Haematology Flashcards
A 65-year-old man who has smoked heavily all his life presents with lymph node over sub-mandibular gland.
Which of the following is the most likely diagnosis?
(Please select 1 option)
Cancer of the floor of maxilla
Cancer of the lateral anterior two thirds of tongue
Cancer of the mandible
Cancer of the pancreas
Testicular teratoma
Cancer of the lateral anterior two thirds of tongue
Seventy five per cent of lingual cancers arise on the anterior two thirds of the tongue.
Metastasis occurs unilaterally to the submental, submandibular and then the lower deep cervical lymph nodes.
Rare posterior third tumours spread bilaterally and have a poor prognosis due to their late presentation.
A 30-year-old woman is seen in the haematology clinic for follow up of a coagulation defect of the extrinsic pathway. Which of the following factors is likely to be affected as part of her condition? (Please select 1 option) Factor VII Factor VIII Factor IX Factor XI Factor XII
FVII
Factor VII and tissue factor are involved in the extrinsic pathway of the coagulation cascade whereas the intrinsic pathway is dependent on factors XII, XI, IX and VIII.
A defect in the extrinsic pathway will show as a prolonged prothrombin time whereas an activated partial thromboplastin time (APTT) will be prolonged with a defect of the intrinsic pathway.
A 28-year-old female attends clinic with a history of multiple venous thromboses.
You suspect she has a prothrombotic disorder.
Which one of the following disorders is most likely to be responsible?
(Please select 1 option)
Antithrombin III deficiency
Disseminated intravascular coagulation (DIC)
Thrombocytopenia
Vitamin C deficiency
Vitamin K deficiency
Antithrombin III deficiency is an autosomal dominant disorder.
Less than 70% of the normal value increases the risk of venous thrombosis because of the role of antithrombin in the inactivation of thrombin, factors VIIa, IXa, Xa, XIa, kallikrein and plasma.
DIC is a systemic thrombohaemorrhagic disorder with variable clinical presentation. Most patients present with easy bruising and haemorrhage and this results from serious underlying disease, such as obstetric complications or massive trauma.
Thrombocytopenia and vitamin K deficiency are types of acquired bleeding disorders.
Vitamin C deficiency can result in an acquired vascular defect.
A 27-year-old woman undergoes excision of a lipoma on the day list and is surprised how quickly the wound heals. Which cell type is involved almost immediately in wound healing following tissue injury? (Please select 1 option) Basophils Fibroblasts Macrophages Monocytes Platelets
Platelets This is the correct answerThis is the correct answer
Damage to blood vessel walls during tissue injury leads to the rapid formation of a platelet plug to prevent bleeding.
Vasodilation and increased vascular permeability is then followed by the influx of inflammatory cells and fibroblasts.
Neutrophils initiate phagocytosis and are the predominant cell type in the first 24 hours.
Macrophages secrete cytokines and continue phagocytosis as they become the predominant cell type after 48 hours.
On an evening ward round you glance at the blood results of a patient from another team.
The results show an increased prothrombin time (PT), an increased activated partial thromboplastin time (APPT), an increased thrombin time (TT) and a low platelet count.
Which of the following is the most likely underlying explanation for this set of blood results?
(Please select 1 option)
Disseminated intravascular coagulation (DIC)
Haemophilia
Heparin
Liver failure
Warfarin
DIC is a systemic thrombo-haemorrhagic disorder with variable clinical presentation.
There is activation of the coagulation process with consumption of platelets and coagulation factors with thrombin formation overwhelming the normal inhibition system and resulting in systemic fibrin deposition.
Most patients present with easy bruising and haemorrhage as a pathological response to serious underlying disease.
Conditions associated with DIC include
Malignancy
Massive tissue injury and trauma
Obstetric complications and infections.
Liver disease can result in a similar pattern of blood results but the thrombin time is usually normal.
A 70-year-old gentleman was admitted with difficulty swallowing, abdominal pain and vomiting.
Past medical history included a gastrectomy for carcinoma.
Initial investigations showed a macrocytic anaemia and thrombocytopenia.
Of the following, which is the likely diagnosis?
(Please select 1 option)
B12 deficiency
Iron deficiency anaemia
Pernicious anaemia
Sickle cell anaemia
Sideroblastic anaemia
B12
Intrinsic factor of Castle which is produced by the parietal cells is required for the normal absorption of vitamin B12.
Lack of intrinsic factor may arise from deficient production by parietal cells due to antiparietal cell antibodies in pernicious anaemia or following gastrectomy.
In the absence of intrinsic factor vitamin B12 will not be absorbed in the terminal ileum, and megaloblastic anaemia will result.
An 80-year-old lady with a history of ischaemic heart disease is transfused three units.
Following this she starts complaining of increasing shortness of breath. It is noted that her saturations have dropped and on examination she had bilateral basal crackles.
Of the following, which is the likely diagnosis?
(Please select 1 option)
Air embolism
Anaphylaxis
Circulatory overload
Hyperkalaemia
Thrombophlebitis
Circulatory overload
In elderly patients fluid overload is a potential complication following a transfusion.
Furosemide may be given in these cases to reduce the risk.
A 75-year-old lady who has had four units of blood prior to surgery is found to have peaked T waves and a slightly prolonged QRS complex on an ECG. What is the cause of her condition? (Please select 1 option) Anaphylaxis Haemolytic reaction Hyperkalaemia Iron overload Thrombophlebitis
Hyperkalaemia
The most common changes associated with hyperkalaemia are
Tall peaked T-waves
Reduction in amplitude
and eventually
Loss of the P-wave and
Bizarre widening of the QRS interval.
A 60-year-old gentleman is transfused two units prior to surgery.
Within thirty minutes of its commencement he starts complaining of feeling generally unwell with back pain and shortness of breath.
Observations reveal hypotension, reduced saturations and pyrexia.
What is the cause of his condition?
(Please select 1 option)
Air embolism
Haemolytic reaction
Hyperkalaemia
Iron overload
Thrombophlebitis
Haemolytic reaction
Acute haemolytic reactions may occur after infusion of a small volume of incompatible blood.
It is associated with a high morbidity and mortality and occurs soon after the start of transfusion.
The patient feels unwell and agitated and symptoms include back pain and pain at infusion site with associated shortness of breath and rigors.
Management includes discontinuation of the transfusion and fluid resuscitation.
A 30-year-old gentleman of African origin was admitted with severe back pain.
Investigations revealed a normocytic normochromic anaemia with a high reticulocyte count.
Which of the following is the likely diagnosis?
(Please select 1 option)
B12 deficiency
Iron deficiency anaemia
Pernicious anaemia
Sickle cell anaemia
Sideroblastic anaemia
Sickle cell anaemia is a disorder of varying degrees.
It may present in childhood with anaemia and mild jaundice. In the older patient, vaso-occlusive problems occur due to sickling in the small vessels of any organ, mimicking many medical and surgical emergencies.
Typical infarctive sickle crises include
Bone pain Pleuritic pain Hemiparesis Fits Splenic infarcts and Priapism.
A 20-year-old Caucasian man presents with a six month history of upper abdominal pain and leg ulcers.
He was noted by his family to be jaundiced.
Investigations showed a slight rise in bilirubin and mild anaemia.
An ultrasound scan of the abdomen showed evidence of splenomegaly.
Of the following, which is the likely diagnosis?
(Please select 1 option)
Hereditary spherocytosis
Iron deficiency anaemia
Sickle cell anaemia
Sideroblastic anaemia
Thalassaemia
Hereditary spherocytosis is the most commonly inherited haemolytic anaemia in northern Europe, affecting 1 in 5000.
It is inherited in an autosomal dominant manner but in 25% of patients neither parent is affected.
It may present with jaundice at birth however sometimes it is delayed for many years and some patients may go through life with no symptoms.
The patient may eventually develop anaemia, splenomegaly and ulcers on the leg.
A 65-year-old gentleman was admitted with a two month history of jaundice, pale stool, dark urine and bruising following minimal trauma.
A coagulation screen revealed a prolonged APTT and PT.
Which of the following is the likely diagnosis?
(Please select 1 option)
DIC
Ehlers-Danlos syndrome
Haemophilia A
Vitamin K deficiency
von Willebrand disease
Vitamin K is necessary for the y-carboxylation of glutamic acid residues on factors II, VII, IX and X and on proteins C and S.
The prothrombin time (PT) and activated partial thromboplastin time (APTT) are prolonged and there may be bruising, haematuria and gastrointestinal or cerebral bleeding.
A 30-year-old gentleman was in an industrial accident leading to 60% burns requiring intensive care and surgery.
A coagulation screen revealed a prolonged APTT, PT and low levels of fibrinogen.
Which of the following is the likely diagnosis?
(Please select 1 option)
DIC
Haemophilia A
Haemophilia B
Vitamin K deficiency
von Willebrand disease
Disseminated intravascular coagulation (DIC) is an acquired syndrome characterised by the intravascular activation of coagulation with loss of localisation arising from different causes.
It can originate from and cause damage to the microvasculature which can produce organ dysfunction.
DIC is seen in sepsis, major trauma, and placental abruption.
An 18-year-old boy was admitted with RIF pain.
On a recent visit to the dentist he had problems with bleeding after a tooth extraction. A clotting screen was requested which showed a prolonged APTT, normal PT and bleeding time. A further coagulation screen showed low levels of factor VIII.
Which of the following is the correct diagnosis?
(Please select 1 option)
DIC
Ehlers-Danlos syndrome
Haemophilia A
Haemophilia B
von Willebrand disease
Haemophilia A is an X linked disorder. The clinical features depend on the level of factor VIII.
Levels of less than 1% are associated with frequent spontaneous bleeding from early life.
Levels of less than 5% are associated with severe bleeding following injury and occasional spontaneous episodes.
Levels above 5% produce mild disease, usually with bleeding after injury or surgery.
A 36-year-old female who is on warfarin after suffering a deep vein thrombosis, presents with an INR of 8.2 and a conjunctival haemorrhage.
The blood pressure is 125/55 mmHg, heart rate is 65 bpm and the ECG reveals a normal sinus rhythm.
Which of the following is the most appropriate treatment for this patient?
(Please select 1 option)
Factor VII
FFP
Oral vitamin K 1 mg
Prothrombin complex concentrate
Stop warfarin only
oral vit K 1mg
A conjunctival bleed is defined as a minor bleed, and current guidelines suggest that oral or IV vitamin K together with the omission of warfarin, is the treatment of choice. Local guidelines should also be available.
One must always weigh up the risks and benefits of reversing the anticoagulation. The patient described is at low risk if the warfarin-induced coagulopathy is reversed. There is no suggestion from the ECG and the haemodynamic status that there is pulmonary embolus.
Major bleeds are defined as
Intraorbital Intracranial Retroperitoneal or Muscular bleeding causing compartment syndrome.
Any acute bleeding with BP less than 90 mmHg, oliguria or Hb drop to less than 9 g/l, also count as major bleeding episodes and require aggressive reversal of the coagulopathy with vitamin K, stopping warfarin and prothrombin complex concentrate or fresh frozen plasma (FFP).
A 78-year-old female who is on warfarin for atrial fibrillation presents with melaena.
Her blood pressure is 90/60 mmHg and heart rate is 100 bpm.
Investigations show:
Haemoglobin 9 g/L (12-16)
MCV 87 fL (83-95)
INR 7.2 (
Stop warfarin and give IV vitamin K and prothrombin complex concentrate This is the correct answerThis is the correct answer
This patient is hypotensive and tachycardic with melaena suggesting a major bleeding episode on warfarin.
In these circumstances guidelines suggest stopping warfarin, giving IV vitamin K, and either fresh frozen plasma (FFP) or prothrombin complex concentrate.
Local guidelines will be available and if in doubt consult with the haematologist on call. FFP may not completely reverse the effects of warfarin so it may now be preferable to consider prothrombin complex concentrate (PCC) if available.
The rate of fatal haemorrhage in patients receiving warfarin approaches 1%.
A 17-year-old girl with mild von Willebrand’s disease is scheduled for dental extraction.
A previous dental extraction resulted in bleeding that had required two unit transfusion.
What is the most appropriate treatment prior to dental surgery?
(Please select 1 option)
Cryoprecipitate
DDAVP
Fresh frozen plasma
High purity factor VIII concentrate
Recombinant factor VIII concentrate
DDAVP is the choice treatment for mild von Willebrand’s disease, which would include type I, and the majority of type II, although there is some controversy in type II B as it is thought that DDAVP can exacerbate thrombocytopenia that can accompany this type of von Willebrand’s.
It is of no use in type III - severe von Willebrand’s disease. The history tells us that she has mild disease.
You would not use cryoprecipitate or fresh frozen plasma in these patients in this era due to potential viral transmission risk from blood products.
For severe disease you would use a von Willebrand factor concentrate, not factor VIII concentrate.
A 25-year-old female with a history of type 1 von Willebrand’s disease (vWD) is referred for an opinion.
She is to have a cervical cone biopsy and the admitting team are concerned about her clotting.
You find that she has a past history of menorrhagia and has had two dental extractions as an adolescent that were uncomplicated.
What is the most useful test to assess her bleeding tendency?
(Please select 1 option)
Activated partial thromboplastin time
Bleeding time
Plasma factor VIII activity
Platelet aggregation
Prothrombin time
Plasma factor VIII activity
In type I vWD the prothrombin time (PT) and platelet aggregation will be normal.
Bleeding time, partial thromboplastin time (APTT) and factor VIII-coagulant (FVIIIc) are likely to be abnormal.
The bleeding time would be a good screening test but as we already know she has type I vWBD it will not give a quantitative measurement of her bleeding tendency.
Similarly APTT will not be that useful.
The most useful test in practice is to do the vWB antigen and activity (RICOF); but you would also do FVIIIc as this is also low in vWD.
You are asked to provide advice on a 35-year-old woman who is admitted under the maxillo-facial surgeons for extraction of wisdom teeth. The only concern was that she had developed prolonged bleeding following a tooth extraction 10 years previously and had required suturing. Besides this, she gave no other history of bleeding. What is the most likely diagnosis? (Please select 1 option) Factor V Leiden Factor IX deficiency Factor XII deficiency Primary antiphospholipid syndrome von Willebrand's disease
von Willebrand’s disease This is the correct answerThis is the correct answer
Not that much is given away by this history just the issue of a prolonged bleed after prior dental extraction.
The most likely diagnosis when considering this patient is von Willebrand’s disease which is an autosomal dominant condition and is one of the commonest bleeding disorders. Most cases are mild, with bleeding after only mild injury, particularly mucosal membrane injuries.
The condition is due to a reduction or structural abnormality of von Willebrand’s factor, which has the dual role of promoting normal platelet function and stabilising coagulation factor VIII.
von Willebrand’s disease can give normal results on screening tests and diagnosis may require specialist investigation.
Most patients with mild disease respond to desmopressin (DDAVP) but clotting factor concentrates are needed for a minority.
In transfusion, which of the following is a clinically important blood group? (Please select 1 option) Kell Lewis Li Lutheran Scianna
Kell
n addition to the ABO and rhesus systems, another eight blood group systems have been identified.
The Kell, Duffy and Kidd systems can cause both haemolytic transfusion reactions and the haemolytic disease of the newborn.
The P and MN systems can also cause both of these reactions, but they are rare events.
The Lutheran, Lewis, Scianna and Li systems are clinically of less importance, since they do not cause the haemolytic disease of the newborn, and are unlikely (or rarely) to cause haemolytic transfusion reactions.
Which of the following is true of low molecular weight (LMW) heparin?
(Please select 1 option)
Exerts its anticoagulant effect by binding with Factor VIII
Has a more potent effect on platelets than does unfractionated heparin
Inactivates thrombin more readily than unfractionated heparin
Is excreted in the urine
Is less efficacious compared with unfractionated heparin
Is excreted in the urine
By binding to antithrombin low molecular weight heparin (LMWH) creates a complex which then binds with and inactivates Xa.
LMWH binds less to platelets, endothelium and von Willebrand factor, hence less bleeding risk.
The shorter chains are less likely to bind both antithrombin and thrombin (LMW binds Xa more readily). LMHW is excreted in the urine (and partly by hepatic metabolism, though less so than unfractionated). This needs monitoring in renal impairment.
LMWH have been shown to be as efficacious, safer and have improved inpatient stay and hospital cost.
A prolonged thrombin clotting time may occur in which of the following? (Please select 1 option) Disseminated intravascular coagulation Presence of factor V Leiden Prothrombin deficiency Renal disease Warfarin therapy
DIC
Thrombin time compares a patient’s rate of clot formation to that of a sample of normal pooled plasma. Thrombin is added to the samples of plasma. If the plasma does not clot immediately, a fibrinogen deficiency is present.
If a patient is receiving heparin, a substance derived from snake venom called reptilase is used instead of thrombin. Reptilase has a similar action to thrombin but, unlike thrombin, it is not inhibited by heparin.
Thrombin is added to platelet-poor plasma at 37°C; the clotting time is recorded. Typically 14-16 seconds is the normal time. It is prolonged in
Afibrinogenaemia
Hypofibrinogenaemia
Dysfibrinogenaemia.
It is prolonged by
Heparin (corrects with protamine)
Fibrinogen degradation product (FDP)
Paraproteins (partial correction with protamine).
A young man has undergone splenectomy five years ago for traumatic splenic rupture. What blood picture would be expected on examination of the peripheral blood film? (Please select 1 option) Auer's bodies Eliptocytosis Howell-Jolly bodies Microcytosis Thrombophilia
Howell Jolly bodies
Splenectomy is associated with:
Howell-Jolly bodies
Thrombocytosis
Macrocytosis (persistence of larger red blood cells [RBCs])
Acanthocytes
Target cells
Leucocytosis.
Auer’s bodies are inclusions in leukaemic white cells and are pathogonomic of leukaemia.
Which of the following conditions is not associated with an increased tendency to thrombosis? (Please select 1 option) Behçet's disease Homocystinuria Kawasaki disease Metastatic malignancy von Willebrand disease
von Willebrand disease This is the correct answerThis is the correct answer
Other acquired causes include
Congestive cardiac failure (CCF) Trauma Surgery Myeloproliferative disorders Oral contraceptives. Other inherited causes include
Antithrombin III/protein C/protein S deficiency
Factor V Leiden
Dysplasminogenaemia
Dysfibrinogenaemia
Heparin cofactor II deficiency.
von Willebrand disease (vWD) is a congenital or acquired condition. It arises from a deficiency of von Willebrand factor (vWF), a protein that is required for platelet adhesion.
