Motivation: Zebras are growing more frequent these days. Though thought to be quite rare, I have seen two of my favorite patients diagnosed with intravascular lymphoma within the past year. In both, the diagnosis could not be made for months because of the non-traditional lymphoma findings - for instance, no enlarged lymph nodes. Intravascular lymphoma is characterized by growth of lymphoid neoplastic cells with the lumen of blood vessels. Given lack of systemic masses, are there clinical clues to its diagnosis? We will review here one of the largest review of cases for this rare disorder.
Paper: Ferreri, A.J.M., Campo, E., Seymour, J.F. et. al. "Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'" Brit. J. of Haematol. (2004); 127: 173-183
Methods: Data gathered from 22 centers forming the International Extranodal Lymphoma Study Group (IELSG) on patients who were HIV negative and were diagnosed with intravascular lymphoma between 1985 and 2003.
Results:
Cohort: Intravascular lymphoma was diagnosed in 38 patients - postmortem in eight (21%) and while living in 30 (79%). The median age was 70 years with male/female ratio of 0.9.
Clinical Presentation: The most common presenting symptom was fever in 45%. Systemic symptoms including fever, weight loss, or night sweats were present in 55%. Cutaneous lesions were present in 39% made up of a wide variety of lesions including erythematous eruption, plaques, swelling, nodular discolorations, or small red palpable spots situated in upper arms, thighs, legs, lower abdomen, or breast. The next most common finding was neurological symptoms present in 34% with a wide range of symptoms from focal deficits to meningoradiculitis.
Laboratory Variables: The most common lab abnormality was elevated serum LDH (86%) followed by elevated beta-2 microglobulin (82%). ESR was elevated in 43%. Anemia was present in 63% while leukopenia and thrombocytopenia were present in 24 and 29% respectively.
Discussion: Intravascular lmphoma remains a difficult disorder to diagnose. I think that patients with unclear lesions in the brain and skin with systemic symptoms should raise suspicion of intravascular lymphoma. The present large series also contrasts with previous reports from Japan where intravascular lymphoma presented more commonly with hemophagocytic syndrome and less commonly with cutaneous or neurological symptoms suggesting that there may be ethnic variance versus diagnostic bias in the previous report. Laboratories are helpful for elevated beta-2 microglobulin present in more than 80%. In summary, I learnt that if I suspect patient of having intravascular lymphoma in the brain, I will conduct a skin exam.
Paper: Ferreri, A.J.M., Campo, E., Seymour, J.F. et. al. "Intravascular lymphoma: clinical presentation, natural history, management and prognostic factors in a series of 38 cases, with special emphasis on the 'cutaneous variant'" Brit. J. of Haematol. (2004); 127: 173-183
Methods: Data gathered from 22 centers forming the International Extranodal Lymphoma Study Group (IELSG) on patients who were HIV negative and were diagnosed with intravascular lymphoma between 1985 and 2003.
Results:
Cohort: Intravascular lymphoma was diagnosed in 38 patients - postmortem in eight (21%) and while living in 30 (79%). The median age was 70 years with male/female ratio of 0.9.
Clinical Presentation: The most common presenting symptom was fever in 45%. Systemic symptoms including fever, weight loss, or night sweats were present in 55%. Cutaneous lesions were present in 39% made up of a wide variety of lesions including erythematous eruption, plaques, swelling, nodular discolorations, or small red palpable spots situated in upper arms, thighs, legs, lower abdomen, or breast. The next most common finding was neurological symptoms present in 34% with a wide range of symptoms from focal deficits to meningoradiculitis.
Laboratory Variables: The most common lab abnormality was elevated serum LDH (86%) followed by elevated beta-2 microglobulin (82%). ESR was elevated in 43%. Anemia was present in 63% while leukopenia and thrombocytopenia were present in 24 and 29% respectively.
Discussion: Intravascular lmphoma remains a difficult disorder to diagnose. I think that patients with unclear lesions in the brain and skin with systemic symptoms should raise suspicion of intravascular lymphoma. The present large series also contrasts with previous reports from Japan where intravascular lymphoma presented more commonly with hemophagocytic syndrome and less commonly with cutaneous or neurological symptoms suggesting that there may be ethnic variance versus diagnostic bias in the previous report. Laboratories are helpful for elevated beta-2 microglobulin present in more than 80%. In summary, I learnt that if I suspect patient of having intravascular lymphoma in the brain, I will conduct a skin exam.
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