Haematology Flashcards
What is polycythaemia?
A condition when you have too many RBC in the blood.
What is an example of true polycythaemia (primary)?
Myeloproliferative disorder i.e. rubra Vera polycythaemia
What is the gene that associated with rubra Vera polycythaemia?
Jack 2 gene
What is a cause of secondary true polycythaemia ?
Respiratory
CHD disease
Leads to tissue hypoxia and increased EPO release
What are some causes of apparent polycythaemia?
Dehydration
Diuretics
Alcohol consumption
What are
3 symptoms
3 signs of polycythaemia?
3 symptoms: red face, dizzy, headache, pruritus
3 signs: bleeding from mucosa, hepatomegaly and thrombosis of blood clots
What are the 2 things involved in polycythaemia treatment?
- Bone Marrow Suppression: chemotherapy
2. Aspirin: reduces the chance of clot formation
What factor is low in haemophilia A?
What would you see on blood tests in someone with haemophilia A ?
Factor 8
Low factor 8, prolonged APTT and PT time
How can haemophilia A present in neonates?
- Umbilical Cord bleeding
- Cephalohaematoma
- Iatrogenic bleeding
Later on you can soft tissue haematoma, joint bleeds and mucosal bleeds
How would you treat Haemophilia A
A. Long Term
B. Acutely
A. Factor 8 infusions
B. Desmopressin, Factor 8 and tranexamic acid
What is the factor which is low in Haemophilia B?
Factor 9
What process causes your reticulocytes to go down?
Anaemia
What two things cause reticulocytes levels to increase ?
Increased RBC destruction: sickle cell anaemia and haemolytic anaemia
Increased bone marrow activation
What would APTT and PT be like in a patient with VwF deficiency?
They would be normal
What is the purpose of VwF?
It stabilises Factor 8
What clotting factors does Vit K make?
2 7 9 and 10
Protein C and S
What will the APTT and PT appear like in a patient with Vit K deficiency
Prolonged APTT and PT
What are 5 causes of Iron deficiency anaemia?
- Hook worm
- Menstruation
- Coeliac and IBD
- Pregnancy
- Poor Diet
What are 3 symptoms and 3 signs of iron deficient anaemia?
3 symptoms: pallor, SOB, palpitations, pruritus and hair loss
3 signs: spoon shaped nails, dry hair, atrophic glottis and angular stomatitis
What will the blood tests in a patient with iron deficiency anaemia show?
- Low Ferritin
- Microcytic Anaemia
- High Transferrin
- High Total iron binding capacity
Macrocytic Anaemia:
What are 5 of causes of macrocytic anaemia?
- Pernicious Anaemia
- Vegan Diet
- Low Folate
- B12 deficiency
- Malabsorption
- Methotrexate and pregnancy
What are some symptoms of macrocytic anaemia that distinguish it from other anaemias?
- Peripheral Neuropathy
- Depression
- Weight Loss and SOB
What type of tongue will you have in low B12 or folate?
Smooth red and sore tongue
What investigations should you do in someone one you find out they have macrocytic anaemia?
Gastroscopy and look at anti-parietal cell antibodies
What are 3 causes of normocytic anaemia?
How do we treat it?
Haemorrhage, combined B12 and folate deficiency and chronic disease
Management: EPO enzyme
What is the inheritance of membranopathies?
Autosomal Dominant
What is the name given to a horizontal protein interaction?
What is the name given to a vertical protein interaction?
In relation to membranopathies?
- Elliptocytosis
2. Spherocytosis
What does parvo virus cause ?
Bone Marrow Supression
What is the inheritance pattern of Glucose 6 Phosphate Deficiency?
X linked inheritance
What are 3 triggers for someone with G6PD deficiency?
Broad Beans
Infections
Quinolines
How does G6PD deficiency present?
Jaundice
Haemolysis
Splenomegaly
Anaemia
When should you suspect G6PD deficiency?
In someone who has sudden onset jaundice, anaemia etc.
Around the same time as having an infection
What can we measure in the blood to confirm G6PD deficiency?
Measure the amount of NADPH
What is pernicious anaemia?
What is the average age of presentation?
What is it associated with?
Autoimmune disorder.
60
Other Autoimmune disorders
How does Pernicious Anaemia present?
It presents with fatigue, pallor and SOB. Proceeds to get Headaches, palpitations and jaundice.
What type of anaemia will you get in pernicious anaemia?
What antibody will be present in the blood?
Megloblastic anaemia
Intrinsic factor antibody
What is the management of someone with pernicious anaemia?
Folate and B12 supplements
Hydroxocobalamin
Will you get higher or lower values of reticulocytes in haemolytic anaemia?
Higher level of reticulocytes
What are the two types of haemolytic anaemia?
Explain both..
Intravascular: within the circulation
Extravascular: spleen, liver, bone marrow and reticuloendothelial system.
What will happen to lactate, urobilinogen and bilirubin and lactate dehydrogenase in Haemolytic anaemia?
They will all increase!!
Is intravascular haemolytic anaemia described as warm or cold?
What are some common causes?
What sign do you get ?
How do you manage the condition?
Intravascular haemolytic anaemia is described as cold
Common causes: lymphoma, autoimmune and drug reactions
You get very cold hands and feet
Management: prednisolone and blood transfusions/
Is extravascular haemolytic anaemia warm or cold?
What are some common causes of it?
How do you manage it?
Warm
Cancer, HIV and UC
Prednisolone and splenectomy
What are some inherited conditions that are associated with haemolytic anaemia?
G6PD
Sickle Cell
Thalassaemia
What are two drugs that can cause acquired haemolytic anaemia?
Penicillin
Quinine
How do you diagnose haemolytic anaemia? 4 ways
Thin and Thick Blood Film: rules out malaria
Auto antibody test: direct Coombs test
FBC
Reticulocytes, LDH, lactate dehydrogenase, bilirubin and urobilinogen will all be raised.
What is the management of sickle cell anaemia?
Hydroxycarbamide
Give blood transfusions
How do you diagnose Sickle Cell anaemia?
Hb electrophoresis
Blood film
FBC: microcytic anaemia
What are the 3 different types of Alpha Thalassaemia?
Four gene deletion: not compatible with life can cause hydrops fetalis
Three gene deletion: moderate splenomegaly and anaemia
Two gene deletion: with or without mild anaemia
What is the treatment for alpha thalassaemia?
Blood transfusions and Iron chelating agents via ascorbic acid
What is the inheritance of thalassaemia?
Autosomal Recessive
What are the 3 different types of Beta Thalassaemia? Describe the presentation of each:
Beta minor: mild microcytic anaemia
Beta intermedia: moderate anaemia, splenomegaly, recurrent leg ulcers and infections
Beta major: failure to thrive. Presents in first year of life. Severe anaemia, hepatosplenomegaly, extra medullary haematopoesis
How do you investigate thalassaemia?
Haemoglobin electrophoresis
What is the 4 step management of a patient in a sickle cell crisis?
- IV fluids + IV pain relief
- Oxygen
- Broad spectrum abx and a blood transfusion
What is hyperviscosity?
How can it present?
How do you manage it?
Hyper viscosity: is when you get too much Ig M in the blood
Cause: myeloma
Symptoms: headaches, SOB and blurred vision, fatigue and mucosal bleeding
Management: fluids and on call haematologist
How long do platelets last for ?
7 to 10 days
What controls platelet formation? 2 things
Thrombopoieten
Stimulates Megakaryocytes stimulates the release of platelets
What is released by platelet alpha granules?
VwF
Fibrinogen
PDGF
What is released by platelet dense granules?
Serotonin
Ca2+
What does thromboxane synthesis?
COX-1
What is an inhibitor of GPIIb/IIIa ?
Tirofiban
What are 3 examples of substances that lead to reduced platelet production?
Aplastic anaemia
Alcohol
Chemo
Low B12
What are 3 causes of increased platelet destruction?
Hyper spleen
DIC
Warfarin
What are the two treatment options for aplastic anaemia ?
Immunosupression
Bone Marrow Transplant
What are 3 conditions that lead to the destruction of platelets via consumption?
- DIC
- TTP
- Haemolytic Uraemic Syndrome
Who is thrombotic thrombocytopenia purpura more common in- males or females?
What are two main causes of it?
Females in 40s +
Two main causes: postpartum, cancer and congenital
What are the 3 pathophysiological steps that make up thrombotic thrombocytopenic purpura?
Small (thrombotic) clots in the vessels
Low platelets and
Red Blood Cell Destruction.
What gene is associated with TTP?
ADAMTS13
What are 5 ways that TTP can present?
Purple Petechiae Bruises and Thrombocytopenia
Renal impairment
Haemolytic anaemia
Fever
Abdominal and chest pain
Confusion/Headache
What levels will be high in TTP?
High reticulocytes
High bilirubin
High LDH
What is the 4 step management of TTP?
- Full plasma tranfusion
- High Dose Steroids
- Monoclonal Antibody
- Splenectomy
Idiopathic Thrombocytopenic Purpura:
What are the auto antibodies against ?
Glycoproteins IIb/IIIa
When does Idiopathic Thrombocytopenic Purpura present?
A. Children
B. Adults
A. Normally 2 weeks post viral infection. Common in those 5-7 yrs old
B. Autoimmune related, HIV or Hep C
What viruses will you want to test in a patient with ITP?
Hep C
HIV
How do you treat ITP in:
- Children
- Adults
- Prednisolone and platelet infusions
2. IV immunoglobulin and IV anti D
How does ITP present?
- Bruising
- Nose Bleeds
- Gastric Bleeds + pain
- Heavy Periods
What are 3 causes of DIC?
Sepsis
Complications of pregnancy: pre eclampsia or placental abruption
Malignancy: leukaemia or Cancer of pancreas, lung or prostate
What are 5 signs of DIC ?
Petechiae and Necrosis of the tissue
Bleeding
Fever
Signs of haemorrhage: low BP and sweaty
Confusion
What are blast cells?
Non functioning cells that build up in the bone marrow
What are the two ways you can classify Acute Lymphoblastic Leukaemia (ALL)?
- FAB system.
2. Immunological
What are 4 ways that ALL normally presents?
- Bilateral parotid infiltration
- Anaemia
- Infection
- Bleeding
What investigations would you like to do in a patient with suspected ALL?
FBC
Blood Film
Bone Marrow Biopsy
CXR and CT to look for lymphadenopathy
LP to check for CNS involvement
How do you treat ALL?
Blood and Platelet transfusions
Allopurinol and IV fluids to prevent tumour lysis syndrome
Chemotherapy: IV and into the spine
Prophylactic: anti- virals, antibacterials and anti-fungal
Bone Marrow transplantation
Who is AML more common in?
Adults
Which version of leukaemia do you get CNS involvement in?
ALL
What is the common presentation of AML leukaemia?
Gum hypertrophy
Bruising, anaemia and infection
Hepatomegaly
What are people with AML prone to get? ( 2 things)
- DIC
2. Rash
What leukaemia do you get Auer Rods seen on Blood film in?
Acute Myeloid Leukaemia (AML)
What form of leukaemia is associated with the Philadelphia chromosome?
Chronic Myeloid Leukaemia (CML)
What is the most common form of Leukaemia?
Chronic Lymphocytic Leukaemia (CLL)
What age is CLL common in?
60-70s
More common in men
How does CLL normally present?
Normally asymptomatic and just appears on routine FBC
However, it can also present with enlarged rubbery or tender nodes with hepato or splenomegaly
Describe the categorisation of CLL : i.e. Stage A B and C
Stage A no anaemia or thrombocytopenia Less than 3 enlarged nodes
Stage B no anaemia or thrombocytopenia. More than 3 enlarged nodes
Stage C anaemia or thrombocytopenia. Doesn’t matter about enlarged nodes.
How do you treat CLL?
Stage A: watch and wait
Stage B and Stage C: treat with chemotherapy and alkylating agents
Is haemolytic anaemia more common in CML or CLL ?
CLL need to fo a direct Coombs test
How does CML present?
Very much like lymphoma:
Fever, Fatigue. Sweats. Weight loss
Chronic and insidious
Anaemia, hepatomegaly and bruising
What is the management of CML?
Tyrosine kinase inhibitor (imatinib)
Allogenic stem cell transplant
What is Richter’s Transformation?
This is the progression of CLL to diffuse Large Cell B Lymphoma
Rule of 3s
1/3 never progress
1/3 progress with time
1/3 actively progress
What form of lymphoma are reed sternberg cells associated with?
Hodgkin’s Lymphoma
What is the Ann Arbor classification used to stage?
Hodgkin’s Lymphoma
What age groups is Hodgkin’s Lymphoma associated with?
It has two peaks
One peak: 20-35
Second peak- 50-70s
What are 3 RF of Hodgkin’s lymphoma?
Primary immunodeficiency: SLE
Secondary immunodeficiency: HIV or transplant
EBV virus
Which lymphoma is associated with an increased risk of SVOC?
Hodgkin’s Lymphoma
How does Hodgkin’s lymphoma present?
Enlarged rubbery non painful lymph nodes
Alcohol induced lymph node pain
Weight loss, fever and night sweats
SVCO
Explain the 4 stages of the Ann Arbor Classification:
1: confined to one lymph node
2. >2 lymph nodes same diaphragm side
- > 2 lymph nodes on different diaphragm sides
- Spread beyond lymph nodes
A or B (presence of weight loss + other systemic symptoms)
How do you manage Hodgkin’s Lymphoma?
Radiotherapy
Chemotherapy in those in stage 2 or higher or those with B symptoms + also give Rituximab
What leukaemia is associated with non Hodgkin’s lymphoma?
Chronic LL
Is non Hodgkin’s lymphoma more common in males or females?
What age does it typically present?
Males
65-75 yrs old
What virus is associated with non hodgkins associated with?
EBV
What is the difference between
Low Grade Follicular Non Hodgkin’s Lymphoma and
High Grade Diffuse Non Hodgkin’s lymphoma ?
Low grade: low growth and asymptomatic
High Grade: high growth rate and symptoms like weight loss, fever, night sweats
Describe the course of disease for low grade and high grade non Hodgkin’s lymphoma..
- Low Grade: common to get relapses. Normally do nothing. Treatment if you do is with rituximab and chemo
- High Grade: need to treat! With CHOP (chemo + pred), radiotherapy, rituximab and a stem cell transplant.
What type of proteins are paraproteins?
M proteins
What is MGUS?
Gammopathy of the blood with no symptoms
Present in those over 50
High chance of developing myeloma
How would you tell myeloma if you did electrophoresis?
- Large M spike
2. Lots of Ig M and Ig G
When does myeloma become multiple myeloma?
When it affects the bones
What is the pathophysiology of myeloma?
You get malignant cells in the bone marrow. Causes low RBC
Stimulates osteoclasts activity. Hypercalcaemia and lytic lesions. Can lead to spinal cord compression
Increased paraprotein leads to hyper-viscosity and can cause kidney damage.
Increased
What diagnostic tests would you do on myeloma?
Serum electrophoresis: show M spike, high Ig M and Ig G and Bence Jones Proteins
Blood Film: rouleaux of RBC
Skeletal Survery
Bone Marrow Biopsy
FBC
How do you treat myeloma?
- Chemotherapy
- Radiotherapy
- Bisphosphonates
- Plasmapheresis
- Thalidomide: anti angiotensin effects
What clinical presentation would you get with a patient who has P Vivax and P OVALE ?
Slowly developed anaemia and tender hepatomegaly
What symptoms would you get with P Malariae?
Mild Symptoms but for a longer amount of time
Malaise, Headache , Vomiting and Diarrhoea
How would P Falciparum present?
- Less marked fever
- Cerebral oedema, causes confusions, convulsions and coma
- Biggest cause of death
What test would you want to do in someone with suspected malaria ?
Thin and Thick Blood film
What is a good abx prophylactic for malaria?
Doxycycline
Chloroquine
How would treat a complicated form of malaria?
IV quinine dihydrochloride
What is the treatment for uncomplicated malaria ?
ORAL quinine + doxycycline
