Intravascular lymphoma: a neoplasm of ‘homeless’ lymphocytes?

Ponzoni, Maurilio; Ferreri, Andrés J.M.

doi:10.1002/hon.776

Cited by 110 publications

(144 citation statements)

References 78 publications

(125 reference statements)

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“…The median age of presentation is 70 with similar prevalence in males and females [6]. It has been reported that about 15% of IVL patients have had a previous or concomitant malignancy with NHL being the most commonly associated hematopoietic malignancy [6].…”

Section: Commentarymentioning

confidence: 99%

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A 69‐year‐old male presenting with hypotension and anasarca

et al. 2007

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A 69-year-old Caucasian male with a past medical history remarkable for hypertension and testicular nonHodgkin's lymphoma (NHL) presented to the emergency department with a 1-week history of fatigue and new onset lower extremity edema. For the past 8 months, the patient was having a nonproductive cough. Work up by his primary care physician was negative, and the patient was switched from enalapril to losartan 1 week before presentation. The cough resolved, however, 2 days later the patient began to experience fatigue and lightheadedness that persisted despite decreasing the dose of losartan. He continued to develop lower extremity edema and was again seen by his primary care physician who noted the patient to be hypotensive. He was referred to the emergency department for evaluation.In a 69-year-old male presenting with fatigue, lower extremity edema, and hypotension, our first concern was for a cardiac process such as heart failure. The differential for his hypotension also included sepsis, adrenal insufficiency, hemorrhage, or possibly an adverse drug reaction. We suspected that his cough was secondary to the enalapril as his symptoms had resolved with discontinuation of this medication; however, we remained concerned that an underlying pulmonary process could also be present.The patient denied chest pain, orthopnea, paroxysmal nocturnal dyspnea, fever, weight loss, or night sweats. His testicular NHL was treated 16 years prior with orchiectomy and four cycles of CHOP without evidence of recurrence. In addition to losartan, the patient also took a daily aspirin. He was a retired carpenter who had a 19-pack year smoking history and had quit 37 years before presentation. He occasionally drank alcohol. He denied illicit drug use. On examination, he was not in distress. His weight was 80.2 kg. His temperature was 36.48C, pulse 130 and regular, respiratory rate 18, blood pressure 78/46, and oxygen saturation was 97% while breathing ambient air. Physical examination was remarkable for a normal jugular venous pressure, tachycardia without extra heart sounds and generalized anasarca including 2+ pitting edema of his lower extremities. There was no lymphadenopathy, palpable masses, or cutaneous lesions. His neurologic examination was normal.The patient's review of systems was negative for symptoms of an acute coronary syndrome or heart failure, as well as for B-symptoms or a rapidly progesssive infectious process. On physical examination, his vital signs suggested intravascular volume depletion with tachycardia and hypotension; however, he appeared to have a component of fluid overload or third spacing of fluids with the marked degree of anasarca. His normal jugular venous pressure suggested normal right heart pressure making a cardiac cause of his anasarca less likely.The patient's white cell count was 12,300/mm 3 , with 93% neutrophils, 1% bands, and 6% monocytes. The hemoglobin level was 11.3 g/dl, with a mean corpuscular volume of 78.8 mm 3 . The platelet count was 554,000/ mm 3 . The serum sodium level was 126 mmo...

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Section: Commentarymentioning

confidence: 99%

“…It has been reported that about 15% of IVL patients have had a previous or concomitant malignancy with NHL being the most commonly associated hematopoietic malignancy [6]. Clinical features are heterogeneous and frequently include B symptoms, fever of unknown origin, and poor performance status [6][7][8][9][10][11].…”

Section: Commentarymentioning

confidence: 99%

A 69‐year‐old male presenting with hypotension and anasarca

et al. 2007

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“…Besides the unusual association of HLH with IVL in European patients, there are other features that do not favor a classical IVL in this case. In fact, brain pathology resembled primary diffuse large B‐cell lymphoma of the CNS and while some cases of IVL have brain mass‐like lesions 11, IVL with secondary CNS involvement usually have brain vessel and leptomeningeal infiltration 9 not seen in this case. It is thus plausible to assume a primary diffuse large B‐cell lymphoma of the CNS with synchronous IVL.…”

Section: Discussionmentioning

confidence: 77%

“…T‐cell lymphoma and Epstein‐Barr virus infection are the most common causes of adult HLH 1, 2, 3. B‐cell lymphoma presenting with HLH is less common and it is mostly seen in the Asian‐variant of IVL 9, but not in western countries 9, 10. Besides the unusual association of HLH with IVL in European patients, there are other features that do not favor a classical IVL in this case.…”

Section: Discussionmentioning

confidence: 99%

Synchronous brain and intravascular B‐cell lymphoma after remission of an adult hemophagocytic syndrome

Fonseca

Espada

Geraldes

et al. 2016

Clinical Case Reports

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“…That biopsy was characteristic of IVL with multiple cortical vessels (M4 branches) pathologically distended with neoplastic lymphocytes positive for CD20 by immunohistochemistry. Biopsies of the central nervous system in IVL patients have revealed neoplastic lymphocytes within large and medium arteries, distal cortical vessels, leptomeningeal perforators, capillaries, and even veins [2][3][4][5][6] Department of Radiology, University of Utah, Salt Lake City, UT 84132, USA also highly variable. The resolution of current vwMRI protocols, 0.5-0.7 mm voxels, does not allow reliable assessment of the distal intracranial vessels, which introduces the possibility of a false negative study, as in the case of our patient.…”

mentioning

confidence: 99%

Response to “High-Resolution Vessel Wall MRI: Appearance of Intravascular Lymphoma Mimics Central Nervous System Vasculitis”

Havenon

McNally

2016

Clin Neuroradiol

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System Vasculitis," [1] Schaafsma et al. describe two interesting cases of biopsy-proven intravascular lymphoma (IVL) that mimicked the vessel wall MRI (vwMRI) appearance of vasculitis, with concentric arterial wall thickening and enhancement. To our knowledge these are the first reported cases of IVL that have had vwMRI. We have scanned over 200 patients with vwMRI at our institution, including a 47-year-old female with biopsy-proven large B-cell IVL. Her vwMRI showed multiple areas of evolving ischemic infarction, parenchymal hemorrhage, edema, and leptomeningeal enhancement; however, on vwMRI there was no vessel wall thickening or enhancement, including the right parietal lobe, where she had infarction with edema and the location of her brain biopsy. That biopsy was characteristic of IVL with multiple cortical vessels (M4 branches) pathologically distended with neoplastic lymphocytes positive for CD20 by immunohistochemistry. Biopsies of the central nervous system in IVL patients have revealed neoplastic lymphocytes within large and medium arteries, distal cortical vessels, leptomeningeal perforators, capillaries, and even veins [2][3][4][5][6] Department of Radiology, University of Utah, Salt Lake City, UT 84132, USA also highly variable. The resolution of current vwMRI protocols, 0.5-0.7 mm voxels, does not allow reliable assessment of the distal intracranial vessels, which introduces the possibility of a false negative study, as in the case of our patient. Furthermore, as the authors note, in vasculitis the intramural lymphocytic invasion provides a pathophysiologic mechanism for concentric vessel wall enhancement, whereas in IVL the vessel wall itself is spared. Given these challenges, we would suggest that, for diagnostic purposes, their findings be interpreted with caution. Conflict of interest

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Intravascular lymphoma: a neoplasm of ‘homeless’ lymphocytes?

Cited by 110 publications

References 78 publications

A 69‐year‐old male presenting with hypotension and anasarca

A 69‐year‐old male presenting with hypotension and anasarca

Synchronous brain and intravascular B‐cell lymphoma after remission of an adult hemophagocytic syndrome

Response to “High-Resolution Vessel Wall MRI: Appearance of Intravascular Lymphoma Mimics Central Nervous System Vasculitis”

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