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[Intravascular lymphomatosis: a case report]
H Adachi1, M Tsuboi, K Hayashi
1Department of Neurosurgery, Bizen Municipal Hospital, 2245 Ibe, Bizen-city, Okayama 705-0001, Japan.
Abstract:
We report a case of a 64-year-old man with intravascular lymphomatosis. He had dementia and generalized seizure. Computed tomogram scan and magnetic resonance imaging showed multiple infarctions in the right frontal lobe, right cerebellar hemisphere and left occipital lobe. A month after admission he had a daily high fever and frequent convulsions. The level of serum LDH was progressively getting higher and the level of beta 2-microglobulin in both serum and CSF was high. One and a half months after admission we performed an open biopsy for making a definite diagnosis by pathological examination. Histological diagnosis was intravascular lymphomatosis (angiotrophic lymphoma) of large B cell type. He was treated with regimens of combined chemotherapy with M-CHOP (methotrexate, cyclophosphamide, adriamycin, vincristine, prednisolone). After chemotherapy his consciousness was gradually improving better and the level of serum LDH and beta 2-microglobulin was reduced to a low level. Three months after admission he died of respiratory failure. After a review of the literature and experience with this case, it is obrious that early diagnosis of this disease is difficult. However, we think that multiple cerebral infarctions on neuroradiological examinations and high levels of serum LDH (especially LDH2 and LDH3) and beta 2-microglobulin in CSF and serum should lead to a presumptive diagnosis of this disease, and biopsy should be used to make a difinite diagnosis.
Insights
Intravascular lymphomatosis is a rare condition causing neurological issues like dementia and seizures. Early diagnosis is challenging but suggested by brain imaging and elevated biomarkers, confirmed by biopsy.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Intravascular lymphomatosis (IVL) is a rare extranodal non-Hodgkin lymphoma characterized by proliferation of lymphoid cells within small blood vessels.
- Diagnosis can be challenging due to nonspecific symptoms and the deep-seated nature of the lesions.
Observation:
- A 64-year-old male presented with dementia, seizures, and neurological deficits attributed to multiple cerebral infarctions on imaging.
- Progressive high fever, convulsions, elevated serum lactate dehydrogenase (LDH), and high beta-2 microglobulin in serum and cerebrospinal fluid (CSF) were noted.
Findings:
- Histopathological examination via open biopsy confirmed intravascular lymphomatosis of the large B-cell type.
- Treatment with M-CHOP chemotherapy led to clinical improvement and reduction of biomarkers, but the patient ultimately succumbed to respiratory failure.
Implications:
- Early diagnosis of IVL is difficult, necessitating a high index of suspicion.
- Neuroradiological findings of multiple cerebral infarctions, coupled with elevated serum LDH (particularly LDH2 and LDH3) and beta-2 microglobulin, suggest IVL.
- Biopsy remains crucial for definitive diagnosis and guiding treatment strategies for this aggressive lymphoma.