Domain – Haematological presentations (case)

Communication and consultation skills

What strategies would you use to communicate with a teenager who presents with a parent?

If Lisa was an Aboriginal or Torres Strait Islander, how would you carry out the consultation?

What if they were both recent immigrants who spoke little English? How would you sensitively obtain a menstrual and sexual history from Lisa?

• Communicating with a teenager with a parent present:
  • Establish rapport with both Lisa and her mother.
  • Explain the importance of confidentiality and offer Lisa some time alone to discuss sensitive issues.
  • Use open-ended questions to encourage Lisa to express her concerns.
• If Lisa was an Aboriginal or Torres Strait Islander:
  • Be culturally sensitive and aware of health beliefs and practices.
  • Involve an Aboriginal Health Worker or Liaison Officer if available.
  • Ensure the consultation environment is culturally safe.
• If they were recent immigrants who spoke little English:
  • Use a professional interpreter, preferably of the same gender as Lisa.
  • Speak clearly and avoid medical jargon.
  • Be patient and check understanding frequently.
• Obtaining a menstrual and sexual history:
  • Explain the importance of these questions for her health.
  • Assure Lisa of confidentiality.
  • Use a non-judgmental and empathetic approach.
  • Allow Lisa to answer these questions privately if she prefers.

Clinical information gathering and interpretation

What other information would you want to know to identify the possible cause of Lisa’s pallor and lethargy?

What examination would you do? What other information would you need to understand about how the symptoms are impacting Lisa?

What dietary or family history would you like to know in relation to Lisa’s symptoms?

• Additional information to identify the cause of pallor and lethargy:
  • Detailed history of the onset, duration, and severity of symptoms.
  • Inquiry about recent infections, weight loss, or changes in appetite.
  • Review of Lisa’s menstrual history including menarche, cycle regularity, and menstrual flow.
  • Detailed dietary history, focusing on iron and vitamin intake.
  • Family history of anemia or other hematological disorders.

• Physical examination:
  • General appearance and vital signs.
  • Paleness of skin, palm creases and conjunctiva
  • Iron def specific
    • Angular cheilitis, painful cracks  at the corners of the mouth
    • Atrophic glossitis, loss of tongue papillae (smooth, shiny tongue)
    • Pruritus and dry skin
    • Nail disorders, including koilonychia
    • Dry and brittle hair 
    • Increased hair shedding (telogen effluvium) resulting in diffuse alopecia.
  • Examination of the abdomen for organomegaly.
  • Evaluation for signs of bleeding or bruising.
  • Neurological examination if indicated by history.
• Understanding symptom impact:
  • Impact on school performance and attendance.
  • Effects on physical activity and social interactions.
  • Psychological effects, including mood and energy levels.

Making a diagnosis, decision making and reasoning

What preliminary investigations would you consider to confirm your provisional diagnosis?

If you consider anaemia as your diagnosis, what would you consider and look for to identify the type of anaemia?

Are there any other investigations you might consider if Lisa were a refugee? What if she were 70 years old, or 5 years old?

• Preliminary investigations:
  • Full blood count (FBC) including hemoglobin, hematocrit, MCV, MCH, and RDW.
  • Reticulocyte count.
  • Iron studies including serum iron, ferritin, transferrin saturation, and total iron-binding capacity (TIBC).
  • Blood film to look for morphological abnormalities.
  • Vitamin B12 and folate levels
• Identifying type of anemia:
  • Microcytic anemia (low MCV) suggests iron deficiency or thalassemia.
  • Normocytic anemia (normal MCV) suggests chronic disease or acute blood loss.
  • Macrocytic anemia (high MCV) suggests vitamin B12 or folate deficiency.
• Additional investigations for refugees:
  • Stool occult blood test (if gastrointestinal bleeding is suspected)
  • Screening for hemoglobinopathies (e.g., sickle cell disease, thalassemia).
  • Infectious diseases screening (e.g., malaria, tuberculosis).
• Age-specific considerations:
  • For a 70-year-old: consider chronic diseases, malignancies, and nutritional deficiencies.
  • For a 5-year-old: consider dietary intake, growth parameters, and congenital conditions.

Clinical management and therapeutic reasoning

What factors would you consider when treating anaemia?

• Duration and progression of symptoms (pallor, lethargy)
• Dietary habits (e.g., vegetarian or vegan diet)
• Menstrual history (e.g., heavy periods)
• Family history of anemia or bleeding disorders
• Recent illnesses or infections
• Medication use (e.g., NSAIDs, proton pump inhibitors)

How would you manage the underlying causes of these symptoms? To manage anaemia, what guidelines or protocols would you refer to?

Potential Underlying Causes:

• Nutritional deficiency (iron, vitamin B12, folate)
• Menorrhagia (heavy menstrual bleeding)
• Chronic diseases (e.g., inflammatory bowel disease)
• Malabsorption syndromes (e.g., celiac disease)
• Hemolytic anemias or bone marrow disorders

Management of Underlying Causes

1. Dietary Management:
   • Ensure adequate intake of iron-rich foods (red meat, poultry, fish, leafy greens, legumes)
   • Iron supplementation (oral ferrous sulfate or ferrous gluconate) if dietary intake is insufficient
   • Vitamin C to enhance iron absorption
2. Menstrual Management:
   • Address heavy menstrual bleeding (e.g., hormonal therapy, tranexamic acid)
   • Referral to a gynecologist if necessary
3. Treatment of Gastrointestinal Causes:
   • Investigate and treat potential sources of gastrointestinal bleeding (e.g., endoscopy for peptic ulcers)
   • Treat any identified infections (e.g., Helicobacter pylori eradication therapy)
4. Management of Malabsorption:
   • Treat underlying conditions (e.g., gluten-free diet for celiac disease)
   • Supplement deficiencies (e.g., vitamin B12 injections for pernicious anemia)
5. Addressing Chronic Conditions:
   • Manage any chronic inflammatory or renal diseases contributing to anemia

Guidelines and Protocols in Australia

1. Therapeutic Guidelines (eTG Complete):
2. Australian Red Cross Lifeblood: Provides guidelines on the management of anemia, particularly related to iron deficiency and blood transfusion protocols.
3. National Blood Authority (NBA): NBA offers guidelines on the use of blood and blood products, including the management of anemia.
4. Royal Australian College of General Practitioners (RACGP)
5. Australian Government Department of Health
6. State Health Departments:

Preventive and population health 

What strategies would you use to prevent dietary-induced anaemia?

Strategies to Prevent Dietary-Induced Anemia

1. Dietary Education:
   • Educate Lisa and her family about iron-rich foods, including red meat, poultry, fish, legumes, dark leafy greens, and fortified cereals.
   • Encourage the consumption of vitamin C-rich foods (e.g., citrus fruits, tomatoes, bell peppers) to enhance iron absorption.
2. Balanced Diet:
   • Promote a balanced diet that includes a variety of nutrients necessary for overall health, such as vitamin B12 and folate.
   • Ensure adequate intake of protein and other micronutrients that support hematopoiesis.
3. Regular Monitoring:
   • Schedule regular follow-up appointments to monitor iron levels and overall nutritional status.
   • Use growth charts and dietary assessments to ensure Lisa is meeting her nutritional needs.
4. Supplementation:
   • If dietary intake is insufficient, consider iron supplements under medical supervision.
   • Educate on proper use and potential side effects of iron supplements.
5. Addressing Specific Dietary Patterns:
   • If Lisa follows a vegetarian or vegan diet, provide guidance on plant-based sources of iron and how to combine them with vitamin C-rich foods to enhance absorption.
   • Discuss the role of fortified foods and the potential need for supplementation.

If Lisa was of Mediterranean descent, would you consider any additional screening for her and her family?

Additional Screening for Mediterranean Descent

If Lisa is of Mediterranean descent, consider screening for:

1. Thalassemia:
   • Perform hemoglobin electrophoresis or genetic testing to screen for thalassemia traits, as they are more prevalent in Mediterranean populations.
   • Family screening may be indicated to identify carriers and provide genetic counseling.
2. Glucose-6-Phosphate Dehydrogenase (G6PD) Deficiency:
   • Screen for G6PD deficiency, which is also more common in individuals of Mediterranean descent, as it can contribute to hemolytic anemia.

If Lisa lived in a remote community, how would you change your approach?

Approach for Lisa Living in a Remote Community

1. Access to Healthcare:
   • Ensure Lisa has regular access to healthcare providers, possibly through telemedicine or mobile health clinics.
   • Arrange for local health workers to provide ongoing monitoring and support.
2. Nutritional Support:
   • Work with community programs to provide access to nutritious foods, including food banks and local agriculture initiatives.
   • Consider government or NGO programs that provide fortified foods or supplements.
3. Education and Awareness:
   • Conduct community education sessions on nutrition and anemia prevention.
   • Provide culturally appropriate educational materials.
4. Coordination with Local Services:
   • Collaborate with local health services and community leaders to ensure a comprehensive approach to Lisa’s health and nutrition needs.

If she was an Aboriginal or Torres Strait Islander, how would that change your approach?

Cultural Sensitivity:

• Approach care with awareness of traditional beliefs and practices.
• Engage Aboriginal or Torres Strait Islander health workers.

Community Engagement:

• Collaborate with community leaders for health education and anemia prevention.
• Provide culturally appropriate resources.

Access to Services:

• Ensure regular health check-ups via Aboriginal Community Controlled Health Services (ACCHS).
• Advocate for better healthcare infrastructure in remote areas.

Addressing Social Determinants of Health:

• Address housing, education, and employment to improve health outcomes.
• Support food security programs to increase access to nutritious foods.

Holistic Care:

• Integrate traditional healing practices with medical treatment.
• Respect cultural practices and beliefs in health interventions.

Professionalism

How would you manage Lisa’s confidentiality if her mother insisted on staying during the consultation?

1. Initial Discussion with Mother Present:
   • Begin the consultation with both Lisa and her mother to gather initial information and ease any concerns.
2. Private Time with Lisa:
   • Politely request some time alone with Lisa to discuss confidential matters.
   • Explain the importance of respecting Lisa’s privacy and ensuring she has the opportunity to speak freely.
3. Reassure Confidentiality:
   • Reassure Lisa that her privacy will be respected and explain the limits of confidentiality, especially regarding safety concerns.

General practice systems and regulatory requirement

How would you assess Lisa’s capacity to consent for an iron infusion if this was needed? What would your approach be if the patient was a child who needed a transfusion of blood products, but the parents refused based on their beliefs?

Managing Lisa’s Confidentiality with Her Mother Present

1. Confidentiality Policies:
   • Follow the clinic’s policies on patient confidentiality and privacy.
   • Adhere to the Australian Privacy Principles under the Privacy Act 1988.
2. Documentation:
   • Document any discussions about confidentiality and ensure that patient records are kept secure.
3. Consent Procedures:
   • Obtain informed consent for any procedures or sharing of information.

General Practice Systems and Regulatory Requirements

1. Confidentiality Policies:
   • Follow the clinic’s policies on patient confidentiality and privacy.
   • Adhere to the Australian Privacy Principles under the Privacy Act 1988.
2. Documentation:
   • Document any discussions about confidentiality and ensure that patient records are kept secure.
3. Consent Procedures:
   • Obtain informed consent for any procedures or sharing of information.

Assessing Lisa’s Capacity to Consent for an Iron Infusion

1. Age and Maturity:
   • Consider Lisa’s age (15 years) and assess her maturity level.
   • Evaluate her understanding of the procedure, its benefits, risks, and alternatives.
2. Capacity Assessment:
   • Use tools and guidelines to assess her capacity to make informed decisions.
   • Ensure she understands the information provided, can weigh the consequences, and communicate her decision.
3. Informed Consent:
   • If Lisa demonstrates capacity, obtain her informed consent for the iron infusion.
   • If there are doubts about her capacity, involve a senior colleague or specialist for further assessment.

Approach if Parents Refuse a Blood Transfusion Based on Beliefs

1. Assess the Situation:
   • Understand the parents’ beliefs and concerns regarding the blood transfusion.
   • Explain the medical necessity and potential consequences of refusing the treatment.
2. Seek Alternatives:
   • Explore alternative treatments if available and appropriate.
   • Consult with a hematologist or relevant specialist for options.
3. Legal and Ethical Considerations:
   • If the refusal poses a significant risk to the child’s health or life, consider legal and ethical obligations to act in the child’s best interest.
   • Seek legal advice or involve child protection services if necessary.
4. Involve Ethics Committee:
   • If the situation is complex, consult the hospital’s ethics committee for guidance.
5. Document Thoroughly:
   • Document all discussions,

Procedural skills

What do you need to consider before planning for an iron infusion? How would you gain the skills to do a transfusion or an iron infusion? How would you manage and report an adverse reaction after an iron infusion?

Managing uncertainty

What factors would you consider while managing an asymptomatic patient with a haemoglobin level (Hb) of 80g/l?

• closely monitor and reassess frequently. If there are signs of worsening or if the patient becomes symptomatic, a blood transfusion may be considered.
• Blood transfusions may be used for decompensated patients (eg those with heart failure or angina) who are too unwell to wait for the predictable increase in haemoglobin from iron supplementation.
• When determining if a blood transfusion is indicated, it is important to consider the clinical situation, rather than making a decision based on haemoglobin and ferritin concentration alone.
• Severe anaemia caused by iron deficiency can be quite well tolerated, because it has developed slowly.
• In the very rare situation where a blood transfusion is indicated, iron supplementation is still necessary to prevent recurrence of iron-deficiency anaemia.

Identifying and managing the significantly ill patient

How would you manage a symptomatic patient with an Hb of 60? With an Hb of 100? Is there any other information apart from the Hb level that might change your management?

Identifying and Managing the Significantly Ill Patient

Management of a Symptomatic Patient with Hb of 60 g/dL

1. Immediate Assessment:
   • Symptoms: Assess for symptoms such as shortness of breath, dizziness, chest pain, fatigue, and pallor.
   • Vital Signs: Monitor heart rate, blood pressure, respiratory rate, and oxygen saturation.
2. Stabilization:
   • Oxygen Therapy: Administer oxygen if the patient is hypoxic.
   • IV Access: Establish intravenous access for fluid resuscitation and potential blood transfusion.
3. Transfusion:
   • Red Blood Cell Transfusion: Typically indicated for symptomatic patients with Hb ≤ 7 g/dL. Start with 1-2 units of packed red blood cells (PRBCs) and reassess.
   • Monitoring: Closely monitor for transfusion reactions and hemodynamic response.
4. Investigate Underlying Cause:
   • Blood Tests: Complete blood count (CBC), reticulocyte count, iron studies, liver function tests, renal function tests, and coagulation profile.
   • History and Physical Examination: Identify possible causes such as gastrointestinal bleeding, chronic disease, nutritional deficiencies, or hemolysis.
5. Supportive Care:
   • IV Fluids: Administer fluids if the patient is hypotensive.
   • Symptom Management: Address pain, nausea, or other distressing symptoms.

Management of a Symptomatic Patient with Hb of 100 g/dL

1. Assessment:
   • Symptoms: Evaluate for any symptoms that might suggest inadequate oxygen delivery (e.g., fatigue, dyspnea on exertion, palpitations).
   • Vital Signs: Monitor vitals to ensure stability.
2. Conservative Management:
   • Observation: If the patient is stable and mildly symptomatic, monitor closely without immediate transfusion.
   • Oral Iron Supplementation: If iron deficiency is identified, start oral iron therapy.
   • Dietary Advice: Encourage intake of iron-rich foods and vitamin C to enhance absorption.
3. Investigate Underlying Cause:
   • Conduct a thorough history and physical examination to identify any underlying cause of anemia.
   • Perform relevant laboratory tests to identify deficiencies or underlying conditions.

Factors Influencing Management Beyond Hb Level

1. Clinical Presentation:
   • Severity and type of symptoms (e.g., chest pain, shortness of breath, dizziness).
   • Signs of hemodynamic instability or cardiovascular compromise.
2. Underlying Conditions:
   • Chronic diseases such as chronic kidney disease, heart failure, or cancer.
   • Acute conditions like gastrointestinal bleeding or hemolysis.
3. Patient’s Overall Health:
   • Age and comorbidities.
   • Nutritional status and any ongoing treatments.
4. Response to Treatment:
   • Previous response to iron supplements or other treatments.
   • Presence of contraindications or adverse reactions to treatments like transfusions.

If Lisa was diagnosed with acute leukaemia and lived in a remote location, how would that change your management approach?

Management of Lisa with Acute Leukemia in a Remote Location

1. Initial Stabilization and Diagnosis:
   • Immediate Referral: Arrange urgent referral to a tertiary care center for further evaluation and treatment.
   • Stabilization: Provide supportive care, including blood transfusions if severely anemic, hydration, and infection management.
2. Coordination with Specialists:
   • Hematology Consultation: Liaise with a hematologist for guidance on managing acute leukemia.
   • Telemedicine: Use telemedicine services to facilitate specialist consultations and ongoing management.
3. Transport and Accessibility:
   • Medical Transport: Arrange for safe medical transport to a tertiary center, if required.
   • Local Support: Coordinate with local health services to ensure continuity of care and management during transit.
4. Ongoing Care in Remote Location:
   • Local Healthcare Providers: Train and support local healthcare providers to manage aspects of care that can be handled locally, such as transfusions and symptom management.
   • Community Health Programs: Engage community health programs to provide supportive care and monitoring.
5. Psychosocial Support:
   • Patient and Family Support: Provide psychological and social support to Lisa and her family, considering the emotional and logistical challenges of managing a serious illness in a remote location.

Blood Cell Indices in Acute Leukemia

Parameter	Typical Findings in Acute Leukemia	Description & Clinical Significance
White Blood Cells (WBC)	Leukocytosis or Leukopenia	Elevated WBC count is common, indicating high levels of immature blasts. Occasionally, WBC count may be low if bone marrow is severely infiltrated.
Blasts in Peripheral Blood	Present	Presence of immature cells (blasts) is a hallmark of acute leukemia.
Hemoglobin (Hb)	Low (Anemia)	Reduced hemoglobin levels due to bone marrow infiltration and decreased erythropoiesis.
Platelets	Thrombocytopenia	Reduced platelet count due to bone marrow infiltration and impaired megakaryocyte function.
Red Blood Cell (RBC) Indices	Normocytic, Normochromic Anemia	Red blood cells are usually of normal size (MCV) and normal color (MCHC) but in reduced numbers.
Reticulocyte Count	Low	Reflects reduced red cell production.
Peripheral Blood Smear	Blasts with high nuclear-to-cytoplasmic ratios, prominent nucleoli, possible Auer Rods (in AML)	Large numbers of immature white cells (blasts) with specific morphological features.
Bone Marrow Biopsy	Hypercellular marrow with high percentage of blasts	Essential for confirming the diagnosis.
Flow Cytometry	Characterization of immunophenotype	Differentiates between AML and ALL, important for diagnosis and treatment planning.
Cytogenetics and Molecular Studies	Specific chromosomal abnormalities and mutations	Important for prognosis and guiding targeted therapy.