088 Cryoglobulinemic neuropathy associated with lymphoplasmacytic lymphoma. Issue 6 (24th May 2018)

• Record Type: Journal Article
• Authors: Dang, Yew Li; Foster, Emma; Finlay, Moira; Evans, Andrew
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 89:Issue 6(2018)
• Page Start: A35
• Page End: A36
• Publication Date: 2018-05-24
• Subjects: Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp-2018-ANZAN.87
• Languages: English
• ISSNs: 0022-3050

Abstract

Abstract : Introduction: A 64 year old woman presented with livedo reticularis and peripheral neuropathy secondary to Type 1 (IgM paraproteinemia) cryoglobulinemia, associated with lymphoplasmacytic lymphoma. Type 1 cryoglobulinemia is rare and remains poorly studied. However, given the common association of Type 1 cryoglobulinemia with lymphoproliferative diseases, it is important to consider Type 1 cryoglobulinemia as a differential diagnosis in presentations of a rash and peripheral neuropathy, and search for underlying malignancy. Case: Our patient presented with a leukocytoclastic vasculitic rash over bilateral lower limbs, which improved with topical betamethasone and oral prednisolone. Two months later, she had rapidly progressive right upper limb and bilateral lower limb weakness, absent reflexes in these limbs, and right arm reduced sensation. Nerve conduction studies revealed generalised axonal sensorimotor peripheral neuropathy in all four limbs, most prominent in the right upper limb. She then developed a livedo reticularis rash over her right forearm and punch biopsy revealed luminal pseudo-thrombi in small vessels consistent with Type 1 cryoglobulinemia. Her cryoglobulin (1558 mg/L, N 0–50 mg/L) and IgM paraprotein levels (4 g/L, N 0.4–2.3) were elevated. Vasculitic and other infective screens were unremarkable. Computer tomography imaging of chest, abdomen and pelvis found widespread lymphadenopathy. Subsequent lymph node core biopsy and bone marrow aspirate revealed lymphoplasmacytic lymphoma—the likely underlying cause of the Type 1 (IgM paraproteinemia) cryoglobulinemia. Conclusion: Peripheral neuropathy is commonly associated with Type 2 and 3 cryoglobulinemia, especially in the presence of hepatitis C infection. However, reports of vasculitic peripheral neuropathy due to Type 1 cryoglobulinemia are limited. Early recognition is essential to allow the identification and treatment of the underlying haematological malignancy, commonly associated with Type 1 cryoglobulinemia. Treatment of the underlying cause indirectly treats the cryoglobulinemia, and avoids or reduces the associated; sometimes severe; cutaneous, neurological, and renal manifestations of this condition.