A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME

Objective: Although plasma cell neoplasms occupy a large place in hematology practice, POEMS syndrome is very rare. Serum lambda light chain elevation and polyneuropathy, together with organomegaly, endocrinopathy, and skin lesions are the main components of the syndrome. We share our case, which we...

Descripción completa

Guardado en:

Detalles Bibliográficos
Autores principales:	Buğra Sağlam, Murat Albayrak, Hacer Berna Afacan Öztürk, Merih Reis Aras, Pınar Tığlıoğlu, Mesut Tığlıoğlu, Fatma Yılmaz, Senem Maral, Mustafa Önder
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Diseases of the blood and blood-forming organs RC633-647.5
Acceso en línea:	https://doaj.org/article/d8a67099b2d04f88be082e541da8eff2
Etiquetas:	Agregar Etiqueta Sin Etiquetas, Sea el primero en etiquetar este registro!

id	oai:doaj.org-article:d8a67099b2d04f88be082e541da8eff2
record_format	dspace
spelling	oai:doaj.org-article:d8a67099b2d04f88be082e541da8eff22021-11-10T04:36:31ZA RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME2531-137910.1016/j.htct.2021.10.1045https://doaj.org/article/d8a67099b2d04f88be082e541da8eff22021-11-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S2531137921011925https://doaj.org/toc/2531-1379Objective: Although plasma cell neoplasms occupy a large place in hematology practice, POEMS syndrome is very rare. Serum lambda light chain elevation and polyneuropathy, together with organomegaly, endocrinopathy, and skin lesions are the main components of the syndrome. We share our case, which we diagnosed in our clinic, with the belief that it will contribute to the literature. Case report: A 51-year-old male patient, who had no history of co-morbidity, drug use, or exposure to toxic substances, was started on supportive treatment in February 2021, who first developed the complaint of impotence. Later, he applied to the neurology outpatient clinic with complaints of weakness and weakness in the feet. After detecting polyneuropathy in his evaluation, IgG Lambda monoclonal gammopathy was detected in serum immune electrophoresis in his evaluation for etiology. Methodology: Thereupon, it started to be investigated in terms of plasma cell neoplasms. In the examinations performed, immunoglobulin levels, serum-urine kappa and lambda light chain levels, plasma increase in the bone marrow biopsies and a solitary 3.3 cm sclerotic lesion in the sacral region were detected in the PET-CT of the patient, whose ethology could not be diagnosed. Results: A tru-cut biopsy was taken from the sclerotic lesion of the patient, who was thought to be a plasmacytoma and a 20% monoclonal IgG lambda plasma increase was detected. In his physical examination, it was seen that he had increased lesions (Figure-1) and acrocyanosis (Figure-2) on the skin for the last 3-4 months. The patient's current complaints and laboratory results were evaluated with a preliminary diagnosis of POEMS syndrome (Table-1). Conclusion: POEMS syndrome is a rare disease and its exact incidence is unknown. It is frequently seen in 5-6 decades, with a median age of 51 years, and 63% of cases are male patients [1]. Chronic and excessive production of proinflammatory and other cytokines (IL-1β, TNFα, IL-6, vascular endothelial growth factor (VEGF) etc.), microangiopathy, edema, effusions, increase in vascular permeability, increase in neovascularization are important in the pathophysiology of the disease.Buğra SağlamMurat AlbayrakHacer Berna Afacan ÖztürkMerih Reis ArasPınar TığlıoğluMesut TığlıoğluFatma YılmazSenem MaralMustafa ÖnderElsevierarticleDiseases of the blood and blood-forming organsRC633-647.5ENHematology, Transfusion and Cell Therapy, Vol 43, Iss , Pp S45-S46 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Diseases of the blood and blood-forming organs RC633-647.5
spellingShingle	Diseases of the blood and blood-forming organs RC633-647.5 Buğra Sağlam Murat Albayrak Hacer Berna Afacan Öztürk Merih Reis Aras Pınar Tığlıoğlu Mesut Tığlıoğlu Fatma Yılmaz Senem Maral Mustafa Önder A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME
description	Objective: Although plasma cell neoplasms occupy a large place in hematology practice, POEMS syndrome is very rare. Serum lambda light chain elevation and polyneuropathy, together with organomegaly, endocrinopathy, and skin lesions are the main components of the syndrome. We share our case, which we diagnosed in our clinic, with the belief that it will contribute to the literature. Case report: A 51-year-old male patient, who had no history of co-morbidity, drug use, or exposure to toxic substances, was started on supportive treatment in February 2021, who first developed the complaint of impotence. Later, he applied to the neurology outpatient clinic with complaints of weakness and weakness in the feet. After detecting polyneuropathy in his evaluation, IgG Lambda monoclonal gammopathy was detected in serum immune electrophoresis in his evaluation for etiology. Methodology: Thereupon, it started to be investigated in terms of plasma cell neoplasms. In the examinations performed, immunoglobulin levels, serum-urine kappa and lambda light chain levels, plasma increase in the bone marrow biopsies and a solitary 3.3 cm sclerotic lesion in the sacral region were detected in the PET-CT of the patient, whose ethology could not be diagnosed. Results: A tru-cut biopsy was taken from the sclerotic lesion of the patient, who was thought to be a plasmacytoma and a 20% monoclonal IgG lambda plasma increase was detected. In his physical examination, it was seen that he had increased lesions (Figure-1) and acrocyanosis (Figure-2) on the skin for the last 3-4 months. The patient's current complaints and laboratory results were evaluated with a preliminary diagnosis of POEMS syndrome (Table-1). Conclusion: POEMS syndrome is a rare disease and its exact incidence is unknown. It is frequently seen in 5-6 decades, with a median age of 51 years, and 63% of cases are male patients [1]. Chronic and excessive production of proinflammatory and other cytokines (IL-1β, TNFα, IL-6, vascular endothelial growth factor (VEGF) etc.), microangiopathy, edema, effusions, increase in vascular permeability, increase in neovascularization are important in the pathophysiology of the disease.
format	article
author	Buğra Sağlam Murat Albayrak Hacer Berna Afacan Öztürk Merih Reis Aras Pınar Tığlıoğlu Mesut Tığlıoğlu Fatma Yılmaz Senem Maral Mustafa Önder
author_facet	Buğra Sağlam Murat Albayrak Hacer Berna Afacan Öztürk Merih Reis Aras Pınar Tığlıoğlu Mesut Tığlıoğlu Fatma Yılmaz Senem Maral Mustafa Önder
author_sort	Buğra Sağlam
title	A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME
title_short	A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME
title_full	A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME
title_fullStr	A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME
title_full_unstemmed	A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME
title_sort	rare and complex cause of impotence poems syndrome
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/d8a67099b2d04f88be082e541da8eff2
work_keys_str_mv	AT bugrasaglam arareandcomplexcauseofimpotencepoemssyndrome AT muratalbayrak arareandcomplexcauseofimpotencepoemssyndrome AT hacerbernaafacanozturk arareandcomplexcauseofimpotencepoemssyndrome AT merihreisaras arareandcomplexcauseofimpotencepoemssyndrome AT pınartıglıoglu arareandcomplexcauseofimpotencepoemssyndrome AT mesuttıglıoglu arareandcomplexcauseofimpotencepoemssyndrome AT fatmayılmaz arareandcomplexcauseofimpotencepoemssyndrome AT senemmaral arareandcomplexcauseofimpotencepoemssyndrome AT mustafaonder arareandcomplexcauseofimpotencepoemssyndrome AT bugrasaglam rareandcomplexcauseofimpotencepoemssyndrome AT muratalbayrak rareandcomplexcauseofimpotencepoemssyndrome AT hacerbernaafacanozturk rareandcomplexcauseofimpotencepoemssyndrome AT merihreisaras rareandcomplexcauseofimpotencepoemssyndrome AT pınartıglıoglu rareandcomplexcauseofimpotencepoemssyndrome AT mesuttıglıoglu rareandcomplexcauseofimpotencepoemssyndrome AT fatmayılmaz rareandcomplexcauseofimpotencepoemssyndrome AT senemmaral rareandcomplexcauseofimpotencepoemssyndrome AT mustafaonder rareandcomplexcauseofimpotencepoemssyndrome
_version_	1718440602160332800

A RARE AND COMPLEX CAUSE OF IMPOTENCE POEMS SYNDROME

Ejemplares similares