Haematology Flashcards
What is Microcytic anaemia
This is anaemia associated with an MCV of <80fL
What are the causes of a Microcytic anaemia
Iron deficiency - Most common
- Blood loss - e.g. GI
- Reduced absorption - Small bowel disease
- Increased demands - Growth, pregnancy
- Reduced intake - Vegans
Anaemia of chronic disease - Microcytic anaemia associated with chronic disease
Thalassemia
Sideroblastic anaemia - Abnormality of haem synthesis
Lead poisoning
What are the signs and symptoms of a Microcytic anaemia
Asymptomatic
Fatigue
Faitness
Dyspnoea
Angina pectoris, intermittent claudication if coexistent arterial disease
Pale skin Pale mucous membranes Tachycardia Koilonychia (Spoon shaped) Cardiac failure Glossitis Angular stomatitis
How is Iron deficiency anaemia diagnosed
FBC: Low Hb, Low MCV
Serum iron: Low
Ferritin: Low
Transferrin: High
Transferring saturation: Low
Blood film:
- Microcytic
- Hypochromic
- Anisocytosis (variations in size) - Poikilocytosis (variations in shape)
Cause: OGD + Colonoscopy if: - Male - Post-menopausal women - Women with minimal menstrual loss
IgA-TTG - For coeliac (Malabsorption)
How is Iron deficiency anaemia treated
Treat underlying causes
Oral iron supplements
IM/IV iron if intolerant or poorly responsive to oral iron
What are causes of Anaemia of chronic disease
Chronic inflammatory disease - RA
Chronic infection - TB
Chronic malignancy
Due to cytokine release:
- Blocking erythropoietin production
- Blocking iron flow out of cells
- Increasing iron stores intracellularly
- Increasing red cell turnover
How is Anaemia of chronic disease diagnosed
CRP/ESR - Raised
FBC - Low Hb, Low or Normal MCV
Iron studies: Serum iron: Low Ferritin: High or Normal (Acute phase protein) Transferrin: Normal or Low Transferrin saturation: Normal
Outline Sideroblastic anaemia
This is a microcytic anaemia due to a problem with them synthesis
The blood film will be dimorphic and show hypochromic microcytic cells and there will be ring sideroblasts in the bone marrow
To manage it one would five Pyridoxine if it is an inherited form and if all else fails blood transfusions with chelation should be considered
Outline Lead poisoning
Lead poisoning will lead to:
Anorexia, N+V, Abdominal pain, constipation and peripheral nerve lesions
Signs: Blue gumline Peripheral verne lesions (Wrist or foot drop) Encephalopathy Convulsions Reduced consciousness
Finding:
Basophilic stippling
Management:
Dimercaprol
D-penicillinamine
What is Thalassemia
A group of genetic disorders characterised by reduced global chain synthesis - Autosomal recessive
5% of the world thought to be carriers - Greek, Turkish, Cypriot, South East Asain and Chinese
What are the 2 different types of Thalassemia
Alpha-Thalassemia - Reduced synthesis of alpha chain
Beta-Thalassemia - Reduced synthesis of beta chain
Outline the different types of Alpha-thalassemia
The are 4 alpha-globin genes on the chromosome
4 deletions = Haemoglobin Barts Hydrops Fetalis (interuterine death)
3 deletions = Haemoglobin H
- Microcytic Hypochromic anaemia
- Target cells and Heinz bodies.
- Hepatosplenomegaly - Lifelong transfusions
2 deletions = Alpha 0 thalassemia
- Microcytic Hypochromic RBCs - No anaemia
1 deletion = Alpha+ thalassemia
- Microcytic Hypochromic RBCs - No anaemia
Outline the different types of Beta-thalassemia
Major (Homozygous)
- Presents at 2-3 months with profound anaemia failure to thrive and recurrent infections.
- Bony abnormalities due to bone marrow hypertrophy
- Extramedullary hematopoiesis causes hepatosplenomegaly
Intermedia (Homozygous beta and alpha thalassemias)
- Moderate anaemia that doesn’t require transfusion.
- Splenomegaly
- Gallstones
- Bone deformities
- Recurrent leg ulcers
Minor/Trait (Heterozygous)
- Asymptomatic carrier
- Mild or absent anaemia can be confused with IDA
What are signs and symptoms of Thalassemia
Beta - Anaemia, presenting in first few months of life. Failure to thrive. Prone to infection
Beta thalassemia major/Intermedia Pallor Malaise Dyspnoea Mild jaundice Frontal bossin Thalassaemia facies Hepatosplenomegaly
What are the investigative findings in Thalassemia
FBC - Low Hb, MCV, MCH
Iron studies:
All normal
Blood film:
- Hypochromic microcytic anaemia
- Target cells
- Nucleated red cells
- Reticulocytosis
Hb electrophoresis: - Alpha: Low or normal HbA2 - Beta: High HbA2 and HbF Major: No/Minimal HbA Intermedia: Decreased HbA Trait: Mostly HbA
How is Thalassemia treated
Blood transfusions to keep Hb>10
Iron chelators to prevent iron overload
Bone marrow transplant - Curative
What is Macrocytic anaemia
This is anaemia associated with an MCV of >96fL
What are the 2 types of Macrocytic anaemia
Megaloblastic = Developing RBCs in bone marrow with delayed nuclear maturation relative to their cytoplasm - Defective DNA synthesis
Normoblastic
What causes of a megaloblastic Macrocytic anaemia
Vitamin B12 deficiency
Folate deficiency
Drugs: Methotrexate, Hydroxyurea, Azathioprine, Zidovudine
What causes of a normoblastic Macrocytic anaemia
- Pregnancy
- Alcohol excess + Liver disease
- Reticulocytosis (Haemolytic processes) - Red cell immature form
- Hypothyroidism
- Haematological disorders (Aplastic anaemia)
- Drugs (Hydroxycarbamide, Tyrosin kinase inhibitors)
- Multiple myeloma + Myelodysplasia
What are the signs and symptoms of Macrocytic anaemia
Asymptomatic
Fatigue
Faitness
Dyspnoea
Angina pectoris, intermittent claudication if coexistent arterial disease
Pale skin Pale mucous membranes Tachycardia Koilonychia (Spoon shaped) Cardiac failure Glossitis Angular stomatitis
How is Macrocytic anaemia diagnosed
FBC - Low Hb, High MCV
- Pancytopenia - Megaloblastic anaemia
- Different degrees off cytopenia - Myelodysplasia
- Exclude reticulocytosis
LFT - High bilirubin - Due to ineffective erythropoiesis or haemolysis
B12
Folate
TFT
Anti-parietal cell and Anti-intrinsic factor antibodies
Blood film:
Megaloblastic anaemia:
- Megaloblasts
- Hypersegmented neutrophil nuclei
Schilling test - Method testing for pernicious anaemia - B12 will only be absorbed when given with intrinsic factor
How is a Macrocytic anaemia treated
Pernicious anaemia - IM hydroxycobalamin for life
Folate deficiency - Oral folic acid
If B12 deficiency is present it must be treated before the folic acid deficiency
What are complications of Macrocytic anaemia
Pernicious anaemia gives and increased risk of gastric cancer
In pregnancy a folate deficiency increases risk of neural tube defects