Table 2 Clinical characteristics of peripheral neuropathy associated with CLPD-NK
Reference | Age, years | Gender | Symptoms/signs | WBC (×109/L) | ALC (×109/L) | LYMR, (%) | EMG | WBC (CSF) (106/L) | Protein (CSF) (g/L) | Sural nerve biopsy | Treatment | Prognosis | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Wex et al.7 | 16 | F | generalized muscle weakness, Lymphadenopathy, Allergic skin, Headaches, Recurrent infections, Delayed puberty | NA | 32 (NK: 10.72) | NA | Decreased motor nerve conduction velocity | Increased NK cells | NA | Neuritic disease with perivascular infiltrates affecting small neural vessels | Prednisone | Achieved transient improvement | |
Boer et al.26 | 23 | M | Lethargy, Anorexia, Headaches, Lower limb paraesthesia, Marked weight loss, Postural hypotension, Diarrhoea, Abnormal sweating, Mildly splenomegaly | 16.25 | NA | 64 | Reduced common peroneal distal motor amplitudes, mildly prolonged F-response latencies and absent H-reflexes. The sural amplitudes were at lower limit of normal. Suggesting a mild predominantly motor, axonal neuropathy or polyradiculopathy. | Found NK cells in CSF (no detail) | 1.59 | NA | MTX, CTX, teniposide prednisolone fludrocortisone | Achieved partial improvement of symptoms, left mild diarrhoea and postural hypotension, without obvious changes of blood white cell count and lymphocytes rate. | |
Rabbani et al.1 | 34 | M | Skin lesions and peripheral neuropathy (bilateral extremity dyesthesias, loss of motor control and coordination) (Biopsy of skin lesions showed perivascular and perineural infiltration with atypical lymphoid cells) | 5.5 | 1.88 (NK: 1.2) | NA (NK:64) | Sensorimotor peripheral neuropathy | NA | NA | Multifocal epineural perivascular lymphocytic infiltrates, focal areas of axonal loss consistent with ischaemic neuropathy | CHOP chemotherapy Prednisone | Improved | |
Rabbani et al.1 | 33 | F | Diffuse macular erythematous skin lesions and peripheral neuropathy, (Biopsy of skin lesions revealed livedoid vasculitis) | 11 | 7.32 (NK: 5.49) | NA (NK:75) | NA | NA | NA | NA | prednisone | Improved | |
Leitenberg et al.27 | 66 | F | Upper and lower extremity paresthesias and progressive weakness | 14.6 | NA | 67 | Decreased motor nerve conduction velocities and prolonged F-wave latencies consistent with a demyelinating sensorimotor polyneuropathy | NA | NA | Inflammatory polyneuropathy with myelin loss and a mononuclear cell infiltrate | Azathioprine Prednisone | Achieved improvement in both neurologic symptoms and hematologic tests | |
Leitenberg et al.27 | 65 | M | Slowly progressive tingling and weakness in the lower extremities, Mild splenomegaly | 6.7 | NA | 6% lymphocytes;17% atypical lymphocytes | Decreased motor nerve conduction velocities and prolonged F-wave latencies consistent with a demyelinating sensorimotor polyneuropathy | NA | NA | Inflammatory polyneuropathy with myelin loss and NK cell infiltrate | Azathioprine Prednisone | Achieved improvement in both neurologic symptoms and hematologic tests | |
Noguchi et al.15 | 70 | F | Progressive hypesthesia and weakness of upper and lower extremities and difficulty in walking, General malaise, Body weight loss, Hepatomegaly | 17 | NA | 68 | A mixed axonal and demyelinating neuropathy; | 10 | 0.93 | Infiltration of NK cells into the nerve fascicles, demyelinating changes combined with axonal degeneration | Prednisolone | Achieved both clinical and hematologic improvement | |
Chee et al.20 | 62 | M | Livedo reticularis, Cutaneous polyarteritis nodosa, Peripheral neuropathy | 14.6 | 8.91 | 82% | NA | NA | NA | Vasculitis, increased abnormal NK cell clone | Corticosteroids, MTX Alemtuzumab | Failed therapy with corticosteroids and MTX, but achieved both clinical and hematologic improvement by alemtuzumab | |
Richelli et al.28 | 65 | M | Painful paresthesias and sensory loss in his feet, later involving volar surface of both hands, Gait disturbance, Weakness | NA | NA (NK:1.5) | NA | A diffuse demyelinating process with sighs of axonal degeneration especially at sural nerves. | 3 | 1.53 | Axonal degeneration and endoneurial mononuclear cell infiltrates, mainly composed of NK cells. | Immune globulin | Achieved both clinical and hematologic improvement | |
Sano et al.29 | 67 | F | Progressive, asymmetric weakness and numbness in all four extremities | 22.8 | NA (NK:19.5) | 94 | Asymmetric demyelination in both motor and sensory nerves | 1 | 0.27 | Demyelination, NK cells infiltrated in the endoneurium. | Corticosteroids | Neurological, electrophysiological and hematological improvement | |