POEMS syndrome characterized by peripheral neuropathy as the first symptom:a rare case report

POEMS(Polyneuropathy,Organomegaly,Endocrinopathy,M-Protein and Skin Changes)syndrome is a rare multi-system disease associated with plasma cell abnormality.The etiology and pathogenesis of POEMS syndrome are unclear.Up to 11%-30%of POEMS patients have documented clonal plasma cell disorder with idiopathic multicentric Castleman disease(iMCD)histology.We report a case of POEMS syndrome characterized by plasma cell iMCD in a 64-year-old Asian woman.She was admitted with complaints of numbness and pain in both legs for the last one year.Over the last two months,she developed symptoms associated with respiratory,digestive,and endocrine system disorders.Finally,the POEMS syndrome was diagnosed by lymph node biopsy,electromyography,vascular endothelial growth factor,serum immunofixation electrophoresis,and other auxiliary examinations.POEMS syndrome lacks a standard treatment.This study contributes to providing a reference value for the diagnosis and treatment of this rare disease.

Gastromegaly infiltrated with plasma cells： a new feature of organomegaly in patients with POEMS syndrome

Organomegaly is a major component of POEMS syndrome （an acronym of polyneuropathy,organomegaly, endocrinopatfiy, M protein, and-skin changes）, which is a rare multisystem disorder of unknown pathogenesis, In patients with POEMS syndrome, the organs involved in organomegaly usually are the liver, spleen, and lymph nodes,a-4 Three studies have reported the frequencies of hepatomegaly as 25%, 68%, and 78%; of splenomegaly as 22%, 52%, and 35%;

POEMS syndrome associated with Castleman disease:a case report and literature review

Polyneuropathy,organomegaly,endocrinopathy,M proteins,and skin changes(POEMS)syndrome is a multisystemic disorder that clinically manifests as paraneoplastic and monoclonal plasma cell dyscrasia.Its acronym is derived from its principal characteristics:polyneuropathy,organomegaly,endocrinopathy,M proteins,and skin changes.Here,the authors reported a case of POEMS syndrome that was also associated with Castleman disease.A 53-year-old female patient was admitted to our hospital with limb weakness,numbness,edema,abdominal distention,and fever.Physical examination revealed tetraplegia,paraesthesia,and hyporeflexia in all four limbs,in addition to lymphadenectasis,splenomegaly,skin hyperpigmentation,hypertrichosis,and pitting edema.Laboratory tests and imaging revealed thrombocytosis,hypothyroidism,diabetes,hydropericardium,hydrothorax,splenomegaly,and lymphadenectasis.Electromyography showed the characteristic patterns of both demyelinating disease and axonal degeneration.Serum protein electrophoresis revealed monoclonal immunoglobulin G-lambda paraproteins.Histological examination clearly diagnosed the disease as the hyaline vascular subtype.The final diagnosis in this case was POEMS syndrome in association with Castleman disease.

The unclear role of VEGF in POEMS syndrome: therapeutic implications of neoangiogenesis in a rare plasma cell disorder

POEMS syndrome is a rare paraneoplastic disorder due to an underlying clone of aberrant plasma cells.The name POEMS is an acronym for some of the major disease manifestations,namely polyneuropathy,organomegaly,endocrinopathy,presence of monoclonal component,and skin changes.The clinical presentation can be various and could lead to delayed diagnosis and treatment.Little is known about the pathogenic mechanism,although the neoangiogenesis due to overproduction of vascular endothelial growth factor(VEGF)by plasma cells seems to play a key role.The latest evidence suggests that the blood concentration of this cytokine correlates with the activity of the syndrome:VEGF could then be used as a therapeutic target and a marker to monitor response.Several case reports have shown the efficacy of this approach,but extended studies are required to better define the use of anti VEGF in patients affected by POEMS syndrome.