This 63-year-old male patient had a history of B-CLL (CD5+, CD23+, FMC7+, F79+, and CD38+, with Kappa light chains) diagnosed 4 years earlier and in therapeutic abstention (grade A). The medical history comprised tuberculosis (total recovery) and a restless legs syndrome. He was admitted to the neurology department for paresthesia, pain, and hypoesthesia of the lower limbs. Hemopathy was still biologically stable without any tumoral syndrome, but the patient presented with weight loss. Clinical examination showed a hypoesthesia of the external part of the left leg and foot, but also a hyperesthesia of the left calf and of both soles of the feet (ONLS = 1/12; MRC sum score = 100/100). Achilles deep tendon reflexes were absent. Ancillary tests confirmed the presence in the serum of a B-cell clone of CD5+ and CD20+ (with Kappa light chains). Serum protein electrophoresis, Lyme serology, QuantiFERON, and anti-MAG antibodies were negative. Nerve conduction studies showed a demyelinating sensorimotor polyneuropathy of the lower limbs, with axonal loss (Table 2). A sural NB was finally performed.
We observed small B-cell infiltrates (Figure 2A). Semi-thin and ultra-thin sections showed a severe loss of large myelinated fibers and demyelinating-remyelinating lesions.
Six courses of monthly association of rituximab, fludarabine, and cyclophosphamide were done. The course was marked by an improvement in the general condition of the patient, and a regression of the sensory signs and symptoms (ONLS: 0/12; MRC: 100/100). The patient's state remained stable thereafter.