Haematological

A 65-year-old woman presents to the emergency department with a 3-month history of progressive fatigue, lethargy and exertional dyspnoea. She denies overt rectal bleeding or melaena but reports vague lower abdominal discomfort, early satiety and an unintentional 4 kg weight loss over the same period. She has no significant past medical history, takes no regular medications and does not drink alcohol. On examination she is afebrile with HR 102/min, BP 118/76 mmHg, RR 16/min and SpO2 98% on room air. She appears pale with conjunctival pallor, koilonychia and a smooth, atrophic tongue. Abdominal examination is soft and non-tender without palpable masses or organomegaly. Digital rectal examination reveals no fresh blood but the stool is noted to be dark. Investigations show Hb 95 g/L, MCV 65 fL, ferritin 5 µg/L, with normal renal and liver function tests. Which of the following is the most likely diagnosis?

A 17-year-old girl presents to the emergency department with heavy, prolonged menstrual bleeding since menarche. She mentions that her mother and grandmother have also had similar problems with easy bruising and heavy periods. Which of the following laboratory investigations is MOST likely to be abnormal in this patient?

A 25-year-old man presents to the emergency department with a 3-week history of worsening fatigue, gum bleeding when brushing his teeth, and two episodes of low-grade fever with a sore throat that did not resolve with antibiotics. He denies significant weight loss, night sweats or bone pain. He takes no regular medications and has had no recent overseas travel. On examination he is pale with scattered petechiae over both shins; there is no lymphadenopathy and no hepatosplenomegaly. Observations are: HR 102 bpm, BP 108/68 mmHg, temp 37.8°C, SpO2 98% on room air. FBE shows haemoglobin 72 g/L, white cell count 1.2 × 10⁹/L (absolute neutrophil count 0.3 × 10⁹/L), platelets 18 × 10⁹/L, and a low reticulocyte count. The peripheral blood film shows no blasts, dysplastic cells or abnormal circulating cells. A bone marrow trephine biopsy reveals a markedly hypocellular marrow (<10% cellularity) with fatty replacement and no increase in blasts, fibrosis or abnormal infiltrate. What is the most likely diagnosis?

A 30-year-old man with homozygous sickle cell disease (HbSS) is reviewed in the emergency department short-stay unit after his third presentation this year with diffuse limb and back pain. He describes a background of near-daily chronic pain for the past 18 months, superimposed on intermittent acute vaso-occlusive flares, and reports increasing opioid requirements at home with associated low mood and reduced function. He is haemodynamically stable, afebrile, with oxygen saturation of 98% on room air, and his examination is unremarkable apart from diffuse musculoskeletal tenderness. Baseline haemoglobin is 82 g/L with reticulocytosis, consistent with his known chronic haemolytic anaemia. He has never been on a disease-modifying agent. What is the most appropriate long-term strategy to reduce his disease burden and chronic pain?

A 60-year-old man presents to the emergency department with haematemesis and melaena. He is alert and oriented, with a heart rate of 118 bpm and blood pressure of 92/58 mmHg. His haemoglobin returns at 62 g/L. He is a practising Jehovah's Witness and carries a signed advance directive card refusing all blood and blood products, including packed red cells. On assessment he demonstrates full capacity: he understands his diagnosis, the risk of exsanguination without transfusion, and the consequences of his refusal, and he consistently reaffirms this decision when questioned alone, without family present. He is haemodynamically responding to crystalloid resuscitation and urgent endoscopy has been arranged. What is the most appropriate approach to his ongoing management?

A 40-year-old man with a 15-year history of severe aplastic anaemia, previously managed with immunosuppressive therapy and long-term transfusion support, is referred to the bone marrow transplant unit after failing a second course of anti-thymocyte globulin and ciclosporin. He reports increasing fatigue and easy bruising but denies fever, night sweats, weight loss or recent infection. On examination he is pale with scattered petechiae on the shins; there is no lymphadenopathy, hepatosplenomegaly or focal neurological deficit. Observations are HR 88 bpm, BP 118/76 mmHg, SpO2 98% on room air, temperature 36.6°C. Blood tests confirm ongoing pancytopenia, and a fully HLA-matched sibling donor has now been identified. Before proceeding to conditioning chemotherapy for allogeneic haematopoietic stem cell transplantation (HSCT), what is the most important next step in his management?