Haematology Flashcards
What is the lifespan of a RBC?
3 months
What is the lifespan of a platelet?
10 days
What does anisocytosis on a blood film indicate?
Myelodysplastic syndrome
What do target cells on a blood film indicate?
Iron deficiency anaemia
Post-splenectomy
What do Howell-Jolly bodies on a blood film indicate?
Post-Splenectomy
Severe anaemia
What do reticulocytes on a blood film indicate?
Haemolytic anaemia
What do smudge cells on a blood film indicate?
Chronic Lymphocytic Leukaemia
Causes of Microcytic anaemia
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
Causes of Normocytic anaemia
Acute blood loss
Haemolytic anaemia
Aplastic anaemia
Hypothyroidism
Anaemia of chronic disease
Causes of Megaloblastic anaemia?
B12 deficiency
Folate deficiency
Causes of Normoblastic, macrocytic anaemia?
Reticulocytosis (e.g. due to Haemolysis)
Alcohol
Hypothyroidism
Liver disease
Drugs (e.g. Azathioprine)
Possible underlying causes for iron deficiency anaemia?
- Inadequate dietary iron
- Inadequate iron absorption (e.g. Coeliac)
- Increased iron requirements (e.g. pregnancy)
- Bleeding (e.g. heavy periods or GI cancer)
Where is intrinsic factor produced?
Parietal cells of the stomach
In pernicious anaemia what is the order of treatment steps and why?
You should treat the B12 deficiency first as treating low folate first can cause Subacute combined degeneration of the cord
What are the types of autoimmune haemolytic anaemia and which is more common?
Warm types AHA (most common)
Cold type AHA
What is the management for autoimmune haemolytic anaemia?
- Blood transfusions
- Steroids (Prednisolone)
- Rituximab
- Splenectomy
What are some inherited causes of haemolysis?
- Hereditary spherocytosis
- Hereditary elliptocytosis
- Thalassaemia
- Sickle cell anaemia
- G6PD deficiency
What is the inheritance pattern of Thalassaemia
Autosomal recessive
What is the diagnostic test of Thalassaemia?
Haemoglobin electrophoresis
Why do you monitor serum ferritin in thalassaemia?
Risk of Iron overload
What is the inheritance pattern of Sickle cell disease?
Autosomal recessive
Affecting the gene for Beta-Globin on Chromosome 11
What is the medication used to stimulate HbF production in sickle cell anaemia?
Hydroxycarbamide
What are some complications of Sickle Cell disease?
- Anaemia
- Increased risk of infection
- CKD
- Sickle cell crisis
- Acute chest syndrome
- Stroke
- Avascular necrosis in large joints such as the hip
- Pulmonary hypertension
- Gallstones
- Priapism
What is a vaso-occlusive crisis?
Most common type of sickle cell crisis caused by the sickle-shaped red blood cells clogging capillaries causing distal ischaemia.
What is a splenic sequestration crisis?
Splenic sequestration crisis is caused by red blood cells blocking blood flow within the spleen. It causes an acutely enlarged and painful spleen. Blood pooling in the spleen can lead to severe anaemia and hypovolaemic shock.
What is an aplastic crisis?
Aplastic crisis describes a temporary absence of the creation of new red blood cells. It is usually triggered by infection with parvovirus B19.
It leads to significant anaemia (aplastic anaemia).
What is acute chest syndrome?
Acute chest syndrome occurs when the vessels supplying the lungs become clogged with red blood cells. A vaso-occlusive crisis, fat embolism or infection can trigger it.
How does acute chest syndrome present?
Acute chest syndrome presents with fever, shortness of breath, chest pain, cough and hypoxia. A chest x-ray will show pulmonary infiltrates.
What is the management for acute chest syndrome?
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
What type of lymphoma is most associated with EBV?
Burkitts Lymphoma
What type of lymphoma is most associated with H. Pylori in the stomach?
MALT lymphoma
What type of lymphoma is most associated with a rapidly-growing painless mass?
Diffuse Large B-cell lymphoma
What are the investigations for Multiple Myeloma?
- Serum protein electrophoresis
- Serum-free light chain assay
- Urine Bence-Jones protein
What is the description of a skull on XRay in multiple myeloma?
‘Raindrop skull’ `
What is the first line chemotherapy for Multiple Myeloma?
- Bortezomid
- Thalidomide
- Dexamethasone
What is the presentation of polycythaemia vera?
Itching
Excessive Sweating
Bruising
Conjunctival plethora
‘‘Ruddy Complexion’’
Splenomegaly
What is the proliferating cell line in Polycythaemia vera?
Erythroid cells
What is the key gene mutation in polycythaemia vera?
JAK2
What is the potential malignant transformation associated with polycythaemia vera?
Acute Myeloid Leukaemia
What are some causes of abnormal or prolonged bleeding?
- Thrombocytopaenia
- Von Willebrand disease
- Haemophilia A or B
- Disseminated intravascular coagulopathy
What are the treatments for Von Willebrand disease?
- Desmopressin
- Von Willebrand factor infusion
- Factor VIII Infusion
What difference in leg circumference is significant in DVT?
> 3cm
What is anisocytosis?
Red blood cells of different sizes.
Diagnosed using RDW
What is the management of a minor allergic reaction during a blood product transfusion?
Temporarily stop the transfusion, antihistamine and monitor
What is Haemophilia B a deficiency of?
Factor 9
What is Haemophilia A a deficiency of?
Factor 8
What is Richter’s transformation?
Occurs when leukaemia cells enter the lymph node and change into a high-grade, fast-growing non-Hodgkin’s lymphoma.
What is the pathophysiology of Acute haemolytic transfusion reaction?
Results from a mismatch of blood group (ABO) which causes massive intravascular haemolysis. This is usually the result of red blood cell destruction by IgM-type antibodies
What are the symptoms of acute haemolytic transfusion reaction and how quickly do they occur?
Symptoms begin minutes after the transfusion is started and include
- Fever
- Abdominal pain
- Chest pain
- Agitation
- Hypotension
What is the treatment of acute haemolytic transfusion reaction and the complications if not started?
- Treatment should include immediate transfusion termination, generous fluid resus with 0.9% NaCl
- Complications include disseminated intravascular coagulation and renal fsilure
What are the features of transfusion-related acute lung injury (TRALI)?
Characterised by the development of hypoxaemia/ARDS within 6 hours of transfusion
- Hypoxia
- Pulmonary infiltrates on CXR
- Fever
- Hypotension
What is TACO?
Transfusion-associated circulatory overload
What is Hereditary spherocytosis?
- Most common hereditary haemolytic anaemia in people of northern European descent
- Autosomal dominant defect of red blood cell cytoskeleton
- Normal biconcave disc shape is replaced by a sphere-shaped red blood cell
What is the presentation of hereditary spherocytosis?
- Failure to thrive
- Jaundice, gallstones
- Splenomegaly
- Aplastic crisis precipitated by parvovirus infection
- Degree of haemolysis variable
- MCHC elevayed
What is the diagnostic test for Hereditary Spherocytosis?
EMA binding test
What condition is associated with ‘tear-drop’ poikilocytes?
Myelofibrosis
What do hypersegmented neutrophils indicate?
Megaloblastic anaemia
What is the inheritance of G6PD deficiency?
X-linked recessive fashion
What drugs may cause haemolysis in G6PD deficiency?
- Anti-malarials: primaquine
- Ciprofloxacin
- Sulph-group drusg such as sulphonamides and sulphasalazine, sulfonylureas
What is the management for Essential thrombocytosis?
Hydroxyurea (hydroxycarbamide) is widely used to reduce platelet count
What would you see on a blood film in CLL?
Smudge cells also known as smear cells
What is the pathophysiology of polycythaemia vera?
It is a myeloproliferative disorder caused by clonal proliferation of a marrow stem cell leading to an increase in red cell volume, often accompanied by overproduction of neutrophils and platelets.
What are the features of Polycythaemia vera?
- Pruritus, especially after a hot bath
- Splenomegaly
- Hypertension
- Hyperviscosity
- Haemorrhage
- Low ESR
What tests should be performed when trying to identify polycythaemia vera?
FBC/Film
-JAK2 mutation
- Serum ferritin
- U&E, LFT
What are the features of lead poisoning?
- Abdominal pain
- Peripheral neuropathy (mainly motor)
- Neuropsychiatric features
- Fatigue
- Constipation
- Blue lines on gums
What conditions are associated with target cells?
- Sickle-cell
- Thalassaemia
- Iron-deficiency anaemia
- Hyposplenism
- Liver disease
What Chromosome is associated with CML?
The Philadelphia chromosome.
Due to a translocation betweent eh long arm of chromosome 9 and 22.
What is the first-line management of CML?
Imatinib is now considered first-line treatment
- Hydroxyurea
- Interferon-alpha
- Allogenic bone marrow transplant
What is the pathophysiology of ITP?
Antibodies are directed against the glycoprotein IIb/IIIa or Ib-V-IX complex
What is the underlying aetiology of factor V Leiden?
Activated protein C resistance
What is ALL a proliferation of?
Uncontrolled proliferation of immature lymphoid precursor cells within the bone marrow.
This unregulated cell growth leads to bone marrow failure and an increase presence of lymphoblasts in the peripheral blood
What are some symptoms of ALL?
- Of marrow failure, such as fatigue, abnormal bleeding/bruising and infections
- From organ infiltration, such as bone pain
What would you expect to see on blood film and bone marrow in ALL?
- Blast cells
- Lymphoblasts are typically large with nucleoli, with very little cytoplasm and no granularity
What are some poor prognostic factors in ALL?
- Age <1 year and >10 years
- Male
- WCC >50 x10 9/L (higher pretreatment WCC predicts poor prognosis)
- CNS disease
- Poor Cytogenic features, such as t(9,22)
- T-ALL - prognosis is worse than for B-ALL
- Incomplete response to therapy
What cells does AML affect?
Uncontrolled proliferation of myeloid precursors in the bone marrow, leading to bone marrow failure and the accumulation of immature WBC in the blood
What is the findings on Bone marrow biopsy in AML?
- Hypercellular bone marrow
- The presence of blasts (>50%)
- Sometimes Auer rods
What is the prognosis of someone with AML?
- Death typically occurs within 2 months without treatment
- Prognosis is poor even if undergoing treatment
- 3-year survival rate 20%
What are the top causes of raised ferritin?
- Inflammation
- Liver disease
- Iron supplementation
- Haemochromatosis
What name is given to immature red blood cells that still contain RNA material, giving a “mesh-like” appearance inside the cell?
Reticulocytes
What does a high percentage of reticulocytes indicate?
- A rapid turnover of red blood cells (e.g., with haemolytic anaemia) where the bone marrow is actively trying to replace lost cells
What name is given to undifferentiated stem cells that can transform into many blood cell types?
Pluripotent haematopoietic stem cell
What term refers to fragments of red blood cells on a blood film and what type of anaemia does this indicate?
- Schistocytes
- Haemolytic anaemia
Where in the GI tract is iron absorbed?
The duodenum and the Jejunum
Where do platelets originate?
They are made by megakaryocytes which develop from the myeloid stem cells
How do you diagnose a vaso-occlusive crisis?
Clinically, no tests needed
What cancer is associated with Reed-Sternberg cells?
Hodgkins lymphoma
What causes an aplastic crisis in patients with hereditary spherocytosis?
Parvovirus
What is the reversal agent for Dabigatran?
Idarucizumab
What is the reversal agent for Heparin
Protamine
What would a biopsy of Burkitt’s lymphoma show?
'’Starry sky’’ Appearance
What is the presentation of Transfusion associated cardiac overload?
Hypertension, raised JVP, Afebrile, S3 present
What drug is useful prophylactically for sickle cell anaemia?
Hydroxyurea
Why and when do we use irradiated blood products?
Used in immunocompromised patients.
They have depleted T-Lymphocyte numbers to reduce the risk of Transfusion graft versus host disease
Which groups of patients require CMV- negative blood?
- Intra-uterine transfusions
- Neonates up to 28 days post expected date of delivery
- Pregnancy
What is Lugano stage 1?
Involvement of a single lymph node region or a single extralymphatic organ or site
What is Lugano Stage 2?
Involvement of two or more lymph node regions on the same side of the diaphragn or localised involvement of an extralymphatic organ on the SAME side of the Diaphragm
What is Lugano Stage 3?
Involvement of lymph node regions on both sides of the diaphragm which may also be accompanied by localised involvement of an extra lymphatic organ or site, involvement of the spleen or both
What is Lugano Stage 4?
Diffuse or disseminated involvement of one or more extra lymphatic organs
What are the signs of Polycythaemia vera?
Raised haemoglobin
Plethoric appearance
Pruritus
Splenomegaly
Hypertension
What kind of anaemia can Methotrexate result in?
Megaloblastic macrocytic anaemia secondary to folate deficiency
How do you treat TACO?
IV Furosemide
What can trigger haemolysis in G6PD deficiency?
sulph- drugs: sulphonamides, sulphasalazine and sulfonylureas can trigger haemolysis
