Marion Alhenc-Gelas1, Luc Cabel1,2, Frederique Berger3, Suzette Delaloge4, Jean-Sebastien Frenel5, Christelle Levy6, Nelly Firmin7, Sylvain Ladoire8, Isabelle Desmoulins9, Pierre-Etienne Heudel9, Florence Dalenc10, Delphine Loirat1, Coraline Dubot1, Perrine Vuagnat1, Elise Deluche4, Meriem Mokdad-Adi4, Anne Patsouris5, Josselin Annic5, Lounes Djerroudi11, Marion Lavigne11, Jean-Yves Pierga1,12, Paul Coppo13,14, Francois-Clement Bidard15,16. 1. Department of Medical Oncology, Institut Curie, Paris and Saint Cloud, France. 2. UVSQ, Université Paris-Saclay, 35 rue Dailly, Saint Cloud, 92210, France. 3. Institut Curie, Biometry Unit, Paris and Saint-Cloud, France. 4. Department of Cancer Medicine, Institut Gustave Roussy, Villejuif, France. 5. Department of Medical Oncology, Institut de Cancérologie de l'Ouest, Saint-Herblain, France. 6. Department of Medical Oncology, Centre François Baclesse, Caen, France. 7. Department of Medical Oncology, Institut du Cancer de Montpellier, Institut de cancérologie de Montpellier INSERM U1194, Montpellier, France. 8. Department of Medical Oncology, Centre Georges-François Leclerc, Dijon, France. 9. Department of Medical Oncology, Centre Léon Bérard, Lyon, France. 10. Department of Medical Oncology, Institut Claudius Regaud, Institut Universitaire du Cancer de Toulouse-Oncopole (IUCT-Oncopole), Toulouse, France. 11. Department of Pathology, Institut Curie, Paris, France. 12. Université de Paris, Paris, France. 13. Reference Center for Thrombotic Microangiopathies (CNR-MAT), AP-HP.SU, INSERM UMRS, 1138, Paris, France. 14. Sorbonne University, Paris, France. 15. Department of Medical Oncology, Institut Curie, Paris and Saint Cloud, France. fcbidard@curie.fr. 16. UVSQ, Université Paris-Saclay, 35 rue Dailly, Saint Cloud, 92210, France. fcbidard@curie.fr.
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.
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.
Entities:
Keywords:
Breast cancer; Microangiopathic haemolytic anaemia; Prognostic factors; Survival
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