Intravascular lymphomatosis presenting as an ascending cauda equina: conus medullaris syndrome: remission after biweekly CHOP therapy

Abstract

A 63 year old man developed dysaesthesia in the legs followed by a subacute ascending flaccid paraparesis with sacral sensory and autonomic involvement. Intravascular lymphomatosis (IVL) was favoured by the presence of low grade fever and raised serum C reactive protein, CSF pleocytosis, raised lymphoma markers (serum LDH, soluble IL-2 receptor), and steroid responsiveness. Only muscle, among several organ biopsies, confirmed IVL. A cytogenetic study of the bone marrow showed chromosome 6 monosomy, as previously reported. The monosomy of chromosome 19, which bears the intercellular cell adhesion molecule-1, newly found in this case, may be related to the unique tumour embolisation of IVL. The CHOP regimen (six courses in 12 weeks) using granulocyte colony stimulating factor (G-CSF) led to gradual resolution of myeloradiculopathy and laboratory supported remission lasting for more than 13 months. The biweekly CHOP with G-CSF support may be a choice of chemotherapy in averting rapidly fatal IVL.

Intravascular lymphomatosis presenting with a conus medullaris syndrome mimicking disseminated encephalomyelitis

“…In contrast to disseminated encephalomyelitis, dementia, aphasia, seizures, and psychosis are frequent findings (Demirer et al, 1994;Glass et al, 1993). Typically, the spinal cord seems to be involved, and there are several reports of patients who presented with spinal symptoms (Hamada et al, 1991;Levin and Lutz, 1996;Nakahara et al, 1999;Ojeda, 1983;Ossege et al, 2000). However, an isolated conus medullaris syndrome as the presenting syndrome has not been described until now.…”

“…Untreated, the prognosis is very poor with an almost invariably fatal course within 1 year (Demirer et al, 1994). When various high-dose chemotherapy regimens are used, a signi cantly better prognosis has been reported if the diagnosis of intravascular lymphomatosis was established early in the disease (Demirer et al, 1994;Glass et al, 1993;Levin and Lutz, 1996;Nakahara et al, 1999). Therefore, intravascular lymphomatosis should be included in the differential diagnosis of every atypical case of presumed myelitis, including conus medullaris syndrome or disseminated encephalomyelitis.…”

“…Without therapeutic intervention, the neurologic involvement is usually rapidly progressive and inevitably fatal. There are now several reports suggesting that an aggressive therapy may be bene cial and, therefore, an early diagnosis is probably crucial for patient outcome (Baumann et al, 2000;Harris et al, 1994;Levin and Lutz, 1996;Nakahara et al, 1999). We report on a patient with intravascular lymphomatosis and with symptoms limited to the CNS.…”

Successful treatment of intravascular malignant lymphomatosis with high-dose chemotherapy and autologous peripheral blood stem cell transplantation

“…If this hypothesis is correct, a strong induction regimen like CHOP or biweekly CHOP is essential 4 and consolidation therapy with stem cell transplantation may be as desirable as in the therapy of other aggressive non-Hodgkin's lymphomas.…”

Intravascular Lymphoma: The Oncologist's “Great Imitator”

“…In the largest series of IVL, 13 of 38 patients presented with neurologic symptoms as either their exclusive symptoms or prominent associated symptoms, and the literature is replete with individual case reports of IVL affecting the CNS [4,[24][25][26]. Patients can present with focal www.TheOncologist.com sensory or motor deficits, generalized weakness, altered sensorium, rapidly progressive dementia, seizures, hemiparesis, dysarthria, ataxia, vertigo, and transient visual loss [24,[27][28][29].…”