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

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.

Therapeutic Plasma Exchange in Postpartum Hemolytic Uremic Syndrome: A Case Report

BACKGROUND AND AIM: Adult, non-infective, haemolytic-uremic syndrome (HUS) although a rare disease in itself, has a high likelihood of occurrence in pregnancy and immediate post partum period. It is an important differential diagnosis in the evaluation of thrombotic microangiopathies. Patients with post-partum HUS display a classical triad of microangiopathic haemolytic anaemia, acute nephropathy and thrombocytopenia. I hereby present a case of post partum HUS treated with therapeutic plasma exchange (TPE) MATERIAL AND METHODS: A total of six sessions of TPE were performed daily, three sessions for consecutive days and remaining three sessions were performed on alternate days. All the procedures were carried out with Haemonetics MCS+ exchanging one plasma volume using fresh frozen plasma and saline as replacement fluid. Haemodialysis was started and four sessions were carried out on alternate days. RESULT: A 37 year old, 85 kg female, G2 P1, underwent emergency LSCS because of foetal distress at 38 weeks of pregnancy. Post surgery she developed decreasing urine output, anuria ensued. Emergency therapeutic plasma exchange was carried out within 24 hours of diagnosis. It could be found that with TPE, patient had improvement in renal function, decrease in LDH levels and increase in platelet count. Patient had sustained remission and discontinuation of haemodialysis. CONCLUSION: HUS is a disorder with high mortality and long term morbidity, if prompt treatment is not instituted. The decision to intervene with plasma exchange should be based upon the severity of thrombocytopenia, microangiopathic haemolytic anaemia and neurological abnormalities, even if the diagnosis and nomenclature is uncertain. Improved survival after this disorder has been attributed to aggressive treatment with plasma exchange therapy.