Haematology Flashcards
What is polycythaemia rubra vera?
Polycythaemia vera is a neoplasm of the bone marrow which results in the production of excessive red blood cells.
How does polycythaemia rubra vera present?
The classic symptom of this condition is intense itching which usually occurs after exposure to hot water or hot and humid weather. It is believed that this is due to abnormal histamine or prostaglandin production.
Due to the excess of red cells, patients who suffer from polycythaemia vera are predisposed to blood clots which could explain the deep vein thrombosis this patient had. Also in polycythaemia vera, roughly 20% of patients will also suffer from gouty arthritis.
How does DIC present on blood film?
The combination of low platelets, increased clotting time and raised fibrin degradation products (FDPs) in this setting make DIC the most likely diagnosis.
Describe the pathophysiology of DIC in sepsis
One critical mediator of DIC is the release of a transmembrane glycoprotein known as tissue factor. Tissue factor (TF) is present on the surface of many cell types and is not normally in contact with the general circulation, but is exposed to the circulation after vascular damage. For example, TF is released in response to exposure to cytokines (particularly interleukin 1), tumour necrosis factor, and endotoxin. This plays a major role in the development of DIC in septic conditions.
How does acute chest syndrome present?
This condition is a severe complication of sickle cell anaemia, characterised by the presence of new pulmonary infiltrates on chest x-ray, accompanied by symptoms such as fever, cough, sputum production, dyspnoea or hypoxia. The patient’s history of sickle cell anaemia, presenting symptoms and radiological findings are all indicative of this diagnosis.
How does DIC present on FBC and coag screen?
↓ platelets
↓ fibrinogen
↑ PT & APTT
↑ fibrinogen degradation products
How can we lower the risk of tumour lysis syndrome?
Giving allopurinol or rasburicase prior to chemotherapy is a way to reduce the risk of developing tumour lysis syndrome, as they stop/reduce uric acid from crystalising which is released from cancer cells
How does polycythaemia rubra vera present?
Pruritis (worst after taking showers or hot baths).
Tingling, burning, and numbness in arms, hands, and feet.
Headaches and lethargy.
Splenomegaly.
Elevated haemoglobin on full blood count.
What is the initial tx for polycythaemia rubra vera?
Phlebotomy or venesection is performed to remove a patient’s blood in order to reduce the haemoglobin levels in patients. Initially, patients may need this procedure every week; however when the condition is under control, most patients require venesection every 6 to 12 weeks.
What is a sequestrian crisis?
Sequestration crises
sickling within organs such as the spleen or lungs causes pooling of blood with worsening of the anaemia
associated with an increased reticulocyte count
How do we treat post-thrombotic syndrome?
However, once post-thrombotic syndrome has developed compression stockings are a recommended treatment. Other recommendations including keeping the leg elevated.
What must we counsel patients on when starting tamoxifen
Tamoxifen increases the risk of VTE + endometrial cancer
How do we manage sickle cell crisis?
The main components for managing sickle cell crisis should be analgesia, oxygen, and IV fluids. You can also consider antibiotics if you suspect an infection, and transfusion if the Hb is low
What mode of inheritance produces sickle cell anaemia?
Autosomal recessive
What is the reversing agent for dabigatran?
Idarucizumab
What do we use to reverse heparin?
Protamine is used to reverse the heparin effect in those on heparin who are bleeding or need an urgent operation, however, it is ineffective in reversing DOAC effect.
How does multiple myeloma present?
‘CRAB’ features of multiple myeloma = hyperCalcaemia, Renal failure, Anaemia (and thrombocytopenia) and Bone fractures/lytic lesions
What do you see on blood film in multiple myeloma?
Rouleaux formation
Why may you see a raised reticulocyte count in sickle cell?
Sickle cell disease causes a normocytic anaemia with raised reticulocyte count - due to haemolysis
What does a high TIBC reflect?
Low iron stores
(Total iron binding capacity)
What does cryoprecipitate contain?
factor VIII, fibrinogen, von Willebrand factor and factor XIII
Where do we use cryoprecipitate?
Patients with haemophilia A are genetically deficient in factor VIII, which is what the cryoprecipitate would be used to replace when actively haemorrhaging
Where do you see Auer rods on blood film?
Auer rods on blood film strongly suggests acute promyelocytic leukaemia
Where do you see smear cells on blood film?
Smear cells are typically seen in chronic lymphocytic leukaemia (CLL). They are remnants of cells that have no identifiable plasma membrane or nuclear structure.
What do we give alongside regular blood transfusions in beta thalassaemia major?
In beta-thalassaemia major, iron chelation therapy (e.g. desferrioxamine) is important to prevent the complications of iron overload due to repeat transfusions
Which electrolyte abnormality can be seen in red cell transfusions?
Hyperkalaemia
RBC units often has higher potassium levels than normal human plasma
How does beta thalassaemia trait present?
Features
mild hypochromic, microcytic anaemia - microcytosis is characteristically disproportionate to the anaemia
HbA2 raised (> 3.5%)
Why do we treat vitamin B12 deficiency first when both B12 and iron are deficient?
In patients with both vitamin B12 and folate deficiencies, the vitamin B12 deficiency must be treated first to avoid subacute combined degeneration of spinal cord
Where do you see tear drop cells?
Tear drop cells are red blood cells with a tapered end. They are associated with thalassaemia, megaloblastic anaemia and myelofibrosis.
How does myelofibrosis present?
constitutional symptoms (fatigue and weight loss), splenomegaly (indicated by the stomach fullness), and easy bleeding points towards myelofibrosis. This reduces the ability of the bone marrow to produce normal cells, thus causing thrombocytopenia, anaemia and extramedullary haematopoiesis in the spleen.
How does haemolytic transfusion reaction present?
Fever, abdominal pain, hypotension during a blood transfusion → acute haemolytic reaction
What are signs of poor prognosis in Hodgkin’s lymphoma?
Hodgkin’s lymphoma: signs of poor prognosis: B-symptoms, increasing age, male sex, stage IV disease and lymphocyte depleted subtype
How does ITP present?
The patient presented with petechiae, purpura and epistaxis, with a blood result of thrombocytopenia. This is a typical picture of immune thrombocytopenic purpura (ITP). This is an autoimmune condition characterised by low platelets. The history of respiratory tract infection also hints at ITP.§
How do we treat ITP?
The first-line treatment for ITP is oral prednisolone.
Which abdominal condition is spherocytosis linked to?
Biliary colic
How does VWD affect platelet levels
Normal
What are the B symptoms
‘B’ symptoms in Hodgkin’s lymphoma are associated with a poor prognosis
weight loss > 10% in last 6 months
fever > 38ºC
night sweats
What is Behçet’s disease?
Behcet’s syndrome, also known as Behcet’s disease, is a rare and chronic condition that causes inflammation of the blood vessels (vasculitis) throughout the body. It can lead to numerous symptoms, including painful mouth sores, genital sores, and eye inflammation.
Which conditions are associated with Behçet’s disease?
Importantly for this question, it can increase the risk of venous thromboembolism due to the inflammation of the blood vessels. This inflammation can cause damage to the vessel walls leading to activation of the coagulation cascade and the formation of blood clots.
Where do you see Heinz bodies?
associated with G6PD deficiency.
Where do you see Howell-Jolly bodies?
These are usually present in hyposplenic or asplenic disorders, including megaloblastic anaemia and post-splenectomy patients.
Where do you see schistocytes?
Schistocytes is the wrong answer. These are fragmented and irregularly-shaped red blood cells which occur in patients with metallic heart valves or haemolytic anaemia.
What may be seen on a blood film in someone with Coeliac’s disease?
Target cells and Howell-Jolly bodies may be seen in coeliac disease → hyposplenism
Crazy massive spleen equals…
CML!
What is a thrombotic crisis?
Thrombotic crises in sickle cell can be precipitated by infection, dehydration or deoxygenation
acute, severe pain in the left hip
What are the electrolyte abnormalities seen in tumour lysis syndrome?
High potassium, high phosphate, and low calcium are the metabolic abnormalities in tumour lysis syndrome
Why do we use irradiated blood products?
Irradiated blood products are used to avoid transfusion-associated graft versus host disease
How does ITP present?
may be detected incidentally following routine bloods
symptomatic patients may present with
petechiae, purpura
bleeding (e.g. epistaxis)
catastrophic bleeding (e.g. intracranial) is not a common presentation
How do we investigate ITP?
full blood count: isolated thrombocytopenia
blood film
a bone marrow examination is no longer used routinely
antiplatelet antibody testing has poor sensitivity and doesn’t affect clinical management so is not commonly done
How does glandular fever impact haematology results?
An increase in the number of circulating activated T and B lymphocytes seen in the virus causes lymphocytosis, and neutropenia
What would the factor VIII be in VWD?
Normal
Which transfusion carries the highest risk of bacterial contamination?
Platelet transfusions have the highest risk of bacterial contamination compared to other types of blood products
How does DIC present on blood tests?
The blood tests show a depletion of platelets and coagulation factors, characteristic of DIC. The condition is associated with schistocytes due to microangiopathic haemolytic anaemia.
What is the most common inherited thrombophilia?
Factor V Leiden
When should sickle cell pts receive the pneumococcal polysaccharide vaccine?
Every 5 years
Where do you see blast cells?
Acute leukaemia
What is the first-line tx for CML?
The first-line treatment for CML is the tyrosine kinase inhibitor imatinib.
How does CML present?
tiredness, significant weight loss and easy bruising
high white cell count which is due to high numbers of myeloid cells and the chronic nature of the presentation
