Intravascular B-cell lymphoma: report of two cases with different clinical presentation but rapid central nervous system involvement.
Intravascular lymphomatosis (IVL) is a rare large-cell lymphoproliferative disorder characterized by a widespread lymphoma proliferation within the lumen of medium and small vessels, frequently presenting with skin and/or central nervous system (CNS) manifestations. The tumor is of B-cell origin in most cases. Prognosis is poor with a reported median survival of 5-7 months. We describe here two cases of IVL. The first was that of a 55-year-old woman with a large B-cell lymphoma of the leg, successfully treated with conventional chemotherapy (CHT) followed by autologous peripheral stem cell transplantation. At 3 months from the autograft she relapsed with a picture of hemophagocytic syndrome (HPS) and CNS symptoms. She died before any specific treatment, and post-mortem examination revealed the intravascular proliferation of lymphoma B-cells in the brain and bone marrow. The second case was that of a 60-year-old male with CNS involvement at diagnosis. He responded poorly to CHOP-like CHT, and died 2 months after diagnosis and 6 months after onset of symptoms. Failure of CHT at least in some IVL patients may be related to a delay in the initiation of therapy due to non-specific neurological symptoms. Therefore, early diagnosis based upon aggressive attempts immediately followed by adequate therapy may prove beneficial to these patients. In the present report, we performed an extended medline-based review of the published series of patients with IVL.
Anghel
G
G
Hematology and Bone Marrow Transplantation Unit, San Camillo-Forlanini Hospital, Circonvalazione Gianicolense 87, Rome 00152, Italy.
Petrinato
G
G
Severino
A
A
Remotti
D
D
Insabato
L
L
De Renzo
A
A
Rotoli
B
B
Majolino
I
I
D001344:Autopsy; D016543:Central Nervous System Neoplasms / Q000556:secondary*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007866:Leg / Q000098:blood supply; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness / Q000473:pathology; D019043:Vascular Neoplasms / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy