Intravascular large B-cell lymphoma with hemophagocytic syndrome (Asian variant) in a Caucasian patient.
Intravascular lymphoma is an aggressive and extremely rare extranodal lymphoma with neoplastic lymphoid cells confined exclusively within intravascular spaces. The histopathologic findings are subtle due to the rarity of the neoplastic cells in blood vessels. Clinical presentations are non-specific and focal space-occupying lesions or lymphoadenopathy are always lacking. It is a diagnostic challenge. Secondary hemophagocytic syndrome is uncommon and is typically associated with infection, malignancy, and suppressed immune states. Intravascular lymphoma has a strong association with hemophagocytic syndrome in Asian patients, the so-called "Asian variant", but not in Western patients. We report a case of intravascular B-cell lymphoma in a Caucasian patient associated with secondary hemophagocytic syndrome. The patient was diagnosed by core liver biopsy and successfully treated. This case demonstrates the importance of high index of suspicion and astute histopathologic examination in recognition of this unusual clinical and pathologic combination.
Department of Pathology, University of Oklahoma Health Sciences Center, Oklahoma City, OK 73104, USA. karmingfung@ouhsc.edu
Kar-Ming
KM
Fung
Jennifer H
JH
Chakrabarty
William F
WF
Kern
Hany
H
Magharyous
Bradley C
BC
Gehrs
Shibo
S
Li
D044466:Asian People; D001706:Biopsy; D001853:Bone Marrow / Q000473:pathology; D002869:Chromosome Aberrations; D003582:Cytogenetics; D006801:Humans; D008099:Liver / Q000473:pathology; D051359:Lymphohistiocytosis, Hemophagocytic / Q000188:drug therapy / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000188:drug therapy / Q000235:genetics / Q000473:pathology; D008297:Male; D008875:Middle Aged; D014057:Tomography, X-Ray Computed; D044465:White People