Type 1 cryoglobulinemic neuropathy associated with lymphoplasmacytic lymphoma
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LETTER TO THE EDITOR
Type 1 cryoglobulinemic neuropathy associated with lymphoplasmacytic lymphoma Yew Li Dang1,3 · Emma Foster1 · Moira Finlay2 · Andrew Evans1 Received: 25 June 2020 / Accepted: 13 October 2020 © Belgian Neurological Society 2020
Keywords Vasculitis · Lymphoma · Cryoglobulinemia · Peripheral nervous system diseases · Exanthema · Paraproteinemias
Introduction Cryoglobulinemia is characterized by the presence of serum immunoglobulins that precipitate at cold temperatures and dissolve at warmer temperatures [1]. The incidence of cryoglobulinemia is 1:100,000, and more common in females and those aged 45–65 years old [2]. Cryoglobulinemia is associated with infections, autoimmune disorders, and malignancies; the most common precipitant is hepatitis C infection [1]. Cryoglobulinemia can cause systemic vasculitis, which may lead to vascular peripheral neuropathy (VPN). Vascular peripheral neuropathy is most frequently distributed in a distal sensory or sensorimotor pattern [2]. Peripheral neuropathy has been commonly described in association with mixed cryoglobulinemia, and in association with hepatitis C infection [3]. However, there are only limited reports of an association between VPN and type 1 (IgM paraproteinemia) cryoglobulinemia [4].
Case report A 64-year-old woman presented with a 10-week history of purpuric rash over bilateral lower limbs. Medical history included late-onset schizophrenia, treated with depot paliperidone and oral benztropine. Punch biopsy of the rash
* Yew Li Dang [email protected] 1
Neurosciences Department, The Royal Melbourne Hospital, Parkville, Australia
2
Anatomical Pathology Department, The Royal Melbourne Hospital, Parkville, Australia
3
Northern Health, 185 Cooper Street, Epping, VIC, Australia
revealed leukocytoclastic vasculitis with fibrin thrombi and weak reactions for immunoglobulins IgG, IgA and IgM in the upper dermal vessels. She was successfully treated with topical betamethasone and oral prednisolone. Two months later, she developed rapidly progressive right upper limb and bilateral lower limb weakness, resulting in several falls. Relevant examination findings included Medical Research Council 2/5 grade power of right elbow flexion, right wrist flexion and extension, and finger abduction; and 4/5 grade power throughout bilateral lower limbs. Deep tendon reflexes were present in the left upper limb and right knee, but absent elsewhere. Plantar responses were flexor bilaterally. There was reduced sensation over the right C7, C8, and T1 dermatomes. Nerve conduction studies revealed generalized axonal sensorimotor peripheral neuropathy in all four limbs, most prominent in the right upper limb. During her inpatient stay, she developed a livedo reticularis rash over her right forearm. Punch biopsy histology revealed luminal pseudothrombi in small vessels consistent with cryoglobulinemia (Fig. 1a) and immunofluorescence staining was strongly positive for IgM in the lumina of small vessels (Fig. 1b). Subsequent testing for light chain rest
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