Monitoring coagulation-fibrinolysis activation prompted timely diagnosis of hemophagocytic lymphohistiocytosis-related disseminated intravascular coagulation

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Abstract

Background

Timely diagnosis of disseminated intravascular coagulation (DIC) in hemophagocytic lymphohistiocytosis (HLH) patients is crucial but challenging, as HLH interferes with the results of the laboratory tests included in the DIC score system.

Case presentation

Here, we reported a case of lymphoma-associated HLH, in which coagulation-fibrinolysis activation /inhibition markers (TAT, tPAIC, and PIC), prompted timely diagnosis of early stage DIC (initial phase of microvascular thrombosis, yet non-overt), prior to the development of organ failures and/or bleedings.

Conclusions

This report highlights the importance of the implementation of new biomarkers (such as TAT, tPAIC, and PIC), into the diagnostic work-up for coagulation disorders. These biomarkers are directly suggestive of microthrombus formation, therefore they can be of paramount importance in diagnosing DIC with complicated etiologies, such as hematological diseases-related DIC.

Supplementary Information

The online version contains supplementary material available at 10.1186/s12959-021-00338-y.

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Most cited references 17

Record: found
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HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

Jan-Inge Henter, Annacarin Horne, Maurizio Aricó … (2007)

In HLH-94, the first prospective international treatment study for hemophagocytic lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly, bicytopenia, hypertriglyceridemia and/or hypofibrinogenemia, and hemophagocytosis). In HLH-2004 three additional criteria are introduced; low/absent NK-cell-activity, hyperferritinemia, and high-soluble interleukin-2-receptor levels. Altogether five of these eight criteria must be fulfilled, unless family history or molecular diagnosis is consistent with HLH. HLH-2004 chemo-immunotherapy includes etoposide, dexamethasone, cyclosporine A upfront and, in selected patients, intrathecal therapy with methotrexate and corticosteroids. Subsequent hematopoietic stem cell transplantation (HSCT) is recommended for patients with familial disease or molecular diagnosis, and patients with severe and persistent, or reactivated, disease. In order to hopefully further improve diagnosis, therapy and biological understanding, participation in HLH studies is encouraged.

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Towards definition, clinical and laboratory criteria, and a scoring system for disseminated intravascular coagulation.

F Taylor, C. Toh, W K Hoots … (2001)

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Is Open Access

A clinical prediction model for cancer-associated venous thromboembolism: a development and validation study in two independent prospective cohorts

Cihan Ay, Noémie Kraaijpoel, Christoph Zielinski … (2018)

Venous thromboembolism (VTE) is a frequent complication of cancer, however the risk is highly variable among individuals depending on various factors, including types of cancer. To enable a personalized risk prediction of VTE we developed and externally validated a clinical prediction model for cancer-associated VTE. The prospective Vienna Cancer and Thrombosis Study (CATS, n=1,423) was used for model development, and the prospective Multinational cohort study to Identify Cancer patients at risk of VTE (MICA, n=832) was used for external validation. Primary outcome was objectively confirmed VTE at 6 months. The cumulative 6-month VTE risk was 5·7% in CATS (95% CI: 4·5-6·9), and 6·3% (95%CI: 4·7-8·2) in MICA. Tumor sites were categorized into low/intermediate, high, and very high VTE categories. Predictive variables were selected from a broad set of clinical and laboratory factors. The final prediction model included two variables: tumor site category (hazard ratio for “high” vs. “low/intermediate”, and “very high” versus “high” VTE-risk tumor site=1·96 (95% CI: 1·41-2·72, p=0·0001). and D-Dimer (hazard ratio per doubling=1·32, 95% CI: 1·12-1·56, p=0·001). The C-Indices of the model were 0·66 (95%: 0·63-0·67) in internal validation (CATS), and 0·68 (95%: 0·62-0·74) in external validation (MICA), respectively. The clinical prediction model was adequately calibrated in both cohorts. An externally-validated clinical prediction model incorporating only one clinical factor (tumor site category) and one biomarker (D-Dimer) predicts the risk of VTE in ambulatory patients with solid cancers. This simple model considerably improves on previous models for predicting cancer-associated VTE, and can aid physicians in selecting patients who will likely benefit from thromboprophylaxis. Austrian Science Fund, Austrian National Bank Memorial Fund, Unrestricted grants from participating hospitals

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Author and article information

Contributors

Jing Zhou:

ORCID: http://orcid.org/0000-0002-4251-9068

zhoujinghuaxi@163.com

Journal

Journal ID (nlm-ta): Thromb J

Journal ID (iso-abbrev): Thromb J

Title: Thrombosis Journal

Publisher: BioMed Central (London )

ISSN (Electronic): 1477-9560

Publication date (Electronic): 4 November 2021

Publication date PMC-release: 4 November 2021

Publication date Collection: 2021

Volume: 19

Electronic Location Identifier: 82

Affiliations

GRID grid.13291.38, ISNI 0000 0001 0807 1581, Department of Laboratory Medicine, West China Hospital, , Sichuan University, ; No. 37 Guo Xue Alley, Sichuan 610041 Chengdu, China

Author information

Jing Zhou http://orcid.org/0000-0002-4251-9068

Article

Publisher ID: 338

DOI: 10.1186/s12959-021-00338-y

PMC ID: 8569956

PubMed ID: 34736467

SO-VID: 6b3c13c0-00b5-41cf-961c-bf9fc8ef4418

License:

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. 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 in a credit line to the data.

History

Date received : 13 June 2021

Date accepted : 26 October 2021

Funding

Funded by: National Natural Science Foundation of China

Award ID: 81371878

Award Recipient : Jing Zhou

Custom metadata

ScienceOpen disciplines: Cardiovascular Medicine

Keywords: coagulation,fibrinolysis,hemophagocytic lymphohistiocytosis,disseminated intravascular coagulation

Data availability:

ScienceOpen disciplines: Cardiovascular Medicine

Keywords: coagulation, fibrinolysis, hemophagocytic lymphohistiocytosis, disseminated intravascular coagulation

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