Characteristics and outcome of breast cancer-related microangiopathic haemolytic anaemia: a multicentre study

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Abstract

Background

Cancer-related microangiopathic haemolytic anaemia (MAHA) is a rare but life-threatening paraneoplastic syndrome. Only single cases or small series have been reported to date. We set up a retrospective multicentre study focusing on breast cancer-related MAHA.

Methods

Main inclusion criteria were known diagnosis of breast cancer, presence of schistocytes and either low haptoglobin or cytopenia and absence of any causes of MAHA other than breast cancer, including gemcitabine- or bevacizumab-based treatment. Patient characteristics, treatments and outcome were retrieved from digital medical records.

Results

Individual data from 54 patients with breast cancer-related MAHA were obtained from 7 centres. Twenty-three (44%) patients had a breast tumour with lobular features, and most primary tumours were low grade (grade I/II, N = 39, 75%). ER+/HER2−, HER2+ and triple-negative phenotypes accounted for N = 33 (69%), N = 7 (15%) and N = 8 (17%) cases, respectively. All patients had stage IV cancer at the time of MAHA diagnosis. Median overall survival (OS) was 28 days (range 0–1035; Q1:10, Q3:186). Independent prognostic factors for early death (≤ 28 days) were PS > 2 (OR = 7.0 [1.6; 31.8]), elevated bilirubin (OR = 6.9 [1.1; 42.6]), haemoglobin < 8.0 g/dL (OR = 3.7 [0.9; 16.7]) and prothrombin time < 50% (OR = 9.1 [1.2; 50.0]). A score to predict early death displayed a sensitivity of 86% (95% CI [0.67; 0.96]), a specificity of 73% (95% CI [0.52; 0.88]) and an area under the curve of 0.90 (95% CI [0.83; 0.97]).

Conclusions

Breast cancer-related MAHA appears to be a new feature of invasive lobular breast carcinoma. Prognostic factors and scores may guide clinical decision-making in this serious but not always fatal condition.

Supplementary Information

The online version contains supplementary material available at 10.1186/s13058-021-01386-y.

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Most cited references 43

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This review article covers the diverse pathophysiological pathways that can lead to microangiopathic hemolytic anemia and a procoagulant state with or without damage to the kidneys and other organs.

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Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.

J Thachil, Marcel Levi, Clifford H. Watson … (2009)

The diagnosis of disseminated intravascular coagulation (DIC) should encompass both clinical and laboratory information. The International Society for Thrombosis and Haemostasis (ISTH) DIC scoring system provides objective measurement of DIC. Where DIC is present the scoring system correlates with key clinical observations and outcomes. It is important to repeat the tests to monitor the dynamically changing scenario based on laboratory results and clinical observations. The cornerstone of the treatment of DIC is treatment of the underlying condition. Transfusion of platelets or plasma (components) in patients with DIC should not primarily be based on laboratory results and should in general be reserved for patients who present with bleeding. In patients with DIC and bleeding or at high risk of bleeding (e.g. postoperative patients or patients due to undergo an invasive procedure) and a platelet count of <50 x 10(9)/l transfusion of platelets should be considered. In non-bleeding patients with DIC, prophylactic platelet transfusion is not given unless it is perceived that there is a high risk of bleeding. In bleeding patients with DIC and prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT), administration of fresh frozen plasma (FFP) may be useful. It should not be instituted based on laboratory tests alone but should be considered in those with active bleeding and in those requiring an invasive procedure. There is no evidence that infusion of plasma stimulates the ongoing activation of coagulation. If transfusion of FFP is not possible in patients with bleeding because of fluid overload, consider using factor concentrates such as prothrombin complex concentrate, recognising that these will only partially correct the defect because they contain only selected factors, whereas in DIC there is a global deficiency of coagulation factors. Severe hypofibrinogenaemia (<1 g/l) that persists despite FFP replacement may be treated with fibrinogen concentrate or cryoprecipitate. In cases of DIC where thrombosis predominates, such as arterial or venous thromboembolism, severe purpura fulminans associated with acral ischemia or vascular skin infarction, therapeutic doses of heparin should be considered. In these patients where there is perceived to be a co-existing high risk of bleeding there may be benefits in using continuous infusion unfractionated heparin (UFH) due to its short half-life and reversibility. Weight adjusted doses (e.g. 10 mu/kg/h) may be used without the intention of prolonging the APTT ratio to 1.5-2.5 times the control. Monitoring the APTT in these cases may be complicated and clinical observation for signs of bleeding is important. In critically ill, non-bleeding patients with DIC, prophylaxis for venous thromboembolism with prophylactic doses of heparin or low molecular weight heparin is recommended. Consider treating patients with severe sepsis and DIC with recombinant human activated protein C (continuous infusion, 24 microg/kg/h for 4 d). Patients at high risk of bleeding should not be given recombinant human activated protein C. Current manufacturers guidance advises against using this product in patients with platelet counts of <30 x 10(9)/l. In the event of invasive procedures, administration of recombinant human activated protein C should be discontinued shortly before the intervention (elimination half-life approximately 20 min) and may be resumed a few hours later, dependent on the clinical situation. In the absence of further prospective evidence from randomised controlled trials confirming a beneficial effect of antithrombin concentrate on clinically relevant endpoints in patients with DIC and not receiving heparin, administration of antithrombin cannot be recommended. In general, patients with DIC should not be treated with antifibrinolytic agents. Patients with DIC that is characterised by a primary hyperfibrinolytic state and who present with severe bleeding could be treated with lysine analogues, such as tranexamic acid (e.g. 1 g every 8 h).

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Author and article information

Contributors

Francois-Clement Bidard:

ORCID: http://orcid.org/0000-0001-5932-8949

fcbidard@curie.fr

Journal

Journal ID (nlm-ta): Breast Cancer Res

Journal ID (iso-abbrev): Breast Cancer Res

Title: Breast Cancer Research : BCR

Publisher: BioMed Central (London )

ISSN (Print): 1465-5411

ISSN (Electronic): 1465-542X

Publication date (Electronic): 19 January 2021

Publication date PMC-release: 19 January 2021

Publication date (Print): 2021

Volume: 23

Electronic Location Identifier: 9

Affiliations

[1 ]GRID grid.418596.7, ISNI 0000 0004 0639 6384, Department of Medical Oncology, , Institut Curie, ; Paris and Saint Cloud, France

[2 ]GRID grid.12832.3a, ISNI 0000 0001 2323 0229, UVSQ, Université Paris-Saclay, ; 35 rue Dailly, Saint Cloud, 92210 France

[3 ]GRID grid.418596.7, ISNI 0000 0004 0639 6384, Institut Curie, Biometry Unit, ; Paris and Saint-Cloud, France

[4 ]GRID grid.14925.3b, ISNI 0000 0001 2284 9388, Department of Cancer Medicine, , Institut Gustave Roussy, ; Villejuif, France

[5 ]GRID grid.418191.4, ISNI 0000 0000 9437 3027, Department of Medical Oncology, , Institut de Cancérologie de l’Ouest, ; Saint-Herblain, France

[6 ]GRID grid.418189.d, ISNI 0000 0001 2175 1768, Department of Medical Oncology, , Centre François Baclesse, ; Caen, France

[7 ]GRID grid.418189.d, ISNI 0000 0001 2175 1768, Department of Medical Oncology, , Institut du Cancer de Montpellier, Institut de cancérologie de Montpellier INSERM U1194, ; Montpellier, France

[8 ]GRID grid.418037.9, ISNI 0000 0004 0641 1257, Department of Medical Oncology, , Centre Georges-François Leclerc, ; Dijon, France

[9 ]GRID grid.418116.b, ISNI 0000 0001 0200 3174, Department of Medical Oncology, , Centre Léon Bérard, ; Lyon, France

[10 ]GRID grid.417829.1, ISNI 0000 0000 9680 0846, Department of Medical Oncology, , Institut Claudius Regaud, Institut Universitaire du Cancer de Toulouse-Oncopole (IUCT-Oncopole), ; Toulouse, France

[11 ]GRID grid.418596.7, ISNI 0000 0004 0639 6384, Department of Pathology, , Institut Curie, ; Paris, France

[12 ]GRID grid.508487.6, ISNI 0000 0004 7885 7602, Université de Paris, ; Paris, France

[13 ]Reference Center for Thrombotic Microangiopathies (CNR-MAT), AP-HP.SU, INSERM UMRS, 1138 Paris, France

[14 ]GRID grid.462844.8, ISNI 0000 0001 2308 1657, Sorbonne University, ; Paris, France

Author information

Francois-Clement Bidard http://orcid.org/0000-0001-5932-8949

Article

Publisher ID: 1386

DOI: 10.1186/s13058-021-01386-y

PMC ID: 7814553

PubMed ID: 33468209

SO-VID: 70bab4d1-7728-4142-a7cb-99741c1ff585

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 : 24 August 2020

Date accepted : 1 January 2021

Funding

Funded by: FundRef http://dx.doi.org/10.13039/100004336, Novartis;

Award ID: N/A

Award Recipient : Francois-Clement Bidard

Funded by: Institut Curie Siric 2

Award ID: INCa-DGOS-4654

Award Recipient : Francois-Clement Bidard

Custom metadata

ScienceOpen disciplines: Oncology & Radiotherapy

Keywords: microangiopathic haemolytic anaemia,breast cancer,survival,prognostic factors

Data availability:

ScienceOpen disciplines: Oncology & Radiotherapy

Keywords: microangiopathic haemolytic anaemia, breast cancer, survival, prognostic factors

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Characteristics and outcome of breast cancer-related microangiopathic haemolytic anaemia: a multicentre study

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Abstract

Background

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Conclusions

Supplementary Information

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Karger: Oncology

Most cited references 43

Syndromes of thrombotic microangiopathy.

Thrombotic microangiopathies.

Guidelines for the diagnosis and management of disseminated intravascular coagulation. British Committee for Standards in Haematology.

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