POEMS syndrome

 

Overview

Characterized by polyneuropathy (simultaneous malfunction of many peripheral nerves throughout the body), organomegaly (enlargement of an organ or organs), endocrinopathy (endocrine gland disease that disrupts hormone production), monoclonal gammopathy (abnormal proteins found in the blood), and skin changes. While the cause for this condition remains unknown, the reasons that it becomes chronic are easily detected. POEMS causes an increased plasma cell count. This Is alarming because the overproduction of plasma cells results in an excess amount of a specific protein that can cause damage to other parts of the body. Misdiagnosis is common because of its symptomatic similarity to other disorders.

Common Symptoms

Enlarged liver, spleen and/ or lymph nodes, progressive polyneuropathy, hyperpigmentation, weight loss, fatigue, blood clots, endocrine gland disorders, monoclonal plasma cell proliferative disorders, restrictive lung disease, pulmonary hypertension, respiratory muscle weakness and extravascular fluid overload.

Coexisting Diseases and Conditions

Hypogonadism, Castleman’s disease, organomegaly (enlargement of the spleen, liver, or lymph nodes), peripheral edema, ascites, or pleural effusion), endocrinopathy (excluding diabetes mellitus or hypothyroidism), skin changes, papilledema, thrombocytosis, polycythemia, osteosclerotic myeloma, gynecomastia in males, amenorrhoea in females, hypothyroidism, hypoparathyroidism, hyperprolactinemia, diabetes, glucose intolerance, and anemia.

Risk Factors and Prevalence

Males, particularly those of middle age, tend to be more affected by POEMS syndrome than females.

Sources

  1. Article Sources and Footnotes

    1. Mayo Clinic Staff. (2020, April 2). POEMS syndrome. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/poems-syndrome/symptoms-causes/syc-20352678

    2. The Monarch Initiative. (n.d.). Monarch Initiative Explorer. https://monarchinitiative.org/disease/MONDO:0017364

    3. NORD (National Organization for Rare Disorders). (n.d.). POEMS Syndrome. NORD (National Organization for Rare Disorders). https://rarediseases.org/rare-diseases/poems-syndrome/

    4. Tian, T. (2013). POEMS syndrome. https://dermnetnz.org/topics/poems-syndrome/

    5. U.S. Department of Health and Human Services. (2015, August 31). POEMS syndrome. Genetic and Rare Diseases Information Center. https://rarediseases.info.nih.gov/diseases/7411/poems-syndrome