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Pancytopenia

Clinical Discipline(s)/Organ System(s)
Haematology, Oncology
Progress Test Topic(s)
Blood and lymph
Description
A 28 year old man presents to the Emergency Department after being unwell with fever (temperature up to 39.7°C), extreme lethargy, and petechial haemorrhages in his skin for the last 5 days. He is usually fit and well. While he is being assessed, his blood pressure drops to 80/55 mmHg and his heart rate climbs to 134 beats per minute. His full blood count shows marked pancytopenia with haemoglobin 76 g/L, platelets <10 x 109/L, and no detectable segmented neutrophils. In the blood film a number of large, immature cells are observed.
Progress Test-Type Questions:   Question 1 | Question 2
Applied Science for Medicine 
   - Development of mature blood cells from haematopoietic stem cells and the role of cytokines
   - Characteristics and clinical potential of stem cells; embryonic stem cells, adult stem cells, induced pluripotent (iPS) cells
   - Explain developments in the field of 'regenerative medicine'; outline how a haematopoietic stem cell transplant is undertaken
   - Describe the major peripheral blood and bone marrow abnormalities observed in leukaemias
   - Outline the types of genetic abnormalities found in haematological malignancies
   - Outline the pharmacology of chemotherapeutic agents
   - Common infections in immunocompromised patients
   - Design of clinical studies: treatment (randomised, nonrandomised) and observational (cohort, case-control)
   - Pharmacology of chloramphenicol
Clinical and Communication Skills 
   - History from a patient with
pancytopenia; consider malignant and non-malignant causes of pancytopenia
   - Examination of a patient with pancytopenia; examine the lymphatic system, liver and spleen; identify possible source(s) of infection
   - Differential diagnosis of pancytopenia
   - Recognition and management of neutropenic sepsis
   - Interpretation of blood film; recognise findings suggestive of leukaemia
   - Outline the management of acute leukaemias and myeloma
   - Complications of leukaemias and myeloma; recognise tumour lysis syndrome
   - Oral ulceration: recognition and management of aphthous ulcers, traumatic ulcers, ulcers caused by infection, and mucositis secondary to chemotherapy
   - Prognosis of different leukaemias and myeloma
Personal and Professional Skills 
   - Equipoise and enrolling patients in research
   - Delivering life-changing news
   - Management of an acutely unwell patient; interaction with family/whānau
Hauora Māori 
   - Disproportionate impact of socioeconomic deprivation on Māori including access to adequate nutrition or expensive treatments, and appropriate response
Population Health 
   - Epidemiology of leukaemias: age, gender and prevalence
   - Epidemiology of myeloma: age and prevalence
   - Ethics of access to healthcare resources for expensive therapies
Conditions to be considered relating to this scenario
Common
acute myeloid leukaemia, acute lymphoblastic leukaemia, myeloma, hypersplenism, vitamin B12/folate deficiency, sepsis, chronic myeloid leukaemia, chemotherapy toxicity
Less common but 'important not to miss'
myelodysplastic syndrome, myelofibrosis, red cell aplasia, aplastic anaemia, hairy cell leukaemia, solid tumour metastases, HIV, parvovirus B19
Uncommon
Shwachman-Diamond syndrome