Haematology Flashcards
Pluripotent haematopoietic stem cell can differentiated in to which 3 cells?
Myeloid stem cells
Lymphoid stem cells
Dendritic cells (via different intermediate stages)
what is the lifespan of RBC?
4 months (120days)
what is the lifespan of platelets and their normal count ?
ten days
150 – 450 x 109/L
define anaemia
low concentration of haemoglobin in the blood.
what are the normal ranges for Haemoglobin and MCV for male and female
The mean cell volume (MCV) refers to the size of the red blood cells
what kind of anaemia can be shown base on the MCV?
Microcytic anaemia (low MCV)
Normocytic anaemia (normal MCV)
Macrocytic anaemia (large MCV)
What are the cause of microcytic anaemia?
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
what is chronic anaemia can associate with what other disease condition?
*Explain why
* treatment
Chronic kidney disease because it reduced the production of erythropoietin by the kidneys (hormone that responsible for stimulating RBC production)
Treatment –> erythropoietin
what are the causes for normocytic anaemia
There are 3 As and 2 Hs for normocytic anaemia:
A – Acute blood loss
A – Anaemia of chronic disease
A – Aplastic anaemia
H – Haemolytic anaemia
H – Hypothyroidism
What is megaloblastic and normoblastic and what are the causes of them?
megaloblastic and normoblastic are part of the Macrocytic anaemia
Megaloblastic anaemia is caused by:
- B12 deficiency
- Folate deficiency
Normoblastic macrocytic anaemia is caused by:
Alcohol
Reticulocytosis (usually from haemolytic anaemia or blood loss)
Hypothyroidism
Liver disease
Drugs, such as azathioprine
Define reticulocytosis
increased concentration of reticulocytes (immature red blood cells)
what are the symptoms of anaemia?
Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions, such as angina, heart failure or peripheral arterial disease
For iron deficiency anaemia:
- Pica (dietary cravings for abnormal things, such as dirt or soil)
- Hair loss
Signs you can see in patient with anaemia
Generic signs of anaemia include:
Pale skin
Conjunctival pallor
Tachycardia
Raised respiratory rate
Signs of specific causes of anaemia include:
- Koilonychia refers to spoon-shaped nails and can indicate iron deficiency anaemia
- Angular cheilitis can indicate iron deficiency anaemia
- Atrophic glossitis is a smooth tongue due to atrophy of the papillae and can indicate iron deficiency anaemia
- Brittle hair and nails can indicate iron deficiency anaemia
- Jaundice can indicate haemolytic anaemia
- Bone deformities can indicate thalassaemia
- Oedema, hypertension and excoriations on the skin can indicate chronic kidney disease
What investigations you can do in the blood test?
- Colonoscopy and oesophagogastroduodenoscopy (OGD) are indicated for unexplained iron deficiency anaemia
- Bone marrow biopsy is indicated for unexplained anaemia or possible malignancy (e.g., leukaemia or myeloma)
What causes iron deficiency
mainly Blood loss
> menorrhagia (heavy periods)
> blood loss in the GI
Insufficient dietary iron (e.g., restrictive diets)
Reduced iron absorption (e.g., coeliac disease)
Increased iron requirements (e.g., pregnancy)
Loss of iron through bleeding (e.g., from a peptic ulcer or bowel cancer)
explain the mechanisms of reduce iron adsorption in the stomach
Iron is mainly absorbed in the duodenum and jejunum
Inflammation of the duodenum / jejunum (coeliac disease or Crohn’s disease) can also reduce iron absorption.
Medications i.e. PPI can intefere with ron absorption
requires the acid from the stomach to keep the iron in the soluble ferrous (Fe2+) form. When the stomach contents are less acidic, it changes to the insoluble ferric (Fe3+) form.
what to test for patient that have iron deficiency ?
serum iron
serum ferritin
haemoglobin and haematocrit
FBC
MCV
total iron-binding capacity
MCH
MCHC
what could iron overload result in ?
Haemochromatosis
Iron supplements
Acute liver damage (the liver contains lots of iron)
how to manage patient with iron deficiency
Oral iron (e.g., ferrous sulphate or ferrous fumarate)
Iron infusion (e.g., IV CosmoFer)
Blood transfusion (in severe anaemia)
side effect of using oral iron and iron infusions
Oral iron
rise in haemoglobin of 20grams in the first month
constipation &black stools
iron infusions
small risk of allergic reaction and anaphylaxis
avoided during infection
Define haemolytic anaemia
destruction of red blood cells (haemolysis), resulting in a low haemoglobin concentration (anaemia)
Features of haemolytic anaemia
- Anaemia
fatigue
SOB
dizziness - Splenomegaly (the spleen becomes filled with destroyed red blood cells)
- Jaundice (bilirubin is released during the destruction of red blood cells)
what investigation for haemolytic anaemia patient?
Full blood count shows a normocytic anaemia
Blood film shows schistocytes (fragments of red blood cells)
Direct Coombs test is positive in autoimmune haemolytic anaemia (not in other types)
What are the causes of haemolytic anaemia?
Hereditary spherocytosis
Hereditary elliptocytosis
Thalassaemia
Sickle cell anaemia
G6PD deficiency
What are the key findings in hereditary spherocytosis?
What is the treatment for it as well?
Findings:
Raised Mean corpuscular haemoglobin concentration (MCHC) on a full blood count
Raised reticulocyte count
spherocytes on a blood film
TREATMENT:
folate supplementation, blood transfusion
possible splenectomy / cholecystectomy
The different of hereditary spherocytosis and Hereditary elliptocytosis
both are autosomal dominant
have same presentation and management
hereditary spherocytosis - fragile, sphere-shaped RBC
Hereditary elliptocytosis - fragile, ellipse-shaped
1) What is G6PD gene do?
2) what are the factors that G6PD cause haemolytic anaemia?
3) Presentation of G6PD deficiency
4) Investigation of G6PD deficiency
1) enzyme that’s responsible for protecting the cell from oxidative damage
2) infection, drugs, fava beans, medications (ciprofloxacin, fulfonylureas)
3) Jaundice, gallstones, anaemia, splenomegaly & Heinz bodies
4) G6PD enzyme assay
a male patient that turns jaundiced and becomes anaemic after eating fava beans (broad beans), developing an infection or taking antimalarials.
What is the suspecting diagnosis?
G6PD deficiency
What is pernicious anaemia
aka b12 deficiency
very large, immature, nucleated erythrocytes. this is the types of anaemia that is usually lack of Folic acid (B9) and B12 (cyanocobalamin)
Pathophysiology of pernicious anaemia
due to lack of intrinsic factor (IF) produced in the glands of the gastric mucosa
–> can’t bind between B12 and IF
–> can’t get absorb in the ilium and into the bone marrow
–> B12 promotes maturation of erythrocytes…..can’t anymore and lead to megaloblastic erythrocytes
signs and symptoms of Vitamin B12 deficiency
paraesthesia
SOB
fatigue
weakness
malabsorption
loss of vibration sense
loss of proprioception
visual change
mood and cognitive changes
loss of muscle control / coordination (ataxia)
What investigations would you do to find pernicious anaemia?
***Intrinsic factor antibodies (first-line investigation)
FBC
Blood film
Haematinics
Lactate dehydrogenase (LDH)
LFTs
Schilling’s test
In some cases of a patient with co-existing folate and B12 deficiency, What will be the management of it first hand?
B12 deficiency: (however –>need to be treated first***) to prevent neurological complications
Initially administer hydroxocobalamin 1 mg intramuscularly three times a week for 2 weeks.
Folate deficiency:
Prescribe oral folic acid 5 mg daily — in most people, treatment will be required for 4 months.
What is Thalassaemia?
genetic defect in the protein chains that make up haemoglobin. causing haemolytic anaemia due to more fragile and break down easily of RBC
Features of thalassaemia
Microcytic anaemia (low mean corpuscular volume)
Fatigue
Pallor
Jaundice
Gallstones
Splenomegaly
Poor growth and development
investigation on thalassemia
Low MCV –> Microcytic anaemia
FBC
Raise ferritin –> iron overload
haemoglobin electrophoresis
DNA testing
what is iron overload caused from and the complication of it?
cause from:
- increased iron absorption in the GI
- Blood transfusions
Complications:
Liver cirrhosis
Hypogonadism
Hypothyroidism
Heart failure
Diabetes
Osteoporosis
Management of thalassaemia
monitoring
blood transfusion
iron chelation
splenectomy
how can you tell the differences of Beta-thalassaemia minor, intermedia and major?
Minor
They have one abnormal and one normal gene
intermedia
have two abnormal copies of the beta-globin gene
Major
homozygous for the deletion genes. They have no functioning beta-globin genes
what kind of mutation is causing in sickle cell disease
point mutation in the chromosome 11 beta-globin gene.
What are the complications of Sickle cell disease
Anaemia
Increased risk of infection
Chronic kidney disease
Sickle cell crises
Acute chest syndrome
Stroke
Avascular necrosis in large joints such as the hip
Pulmonary hypertension
Gallstones
Priapism (painful and persistent penile erections)
1) what is sickle cell crisis
2) how many types are there?
3) what are the triggering factors
4) what are the management ?
1) acute exacerbations caused by sickle cell disease
2) vaso-occlusive crisis; splenic sequestration crisis; aplastic crisis; acute chest syndrome
3) dehydration, infection, stress or cold weather.
4) no specific treatment. They are managed supportively
what is the most common type in the sickle cell crisis?
Vaso-occlusive crisis
tell me the causes, presentation and management of vaso-occlusive crisis
causes
sickle-shaped red blood cells clogging capillaries, causing distal ischaemia.
Presentation
pain and swelling in the hands or feet but can affect the chest, back, or other body areas. It can be associated with fever. Priapism (urological emergency)
Management
Priapism –> aspirating blood from penis
tell me the causes, presentation and management of Splenic sequestration crisis
causes
RBC blocking blood flow within the spleen
Presentation
spleen enlarged and painful, severe anaemia & hypovolaemic shock
Medical emergency
Management
blood transfusion & fluid resus& treat anaemia and shock, splenectomy
what is the causes & management of Aplastic crisis
a temporary absence of the creation of new red blood cells
causes
Infection with parvovirus B19
management
blood transfusion
What causes acute chest syndrome and what are the management
vessels supplying the lungs become clogged with red blood cells
causes
vaso-occlusive crisis, fat embolism or infection
Management (*Medical Emergency)
Analgesia
Good hydration (IV fluids may be required)
Antibiotics or antivirals for infection
Blood transfusions for anaemia
Incentive spirometry using a machine that encourages effective and deep breathing
Respiratory support with oxygen, non-invasive ventilation or mechanical ventilation
How does hydroxycarbamide works on sickle cell disease?
stimulating the production of fetal haemoglobin (HbF)
It reduces the frequency of vaso-occlusive crises, improves anaemia and may extend lifespan.
How does Crizanlizumab works on Sickle cell disease?
monoclonal antibody that targets P-selectin (adhesion molecules found on endothelial cells.
It prevents red blood cells from sticking to the blood vessel wall and reduces the frequency of vaso-occlusive crises.
what are the general management of sickle cell disease
avoid triggers for crises
vaccination
antibiotic prophylaxis (penicillin V)
Medication
Blood transfusion
Bone marrow transplant
what are the 2 medication that used in patient with Sickle cell disease?
Hydroxycarbamide
Crizanlizumab
What are the 4 types of leukaemia?
Acute myeloid leukaemia (rapidly progressing cancer of the myeloid cell line)
Acute lymphoblastic leukaemia (rapidly progressing cancer of the lymphoid cell line)
Chronic myeloid leukaemia (slowly progressing cancer of the myeloid cell line)
Chronic lymphocytic leukaemia (slowly progressing cancer of the lymphoid cell line)
link these question to what leukaemia it can lead to:
1) has 3 phase and it’s associated with the Philadelphia chromosome
2) associated with haemolytic anaemia, Richter’s transformation and smudge cells
3) transformation from a myeloproliferative disorder and is associated with Auer rods
4) common in children and associated with Down syndrome
1) CML
2) CLL
3) AML
4) ALL
What is pancytopenia?
combination of low red blood cells (anaemia), white blood cells (leukopenia) and platelets (thrombocytopenia).
Presentation of Leukaemia
Fatigue
Fever
Pallor due to anaemia
Petechiae or bruising due to thrombocytopenia
Abnormal bleeding
Lymphadenopathy
Hepatosplenomegaly
Failure to thrive (children)
what are the 3 non-blanching lesion?
Petechiae are less than 3 and caused by burst capillaries
Purpura are 3 – 10mm
Ecchymosis is larger than 1cm
what are the top differentials for a non-blanching rash?
Leukaemia
Meningococcal septicaemia
Vasculitis
Henoch-Schönlein purpura (HSP)
Immune thrombocytopenic purpura (ITP)
Thrombotic thrombocytopenic purpura (TTP)
Traumatic or mechanical (e.g., severe vomiting)
Non-accidental injury
What are the investigations to diagnose WBC cancer?
FBC (first investigation)
blood film
Lactate dehydrogenase (LDH)
Bone marrow biopsy
CT and PET scans
Lymph node biopsy
Genetic tests & immunophenotyping
what are the general management for leukaemia
chemotherapy & target therapies
radiotherapy
bone marrow transplant
surgery
* treat by oncology and haematology multi-disciplinary team * so for you just refer
Define Lymphoma
> Lymphoma is a type of cancer affecting the lymphocytes inside the lymphatic system.
proliferate inside the lymph nodes,
causing the lymph nodes to become abnormally large (lymphadenopathy).
what are the different types of lymphoma?
Hodgkin’s lymphoma (a specific disease)
Non-Hodgkin’s lymphoma (which includes all other types)
presentation of Lymphoma
Lymphadenopathy - enlarged lymph node in neck, axilla/ inguinal region
(non-tender and fell firm or rubbery)
Fatigue
Itching
Cough
Shortness of breath
Abdominal pain
Recurrent infections
Red Flags
* Fever
* Weight loss
* Night sweats
investigations on Lymphoma
Lymph node biopsy (*standard)
Reed-Sternberg cells are the characteristic finding from a biopsy of Hodgkin’s lymphoma
CT, MRI, and PET scans
what is the system that used for Hodgkin’s and non-Hodgkin’s lymphoma
Lugano Classification
Stage 1: Confined to one node or group of nodes
Stage 2: In more than one group of nodes but on the same side of the diaphragm (either above or below)
Stage 3: Affects lymph nodes both above and below the diaphragm
Stage 4: Widespread involvement, including non-lymphatic organs, such as the lungs or liver
management of Hodgkin’s lymphoma
chemotherapy & radiotherapy
