Case Presentation: A 63-year-old man presented with progressive dyspnea associated with new onset lower-extremity weakness, numbness, and low back pain. CXR revealed bilateral, large pleural effusions which were borderline exudative, but with negative cytology. An MRI of his spine showed concern for possible metastatic disease, but a CT of his chest, abdomen, and pelvis failed to show evidence of boney abnormality and no masses were seen. He was discharged home with outpatient follow up and his symptoms were attributed to possible diabetic neuropathy, heart failure, or cirrhosis.He returned to the hospital 2 weeks later with enlarging pleural effusions. He was hypotensive to the 80s/60s. Repeat thoracentesis demonstrated an exudative effusion, again with negative cytology. Despite aggressive fluid resuscitation the patient remained hypotensive. A transthoracic echocardiogram showed a pericardial effusion. He underwent right heart catheterization with pericardiocentesis showing high normal cardiac output and index with decreased systemic vascular resistance. Midodrine was added, but he remained hypotensive. Workup for heart failure, cirrhosis, infection, protein-wasting enteropathy, and endocrine causes of hypotension were negative.Consequently, flow cytometry of pericardial fluid was obtained and returned positive for DLBCL which was confirmed with bone marrow biopsy. He was started on a R-CHOP and his hypotension, effusions, and neuropathy all resolved after initiation of treatment.
Discussion: Diffuse large B cell lymphoma (DLBCL) is the most common histologic subtype of non-Hodgkin lymphoma. Patients usually present with symptomatic nodal enlargement or “mass” in the neck, abdomen, or pelvis associated with classical “B” symptoms. We present a patient with refractory hypotension, neuropathy, and pleural effusions as presenting features of DLBCL without obvious mass lesion or lymphadenopathy.
Conclusions: Hypotension and neuropathy without mass or lymphadenopathy are rare presenting symptoms of DLBCL, making the diagnosis challenging. Additionally, the lack of CT findings to corroborate the MRI was confusing to the initial admission team, but likely secondary to marrow involvement by lymphoma which could not be detected by CT scan. The negative cytology is explained by the presence of normal appearing lymphocytes which were clonal on flow cytometry.Hypotension is rarely associated with lymphoma, but hypothetically related to cytokines release from a clonal B-cell population resulting in peripheral vasodilation. This is supported by the heart catheterization showing high CO/CI despite systemic hypotension and a low systemic vascular resistance. The fact that the hypotension, neuropathy, and effusions all resolved with chemotherapy further solidifies the lymphoma as the ultimate cause of this rare presentation.While refractory hypotension has been rarely reported in Hodgkin lymphoma, to our knowledge it has never been reported with DLBCL. The hypotension may be secondary to direct cytokinerelease into the circulation from a large population of B-cells infiltrating the marrow or a direct effect on the hypothalamus, similar to the paraneoplastic neuropathy which resolved.Physicians should be aware of the variable presenting symptoms of lymphoma, including neuropathy, hypotension, recurrent effusions, and isolated marrow involvement which were all present in our patient.