Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis
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Publication Date: | 2022 |
Other Authors: | , , , |
Format: | Article |
Language: | por |
Source: | Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
Download full: | https://doi.org/10.24950/rspmi.545 |
Summary: | Sarcoidosis is a multisystemic granulomatous disorder of unknown aetiology. Although the lungs are the primary site of involvement, the disease can affect virtually any organ, leading to a wide variety of clinical manifestations. Haematological abnormalities are uncommon, and most frequently include lymphopenia and anaemia. Thrombocytopenia, however, is rare, and three distinct pathophysiological mechanisms have been described: immune platelet destruction, splenomegaly with hypersplenism, and bone marrow infiltration. We report a case of sarcoidosis presenting with pulmonary and cutaneous involvement, and severe acute thrombocytopenia. Bone marrow aspirate was consistent with an immune process, assumed as the primary cause for the cytopenia, but bone marrow biopsy also showed multiple noncaseating granulomas, and a CT scan revealed massive splenomegaly. There are no guidelines for the management of thrombocytopenia in sarcoidosis. In our patient, treatment with intravenous immune globulin and corticosteroids restored normal platelet count. |
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Immune Thrombocytopenia: A Rare Haematological Manifestation of SarcoidosisTrombocitopenia Imune: Uma Manifestação Hematológica Rara de SarcoidosePúrpura Trombocitopênica Idiopática/etiologiaSarcoidose/complicaçõesTrombocitopenia/etiologiaPurpura, Thrombocytopenic, Idiopathic/etiologyThrombocytopenia/etiologySarcoidosis/complicationsSarcoidosis is a multisystemic granulomatous disorder of unknown aetiology. Although the lungs are the primary site of involvement, the disease can affect virtually any organ, leading to a wide variety of clinical manifestations. Haematological abnormalities are uncommon, and most frequently include lymphopenia and anaemia. Thrombocytopenia, however, is rare, and three distinct pathophysiological mechanisms have been described: immune platelet destruction, splenomegaly with hypersplenism, and bone marrow infiltration. We report a case of sarcoidosis presenting with pulmonary and cutaneous involvement, and severe acute thrombocytopenia. Bone marrow aspirate was consistent with an immune process, assumed as the primary cause for the cytopenia, but bone marrow biopsy also showed multiple noncaseating granulomas, and a CT scan revealed massive splenomegaly. There are no guidelines for the management of thrombocytopenia in sarcoidosis. In our patient, treatment with intravenous immune globulin and corticosteroids restored normal platelet count.A sarcoidose é uma doença granulomatosa multissistémica de etiologia desconhecida. Os pulmões representam o local de envolvimento primário; contudo, esta patologia pode afetar virtualmente qualquer órgão, resultando numa grande variedade de manifestações clínicas. As anormalidades hematológicas são incomuns, e mais frequentemente incluem a linfopenia e anemia. A trombocitopenia, por outro lado, é rara, e três mecanismos fisiopatológicos distintos foram descritos: destruição imune das plaquetas, esplenomegalia com hiperesplenismo, e infiltração da medula óssea. Apresentamos um caso de sarcoidose com envolvimento pulmonar e cutâneo, complicado com trombocitopenia aguda grave. O mielograma foi compatível com um processo imune, que foi assumido como a causa primária para a citopenia; adicionalmente, a biópsia óssea mostrou também múltiplos granulomas não caseosos, enquanto uma tomografia computorizada revelou esplenomegalia maciça. Não existem recomendações para a abordagem da trombocitopenia na sarcoidose. No nosso doente, o tratamento com imunoglobulina endovenosa e corticoides resultou na normalização da contagem de plaquetas.Sociedade Portuguesa de Medicina Interna2022-12-19info:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://doi.org/10.24950/rspmi.545https://doi.org/10.24950/rspmi.545Internal Medicine; Vol. 29 No. 4 (2022): Outubro/Dezembro; 278-282Medicina Interna; Vol. 29 N.º 4 (2022): Outubro/Dezembro; 278-2822183-99800872-671Xreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAPporhttps://revista.spmi.pt/index.php/rpmi/article/view/545https://revista.spmi.pt/index.php/rpmi/article/view/545/924Direitos de Autor (c) 2022 Medicina Internainfo:eu-repo/semantics/openAccessMonteiro, ManuelPimentel, JoãoRodrigues, MárioCosta, AnaJesus, Margarida2023-01-07T06:10:34Zoai:oai.revista.spmi.pt:article/545Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T10:45:24.964642Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse |
dc.title.none.fl_str_mv |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis Trombocitopenia Imune: Uma Manifestação Hematológica Rara de Sarcoidose |
title |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis |
spellingShingle |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis Monteiro, Manuel Púrpura Trombocitopênica Idiopática/etiologia Sarcoidose/complicações Trombocitopenia/etiologia Purpura, Thrombocytopenic, Idiopathic/etiology Thrombocytopenia/etiology Sarcoidosis/complications |
title_short |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis |
title_full |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis |
title_fullStr |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis |
title_full_unstemmed |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis |
title_sort |
Immune Thrombocytopenia: A Rare Haematological Manifestation of Sarcoidosis |
author |
Monteiro, Manuel |
author_facet |
Monteiro, Manuel Pimentel, João Rodrigues, Mário Costa, Ana Jesus, Margarida |
author_role |
author |
author2 |
Pimentel, João Rodrigues, Mário Costa, Ana Jesus, Margarida |
author2_role |
author author author author |
dc.contributor.author.fl_str_mv |
Monteiro, Manuel Pimentel, João Rodrigues, Mário Costa, Ana Jesus, Margarida |
dc.subject.por.fl_str_mv |
Púrpura Trombocitopênica Idiopática/etiologia Sarcoidose/complicações Trombocitopenia/etiologia Purpura, Thrombocytopenic, Idiopathic/etiology Thrombocytopenia/etiology Sarcoidosis/complications |
topic |
Púrpura Trombocitopênica Idiopática/etiologia Sarcoidose/complicações Trombocitopenia/etiologia Purpura, Thrombocytopenic, Idiopathic/etiology Thrombocytopenia/etiology Sarcoidosis/complications |
description |
Sarcoidosis is a multisystemic granulomatous disorder of unknown aetiology. Although the lungs are the primary site of involvement, the disease can affect virtually any organ, leading to a wide variety of clinical manifestations. Haematological abnormalities are uncommon, and most frequently include lymphopenia and anaemia. Thrombocytopenia, however, is rare, and three distinct pathophysiological mechanisms have been described: immune platelet destruction, splenomegaly with hypersplenism, and bone marrow infiltration. We report a case of sarcoidosis presenting with pulmonary and cutaneous involvement, and severe acute thrombocytopenia. Bone marrow aspirate was consistent with an immune process, assumed as the primary cause for the cytopenia, but bone marrow biopsy also showed multiple noncaseating granulomas, and a CT scan revealed massive splenomegaly. There are no guidelines for the management of thrombocytopenia in sarcoidosis. In our patient, treatment with intravenous immune globulin and corticosteroids restored normal platelet count. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-12-19 |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://doi.org/10.24950/rspmi.545 https://doi.org/10.24950/rspmi.545 |
url |
https://doi.org/10.24950/rspmi.545 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
https://revista.spmi.pt/index.php/rpmi/article/view/545 https://revista.spmi.pt/index.php/rpmi/article/view/545/924 |
dc.rights.driver.fl_str_mv |
Direitos de Autor (c) 2022 Medicina Interna info:eu-repo/semantics/openAccess |
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Direitos de Autor (c) 2022 Medicina Interna |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Sociedade Portuguesa de Medicina Interna |
publisher.none.fl_str_mv |
Sociedade Portuguesa de Medicina Interna |
dc.source.none.fl_str_mv |
Internal Medicine; Vol. 29 No. 4 (2022): Outubro/Dezembro; 278-282 Medicina Interna; Vol. 29 N.º 4 (2022): Outubro/Dezembro; 278-282 2183-9980 0872-671X reponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia instacron:RCAAP |
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RCAAP |
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RCAAP |
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Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
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Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) |
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Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologia |
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