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

• Main Authors: Marion Alhenc-Gelas, Luc Cabel, Frederique Berger, Suzette Delaloge, Jean-Sebastien Frenel, Christelle Levy, Nelly Firmin, Sylvain Ladoire, Isabelle Desmoulins, Pierre-Etienne Heudel, Florence Dalenc, Delphine Loirat, Coraline Dubot, Perrine Vuagnat, Elise Deluche, Meriem Mokdad-Adi, Anne Patsouris, Josselin Annic, Lounes Djerroudi, Marion Lavigne, Jean-Yves Pierga, Paul Coppo, Francois-Clement Bidard
• Format: Article
• Language: English
• Published: BMC 2021-01-01
• Series: Breast Cancer Research
• Subjects: Microangiopathic haemolytic anaemia; Breast cancer; Survival; Prognostic factors
• Online Access: https://doi.org/10.1186/s13058-021-01386-y

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.