What is POEMS Syndrome?
POEMS Syndrome is an extremely rarefied blood line disorder which tends to significantly damage the nerves of the organic structure and also affects various other body theatrical role . poem stands for the signs and symptoms of Polyneuropathy , Organomegaly , Endocrinopathy , Monoclonal proliferative Disorder , peel change which browse from numbness and prickle along with weakness in the extremities to enlargement of the liver or spleen , to abnormal hormonal horizontal surface , and abnormal skin changes .
POEMS Syndrome often at times is misdiagnosed due to the law of similarity of symptoms with various other medical conditions . POEMS Syndrome is a condition which progresses at a fairly speedy stride and tends to become life sentence threaten if not treated at the appropriate time . There is no cure for POEMS Syndrome but timely treatment can ease the symptoms to a significant arcdegree . Some of the intervention options for treating POEMS Syndrome are radiation andchemotherapyorstem cell transplantation .
What Causes POEMS Syndrome?
What on the button causes POEMS Syndrome is not well known ; however , it is recognise to be a paraneoplastic disorder meaning that the symptoms of this condition appear as a manifestation of a plasma cubicle disorder but why this occurs is not have intercourse . POEMS Syndrome is also believed to be associated with excessive production of inflammatory cytokine which are eff to advance inflammation . Another cause for POEMS Syndrome is the vascular endothelial development component or VEGF which is a vasodilator which tends to lead to leaky blood vessels and is often associated with POEMS Syndrome .
What are the Symptoms of POEMS Syndrome?
As POEMS Syndrome affects multiple systems in the consistence , hence the symptoms of this disorder is also highly varied . The symptoms for POEMS Syndrome are :
Polyneuropathy : The symptom of Polyneuropathy include failing , pain , indifference or tingle generally get down in the lower extremities and then bit by bit work up as the disease advances to the upper extremities .
Organomegaly : In general the liver and spleen are the electric organ enlarged in an someone with POEMS Syndrome and hence the symptoms are related to that .
Endocrine Abnormalities : Individuals with POEMS syndrome may show numerous abnormalities which affect the internal secretion system like unlawful operation of ovary in females and orchis in males along with growing ofdiabetes , hypothyroidism , glucose intolerance , gynecomastia in males .
Monoclonal Gammopathy : Individuals with POEMS Syndrome will also have monoclonal gammopathy which is qualify by uncontrolled growing of plasma cell . This excessive production of plasm cellphone lead to development of tumors .
Skin Abnormalities : soul with POEMS Syndrome will also have significant skin abnormalcy with hyperpigmentation of the skin , excessivehair growthon the skin , severe and thick skin , andhyperhidrosis .
asunder from these symptoms , individual with POEMS Syndrome also run to have papilledema with gradual loss of visual modality .
Some of the other symptom of POEMS Syndrome are :
How is POEMS Syndrome Diagnosed?
diagnose verse form syndrome is quite complex as the symptoms resemble so many other aesculapian weather condition but to name POEMS Syndrome a elaborated story and physical examination is done and a notation is made of the symptom experienced by the patient role . A kin history of the patient is also taken to investigate as to whether there are other family member who have had this problem but a definitive diagnosing of POEMS Syndrome is made normally by identifying sure immunological irregularity on laboratory examination .
These trial are done on the blood or the CSF or cerebrospinal fluid which will show lofty levels of M - proteins which is a clinching grounds of POEMS Syndrome . Additionally , analysis of blood blood plasma will show rarefied levels of vascular endothelial ontogeny factor which will almost confirm the diagnosing of POEMS Syndrome .
How is POEMS Syndrome Treated?
There is no cure for poem Syndrome but treatment is basically two fold with the first discourse totally aimed at treating the plasm jail cell disorder and the second is to process the specific symptoms that the patient experiences . The treatment for POEMS Syndrome need a squad of physician from various forte working in a align way to endeavor to give best potential discussion to the patient .
irradiation and chemotherapy or even operative excision of localized osteosclerotic lesion and which have still not spread is quite efficacious in ameliorating the symptoms of POEMS Syndrome . In some cases chemotherapy , anti - cancer agents , and steroids have also some effectiveness towards treatment of POEMS Syndrome . root cellular phone graft is yet another mode of attempting to treat symptoms of POEMS Syndrome . Otherwise , the treatment is strictly supportive and diagnostic for POEMS Syndrome .
What is the Prognosis or Survival Rate of POEMS Syndrome?
verse form Syndrome as of now can not be bring around completely and the symptom tend to come and go . The consideration advances at a fairly speedy pace to arrogate a aliveness jeopardize precondition if not treated at an appropriate time . POEMS syndrome with time becomes a chronic condition and in majority of case turn in an individual totally disabled due to the symptoms . On intermediate the survival rate has been estimated to be around 14 old age for an individual with POEMS Syndrome , although with timely intervention and a right response to the handling receive an individual can survive for a longer period of clip with POEMS Syndrome .
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