Page 46 - Haematologica Vol. 107 - September 2022

REVIEW ARTICLE - IgM monoclonal gammopathies of clinical significance J. Khwaja et al.
Haematologica | 107 September 2022
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Table 3. Features of IgM and non-IgM-related neuropathies. Non-IgM-related
IgM-related POEMS
Onset
Gradually pro- gressive, relap- sing remitting
Gradually progressive
Gradually progressive
*Rapidly progressive <6 months
Gradually progressive
*Rapidly progressive <6 months
Gradually progressive
Gradually progressive
Peripheral nervous system features
Symmetrical, proximal, sen- sory and motor
Symmetrical, distal, progres- sive
Symmetrical, distal, sensory predominant, mild-moderate distal muscle weakness
Symmetrical, distal, sensory predominant, mild-moderate distal muscle weakness
Symmetrical, sensory, may be painful*
Symmetrical, ascending sen- sorimotor poly- neuropathy
*Symmetrical, painful, length dependent, sensorimotor neuropathy. Autonomic dysfunction
Paraesthesia, hypoesthesia, ataxia, ophthal- moplegia
Rare involve- ment of peri- pheral nervous system
Demyelinating/ axonal
Demyelinating
Axonal
Demyelinating
Demyelinating
Axonal
Mixed
Axonal
Mixed
Neither
Supportive tests
Conduction block and ab- normal tempo- ral dispersion
Timing is key. Most commonly bortezomib
High anti-MAG titer typical
Anti-MAG negative
Clinical features are key (see CG section)
VEGF, clinical features (skin, edema, endo- crinopathy, thrombocytosis)
Organ involvement
Anti-ganglioside antibodies
CNS signs* CSF studies, MRI head
Light chain predominance
Not IgM associated
Not IgM asso- ciated
IgMk 70%-80%77
Not reported
IgMk 85% type I
Not reported in IgM. Overall λ LC restriction in POEMS
IgMλ predominance
No k/λ predominance
IgMk 84%61
CIDP
Therapy related Treatment-
Anti MAG
Non-MAG PN Cryoglobulina emia
Amyloidosis
CANOMAD
Bing-Neel
emergent
*Red flag features. CIDP: chronic inflammatory demyelinating polyneuropathy; MAG: myelin-associated glycoprotein; PN: polyneuropathies; POEMS: polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin abnormalities; CANOMAD: chronic ataxic neuropathy, ophthalmoplegia, IgM M-protein, cold agglutinins and disialosyl ganglioside antibodies; CG: cryoglobulinemia; LC: light chain; CNS: central nervous system; CSF: cerebrospinal fluid; MRI: magnetic resonance imaging.
syndrome