Protecting vulnerable patients with inherited anaemias from unnecessary death during the COVID-19 pandemic.

With the developing COVID-19 pandemic, patients with inherited anaemias require specific advice regarding isolation and changes to usual treatment schedules. The National Haemoglobinopathy Panel (NHP) has issued guidance on the care of patients with sickle cell disease, thalassaemia, Diamond Blackfan anaemia (DBA), congenital dyserythropoietic anaemia (CDA), sideroblastic anaemia, pyruvate kinase deficiency and other red cell enzyme and membrane disorders. Cascading of accurate information for clinicians and patients is paramount to preventing adverse outcomes, such as patients who are at increased risk of fulminant bacterial infection due to their condition or its treatment erroneously self-isolating if their fever is mistakenly attributed to a viral cause, delaying potentially life-saving antibiotic therapy. Outpatient visits should be minimised for most patients, however some, such as first transcranial dopplers for children with sickle cell anaemia should not be delayed as known risk of stroke will outweigh the unknown risk from COVID-19 infection. Blood transfusion programmes should be continued, but specific changes to usual clinical pathways can be instituted to reduce risk of patient exposure to COVID-19, as well as contingency planning for possible reductions in blood available for transfusions. Bone marrow transplants for these disorders should be postponed until further notice. With the current lack of evidence on the risk and complications of COVID-19 infection in these patients, national data collection is ongoing to record outcomes and eventually to identify predictors of disease severity, particularly important if further waves of infection travel through the population.

Recurrent acute lower-limb ischemia with multiple organ infarctions secondary to acute myeloid leukaemia M1.

BACKGROUND: Acute myeloid leukemia (AML) is usually associated with coagulopathy and disorders of hemostasis, but cases of ischemic events have been reported. We present a case of AML with recurrent acute limb ischemia and multiple organ infarctions. METHODS AND RESULTS: A 57-year-old woman diagnosed with AML subtype M1 developed recurrent bilateral acute lower-limb ischemia refractory to multiple thromboembolectomies and bypass grafting. Histopathology revealed that thrombi were composed of leukemic blasts, and computed tomography angiogram incidentally revealed multiple infarctions. She demonstrated a response to chemotherapy, but died of an overwhelming sepsis 22 days after her acute admission. CONCLUSIONS: AML subtype M1 with acute lower-limb ischemia and multiple organ infarctions is associated with a poor prognosis. The role of emergency chemotherapy in reducing the tumour burden and possibly improving the results of vascular interventions needs to be defined. Limb-salvaging surgery should not be delayed but be administered immediately according to the degree of ischemia.