blood-2016-01-643569