Acquired hypofibrinogenemia in a patient with multiple myeloma.
Coagulopathy
Fibrinogen
Multiple myeloma
Journal
International journal of hematology
ISSN: 1865-3774
Titre abrégé: Int J Hematol
Pays: Japan
ID NLM: 9111627
Informations de publication
Date de publication:
Sep 2021
Sep 2021
Historique:
received:
17
12
2020
accepted:
23
03
2021
revised:
18
03
2021
pubmed:
1
6
2021
medline:
1
9
2021
entrez:
31
5
2021
Statut:
ppublish
Résumé
We report a case of acquired hypofibrinogenemia with multiple myeloma presenting λ-type IgG monoclonal protein. The patient had anemia and renal deficiency, and also developed bleeding tendency due to severe coagulopathy. Her fibrinogen level was under the detectable limits in a functional assay. Enzyme-linked immunosorbent assay (ELISA) and immunoblotting analysis results were consistent with functional assay results, and deficiency patterns observed in cross-mixing tests for PT and aPTT confirmed the diagnosis of hypofibrinogenemia. To determine the cause of hypofibrinogenemia, we purified the patient's immunoglobulin via protein A agarose, and confirmed that fibrinogen was included in the bound fraction, strongly indicating paraprotein interference with fibrinogen. As accelerated removal of fibrinogen was indicated, we incubated the patient's plasma up to 48 h, but did not observe significant loss of fibrinogen. In sharp contrast, fibrinogen returned to below the detection level 12 h after infusion of fresh frozen plasma. These findings support leukocyte-mediated fibrinogen removal, rather than paraprotein-triggered fibrinogen instability. Surprisingly, the patient's paraprotein was IgG2, but we speculate the amount of paraprotein (IgG 5346 mg/dL) compensated for lower affinity to Fcγ receptors.
Identifiants
pubmed: 34057670
doi: 10.1007/s12185-021-03143-5
pii: 10.1007/s12185-021-03143-5
doi:
Substances chimiques
Immunoglobulin G
0
Immunoglobulin lambda-Chains
0
Fibrinogen
9001-32-5
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
395-400Informations de copyright
© 2021. Japanese Society of Hematology.
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