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Diagnosis and treatment of intravascular lymphomatosis
T P Baumann1, N Hurwitz, E Karamitopolou-Diamantis
1Department of Neurology, University Hospital, Basel, Switzerland.
Insights
This case report details a rare intravascular lymphomatosis with neurological symptoms. Standard CHOP chemotherapy achieved complete remission, demonstrating a surprisingly positive outcome for this aggressive lymphoma.
Area of Science:
- Neurology
- Oncology
- Hematology
Background:
- Intravascular lymphomatosis is a rare, aggressive non-Hodgkin lymphoma.
- It frequently presents with neurological complications, often mimicking other conditions.
Observation:
- A 70-year-old pharmacist presented with meningoencephalitis-like symptoms and later acute confusional state with status epilepticus.
- Diagnosis was confirmed via bilateral adrenal tumor biopsy, revealing intravascular lymphomatosis.
Findings:
- Polychemotherapy with CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, prednisone) resulted in complete remission.
- The patient achieved a survival exceeding 2.5 years, an unusually good outcome.
Implications:
- Consider intravascular lymphomatosis in adults with unexplained neurological syndromes and systemic disease signs.
- CHOP chemotherapy is a viable standard treatment for intravascular lymphomatosis, offering potential for long-term survival.
Objective:
To describe a patient with unusually good outcome of a rare, high-grade lymphoma that often involves the nervous system.
Design:
Case report.
Setting:
University hospital.
Case:
A 70-year-old pharmacist first presented with meningoencephalitislike symptoms and 6 months later with acute confusional state followed by complex partial status epilepticus. Diagnosis of intravascular lymphomatosis was made using detection and biopsy of a bilateral adrenal tumor.
Main Outcome And Results:
Polychemotherapy consisting of CHOP (cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone) led to complete remission. The patient's survival time currently exceeds 21/2 years.
Conclusions:
The possibility of intravascular lymphomatosis should be considered in adult patients with unclear meningoencephalitic syndrome, acute confusional state, dementia, or other unexplained neurologic conditions with signs of a systemic disease. In intravascular lymphomatosis, as in other high-grade non-Hodgkin lymphomas, CHOP polychemotherapy should be the standard treatment.