POEMS syndrome

POEMS syndrome
Other namesCrow–Fukase syndrome, Osteosclerotic myeloma, PEP syndrome, Polyneuropathy-endocrinopathy-plasma cell dyscrasia syndrome, Takatsuki syndrome.[1]
Multiple myeloma is usually diagnosed because malignant plasma cells continue to produce an antibody that can be detected as a paraprotein.
SpecialtyOncology 
SymptomsPapilledema,[2] polyneuropathy,[1] edema,[3][4] hyperpigmentation, hypogonadism,[1] visceromegaly,[1] amenorrhea,[3][4] gynecomastia,[3][4] erectile dysfunction,[3][4] and testicular atrophy.[3][4]
ComplicationsMultiorgan failure, restrictive lung disease, pulmonary hypertension, cardiorespiratory failure, progressive inanition, infection, capillary leak-like syndrome, and renal failure.[5]
Usual onset5th to 6th decade of life.[6]
DurationChronic.[6]
Diagnostic methodClinical Criteria.[6]
Differential diagnosisChronic inflammatory demyelinating polyradiculoneuropathy, cryoglobulinemia, nodular localized cutaneous amyloidosis, multiple myeloma, monoclonal gammopathy of undetermined significance, waldenstrom hypergammaglobulinemia, scleroderma, raynaud phenomenon, amyloid light chains (AL) amyloidosis, guillain-Barré syndrome.[6][7]
TreatmentCorticosteroids, low-dose alkylators, peripheral blood stem cell transplantation, high-dose chemotherapy.[6]
MedicationThalidomide, lenalidomide, bortezomib, bevacizumab.[5]
PrognosisMedian survival of 13.8 years.[8]
FrequencyRare

POEMS syndrome (also termed osteosclerotic myeloma, Crow–Fukase syndrome, Takatsuki disease, or PEP syndrome) is a rare paraneoplastic syndrome caused by a clone of aberrant plasma cells. The name POEMS is an acronym for some of the disease's major signs and symptoms (polyneuropathy, organomegaly, endocrinopathy, myeloma protein, and skin changes), as is PEP (polyneuropathy, endocrinopathy, plasma cell dyscrasia).

The signs and symptoms of most neoplasms (excessive, abnormal tissue growths) are due to their mass effects (compression of surrounding tissue by the mass of the growth) caused by the invasion and destruction of tissues by the neoplasms' cells. Signs and symptoms of a cancer causing a paraneoplastic syndrome result from the release of humoral factors such as hormones, cytokines, or immunoglobulins by the syndrome's neoplastic cells and/or the response of the immune system to the neoplasm. Many of the signs and symptoms in POEMS syndrome are due at least in part to the release of an aberrant immunoglobulin, i.e. a myeloma protein, as well as certain cytokines by the malignant plasma cells.[9][3][4]

POEMS syndrome typically begins in middle age – the average age at onset is 50 – and affects up to twice as many men as women.

  1. ^ a b c d "POEMS syndrome — About the Disease — Genetic and Rare Diseases Information Center". rarediseases.info.nih.gov. Retrieved 2023-08-14.
  2. ^ Cite error: The named reference pmid21035860 was invoked but never defined (see the help page).
  3. ^ a b c d e f Warsame R, Yanamandra U, Kapoor P (2017). "POEMS Syndrome: an Enigma". Current Hematologic Malignancy Reports. 12 (2): 85–95. doi:10.1007/s11899-017-0367-0. PMID 28299525. S2CID 31324035.
  4. ^ a b c d e f Cite error: The named reference ajh was invoked but never defined (see the help page).
  5. ^ a b Nozza, Andrea (1 September 2017). "Poems Syndrome: An Update". Mediterranean Journal of Hematology and Infectious Diseases. 9 (1): e2017051. doi:10.4084/mjhid.2017.051. ISSN 2035-3006. PMC 5584767. PMID 28894560.
  6. ^ a b c d e "POEMS Syndrome: Background, Pathophysiology, Etiology". 16 October 2021.
  7. ^ "POEMS Syndrome — Symptoms, Causes, Treatment — NORD". rarediseases.org. Retrieved 2023-08-16.
  8. ^ Dispenzieri, Angela (1 January 2005). "POEMS Syndrome". Hematology Am Soc Hematol Educ Program. 2005 (1). ash publications: 360–357. doi:10.1182/asheducation-2005.1.360. ISSN 1520-4391. PMID 16304404. Retrieved 15 August 2023.
  9. ^ Castillo JJ (2016). "Plasma Cell Disorders". Primary Care. 43 (4): 677–691. doi:10.1016/j.pop.2016.07.002. PMID 27866585.
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