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Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia

Overview of attention for article published in BMC Hematology, August 2018
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Title
Rare and unusual case of anti-factor XI antibodies in patient with plasma cell leukemia
Published in
BMC Hematology, August 2018
DOI 10.1186/s12878-018-0100-9
Pubmed ID
Authors

Jean Uwingabiye, Hafid Zahid, Mohamed El Amrani, Fayçal Labrini, Abdelhak Elkhazraji, Driss El Kabbaj, Mohammed Benyahia, Anass Yahyaoui, Rachid Hadef, Nezha Messaoudi

Abstract

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). 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. 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.

Twitter Demographics

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Mendeley readers

The data shown below were compiled from readership statistics for 8 Mendeley readers of this research output. Click here to see the associated Mendeley record.

Geographical breakdown

Country Count As %
Unknown 8 100%

Demographic breakdown

Readers by professional status Count As %
Researcher 2 25%
Unspecified 2 25%
Professor 1 13%
Student > Bachelor 1 13%
Student > Ph. D. Student 1 13%
Other 1 13%
Readers by discipline Count As %
Medicine and Dentistry 3 38%
Business, Management and Accounting 2 25%
Unspecified 2 25%
Immunology and Microbiology 1 13%

Attention Score in Context

This research output has an Altmetric Attention Score of 1. This is our high-level measure of the quality and quantity of online attention that it has received. This Attention Score, as well as the ranking and number of research outputs shown below, was calculated when the research output was last mentioned on 18 August 2018.
All research outputs
#11,870,802
of 13,385,815 outputs
Outputs from BMC Hematology
#58
of 76 outputs
Outputs of similar age
#232,392
of 269,384 outputs
Outputs of similar age from BMC Hematology
#2
of 2 outputs
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