ABSTRACT
Thrombotic Microangiopathies (TM) have been described since the 1960s. They are characterized by presence of mechanical haemolytic anemia associated with peripheral thrombocytopenia. TM in cancer can be related to several causes, whose cancer himself: cancer-related microangiopathic haemolytic anaemia (MAHA). Incidence of cancer related MAHA remains unknown. Cancer-related MAHA are mainly observed in mucin-producer adenocarcinomas, such as gastric (half of reported cases) and breast cancer. We conducted a review of all original published cases of TM reported in breast cancer, and we specifically investigated BC-MAHA cases. A Medline search identified 158 MAHA cases including 118 BC-MAHA, and 40 drug-related MAHA. Most of BC-MAHA occur in disseminated cancers, mainly with medullar involvement, and/or bone metastasis. Patients typically suffer from poor general state, bone pain, and/or dyspnea. Laboratory abnormalities such as myelemia or erythromyelemia in peripheral blood are frequently observed. Incidence of coagulation disorders is increased, compared to other MAHA causes. BC-MAHA prognosis is dramatically poor. Treatments classically used in other MAHA causes, such as plasmapheresis or immunoglobulins, are inefficient. Urgent anti-neoplastic therapy may be the only effective treatment, associated to symptomatic therapies (transfusions, blood pressure control).
Subject(s)
Anemia, Hemolytic/complications , Breast Neoplasms/complications , Thrombotic Microangiopathies/etiology , Adenocarcinoma/metabolism , Anemia, Hemolytic/epidemiology , Anemia, Hemolytic/mortality , Anemia, Hemolytic/therapy , Blood Coagulation Disorders/epidemiology , Bone Neoplasms/secondary , Breast Neoplasms/metabolism , Female , Humans , Incidence , Mucins/biosynthesis , Thrombocytopenia/complications , Thrombotic Microangiopathies/therapyABSTRACT
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.
Subject(s)
Anemia, Hemolytic/epidemiology , Anemia, Hemolytic/etiology , Breast Neoplasms/complications , Breast Neoplasms/epidemiology , Anemia, Hemolytic/diagnosis , Anemia, Hemolytic/therapy , Area Under Curve , Biomarkers, Tumor , Breast Neoplasms/diagnosis , Breast Neoplasms/therapy , Disease Management , Disease Susceptibility , Female , France/epidemiology , Humans , Neoplasm Grading , Neoplasm Staging , Phenotype , Prognosis , Severity of Illness Index , Survival AnalysisABSTRACT
Pancreatic adenocarcinoma remains a cancer associated with a poor prognosis. For locally advanced pancreatic cancer (LAPC), median overall survival is approximately 16 months. Here we report the case of a 52-year-old LAPC patient treated with chemotherapy followed by chemoradiotherapy that was associated with a 14-year complete remission. A peritoneal relapse was then observed and chemotherapy was undergone until the patient died of infectious complications, 17 years after his diagnosis. The tumor was found KRAS, TP53, BRCA1, and BRCA2 wild-type. This KRAS wild-type LAPC-long survivor case report emphasizes the need to develop molecular approaches to predict LAPC patients' prognosis.
