title: Intravascular lymphomatosis--an indolent or aggressive entity?
abstract: Intravascular lymphomatosis (IVL) is a rare malignancy characterized by neoplastic proliferation of lymphoid cells within the lumens of arteries, small veins and capillaries. We report four patients with IVL and review the recent world literature, relating to incidence, clinical features and possible therapy. In these cases diagnosis was established coincidentally in one patient after prostatectomy. This patient eventually had central nervous system involvement. In two other patients IVL was diagnosed from skin lesions. In the fourth case the diagnosis was established at post-mortem examination, where involvement of most organs was evident but particularly kidneys, myocardium, gastrointestinal tract and lymph nodes. Therapy was given to three patients, but the disease progressed in two and they both died with evidence of central nervous system involvement, while the third patient has had a good partial response to combination chemotherapy but has relapsed within two months of completing chemotherapy. As evident from our patients and the literature review IVL has a variable clinical course and currently, there appears to be no effective therapy for this rare disorder.
authors.affiliation: Department of Hematology, Hadassah University Hospital and Hebrew University Medical School, Jerusalem, Israel.
authors.forename: V
authors.initials: V
authors.lastname: Bogomolski-Yahalom
authors.forename: I S
authors.initials: IS
authors.lastname: Lossos
authors.forename: E
authors.initials: E
authors.lastname: Okun
authors.forename: Y
authors.initials: Y
authors.lastname: Sherman
authors.forename: A
authors.initials: A
authors.lastname: Lossos
authors.forename: A
authors.initials: A
authors.lastname: Polliack
mesh_terms: D000368:Aged; D001172:Arthritis, Rheumatoid / Q000150:complications / Q000188:drug therapy; D001249:Asthma / Q000150:complications / Q000188:drug therapy; D001799:Blood Sedimentation; D001853:Bone Marrow / Q000473:pathology; D001932:Brain Neoplasms / Q000175:diagnosis / Q000473:pathology; D018450:Disease Progression; D004890:Erythema / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005335:Fever of Unknown Origin / Q000209:etiology; D006801:Humans; D016867:Immunocompromised Host; D016130:Immunophenotyping; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use; D016393:Lymphoma, B-Cell / Q000175:diagnosis / Q000473:pathology; D008228:Lymphoma, Non-Hodgkin / Q000175:diagnosis / Q000473:pathology*; D008297:Male; D011241:Prednisone / Q000009:adverse effects / Q000627:therapeutic use; D011468:Prostatectomy; D012878:Skin Neoplasms / Q000175:diagnosis / Q000473:pathology; D013848:Thigh; D019043:Vascular Neoplasms / Q000175:diagnosis / Q000473:pathology*
chemical_list: D007166:Immunosuppressive Agents; D011241:Prednisone