Background and aims – Description of two cases of rare
intravascular large B-cell lymphoma and secondary T-cell
lymphoma diagnosed postmortem, that manifested clinically
as longitudinally extensive transverse myelitis (LETM).
We discuss causes of diagnostic difficulties, deceptive radiological
and histological investigations, and outline diagnostic
procedures based on our and previously reported
Case reports – Our first case, a 48-year-old female was
admitted to the neurological department due to paraparesis.
MRI suggested LETM, but the treatments were ineffective.
She died after four weeks because of pneumonia and
untreatable polyserositis. Pathological examination
revealed intravascular large B-cell lymphoma (IVL). Our
second case, a 61-year-old man presented with headache
and paraparesis. MRI showed small bitemporal lesions
and lesions suggesting LETM. Diagnostic investigations
were unsuccessful, including tests for possible lymphoma
(CSF flow cytometry and muscle biopsy for suspected IVL).
Chest CT showed focal inflammation in a small area of
the lung, and adrenal adenoma. Brain biopsy sample
from the affected temporal area suggested T-cell mediated
lymphocytic (paraneoplastic or viral) meningoencephalitis
and excluded diffuse large B-cell lymphoma. The symptoms
worsened, and the patient died in the sixth week of
disease. The pathological examination of the presumed adenoma in the adrenal gland, the pancreatic tail and the
lung lesions revealed peripheral T-cell lymphoma, as did
the brain and spinal cord lesions. Even at histological
examination, the T-cell lymphoma had the misleading
appearance of inflammatory condition as did the MRI.
Conclusion – Lymphoma can manifest as LETM. In cases
of etiologically unclear atypical LETM in patients older
than 40 years, a random skin biopsy (with subcutaneous
adipose tissue) from the thigh and from the abdomen is
strongly recommended as soon as possible. This may
detect IVL and provide the possibility of prompt
chemotherapy. In case of suspicion of lymphoma, parallel
examination of the CSF by flow cytometry is also recommended.
If skin biopsy is negative but lymphoma suspicion
remains high, biopsy from other sites (bone marrow,
lymph nodes or adrenal gland lesion) or from a simultaneously
existing cerebral lesion is suggested, to exclude or
prove diffuse large B-cell lymphoma, IVL, or a rare T-cell