Blog - INTERNET MÉDICA

Síndrome POEMS Online (POEMS Syndrome)

Por Paulo Leite 18/06/2019

¨A síndrome de POEMS é uma entidade clínica única definida pela presença concomitante de doença monoclonal de plasmócitos, polineuropatia periférica e outras manifestações paraneoplásicas tais como organomegalias, endocrinopatias, alterações cutâneas, papiledema, lesões osteoescleróticas e sobrecarga de volume extravascular. Para o diagnóstico da doença não é necessária a presença de todas as manifestações relacionadas e o reconhecimento precoce desta patologia é importante para reduzir a morbidade. A síndrome de POEMS também é conhecida como mieloma osteoesclerótico, síndrome de Crow-Fukase e síndrome de Takatsuki. A causa da síndrome de Poems é desconhecida. Para o diagnóstico da doença não é necessária a presença de todas as manifestações relacionadas; por outro lado, nenhuma prova laboratorial pode ser considerada como patognomônica isoladamente. Após uma revisão sistemática das três maiores séries publicadas sobre a síndrome de Poemsreunindo um total de 226 pacientes, propuseram em 2003 a adoção de um sistema de critérios para o diagnóstico da síndrome de Poems baseado na freqüência e na relevância dos achados clínicos e laboratoriais que compõe esta patologia. O tratamento padrão da síndrome de POEMS ainda não está estabelecido e as principais opções são radioterapia, corticosteróides e agentes alquilantes, inclusive na forma de quimioterapia de altas doses com transplante autólogo de células-tronco hematopoiéticas.¨

■ An update on the diagnosis and management of the polyneuropathy of POEMS syndrome

Federica Cerri, Yuri Matteo Falzone, Nilo Riva, Angelo Quattrini

J Neurol. 2019; 266(1): 258–267

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6343023/

■ POEMS syndrome: clinical update

Rachel Brown, Lionel Ginsberg

J Neurol. 2019; 266(1): 268–277

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6342878/

■ Síndrome de POEMS incompleto asociado a enfermedad de Castleman multicéntrica y amiloidosis

González de la Aleja, P et al

Anales Sis San Navarra 2017, vol 40 (3)

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1137-66272017000300475

■ POEMS syndrome and idiopathic portal hypertension: a possible association

Campos, Sara, Agostinho, Cláudia and Cipriano, Maria Augusta

Rev Esp Enferm Dig 2017, vol 109 (5): 393

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S1130-01082017000500026&lng=es&nrm=iso

■ Endocrine manifestations in POEMS syndrome: a case report and literature review

Hui Li, Yumeng Huang, Yan Li, Baozhong Zheng, Jingqiu Cui, Ming Liu

BMC Endocr Disord. 2019; 19: 33

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6431048/

■ Positron emission tomography-computed tomography in a case of POEMS syndrome

Jose Rafael Infante, Lucía García, Juan Ignacio Rayo et al

Indian J Nucl Med. 2019 Jan-Mar; 34(1): 66–6

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6352633/

■ Frequent central nervous system, pachymeningeal and plexus MRI changes in POEMS syndrome

Oliver J. Ziff, Chandrashekar Hoskote, Stephen Keddie et al

J Neurol. 2019; 266(5): 1067–1072

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6469836

■ High-dose therapy and autologous stem cell transplantation in patients with POEMS syndrome: a retrospective study of the plasma cell disorder sub-committee of the chronic malignancy working party of the European Society for Blood & Marrow Transplantation

Gordon Cook, Simona Iacobelli, Anja van Biezen et al

Haematologica. 2017 Jan; 102(1): 160–16

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5210246/

■ Raised VEGF: high sensitivity and specificity in the diagnosis of POEMS syndrome

Morgane Pihan, Stephen Keddie, Shirley D’Sa, Andrew J. Church, Kwee L. Yong, Mary M. Reilly, Michael P. Lunn

Neurol Neuroimmunol Neuroinflamm. 2018 Sep; 5(5): e486

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6117193/

■ POEMS syndrome: an update

Andrea Nozza

Mediterr J Hematol Infect Dis. 2017; 9(1): e2017051

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5584767/

■ Nerve pathologic features differentiate POEMS syndrome from CIDP

Ezequiel A. Piccione, Janean Engelstad, Peter J. Dyck et al

Acta Neuropathol Commun. 2016; 4: 11

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5088652/

■ Pulmonary morbidity improves after autologous stem cell transplantation in POEMS syndrome

Satish Chandrashekaran, Angela Dispenzieri, Stephen S. Cha, Cassie C. Kennedy

Respir Med. 2015 Jan; 109(1): 122–130

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4758677/

■ An occult malignancyt behing a demyelinatingj disease: POEMS syndrome

Saberio Lo Presti, Prashanth Kanagarajah, Daniela Pirela, Diana Morlote, Mike Cusnir

J Investig Med High Impact Case Rep. 2016 Oct-Dec; 4(4): 2324709616673389

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5072185/

■ Longitudinal bioimpedance assessments to evaluate hydration in POEMS syndrome

Amara Callistus Nwosu, Lauren Morris, Catriona Mayland et al

BMJ Support Palliat Care. 2016 Sep; 6(3): 369–372

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5013159/

■ POEMS syndrome: complex factors contributing to a delayed diagnosis

Rebecca Caroline Robey, Craig Campus, Beverly Ringuette, Mark Shumate

BMJ Case Rep. 2015; 2015: bcr2015213123

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4680602/

■ Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS syndrome): a paraneoplastic syndrome

Sunil Kumar, Shruti Sharma

Oxf Med Case Reports. 2015 Mar; 2015(3): 237–240

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4664845

■ POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link

Dorothee Heck, Miriam Mergen, Athina Ganner et al

BMC Neurol. 2014; 14: 210.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4226878/

■ Outcomes of patients with POEMS syndrome treated initially with radiation

Michael S. Humeniuk, Morie A. Gertz, Martha Q. Lacy, et al

Blood. 2013 Jul 4; 122(1): 68–73

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4067496/

■ POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, multiple myeloma and skin changes) with cranial vault plasmocytoma and the role of surgery in its management: a case report

Julio Plata Bello, Victor Garcia-Marin

J Med Case Rep. 2013; 7: 24

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4016595/

■ Long-term therapy with lenalidomide in a patient with POEMS syndrome

Benjamin F. Chu, Arwa Shana’ah, Craig C. Hofmeister et al

Eur J Case Rep Intern Med. 2014; 1: 2014_000093

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4326104/

■ Alcohol misuse link to POEMS syndrome in a patient

John Neary, Susan E. Goodwin, Lawrence B. Cohen, Manuela G. Neuman

Cancers (Basel) 2017 Oct; 9(10): 129

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5664068/

■ How I treat POEMS syndrome

Angela Dispenzieri

Blood. 2012 Jun 14; 119(24): 5650–565

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3425020/

■ Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findigns in 87 patients

Linda N. Dao, Curtis A. Hanson, Angela Dispenzieri, et al

Blood. 2011 Jun 16; 117(24): 6438–644

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3123015/

■ Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findigns in 87 patients

Linda N. Dao, Curtis A. Hanson, Angela Dispenzieri, et al

■ Papiledema associado a síndrome de POEMS

Barnés, C et al

Arch Soc Esp oftalmol 2008, vol 83 (12): 709-711

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0365-66912008001200005&lng=es&nrm=iso

■ Síndrome de POEMS (mieloma osteoesclerótico).

Pinto Neto, Jorge V.

Rev. Bras. Hematol. Hemoter., Mar 2007, vol.29, no.1, p.98-10

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-84842007000100018&lng=en&nrm=iso

■Dr Paulo Fernando Leite

Cardiologia/Prevenção Cardiovascular

Estratificação de Risco Cardiovascular

Novo endereço:

Consultório: Rua Padre Rolim 815/sala 815

Tel: 33245518 (Consulta Particular/Unimed)

– Centro Médico Unimed BH/Contagem/Pedro I

Belo Horizonte/MG/Brasil

CRMMG: 7026

Email: pfleite1873@gmail.com

Síndrome de Brooke-Splieger Online (Brooke-Splieger Syndrome)

Por Paulo Leite 18/06/2019

¨A síndrome de Brooke-Spiegler é uma doença autossômica dominante, rara, caracterizada pelo aparecimento de neoplasias de anexos cutâneos, habitualmente tricoepiteliomas e cilindromas. Ocorre, em geral, na segunda e terceira décadas de vida e tem prevalência maior em mulheres. A histopatologia revela uma ampla gama de tumores, com diferenciação écrina, apócrina, folicular e sebácea. É causada por mutação no gene CYLD, licalizado no cromossomo 16q12-q13. O tratamento pode ser feito por excisão cirúrgica, laser, crioterapia, eletrofulguração e dermabrasão. Em razão do risco de malignidade, há necessidade de um bom acompanhamento clínico e aconselhamento genético.¨

■ Espiradenoma écrino gigante associado à síndrome de Brooke-Splieger

T M Terra, F T G Miranda, L F F F Junior

Surgical and Cosmetic Dermatology 2019, vol 11 (1)

http://www.surgicalcosmetic.org.br/detalhe-artigo/705/Espiradenoma-ecrino-gigante-associado-a-sindrome-de-Brooke-Splieger

■ A rare case of Brooke-Spiegler syndrome: integrated surgical treatment of multiple giant eccrine spiradenomas of the head and neck in a young girl

Portincasa, L. Cecchino, E.M.C. Trecca et al

Int J Surg Case Rep. 2018; 51: 277–281.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6146587/

■ Turban tumor: a classical presentation of Brooke-Spiegler syndrome

Suman Patra, G. Sethuraman, Ranjit Kumar

Indian Dermatol Online J. 2018 Jul-Aug; 9(4): 284–285

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6042198/

■ Brooke-Spiegler syndrome

Waseem Mohiuddin, Jake Laun, Wayne Cruse

Eplasty. 2018; 18: ic14

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6062842/

■ Brooke-Spiegler syndrome and phenotypic variants: an update

Dmitry V. Kazakov

Head Neck Pathol. 2016 Jun; 10(2): 125–130

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4838966/

■ Brooke-Spiegler syndrome – an underrecognized cause of multiple familial scalp tumors: report of a new germline mutation

André Castro Pinho, Miguel José Pinto Gouveia, Ana Rita Portelinha Gameiro et al

J Dermatol Case Rep. 2015 Sep 30; 9(3): 67–70

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4619162/

■ Syndrome In Question – Brooke-Splieger Syndrome

Lavorato, Fernanda Guedes et al

An. bras. dermatol., Jan 2014, vol.89, no.1, p.175-176

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962014000100175&lng=en&nrm=iso

■ Brooke-Spiegler syndrome: a rare entity

M Rathi, S Awasthi, S K Budania et al

Case Rep Pathol 2014; 2014: 231895

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3920916/

■ Espiradenoma ecrino múltiple

J García-Molina, J González-Martin, E Ruiz-Bravo et al

Revista Espãnola de Cirurgia Oral y Maxilofacial 2014, vol 36 (4)

https://www.elsevier.es/es-revista-revista-espanola-cirugia-oral-maxilofacial-300-articulo-espiradenoma-ecrino-multiple-S1130055814000835

■ Tricoepitelioma múltiple

C G Gómez, R M Wagner, F J L Mendoza

Cir Plást Iberolatinoam 2012, vol 38 (2)

http://scielo.isciii.es/scielo.php?script=sci_arttext&pid=S0376-78922012000200009

■ Brooker-Spiegler syndrome: a rare enti

Kajal Manchanda, Manish Bansal, Aakash Amar Bhayana, SS Pandey

Int J Trichology. 2012 Jan-Mar; 4(1): 29–31

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3358935/

■ Brooke-Spiegler syndrome with multiple scalp cylindromas and bilateral parotid gland adenomas

Peter Kalina, Rokea el-Azhary

Case Rep Radiol. 2012; 2012: 249583

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3350190/

■ Você conhece esta syndrome? Síndrome de Brooke-Splieger

J N T Parente, A P M Schettini, C Massone et al

An Bras Dermatol 2009, vol 84 (5)

http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0365-05962009000500019

■ Cylindroma transforming into basal cell carcinoma in a patient with Brooke-Spiegler syndrome

Justyna Sicinska, Adriana Rakowska, Joanna Czuwara-Ladykowska et al

J Dermatol Case Rep. 2007 Dec 29; 1(1): 4–9

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3157764/