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 | |
WBC: white blood cell, ALC: absolute lymphocyte count, LYMR: lymphocyte rate, NCS: nerve conduction study, CSF: cerebrospinal fluid, CHOP: cyclophosphamide, doxorubicin [adriamycin], vincristine, and prednisone, NA: not available