He was treated empirically with 5 gr of IV methylprednisolone followed
by 50 mg daily oral prednisolone for two weeks.
The histopathological assessment of peroneal nerve biopsy revealed
intravascular proliferation of large atypical lymphocytes. The cells
were positive for CD-20 on IHC staining (figure 1). Further IHC
specification was not feasible due to the few number of available cells.
The final diagnosis was intravascular large B-cell lymphoma, presenting
as mono-neuritis multiplex. The patient was referred to an oncology
clinic where he received chemotherapy with a combination of doxorubicin,
rituximab, cyclophosphamide, and vincristine for about 6 months. Along
with chemotherapy, the patient continued receiving oral prednisolone at
a dose of 100 mg per day.
Outcome and Follow-up
We visited the patient for follow-up 4 months after he completed his
first cycle of chemotherapy. His weakness had subjectively improved and
his Overall Neuropathy Limitations Scale (ONLS) had improved from 1 to 0
in arms and from 4 to 2 in legs.
However follow-up electrodiagnostic studies showed progression in axonal
loss and worsening of polyneuropathy. We postulate that the reason was
disease progression and chemotherapy-induced axonal damage.
He came back to the clinic after 2 years of his first symptoms
complaining of worsening weakness; his ONLS had improved to 2 in arms
and 3 in legs. We performed a head-to-toe examination and found new skin
lesions in his abdomen (Figure 2 ); The skin lesion was biopsied
and the histopathologic study confirmed the recurrence of intravascular
Large-B-cell lymphoma. The timeline in figure 3 summarizes the
disease course in our patient.