Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Institucional da UFRGS |
Texto Completo: | http://hdl.handle.net/10183/204381 |
Resumo: | Background: Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a wide variety of clinical presentations and outcomes. Human herpesvirus-8 (HHV-8) related CD corresponds to the most common subtype of the multicentric Castleman disease (MCD). However, if HHV-8 is negative, POEMS (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) associated with MCD or idiopathic MCD are the cause in a subgroup of patients. Considering the rarity of POEMS and MCD association, we herein describe a patient with a typical presentation based on clinical, laboratory and tissue biopsy data. Case Presentation: We report a diabetic patient who presented with asthenia, edema, skin lesions manifested by scarring in chiropodactyls, multiple lymph node enlargement in the neck, armpits and inguinal areas, splenomegaly, severe anemia, thrombocytopenia, and mixed polyneuropathy. Hematuria and proteinuria were detected. The patient developed progressive renal failure requiring dialysis. Renal biopsy showed mesangial expansion with mesangial hypercellularity, and lymphoplasmacytoid cells focally distributed in tubules and interstitium, which were compatible with acute tubulointerstitial nephritis. In immunofluorescence, no deposits of IgG, IgA, IgM, C1q, C3 or fibrinogen were found, and kappa and lambda were also negative. Lymph node biopsy revealed lymphoid tissue with follicular hyperplasia, sinusoidal and medullary infiltration of plasma cells. Immunohistochemistry confirmed positivity for B lymphocytes, T lymphocytes, and plasma cells in sub-capsular and para-follicular areas. The patient was diagnosed as POEMS-associated MCD variant, and chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was started. The patient did not recover renal function and remained dialysis-dependent. Conclusions: To date, the renal involvement in MCD and POEMS syndrome seems to be uncommon as reported in few case series. Its pathophysiology is not well understood. In the spectrum of MCD, decreased renal function may have impact in patient survival. Early diagnosis and treatment are needed to control the systemic manifestations, and most importantly to avoid chronic organ damage. |
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Ribeiro, Caroline KullmannBresciani, FernandaSilveira, Samile Sallaberry EcheverriaSchaefer, Pedro GuilhermeBarros, Elvino José GuardãoVeronese, Francisco José Veríssimo2020-01-16T04:10:29Z20192251-8363http://hdl.handle.net/10183/204381001107410Background: Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a wide variety of clinical presentations and outcomes. Human herpesvirus-8 (HHV-8) related CD corresponds to the most common subtype of the multicentric Castleman disease (MCD). However, if HHV-8 is negative, POEMS (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) associated with MCD or idiopathic MCD are the cause in a subgroup of patients. Considering the rarity of POEMS and MCD association, we herein describe a patient with a typical presentation based on clinical, laboratory and tissue biopsy data. Case Presentation: We report a diabetic patient who presented with asthenia, edema, skin lesions manifested by scarring in chiropodactyls, multiple lymph node enlargement in the neck, armpits and inguinal areas, splenomegaly, severe anemia, thrombocytopenia, and mixed polyneuropathy. Hematuria and proteinuria were detected. The patient developed progressive renal failure requiring dialysis. Renal biopsy showed mesangial expansion with mesangial hypercellularity, and lymphoplasmacytoid cells focally distributed in tubules and interstitium, which were compatible with acute tubulointerstitial nephritis. In immunofluorescence, no deposits of IgG, IgA, IgM, C1q, C3 or fibrinogen were found, and kappa and lambda were also negative. Lymph node biopsy revealed lymphoid tissue with follicular hyperplasia, sinusoidal and medullary infiltration of plasma cells. Immunohistochemistry confirmed positivity for B lymphocytes, T lymphocytes, and plasma cells in sub-capsular and para-follicular areas. The patient was diagnosed as POEMS-associated MCD variant, and chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was started. The patient did not recover renal function and remained dialysis-dependent. Conclusions: To date, the renal involvement in MCD and POEMS syndrome seems to be uncommon as reported in few case series. Its pathophysiology is not well understood. In the spectrum of MCD, decreased renal function may have impact in patient survival. Early diagnosis and treatment are needed to control the systemic manifestations, and most importantly to avoid chronic organ damage.application/pdfengJournal of nephropathology. Isfahan. Vol. 8, no. 2 (April 2019), e21, [6] p.Hiperplasia do linfonodo giganteSíndrome POEMSParaproteinemiasInsuficiência renalEstudos de casosMulticentric Castleman diseasePOEMS syndromeMonoclonal gammopathyEndocrinopathyRenal failureClinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challengeEstrangeiroinfo:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/openAccessreponame:Repositório Institucional da UFRGSinstname:Universidade Federal do Rio Grande do Sul (UFRGS)instacron:UFRGSTEXT001107410.pdf.txt001107410.pdf.txtExtracted Texttext/plain28402http://www.lume.ufrgs.br/bitstream/10183/204381/2/001107410.pdf.txt97ec195e3974376f54c46a1e5cfc67b3MD52ORIGINAL001107410.pdfTexto completo (inglês)application/pdf1144987http://www.lume.ufrgs.br/bitstream/10183/204381/1/001107410.pdfdac7a151134afb477e75bd83269e6245MD5110183/2043812020-01-17 05:10:54.804013oai:www.lume.ufrgs.br:10183/204381Repositório de PublicaçõesPUBhttps://lume.ufrgs.br/oai/requestopendoar:2020-01-17T07:10:54Repositório Institucional da UFRGS - Universidade Federal do Rio Grande do Sul (UFRGS)false |
dc.title.pt_BR.fl_str_mv |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
title |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
spellingShingle |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge Ribeiro, Caroline Kullmann Hiperplasia do linfonodo gigante Síndrome POEMS Paraproteinemias Insuficiência renal Estudos de casos Multicentric Castleman disease POEMS syndrome Monoclonal gammopathy Endocrinopathy Renal failure |
title_short |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
title_full |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
title_fullStr |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
title_full_unstemmed |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
title_sort |
Clinical spectrum of POEMS-associated multicentric Castleman disease with renal involvement : a diagnostic challenge |
author |
Ribeiro, Caroline Kullmann |
author_facet |
Ribeiro, Caroline Kullmann Bresciani, Fernanda Silveira, Samile Sallaberry Echeverria Schaefer, Pedro Guilherme Barros, Elvino José Guardão Veronese, Francisco José Veríssimo |
author_role |
author |
author2 |
Bresciani, Fernanda Silveira, Samile Sallaberry Echeverria Schaefer, Pedro Guilherme Barros, Elvino José Guardão Veronese, Francisco José Veríssimo |
author2_role |
author author author author author |
dc.contributor.author.fl_str_mv |
Ribeiro, Caroline Kullmann Bresciani, Fernanda Silveira, Samile Sallaberry Echeverria Schaefer, Pedro Guilherme Barros, Elvino José Guardão Veronese, Francisco José Veríssimo |
dc.subject.por.fl_str_mv |
Hiperplasia do linfonodo gigante Síndrome POEMS Paraproteinemias Insuficiência renal Estudos de casos |
topic |
Hiperplasia do linfonodo gigante Síndrome POEMS Paraproteinemias Insuficiência renal Estudos de casos Multicentric Castleman disease POEMS syndrome Monoclonal gammopathy Endocrinopathy Renal failure |
dc.subject.eng.fl_str_mv |
Multicentric Castleman disease POEMS syndrome Monoclonal gammopathy Endocrinopathy Renal failure |
description |
Background: Castleman disease (CD) is a rare and heterogeneous lymphoproliferative disorder with a wide variety of clinical presentations and outcomes. Human herpesvirus-8 (HHV-8) related CD corresponds to the most common subtype of the multicentric Castleman disease (MCD). However, if HHV-8 is negative, POEMS (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) associated with MCD or idiopathic MCD are the cause in a subgroup of patients. Considering the rarity of POEMS and MCD association, we herein describe a patient with a typical presentation based on clinical, laboratory and tissue biopsy data. Case Presentation: We report a diabetic patient who presented with asthenia, edema, skin lesions manifested by scarring in chiropodactyls, multiple lymph node enlargement in the neck, armpits and inguinal areas, splenomegaly, severe anemia, thrombocytopenia, and mixed polyneuropathy. Hematuria and proteinuria were detected. The patient developed progressive renal failure requiring dialysis. Renal biopsy showed mesangial expansion with mesangial hypercellularity, and lymphoplasmacytoid cells focally distributed in tubules and interstitium, which were compatible with acute tubulointerstitial nephritis. In immunofluorescence, no deposits of IgG, IgA, IgM, C1q, C3 or fibrinogen were found, and kappa and lambda were also negative. Lymph node biopsy revealed lymphoid tissue with follicular hyperplasia, sinusoidal and medullary infiltration of plasma cells. Immunohistochemistry confirmed positivity for B lymphocytes, T lymphocytes, and plasma cells in sub-capsular and para-follicular areas. The patient was diagnosed as POEMS-associated MCD variant, and chemotherapy with cyclophosphamide, doxorubicin, vincristine, and prednisone was started. The patient did not recover renal function and remained dialysis-dependent. Conclusions: To date, the renal involvement in MCD and POEMS syndrome seems to be uncommon as reported in few case series. Its pathophysiology is not well understood. In the spectrum of MCD, decreased renal function may have impact in patient survival. Early diagnosis and treatment are needed to control the systemic manifestations, and most importantly to avoid chronic organ damage. |
publishDate |
2019 |
dc.date.issued.fl_str_mv |
2019 |
dc.date.accessioned.fl_str_mv |
2020-01-16T04:10:29Z |
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Journal of nephropathology. Isfahan. Vol. 8, no. 2 (April 2019), e21, [6] p. |
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