A case of a young boy with hyper-fibrinolysis associated with natural fibrin precipitates suspected to have occurred through a novel coagulation and fibrinolysis mechanism
© 2022. Japanese Society of Hematology..
An 8-year-old Japanese boy with no underlying disease presented with severe intramuscular hematoma of the hip, and was admitted for a disseminated intravascular coagulation-like state with fibrinolytic dominance. Laboratory examinations revealed severe hyper-fibrinolysis with elevated markers, markedly shortened euglobulin clot lysis time, mildly decreased prothrombin, and severely decreased fibrinogen and factor XIII. Natural fibrin precipitates rapidly appeared in citrate-treated, ethylene-diamine-tetra-acetic-treated, and heparin-treated samples, but not in argatroban-treated samples, indicating that the mechanism of thrombin and fibrin formation was Ca2+-independent. The precipitates were physically similar to thrombin-triggered plasma fibrin. A global coagulation assay revealed that thrombin generation potentials were normal throughout the clinical course, whereas plasmin generation was already detected before initiation of fibrin formation in the acute phase. This phenomenon disappeared with time. Changes in coagulation abnormalities and nature of fibrinolysis paralleled those seen in specific markers for streptococcal infections. Streptokinase was possibly involved in this disease, as SDS-polyacrylamide gel electrophoresis revealed that plasmin derived from streptokinase-plasminogen complex proteolyzed the prothrombin to approximately 35-kDa α-thrombin consisting of the A-B single chain, which was identified by NH2-terminal sequence analysis. The involvement of streptokinase-plasminogen-prothrombin caused by streptococcal infection may be one mechanism that produces marked hyper-fibrinolysis associated with natural fibrin precipitates.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2022 |
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| Erschienen: |
2022 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:116 |
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| Enthalten in: |
International journal of hematology - 116(2022), 2 vom: 13. Aug., Seite 276-287 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Nishiyama, Atsuko [VerfasserIn] |
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| Links: |
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| Themen: |
9001-26-7 |
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| Anmerkungen: |
Date Completed 27.07.2022 Date Revised 27.07.2022 published: Print-Electronic Citation Status MEDLINE |
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| doi: |
10.1007/s12185-022-03339-3 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM339461128 |
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| 100 | 1 | |a Nishiyama, Atsuko |e verfasserin |4 aut | |
| 245 | 1 | 2 | |a A case of a young boy with hyper-fibrinolysis associated with natural fibrin precipitates suspected to have occurred through a novel coagulation and fibrinolysis mechanism |
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| 520 | |a An 8-year-old Japanese boy with no underlying disease presented with severe intramuscular hematoma of the hip, and was admitted for a disseminated intravascular coagulation-like state with fibrinolytic dominance. Laboratory examinations revealed severe hyper-fibrinolysis with elevated markers, markedly shortened euglobulin clot lysis time, mildly decreased prothrombin, and severely decreased fibrinogen and factor XIII. Natural fibrin precipitates rapidly appeared in citrate-treated, ethylene-diamine-tetra-acetic-treated, and heparin-treated samples, but not in argatroban-treated samples, indicating that the mechanism of thrombin and fibrin formation was Ca2+-independent. The precipitates were physically similar to thrombin-triggered plasma fibrin. A global coagulation assay revealed that thrombin generation potentials were normal throughout the clinical course, whereas plasmin generation was already detected before initiation of fibrin formation in the acute phase. This phenomenon disappeared with time. Changes in coagulation abnormalities and nature of fibrinolysis paralleled those seen in specific markers for streptococcal infections. Streptokinase was possibly involved in this disease, as SDS-polyacrylamide gel electrophoresis revealed that plasmin derived from streptokinase-plasminogen complex proteolyzed the prothrombin to approximately 35-kDa α-thrombin consisting of the A-B single chain, which was identified by NH2-terminal sequence analysis. The involvement of streptokinase-plasminogen-prothrombin caused by streptococcal infection may be one mechanism that produces marked hyper-fibrinolysis associated with natural fibrin precipitates | ||
| 650 | 4 | |a Case Reports | |
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Fibrin | |
| 650 | 4 | |a Fibrinolysis | |
| 650 | 4 | |a Plasminemia | |
| 650 | 4 | |a Streptococcal infection | |
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| 700 | 1 | |a Ogiwara, Kenichi |e verfasserin |4 aut | |
| 700 | 1 | |a Nakajima, Yuto |e verfasserin |4 aut | |
| 700 | 1 | |a Furukawa, Shoko |e verfasserin |4 aut | |
| 700 | 1 | |a Matsumoto, Tomoko |e verfasserin |4 aut | |
| 700 | 1 | |a Takeda, Hiroki |e verfasserin |4 aut | |
| 700 | 1 | |a Nogami, Keiji |e verfasserin |4 aut | |
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