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The Neuropathies of Waldenström's Macroglobulinemia (WM) and IgM-MGUS
- Christopher J. Klein, Joon-Shik Moon, Michelle L. Mauermann, Steven R. Zeldenrust, Yanhong Wu, Angela Dispenzieri, Peter J. Dyck
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- Journal:
- Canadian Journal of Neurological Sciences / Volume 38 / Issue 2 / March 2011
- Published online by Cambridge University Press:
- 02 December 2014, pp. 289-295
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Background:
Neuropathy is common in Waldenström's macroglobulinemia (WM, an IgM-associated lymphoplasmacytic lymphoma) and in IgM-monoclonal gammopathy of undetermined significance (IgM-MGUS). Paraneoplastic or paraimmune mechanisms are thought to be involved in the pathogenesis of these neuropathies. Attempts at distinguishing WM and IgM-MGUS neuropathies are lacking especially among bone marrow (BM) confirmed patients.
Methods:Retrospective analyses were performed on BM confirmed WM (N=30) and IgM-MGUS (N=73) neuropathy patients with neurologic assessments and hematologic features.
Results:The presence of anemia and quantity of IgM monoclonal protein were significantly greater in WM. Based on multiple neurologic assessments differences were not found for: 1) length of time from neurologic symptom onset to evaluation; 2) chief complaint of painless loss of feeling in the feet, Romberg's sign and tremor; and 3) clinical motor, sensory and reflex abnormalities. Autonomic testing was normal in both diseases. Using nerve conduction (NCS) criteria for demyelination, 62% of IgM-MGUS and 27% of WM met this criteria (p=0.013). IgM MGUS patients had greater terminal conduction slowing by ulnar residual latency calculation (<0.01). The degree of axonal loss as measured by summated compound muscle action potentials and available nerve biopsy was not significantly different between diseases.
Conclusion:Although WM and IgM-MGUS must be distinguished for hematologic prognosis and treatment, clinical neuropathy presentations of WM and IgM-MGUS are similar and likely related to comparable axonal loss in both conditions. Despite these similarities, evidence of demyelination was found by electrophysiologic studies much more commonly in IgM-MGUS. This difference may reflect varied immune mechanism(s) in the two disorders.
17 - POEMS syndrome and paraproteinemic syndromes: management and follow-up
- from Section 3 - Myeloma: clinical entities
- Edited by Stephen A. Schey, Kwee L. Yong, Robert Marcus, Kenneth C. Anderson
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- Book:
- Myeloma
- Published online:
- 18 December 2013
- Print publication:
- 05 December 2013, pp 225-244
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Summary
Introduction
POEMS syndrome is an atypical plasma cell disorders, composed of a diverse group of diseases that often share little other than an underlying plasma cell clone. The pathophysiologic relationship between the plasma cell dyscrasia is often cryptic in many of these disorders. However, understanding that there may be relationships between bone marrow and distant organ systems is important to arrive at a diagnosis and management plan. A convenient means of characterizing these disorders is by their dominant clinical feature: neuropathy, dermopathy and nephropathy (Figure 17.1). Some atypical plasma cell disorders will have phenotypes that cross many systems, as will be specified in the following pages. The most common atypical plasma cell disorder, light chain (AL) amyloidosis, may affect almost any organ system but will be discussed in a separate chapter.
Atypical PCD with peripheral neuropathy as dominant phenotype
Chemotherapy-induced peripheral neuropathy, Waldenstrom’s macroglobulinemia, multiple myeloma, and light chain amyloid are important etiologies for plasma cell disorder (PCD)-associated neuropathy, but these are beyond the scope of this review. In this section the focus will be upon POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) syndrome and MGUS (monoclonal gammopathy of undetermined significance) associated peripheral neuropathy.
11 - Amyloidosis and other rare plasma cell dyscrasias
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- By Angela Dispenzieri, Mayo Clinic College of Medicine, Division of Hematology, Mayo Clinic, Rochester, MN, USA, Suzanne R. Hayman, Mayo Clinic College of Medicine, Division of Hematology, Mayo Clinic, Rochester, MN, USA
- Edited by Susan O'Brien, Julie M. Vose, Hagop M. Kantarjian
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- Book:
- Management of Hematologic Malignancies
- Published online:
- 10 January 2011
- Print publication:
- 18 November 2010, pp 184-206
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Summary
Introduction
The spectrum of plasma cell disorders is vast. From a proliferation standpoint, the spectrum extends from the premalignant monoclonal gammopathy of undetermined significance (MGUS) to multiple myeloma, to plasma cell leukemia. The picture, however, is much more complex because there are also low tumor burden and low proliferation plasma cell diseases responsible for a clinical phenotype ranging from troublesome to fatal. These conditions are rare, but must be recognized in order to reduce morbidity and mortality. For most of these diseases, the pathogenesis is not well understood.
The discussion will begin with the three more common and potentially life-threatening disorders, light chain amyloidosis (AL), POEMS syndrome (polyradiculoneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes), and cryoglobulinemia. Subsequently, the monoclonal gammopathy-associated disease entities will be parsed according to their dominant clinical feature – neuropathy, dermopathy, and nephropathy – and will include: MGUS-associated peripheral neuropathy, scleromyxedema, xanthogranulosum necrobiotica, and Schnitzler's syndrome.
Immunoglobulin light chain amyloidosis
Immunoglobulin light chain amyloidosis (AL) is a low tumor burden plasma cell disorder characterized by deposition of insoluble fibrils composed of immunoglobulin light chains. Without treatment, it has an inexorable progressive course due to uncontrolled tissue damage. Not all amyloidosis is related to a plasma cell dyscrasia. Although AL is the most common form of systemic amyloidosis, with an incidence of approximately 1 case per 100,000 person-years in Western countries, there are other forms of systemic amyloidosis as well (Table 11.1).
14 - DIAGNOSIS AND TREATMENT OF POEMS SYNDROME
- Edited by S. Vincent Rajkumar, Robert A. Kyle
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- Book:
- Treatment of Multiple Myeloma and Related Disorders
- Published online:
- 11 July 2009
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- 27 October 2008, pp 182-194
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Summary
INTRODUCTION
POEMS syndrome is a paraneoplastic disorder associated with an underlying plasma cell dyscrasia. The major clinical feature of the syndrome is a chronic progressive polyneuropathy with a predominant motor disability. The acronym POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin changes) captures several dominant features of the syndrome. Important traits not included in the acronym include elevated levels of vascular endothelial growth factor (VEGF), sclerotic bone lesions, Castleman disease, papilledema, peripheral edema, ascites, effusions, thrombocytosis, polycythemia, fatigue, clubbing, and abnormal pulmonary function test. Other names for the syndrome include osteosclerotic myeloma, Crow-Fukase syndrome, and Takatsuki syndrome. Although the vast majority of patients have osteosclerotic myeloma, these same patients usually have only 5% bone marrow plasma cells or less (almost always monoclonal λ), and rarely have anemia, hypercalcemia, or renal insufficiency. These characteristics and the superior median survival differentiate POEMS syndrome from multiple myeloma.
The complexity of the interaction of plasma cell dyscrasia and peripheral neuropathy (PN) became increasingly evident in 1956 with Crow's description of two patients with osteosclerotic plasmacytomas with neuropathy, and other “striking features,” which included clubbing, skin pigmentation, dusky discoloration of skin, white finger nails, mild lymphadenopathy, and ankle edema. As many as 50% of patients with osteosclerotic myeloma were noted to have PN in contrast to 1–8 of multiple myeloma patients. A syndrome distinct from multiple myeloma-associated neuropathy became to be recognized.