HAEM5:Plasma cell neoplasms with associated paraneoplastic syndrome: Difference between revisions

As mentioned earlier, POEMS syndrome is a rare disorder which comprises of constellation of clinical presentations with neuropathy present in almost 100% of the cases. Following are the clinical features:

*'''<u>Specific findings in Females</u>'''- amenorrhea and galactorrhea have been reported in female patients with POEMS syndrome

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==Sites of Involvement==

Plasma cells in BM biopsy ranges from<5% to 60%. 95% of the cases have value <5%.<ref name=":6">{{Cite journal|last=Dispenzieri|first=Angela|date=2012-06-14|title=How I treat POEMS syndrome|url=https://doi.org/10.1182/blood-2012-03-378992|journal=Blood|volume=119|issue=24|pages=5650–5658|doi=10.1182/blood-2012-03-378992|issn=0006-4971|pmc=PMC3425020|pmid=22547581}}</ref>  Usually there are a combination of normal polyclonal plasma cells and monoclonal plasma cells. The monoclonal plasma cells stains positive for λ chains (IgA and IgG). <ref>{{Cite journal|last=Dao|first=Linda N.|last2=Hanson|first2=Curtis A.|last3=Dispenzieri|first3=Angela|last4=Morice|first4=William G.|last5=Kurtin|first5=Paul J.|last6=Hoyer|first6=James D.|date=2011-06-16|title=Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients|url=https://doi.org/10.1182/blood-2010-11-316935|journal=Blood|volume=117|issue=24|pages=6438–6444|doi=10.1182/blood-2010-11-316935|issn=0006-4971|pmc=PMC3123015|pmid=21385854}}</ref>

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==Chromosomal Rearrangements (Gene Fusions)==

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* Individual Region Genomic Gain/Loss/LOH

* Characteristic Chromosomal Patterns

Diagnosis of POEMS syndrome requires meticulous search for clinical features and testing because of clinical simulation with other common conditions. Being one of the rare disease entities it can easily be missed by clinicians.  

*clinical improvement in effusions

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==Individual Region Genomic Gain / Loss / LOH==

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A study conducted at Peking Union Medical college Hospital from November 2011 to June 2012 showed the following chromosomal abnormalities associated with POEMS syndrome.<ref>{{Cite journal|last=Kang|first=Wen-Ying|last2=Shen|first2=Kai-Ni|last3=Duan|first3=Ming-Hui|last4=Zhang|first4=Wei|last5=Cao|first5=Xin-Xin|last6=Zhou|first6=Dao-Bin|last7=Li|first7=Jian|date=2013-12|title=14q32 translocations and 13q14 deletions are common cytogenetic abnormalities in POEMS syndrome|url=https://onlinelibrary.wiley.com/doi/10.1111/ejh.12189|journal=European Journal of Haematology|language=en|volume=91|issue=6|pages=490–496|doi=10.1111/ejh.12189}}</ref>. In this study, BM plasma cells CD138+ were collected using MACS system and then FISH technique was applied.   

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==Characteristic Chromosomal Patterns==

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==Gene Mutations (SNV / INDEL)==

A study was conducted in 20 patients of POEMS syndrome. The study showed 7  gene shaving recurrent somatic gene mutations involved in POEMS syndrome.  It is important to know that none of the important gene mutations involved in MM such as NRAS, KRAS, BRAF, and TP53 were found in POEMS syndrome patients.<ref>{{Cite journal|last=Nagao|first=Yuhei|last2=Mimura|first2=Naoya|last3=Takeda|first3=June|last4=Yoshida|first4=Kenichi|last5=Shiozawa|first5=Yusuke|last6=Oshima|first6=Motohiko|last7=Aoyama|first7=Kazumasa|last8=Saraya|first8=Atsunori|last9=Koide|first9=Shuhei|date=2019-07|title=Genetic and transcriptional landscape of plasma cells in POEMS syndrome|url=https://www.nature.com/articles/s41375-018-0348-x|journal=Leukemia|language=en|volume=33|issue=7|pages=1723–1735|doi=10.1038/s41375-018-0348-x|issn=1476-5551}}</ref> [[HAEM4:Plasma Cell Neoplasms]]

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==Epigenomic Alterations==

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'''<u>PATHOGENESIS OF POEMS SYNDROME</u>'''

*'''<u>λ Immunoglobulins</u>'''

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==Genetic Diagnostic Testing Methods==