Specialist & Consultant Haematology & Oncology oral assessment prep

Knowledge: The working diagnosis is acute promyelocytic leukaemia with DIC and hyperfibrinolysis, and early haemorrhagic death is the main threat. Other leukaemia with DIC and sepsis are considered but must not delay treatment. Diagnosis is confirmed by PML::RARA on FISH or RT-PCR, but ATRA starts on morphology alone. Risk is set by the presenting white count, with 10 x 10^9/L or below being non-high-risk. For non-high-risk disease, ATRA with arsenic trioxide is the standard of care, based on the APL0406 trial, with cure rates above 90 percent. Differentiation syndrome is common and presents with fever, weight gain, dyspnoea, infiltrates and renal impairment.

Management: I would start ATRA 45 mg/m2 daily in two divided doses immediately. I would transfuse platelets to keep them above 30 to 50 x 10^9/L, give cryoprecipitate or fibrinogen concentrate to keep fibrinogen above 1.0 to 1.5 g/L, and plasma for the prolonged PT, rechecking every 6 to 8 hours. I would avoid lumbar puncture, uncorrected central line insertion and intramuscular injections. I would suppress menstruation hormonally and send marrow, FISH and PCR, a pregnancy test, ECG with QTc, potassium and magnesium before arsenic. Once non-high-risk disease is confirmed, I would add arsenic trioxide 0.15 mg/kg daily. If differentiation syndrome develops, I would give dexamethasone 10 mg intravenously twice daily and hold ATRA if severe. I would discuss her at the leukaemia multidisciplinary meeting.

Ethics and communication: She needs an honest explanation that this is a serious but highly curable leukaemia and that treatment must start today, with consent obtained in line with Cabinet Resolution No. 40 of 2019. ATRA and arsenic are teratogenic, so pregnancy, contraception and fertility must be discussed. Her husband can be present if she wishes, but the decisions are hers. I would document the timing of each step, because delay in starting ATRA costs lives.

Reference: Sanz MA et al, Management of acute promyelocytic leukemia: updated recommendations from an expert panel of the European LeukemiaNet (Blood 2019); Hoffbrand AV, Moss PAH, Hoffbrand's Essential Haematology (8th ed, 2019)

Knowledge: This is febrile neutropenia with septic shock, where mortality rises with every hour antibiotics are delayed. Likely sources are Gram-negative bacteraemia, including Pseudomonas and resistant Enterobacterales common in the region, and Gram-positive line infection. MASCC and CISNE scores select low-risk outpatients but do not apply to an unstable patient. A vague childhood penicillin rash should not stop a beta-lactam, as cross-reactivity with carbapenems is very low. The local antibiogram and any prior ESBL or carbapenem-resistant colonisation guide the choice.

Management: I would take blood cultures from each line lumen and a peripheral vein and give the first antibiotic within 60 minutes. Because he is in shock, I would give meropenem 1 g every 8 hours with vancomycin for the line, adding amikacin if carbapenem resistance is common locally. I would give 30 mL/kg balanced crystalloid, start noradrenaline if mean arterial pressure stays below 65 mmHg, and refer early to intensive care. I would send urine, chest radiograph, exit-site swab and repeat lactate, and adjust doses to renal function. I would remove the line for tunnel infection, persistent bacteraemia, or Staphylococcus aureus, Pseudomonas or Candida in the blood. If fever persists beyond 4 to 7 days, I would look for fungal infection with CT chest and galactomannan. I would de-escalate on culture results and use G-CSF prophylaxis for later cycles.

Ethics and communication: If he cannot discuss treatment, emergency care proceeds, as UAE law allows treatment without consent in an emergency. With his permission I would explain the seriousness and plan to his family. I would record the triage and antibiotic times, and report any delay through the facility incident system. Teaching patients to present early with fever is part of safe chemotherapy practice.

Reference: Klastersky J et al, Management of febrile neutropaenia: ESMO Clinical Practice Guidelines (Ann Oncol 2016); Surviving Sepsis Campaign International Guidelines 2021

Knowledge: This is acute chest syndrome, a new pulmonary infiltrate with fever, chest symptoms or hypoxia, often following a vaso-occlusive crisis, opioids and hypoventilation. Causes include infection with atypical organisms, fat embolism from marrow infarction and in-situ thrombosis. Pulmonary embolism and fluid overload are the main differentials. Falling haemoglobin and platelets suggest a severe course with risk of multi-organ failure. Severity is judged by oxygen need, the trend and the number of lobes involved. The BSH guideline supports simple transfusion for moderate disease and urgent exchange transfusion for severe features or progression despite simple transfusion.

Management: I would give oxygen to a target of 94 to 98 percent, start ceftriaxone with clarithromycin, and continue monitored opioid analgesia with incentive spirometry. I would limit fluids to maintenance and start thromboprophylaxis. I would give a simple transfusion of extended phenotype-matched, sickle-negative red cells to no more than about 100 g/L to avoid hyperviscosity. If oxygen needs rise, multiple lobes become involved or he worsens despite transfusion, I would arrange urgent red cell exchange and involve intensive care. I would obtain his antibody history from any other hospital, blood cultures, atypical serology and a viral panel. I would not discharge until he is stable on room air. After recovery, I would start or optimise hydroxycarbamide and review vaccinations.

Ethics and communication: Patients with sickle cell disease are sometimes wrongly labelled as drug-seeking, so I would assess and treat pain without prejudice. Before transfusion I would gain informed consent, explaining alloimmunisation and delayed haemolysis, in line with Cabinet Resolution No. 40 of 2019. He is an adult, so his family is involved only with his agreement. I would offer genetic counselling when relevant, respecting his privacy.

Reference: Howard J et al, Guideline on the management of acute chest syndrome in sickle cell disease, British Society for Haematology (2015); Chou ST et al, American Society of Hematology 2020 guidelines for sickle cell disease: transfusion support

Knowledge: He has stage IV pancreatic cancer with a median survival measured in months, and with a good performance status he may be fit for FOLFIRINOX or gemcitabine with nab-paclitaxel. Germline BRCA testing is recommended in all pancreatic cancer and may open olaparib maintenance. Any treatment needs his informed consent, and he cannot consent to chemotherapy for an infection. Requests for non-disclosure are common in GCC practice and come from love, not bad intent. Patients often know more than families think, and many want information. Early palliative care improves quality of life in advanced cancer.

Management: I would first listen to the sons, acknowledge their fears, and explain that I cannot give him false information or treatment he has not agreed to. I would agree that information will not be forced on him, and that I will ask him how much he wants to know and who he wants involved. I would see him privately, using a structured approach such as SPIKES and starting with what he understands. If he wants to know, I would explain the diagnosis plainly and outline chemotherapy, symptom control and early palliative care. If he prefers his sons to receive details, I would document and respect that. I would arrange germline testing and a multidisciplinary discussion, and plan a second meeting with a specialist nurse.

Ethics and communication: UAE law, under Federal Decree-Law No. 4 of 2016 and Cabinet Resolution No. 40 of 2019, requires informed consent before treatment, and consent based on a false diagnosis is not valid. The WMA Declaration of Lisbon sets the international principle of a right to information, including the right not to be informed if the patient chooses. Confidentiality belongs to him, so the family receives information with his permission. I would document the family meeting, his wishes and whom he nominates.

Reference: Baile WF et al, SPIKES: a six-step protocol for delivering bad news (The Oncologist 2000); WMA Declaration of Lisbon on the Rights of the Patient; Conroy T et al, Pancreatic cancer: ESMO Clinical Practice Guideline (Ann Oncol 2023)

Knowledge: Intrathecal vincristine causes ascending myeloencephalopathy that is almost always fatal, and this exact error has caused repeated deaths worldwide. Vinca alkaloids should be diluted in a minibag for intravenous infusion, never supplied in a syringe, and labelled for intravenous use only. Intrathecal drugs should be issued separately, at a different time, and checked by two trained staff on an authorised register. Non-Luer NRFit connectors for neuraxial devices make wrong-route connection physically difficult. A near miss is a key learning event because the same failures can kill next time.

Management: I would stop the procedure, remove the vincristine from the room and quarantine both syringes with their labels. I would confirm nothing was given. If he agrees, the methotrexate would go ahead only after a fresh two-person check, with vincristine given later on the ward from a minibag. I would report the near miss through the facility incident system the same day and inform pharmacy and the chemotherapy lead. I would support the nurse who spoke up and the junior doctor without blame. I would ask for a root cause analysis covering dispensing, delivery, scheduling and connector use. Fixes should follow the hierarchy of controls, such as minibag-only dispensing and separate delivery, rather than reminders alone.

Ethics and communication: The patient has a right to know, so I would tell him honestly that the drugs were sent together wrongly, that the error was caught before any harm, and what we will change. I would apologise without blaming staff. I would document the facts and the disclosure in his record. Reporting near misses is a professional duty, and hiding this one would put future patients at risk.

Reference: WHO Information Exchange System Alert No. 115, Vincristine (and other vinca alkaloids) should only be given intravenously (2007); Neuss MN et al, 2016 Updated American Society of Clinical Oncology/Oncology Nursing Society Chemotherapy Administration Safety Standards

Knowledge: This is a thrombotic microangiopathy with neurological features and a high PLASMIC score, so immune TTP is the working diagnosis. Untreated mortality is around 90 percent, and with plasma exchange most patients survive. Normal clotting helps exclude DIC, and I must consider complement-mediated HUS, which is associated with the postpartum period, as well as HELLP, sepsis and drug-induced TMA. ADAMTS13 activity below 10 percent confirms TTP, but the result may take days. The ISTH 2020 guideline recommends plasma exchange with corticosteroids and suggests rituximab and caplacizumab for immune TTP. Platelet transfusion is avoided unless there is life-threatening bleeding.

Management: I would advise against the platelet transfusion and send ADAMTS13 activity and inhibitor before any plasma is given. I would arrange urgent central access and start daily plasma exchange at 1 to 1.5 plasma volumes, giving plasma infusion if exchange is delayed by hours. I would start methylprednisolone 1 g daily for three days, or prednisolone 1 mg/kg, and add rituximab and caplacizumab. I would admit her to a high-dependency unit with troponin, ECG and neurological monitoring, as cardiac involvement causes sudden death. I would give folic acid and start thromboprophylaxis once platelets exceed 50 x 10^9/L. I would continue exchange until platelets are normal for 2 days, then use ADAMTS13 to guide caplacizumab duration. If ADAMTS13 is not severely reduced, I would review for complement-mediated HUS and eculizumab.

Ethics and communication: She is confused, so her capacity to consent may be impaired, and urgent treatment should proceed as emergency treatment, which UAE law allows without consent. I would keep her husband informed and record why treatment could not wait. Once she recovers, I would explain the diagnosis, the relapse risk and future pregnancy planning, as TTP can recur in pregnancy.

Reference: Zheng XL et al, ISTH guidelines for treatment of thrombotic thrombocytopenic purpura (J Thromb Haemost 2020); Scully M et al, Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura, British Society for Haematology (2012)