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      Drugs & Diseases > Dermatology

      POEMS Syndrome Workup

      Updated: Sep 17, 2020
      • Author: Joanna L Chan, MD; Chief Editor: Dirk M Elston, MD  more...
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      Workup

      Laboratory Studies

      When POEMS syndrome is suggested clinically, perform a range of laboratory studies to define the extent of involvement and to establish whether other organ systems are involved.

      Patients commonly have thrombocytosis with or without polycythemia.

      Hypercalcemia and renal insufficiency are rarely present.

      Generally, the M protein is IgG-gamma or IgA-gamma and small in size (median, 1.1 g/dL).

      Serum protein immunoelectrophoresis is used to define the nature and the extent of the monoclonal gammopathy.

      Thyrotropin levels, fasting blood glucose levels, a glucose tolerance test, and estrogen levels can be used to screen for endocrinopathy.

      In patients with neuropathy, cerebrospinal fluid test results either are in the reference range or may show elevated levels of protein.

      Erythrocyte sedimentation rate results are in the reference range or slightly elevated.

      Cerebrospinal fluid may demonstrate a cytoalbuminologic dissociation.

      For research purposes, cytokine and growth factor levels can be measured. Based on past studies, TNF-alpha, IL-6, IL-1beta, and VEGF levels are usually elevated, while epidermal growth factor, fibroblast growth factor, and platelet-derived growth factor levels are within the reference range.

      Although rare, it has been suggested that POEMS syndrome may increase the risk of potentially lethal calciphylaxis. Follow-up on the potential occurrence of this symptom is advisable. [25]

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      Imaging Studies

      Plain film radiographs are useful for locating lytic bone lesions caused by osteosclerotic myeloma.

      At least 95% of patients have osteosclerotic lesions, with more than half the patients having multiple lesions. Both osteosclerotic and osteolytic lesions may be present and may be of modest size.

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      Procedures

      Bone marrow biopsy may be indicated because as many as 10% of patients have marrow involvement with plasma cells.

      Lymph node biopsy is indicated in patients with lymphadenopathy and, in most patients, demonstrates findings of Castleman disease. HHV-8 has been demonstrated within the lymphocytes of some of these lymph node biopsy specimens, in addition to being present within endothelial cells and lymphocytes in the glomeruloid hemangioma skin biopsy specimen. [26, 27] Within the HHV-8 genome, a viral homologue to human IL-6 is present, which is believed to induce angiogenesis and hematopoiesis. However, some patients test negative for HHV-8, [28] so the complete role of this virus in the pathogenesis of Castleman disease is unknown.

      Electromyography exhibits findings consistent with polyneuropathy, prominent demyelination, and features of axonal degeneration. One study demonstrated a statistically significant pattern of lower limbs having absent or attenuated amplitudes of compound muscle action potentials and absent sensory nerve action potentials compared with upper limbs. Intermediate nerves segments exhibited abnormal conduction slowing compared with distal portions. These patterns may aid in early diagnosis.

      Nerve biopsies usually reveal evidence of both axonal degeneration and demyelination, characterized by uncompacted myelin lamina without immunoglobulin deposition and minimal cellular infiltration.

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      Histologic Findings

      The histopathologic changes seen in the sclerodermoid lesions are nonspecific, showing hyperpigmentation of the basal layer with an inflammatory infiltrate or dermal fibrosis. Other reports have noted vascular prominence. Sweat glands and collagen are normal, differentiating this condition from scleroderma. A skin biopsy of hyperpigmented lesional skin may demonstrate a nonspecific inflammatory infiltrate composed of a lymphoplasmacytic population. [29]

      Most angiomas seen in persons with POEMS syndrome are histologically consistent with cherry angiomas. The angiomas in a small proportion of patients have the appearance of a glomeruloid hemangioma. [30] This finding may be strongly suggestive of POEMS syndrome, but it is not pathognomonic because the presence of this pathologic entity has been reported in a patient without POEMS syndrome. Multiple ectatic vascular spaces with luminal clusters of congested capillaries are noted in the lesions. The capillaries are surrounded by pericytes and resemble renal glomeruli, hence the term glomeruloid hemangioma.

      A study of bone-marrow histology in 87 patients from the Mayo Clinc concluded that the constellation of lambda-restricted monoclonal gammopathy, plasma cell rimming around lymphoid aggregates, and megakaryocytic hyperplasia in bone marrow is highly suggestive of POEMS syndrome, especially in the context of a peripheral neuropathy. [31]

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