INTRODUCTION: Intravascular large B-cell lymphoma (IVL) is a rare, diagnostically challenging, and usually fatal disease classified as a high-grade malignant lymphoma and characterized by proliferation of malignant cells within the lumen of small blood vessels. While IVL can involve the vasculature of any organ, primary pulmonary involvement is uncommon and secondary pulmonary arterial hypertension has only rarely been described(1,2). We present a case of fatal IVL causing hypoxemic respiratory failure and right ventricular failure due to occlusion of the pulmonary vasculature with a monoclonal B-cell population; e.g. IVL.
CASE PRESENTATION: A sixty-eight year old male was evaluated at our institution with a 3 month history of progressive dyspnea, fatigue, and low grade fevers. His review of systems was positive for anorexia, 40 lb weight loss in 3 months, occasional night sweats, mild headache and depression. Physical exam revealed a grade 3/6 systolic ejection murmur. Laboratory studies were performed and significant for hemoglobin 9.6 g/dL, MCV 84.7, ESR 120 mm/hr, CRP 159 mg/L, LDH 576 U/L and Ferritin 1313ug/L. Initial concerns were for a malignant or infectious process. All cultures remained negative. The patient continued to be more dyspneic and pulmonary function test were done. This showed a nonspecific reduction in his FVC 3.38 (76%), marked reduction of his DLCO 12.5 (48%), and desaturation with exercise (87%). An echocardiogram demonstrated a bicuspid aortic valve, normal ejection fraction (63%) and pulmonary hypertension (right ventricular systolic pressure (RVSP) 41mmHg). A bone marrow biopsy, FDG PET and CT Chest were unrevealing. He was empirically treated with both antibiotics and steroids. Despite this he developed worsening hypoxia, fatigue, delirium and was readmitted to the hospital. On hospital day eight, the patient developed shock and respiratory failure and was transferred to the intensive care unit. An urgent echocardiogram was performed which demonstrated acute changes when compared to his prior echocardiogram. He had developed worsening pulmonary hypertension with a RV failure, D-shaped left ventricle and RVSP of 56mmHg (SBP 73 mmHg). A pulmonary embolism was suspected and thrombolytics were administered. The patient continued to worsen and developed multiorgan failure and died despite multiple attempts at CPR and resuscitation. Autopsy was requested and revealed diffuse intravascular large B-cell lymphoma.
DISCUSSION: Intravascular large B-cell lymphoma is a rare malignant lymphoma characterized by selective growth of neoplastic cells within blood vessel lumina, particularly small vessels, e.g. capillaries. It commonly affects the vessels of the skin and central nervous system although blood vessels in other organs may be occluded(1). Due to its varied and non-specific clinical manifestations, antemortem diagnosis is often difficult. Our case of IVL was not diagnosed despite extensive outpatient evaluation and prolonged symptoms. He presented to the ICU with hypoxic respiratory failure, hemodynamic compromise and echocardiographic findings suggesting acute worsening of right heart failure which progressed despite support and resulted in his death. Post-mortem examination did not reveal PE, rather histopathologic analysis demonstrated obstruction of the pulmonary arterioles by large lymphoma cells. We conclude this was the mechanism leading to decompensated acute right heart failure and death.
CONCLUSIONS: Intravascular lymphoma remains a diagnostic challenge and is commonly unrecognized pre-mortem. Preferential involvement of the lumen of the pulmonary vasculature leading to hypoxia and decompensated right heart failure is a rare presentation of a rare disease. Clinicians should consider IVL within the spectrum of etiologies of acute and subacute right heart failure/PAH in the context of systemic inflammatory illness.
Reference #1 Aouba A, Diop S, Saadoun D, et al. Severe pulmonary arterial hypertension as initial manifestation of intravascular lymphoma: case report. Am J Hematol. 2005;79:46-49.
Reference #2 Snyder LS, Harmon KR, Estensen RD. Intravascular lymphomatosis (malignant angioendotheliomatosis) presenting as pulmonary hypertension. Chest. 1989;96:1199-1200.
DISCLOSURE: The following authors have nothing to disclose: Darlene Nelson, Craig Daniels
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