Weekly Case

Title : case 367

Age / Sex : 54 / M


Chief complaint: Right leg weakness, both hand numbness

1) What is your impression?

Two weeks later, you can see the final diagnosis with a brief discussion of this case.

(Quiz
quiz 뿐이오니 답안은 개만 보내주시기 바랍니다)


 


 


 


Courtesy: 김성준(Sungjun Kim), 강남세브란스병원(Gangnam Severance Hospital)


Diagnosis:

POEMS syndrome



Discussion


Findings:


1. Multiple sclerotic lesion of vertebral bodies and pelvic bone on CT images.


2. Focal FDG uptake at L1 vertebral body on PET scan.


3. Splenomegaly.


 


 


 


Differential Diagnosis:


Sclerotic variant of multiple myeloma, osteoblastic bone metastasis, osteopoikilosis, lymphoma.


 


Diagnosis:  


POEMS syndrome


 


Discussion:


POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. It is first described by Bardwick in 1980. The pathogenesis is unknown. Recently proposed guideline for the diagnosis is like follows: three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria in the following features.

































Mandatory major criteria


1. Polyneuropathy (typically demyelinating)


2. Monoclonal plasma cell-proliferative disorder (almost always λ)


Other major criteria


3. Castleman diseasea


One required


4. Sclerotic bone lesions


5. VEGF elevation


Minor criteria


6. Organomegaly (splenomegaly, hepatomegaly, or lymphadenopathy)


7. Extravascular volume overload (edema, pleural effusion, or ascites)


8. Endocrinopathy (adrenal, thyroid,b pituitary, gonadal, parathyroid, and pancreaticb)


9. Skin changes (hyperpigmentation, hypertrichosis, glomeruloid hemangiomata, plethora, acrocyanosis, flushing, and white nails)


10. Papilledema


11. Thrombocytosis/polycythemiac


Other symptoms and signs


Clubbing, weight loss, hyperhidrosis, pulmonary hypertension/restrictive lung disease, thrombotic diatheses, diarrhea, low vitamin B12 values


 


 


References:


 Dispenzieri A. Am J Hematol. 2014 Feb;89(2):214-23.



Correct Answer
Name Institution
이름:소속병원
Total Applicants: 21
Correct answers:18
박진아:서울대병원
김완태:중앙보훈병원
조병구:삼성창원병원
박선영:서울아산병원
이혜란:서울아산병원
이지현:삼성서울병원
김성윤:으뜸병원
장은호:포항남부 보건소
이지숙:순천향대부천병원
박지원:대구튼튼병원
조신영:웰튼병원
유성혜:고대안암병원
김현수:삼성서울병원
윤성종:강동경희대병원
홍현주:의정부성모병원
장윤희:충북대병원
이상윤:무척나은 병원
강유선:서울대병원

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