Intravascular large B-cell lymphoma with a fulminant clinical course: a case report with definite diagnosis post mortem.
A patient is described who presented with pancytopenia, splenomegaly and excessively elevated lactate dehydrogenase levels in concurrence with signs of extramedullary hematopoiesis. Although initially considered in the differential diagnostic spectrum, a highly aggressive lymphoma could not be identified before the patient died, 6 weeks after admission. Even an intensive diagnostic work-up including splenectomy and repeated bone marrow biopsies was inconclusive. Finally, the diagnosis of an intravascular large B-cell lymphoma, a highly aggressive clinical subtype of a diffuse large B-cell lymphoma, spreading within vascular structures of multiple organs was established by autopsy. Intravascular large B-cell lymphoma is often not diagnosed before death due to the exclusive intravascular growth pattern of the tumor cells and a fulminant clinical course. The heterogeneous clinical features of this lymphoma subtype are discussed.
Fiegl
M
M
Department of Internal Medicine, Division of Hematology and Oncology University of Innsbruk, Innsbruck, Austria. michael.fiegl@unibk.ac.at
Greil
R
R
Pechlaner
C
C
Krugmann
J
J
Dirnhofer
S
S
D001344:Autopsy; D001707:Biopsy, Needle; D003131:Combined Modality Therapy; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D016393:Lymphoma, B-Cell / Q000150:complications / Q000473:pathology* / Q000628:therapy; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012720:Severity of Illness Index; D013156:Splenectomy; D019043:Vascular Neoplasms / Q000150:complications / Q000473:pathology* / Q000628:therapy