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Progressive multifocal cerebral infarction from intravascular large B cell lymphoma presenting in a man: a case report
© Jitpratoom et al; licensee BioMed Central Ltd. 2011
Received: 17 May 2010
Accepted: 20 January 2011
Published: 20 January 2011
Intravascular lymphoma is rare, and may present as ischemic stroke. Diagnosis is difficult due to the non-specific presentation and lack of lymphadenopathy, thus leading to frequent instances of autopsy-proven diagnosis. To the best of our knowledge, this is the first report of progressive stroke from intravascular lymphoma diagnosed antemortem by random skin biopsy.
A 42-year-old Thai man presented to our hospital with progressive multifocal cerebral infarction. Despite taking aspirin (300 mg/day), his neurological symptoms worsened. During admission, he developed an unexplained fever and hypoxemia. Magnetic resonance angiography clearly showed patency of all cerebral arteries including the internal carotid and vertebrobasilar arteries. Echocardiography, an antiphospholipid antibody test, cerebrospinal fluid cytology and a bone marrow study were normal. Other laboratory test results showed an elevated lactate dehydrogenase level, nephrotic range proteinuria (3.91 g/day), hypoalbuminemia (1.9 g/dL), a very low high-density lipoprotein level (7 mg/dL) and hypertriglyceridemia (353 mg/dL). Because of suspected vasculitis, pulse methylprednisolone was given with transiently minimal improvement. A random skin biopsy from both thighs revealed intravascular large B cell lymphoma. Chemotherapy was not given due to our patient having ventilator associated pneumonia. He died 10 days after the definite diagnosis was established.
One etiology of stroke is intravascular lymphoma, in which random skin biopsy can be helpful for antemortem diagnosis.
Intravascular lymphoma (IVL) is a rare type of extranodal lymphoma with an aggressive clinical course characterized by proliferation of lymphoma cells within the lumina of vessels, particularly the capillaries, with the exception of large arteries and veins [1, 2]. Because of its varied clinical symptoms and the absence of lymphadenopathy, a diagnosis of IVL is extremely difficult to make and many of the reported cases were diagnosed postmortem. We report the case of a 42-year-old man with IVL who presented with the clinical features of progressive multifocal cerebral infarction.
The clinical presentation of IVBL is variable, but the disease has a fulminant course that confers a poor prognosis. Patients may present with a spectrum of symptoms ranging from pyrexia and non-specific constitutional symptoms to multi-organ failure [3, 4]. The most common clinical manifestations involve the nervous system and the skin, with at least 68% of patients having involvement of at least one of these organs . Other reported clinical manifestations included fever of unknown origin , hypoxemia , nephrotic syndrome , hemophagocytic syndrome , arthritis , and myocardial infarction . Anemia and elevated lactate dehydrogenase (LDH) were the most common laboratory findings . The clinical diagnosis of IVL is challenging and often difficult to diagnose antemortem, and the poor prognosis for these patients reflects delays in diagnosis and the institution of chemotherapy. Use of random skin biopsy was reported to help diagnose IVL antemortem [10–12].
Our patient presented with neurological manifestations but did not have cutaneous involvement of IVBL. Initial laboratory abnormalities included mild anemia and elevated LDH, which were non-specific for IVL. Other laboratory test results related to IVL, such as soluble interleukin 2 receptor level  and serum ferritin level tests, were not performed. There was no evidence of hemophagocytic syndrome from a bone marrow study. However, histological evidence of IVBL in skin biopsies taken from apparently normal skin has been documented previously. A skin biopsy enabled diagnosis of IVBL in our patient.
The neurological manifestations of IVBL are varied, ranging from focal ischemic events to dementia . Ischemic stroke is a common disease worldwide and is mostly caused by atherosclerotic arterial disease. However, our patient showed some symptoms that guided us to consider IVL as more likely than atherosclerotic arterial disease: our patient had no apparent atherosclerotic risk factor and there was no evidence of a cardiac source of emboli and antiphospholipid syndrome, aspirin did not retard the progression of stroke, he developed an unexplained fever in the second week of hospitalization without an identifiable source of infection and the fever did not respond to empirical antibiotics, he developed unexplained hypoxemia, his serum LDH was elevated to 1203 U/L, he had unexplained nephrotic range proteinuria, hypoalbuminemia and hypertriglyceridemia, and finally, MRA showed normal intracranial arteries.
Even though intravascular lymphoma is uncommon, the disease should be considered in the differential diagnosis of neurological manifestations or fever of unknown origin with elevated level of lactate dehydrogenase. Our patient's case shows that a random skin biopsy from normal skin can help a physician to diagnose IVBL. We recommend random skin biopsy, a safe bedside procedure, be used in the evaluation of patients in whom the diagnosis of IVL is doubtful, particularly before other invasive investigations are used.
Written informed consent was obtained from the patient's next of kin for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
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