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Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia
BACKGROUND: The acquired inhibitors of coagulation have been observed in very rare cases of monoclonal gammopathies. We report a very rare case of anti-factor XI antibodies in patient with plasma cell leukemia (PCL). CASE PRESENTATION: This is a 59-year-old male patient without pathological history,...
Autores principales: | , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6086047/ https://www.ncbi.nlm.nih.gov/pubmed/30116534 http://dx.doi.org/10.1186/s12878-018-0100-9 |
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author | Uwingabiye, Jean Zahid, Hafid El Amrani, Mohamed Labrini, Fayçal Elkhazraji, Abdelhak El Kabbaj, Driss Benyahia, Mohammed Yahyaoui, Anass Hadef, Rachid Messaoudi, Nezha |
author_facet | Uwingabiye, Jean Zahid, Hafid El Amrani, Mohamed Labrini, Fayçal Elkhazraji, Abdelhak El Kabbaj, Driss Benyahia, Mohammed Yahyaoui, Anass Hadef, Rachid Messaoudi, Nezha |
author_sort | Uwingabiye, Jean |
collection | PubMed |
description | BACKGROUND: The acquired inhibitors of coagulation have been observed in very rare cases of monoclonal gammopathies. We report a very rare case of anti-factor XI antibodies in patient with plasma cell leukemia (PCL). CASE PRESENTATION: This is a 59-year-old male patient without pathological history, admitted to the nephrology department for management of renal insufficiency and anemia syndrome. The history and physical examination revealed stigmata of hemorrhagic syndrome including hemothorax and hemoptysis. The hemostasis assessment showed an isolated prolonged activated partial thromboplastin time (APTT) with APTT ratio = 2.0.The index of circulating anticoagulant (37.2%) revealed the presence of circulating anticoagulants. The normalized dilute Russell viper venom time ratio of 0.99 has highlighted the absence of lupus anticoagulants. The coagulation factors assay objectified the decrease of the factor XI activity corrected by the addition of the control plasma confirming the presence of anti-factor XI autoantibodies. In addition, the blood count showed bicytopenia with non-regenerative normocytic normochromic anemia and thrombocytopenia. The blood smear demonstrated a plasma cell count of 49% (2842/mm3) evoking PCL. The bone marrow was invaded up to 90% by dystrophic plasma cells. The biochemical assessment suggested downstream renal and electrolyte disturbances from exuberant light chain production with abnormalities including hyperuricemia, hypercalcemia, elevated lactate dehydrogenase, non nephrotic-range proteinuria and high level of C reactive protein. The serum protein electrophoresis showed the presence of a monoclonal peak. The serum immunofixation test detects the presence of monoclonal free lambda light chains. He was treated with velcade, thalidomide and dexamethasone. The patient died after 2 weeks despite treatment. CONCLUSION: Both PCL and anti-factor XI inhibitors are two very rare entities. To the best of our knowledge, this is the first reported case of a factor XI inhibitor arising in the setting of PCL. Factor inhibitors should be suspected in patients whose monoclonal gammopathies are accompanied by bleeding manifestations. |
format | Online Article Text |
id | pubmed-6086047 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2018 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-60860472018-08-16 Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia Uwingabiye, Jean Zahid, Hafid El Amrani, Mohamed Labrini, Fayçal Elkhazraji, Abdelhak El Kabbaj, Driss Benyahia, Mohammed Yahyaoui, Anass Hadef, Rachid Messaoudi, Nezha BMC Hematol Case Report BACKGROUND: The acquired inhibitors of coagulation have been observed in very rare cases of monoclonal gammopathies. We report a very rare case of anti-factor XI antibodies in patient with plasma cell leukemia (PCL). CASE PRESENTATION: This is a 59-year-old male patient without pathological history, admitted to the nephrology department for management of renal insufficiency and anemia syndrome. The history and physical examination revealed stigmata of hemorrhagic syndrome including hemothorax and hemoptysis. The hemostasis assessment showed an isolated prolonged activated partial thromboplastin time (APTT) with APTT ratio = 2.0.The index of circulating anticoagulant (37.2%) revealed the presence of circulating anticoagulants. The normalized dilute Russell viper venom time ratio of 0.99 has highlighted the absence of lupus anticoagulants. The coagulation factors assay objectified the decrease of the factor XI activity corrected by the addition of the control plasma confirming the presence of anti-factor XI autoantibodies. In addition, the blood count showed bicytopenia with non-regenerative normocytic normochromic anemia and thrombocytopenia. The blood smear demonstrated a plasma cell count of 49% (2842/mm3) evoking PCL. The bone marrow was invaded up to 90% by dystrophic plasma cells. The biochemical assessment suggested downstream renal and electrolyte disturbances from exuberant light chain production with abnormalities including hyperuricemia, hypercalcemia, elevated lactate dehydrogenase, non nephrotic-range proteinuria and high level of C reactive protein. The serum protein electrophoresis showed the presence of a monoclonal peak. The serum immunofixation test detects the presence of monoclonal free lambda light chains. He was treated with velcade, thalidomide and dexamethasone. The patient died after 2 weeks despite treatment. CONCLUSION: Both PCL and anti-factor XI inhibitors are two very rare entities. To the best of our knowledge, this is the first reported case of a factor XI inhibitor arising in the setting of PCL. Factor inhibitors should be suspected in patients whose monoclonal gammopathies are accompanied by bleeding manifestations. BioMed Central 2018-08-10 /pmc/articles/PMC6086047/ /pubmed/30116534 http://dx.doi.org/10.1186/s12878-018-0100-9 Text en © The Author(s). 2018 Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Uwingabiye, Jean Zahid, Hafid El Amrani, Mohamed Labrini, Fayçal Elkhazraji, Abdelhak El Kabbaj, Driss Benyahia, Mohammed Yahyaoui, Anass Hadef, Rachid Messaoudi, Nezha Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia |
title | Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia |
title_full | Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia |
title_fullStr | Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia |
title_full_unstemmed | Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia |
title_short | Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia |
title_sort | rare and unusual case of anti-factor xi antibodies in patient with plasma cell leukemia |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6086047/ https://www.ncbi.nlm.nih.gov/pubmed/30116534 http://dx.doi.org/10.1186/s12878-018-0100-9 |
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