Haem Flashcards
1
Q
- What is the lifespan of a RBC ?
A
- 3 months
2
Q
- What is the lifespan of a platelet ?
A
- 10 days
3
Q
- What condition is anisocytosis associated with ?
A
- Myelodysplastic syndrome
4
Q
- What condition are target cells associated with ?
A
- Iron deficiency anaemia
- Post-splenectomy
5
Q
- What condition are Heinz bodies associated with ?
A
- G6PD deficiency
- Alpha-thalassemia
6
Q
- What conditions are reticulocytes associated with ?
A
- Haemolytic anaemia
7
Q
- What is normal hemoglobin ranges for men and women ?
A
- 130-180g/L Men
- 120 – 165g/L Women
8
Q
- What is the normal MCV range ?
A
- 80-100 femtolitres
9
Q
- What can cause microcytic anaemia ?
A
- Thalassemia
- Anaemia of chronic disease
- Iron deficiency
- Lead poisoning
- Sideroblastic anaemia
10
Q
- What can cause normocytic anaemia ?
A
- Haemolytic anaemia
- Hypothyroidism
- Acute blood loss
- Aplastic anaemia
- Anaemia of chronic disease
11
Q
- Megaloblastic anaemia ?
A
- B12 deficiency
- Folate deficiency
12
Q
- Normoblastic macrocytic anaemia ?
A
- Reticulocytosis e.g. .due to haemolysis
- Alcohol abuse
- Hypothyroidism
- Liver disease
- Drugs e.g. azathioprine
13
Q
- A 28 yo presents with tiredness and hair loss. Blood results should a hypochromic microcytic anaemia. What is the diagnosis ?
A
- Iron deficiency anaemia
14
Q
- What clinical signs appear with iron deficiency anaemia ?
A
- Koilonychia (abnormally thin nails)
- Angular chelitis
15
Q
- What is the initial diagnostic test for iron deficiency anaemia ?
A
- Serum ferritin
16
Q
- What are possible underlying causes of iron deficiency anaemia ?
A
- Inadequate dietary iron
- Inadequate iron absorption e.g. coeliac
- Increased iron requirement e.g. pregnancy
- Bleeding e.g. heavy periods or GI cancer
17
Q
- What is management of iron deficiency anaemia when the cause isn’t clear ?
A
- Urgent OGD and colonoscopy to exclude GI cancer
18
Q
- What condition are smudge cells associated with ?
A
- Chronic lymphocytic leukaemia
19
Q
- What treatment options are available for iron deficiency anaemia ?
A
- Oral iron (ferrous sulfate)
- Iron infusion
- Blood transfusion (if very low)
20
Q
- What condition is +ve intrinsic factor antibodies associated with ?
A
- Pernicious anaemia
21
Q
- Where is intrinsic factor produced ?
A
- Parietal cells of the stomach
22
Q
- What is the key symptom associated with pernicious anaemia ?
A
- Peripheral neuropathy
23
Q
- How does pernicious anaemia present ?
A
- Anaemia features – lethargy, pallor and dyspnoea
- Neurological features – pins and needles, weakness, ataxia and paresthesia
- Neuropsychiatric features – memory loss, poor concentration, confusion, depression and irritability
- Mild jaundice + pallor = lemon tinge
- Atrophic glossitis
24
Q
- How is pernicious anaemia treated and which medication is given first and why ?
A
- Intramuscular hydroxycobalamin injections (IM B12)
- Folic acid supplementation may be required
- Treat B12 first as treating folate first can cause subacute combined degeneration of the spinal cord
25
Q
- What is autoimmune haemolytic anaemia ?
A
- When antibodies develop against RBC cells causing haemolysis
- What activates the antibodies depends on the temperature
- 2 types – hot or cold haemolytic anaemia
26
Q
- What is warm autoimmune haemolytic anaemia ?
A
- The MC
- Haemolysis caused by IgG
- Haemolysis tends to occur in extravascular sites like the spleen
- Can be caused by SLE, lymphoma, CLL and methyldopa (idiopathic)
27
Q
- How is warn autoimmune haemolytic anaemia managed ?
A
- Treatment of underlying disorder
- Steroids (+/- rituximab) are 1st line
28
Q
- What is cold autoimmune haemolytic anaemia ?
A
- The antibody in cold AIHA is usually IgM and causes haemolysis best at 4 deg C.
- Haemolysis is mediated by complement and is more commonly intravascular.
- Features may include symptoms of Raynaud’s and acrocynaosis.
- Patients respond less well to steroids
29
Q
- What can cause cold autoimmune haemolytic anaemia ?
A
- Neoplasia: e.g. lymphoma
- Infections: e.g. mycoplasma, EBV
30
Q
- What is found on blood films of autoimmune haemolytic anaemia ?
A
- Raised reticulocytes
- Schistocytes
31
Q
- What are inherited causes of haemolysis ?
A
- Hereditary spherocytosis
- Hereditary elliptocytosis
- Thalassaemia
- Sickle cell anaemia
- G6PD deficiency
32
Q
- What condition causes defective globin chains ?
A
- Thalassaemia
33
Q
- What types of thalassaemia are there ?
A
- Alpha thalassaemia
- Beta thalassaemia
34
Q
- What is the inheritance pattern of thalassaemia ?
A
- Autosomal recessive
35
Q
- How would thalassaemia present in the abdomen, sclera, conjunctiva and FBC ?
A
- Splenomegaly
- Jaundice
- Pallor
- Microcytic anaemia
36
Q
- What is the diagnostic test for thalassaemia ?
A
- Haemoglobin electrophoresis
37
Q
- What management is there for thalassaemia ?
A
- Blood transfusion
- Splenectomy
38
Q
- Why is serum ferritin monitored in thalassemia ?
A
- Risk of iron overload
39
Q
- What is a potential cure for thalassemia ?
A
- Bone marrow transplant
40
Q
- What is the name for when you have one sickle cell gene ?
A
- Sickle cell trait
41
Q
- What antibiotic is given as prophylaxis in children with sickle cell ?
A
- Penicillin V
42
Q
- What medication stimulates Hbf production ?
A
- Hydroxycarbamide
43
Q
- What is a potential cure of sickle cell ?
A
- Bone marrow transplantation
44
Q
- What types of sickle cell crises are there ?
A
- Acute chest syndrome
- Vaso-occlusive crisis
- Splenic sequestration crisis
- Aplastic crisis
45
Q
- A 75 yo presents with fatigue, pallor and abnormal bruising. What is the diagnosis ?
A
- Acute myeloid leukaemia
46
Q
- What are the types of leukaemia ?
A
- Acute myeloid leukaemia
- Acute lymphoblastic leukaemia
- Chronic myeloid leukaemia
- Chronic lymphocytic leukaemia
47
Q
- What causes bruising in acute myeloid leukaemia ?
A
- Thrombocytopenia (low platelet)
48
Q
- What is the diagnostic test for acute myeloid leukaemia ?
A
- FBC for initial
- Bone marrow biopsy is definitive
49
Q
- What type of leukaemia is associated with Philadelphia chromosome ?
A
- ALL and CML
50
Q
- What type of leukaemia is associated with smudge cells ?
A
- CLL
51
Q
- What type of leukaemia is associated with myelofibrosis ?
A
- AML
52
Q
- What type of leukaemia is associated with Richter’s transformation ?
A
- CLL
53
Q
- What are the main treatments for leukaemia ?
A
- Chemotherapy
- Steroids
54
Q
- What is the cause of increased uric acid in leukaemia ?
A
- Tumour lysis syndrome
55
Q
- A 24 yo presents with enlarged rubbery non-tender lymph nodes. Pain is worse with alcohol use. What is the diagnosis ?
A
- Hodgkins lymphoma
56
Q
- What viruses are associated with Hodgkins lymphoma ?
A
- HIV
- Epstein-Barr
57
Q
- What is the diagnostic test for Hodgkins lymphoma ?
A
- Lymph node biopsy
58
Q
- What is the special cell seen on histology in Hodgkins lymphoma ?
A
- Reed-Sternberg cells
59
Q
- What is the staging system used in Hodgkins lymphoma ?
A
- ANN Arbor has been replaced by Lugano classification
60
Q
- What is the main treatments for Hodgkins lymphoma ?
A
- Chemo and radio
61
Q
- What type of lymphoma is associated with EPV ?
A
- Burkitt Lymphoma
62
Q
- What type of lymphoma is associated with H.pylori ?
A
- Malt lymphoma
63
Q
- What type of lymphoma is associated with a rapidly growing painless mass ?
A
- Diffuse large B-cell lymphoma
64
Q
- A 67 yo presents with vague symptoms of weight loss, tiredness and bone pain. What is the most likely diagnosis ?
A
- Multiple myeloma
65
Q
- What are the key features of multiple myeloma ?
A
- CRAB
- Calcium elevated
- Renal failure
- Anaemia
- Bone lesions/bone pain
66
Q
- What are the initial lab tests for multiple myeloma ?
A
- Serum protein electrophoresis
- Serum-free light chain assay
- Urine Bence-Jones protein
67
Q
- What test is used to confirm the diagnosis of multiple myeloma ?
A
- Bone marrow biopsy
68
Q
- What would the skull look like on X-ray of multiple myeloma ?
A
- Raindrop skull
69
Q
- What is 1st line chemo in multiple myeloma ?
A
- Bortezomib
- Thalidomide
- Dexamethasone
70
Q
- A 62 yo presents with itching, bruising and excessive sweating. His Hb is 208g/L. What is the diagnosis ?
A
- Polycythaemia vera
71
Q
- From what cell line are RBCs produced ?
A
- Erythroid cells
72
Q
- What is the key gene mutation in polycythaemia vera ?
A
- JAK2
73
Q
- What is the diagnostic test for polycythaemia vera ?
A
- Bone marrow biopsy
74
Q
- What is the key complication of polycythaemia vera ?
A
- Thrombosis
75
Q
- What is the key management of polycythaemia vera ?
A
- Venesection
- Aspirin
- Chemotherapy
76
Q
- What myeloproliferative disorders are there ?
A
- Polycythaemia vera
- Primary myelofibrosis
- Essential thrombocythemia
77
Q
- What can potential myeloproliferative disorders potentially malignantly transform into ?
A
- Acute myeloid leukaemia
78
Q
- A 50yo women with 2 weeks of bruising rash and nosebleeds presents with a platelet count of 16x10(9)/L. What is the diagnosis ?
A
- Immune thrombocytopenia purpura
79
Q
- How is immune thrombocytopenia purpura treated ?
A
- Steroids
- IV immunoglobulins
- Rituximab
- Splenectomy
80
Q
- What can cause low platelets ?
A
- B12 or folate deficiency
- Alcohol
- Heparin induced thrombocytopenia
- Thrombotic thrombocytopenic purpura
81
Q
- A 28 yo women with a history of heavy periods and epistaxis presents. Her mother had a hysterectomy at 40 for heavy periods. What is the most likely diagnosis ?
A
- Von Willebrand disease
82
Q
- What can cause abnormal or prolonged bleeding ?
A
- Thrombocytopenia
- Von Willebrand disease
- Haemophilia A or B
- Disseminated intravascular coagulopathy
83
Q
- What treatment is required for Von Willebrand disease ?
A
- Desmopressin
- Von Willebrand factor infusion
- Factor 8 infusion
84
Q
- What is the underlying pathology in haemophilia ?
A
- A Factor 8 deficiency
- B Factor 9 deficiency
85
Q
- A 32 yo with a history of 3 miscarriages presents with a swollen and tender leg. What is the diagnosis ?
A
- DVT
86
Q
- What difference in leg circumference is significant in deep vein thrombosis ?
A
- > 3cm
87
Q
- A 32 yo with a history of 3 miscarriages presents with a swollen and tender leg. What is likely the underlying cause of the DVT ?
A
- Antiphospholipid syndrome
88
Q
- In antiphospholipid syndrome. What is the test, treatment and treatment in pregnancy ?
A
- Test = antiphospholipid antibodies
- Treatment = long term warfarin
- Pregnancy = low molecular weight heparin and aspirin
89
Q
- What is disseminated intravascular coagulopathy
A
- Dysregulation in the process of coagulation and fibrinolysis resulting in widespread clotting with resultant bleeding
90
Q
- What is the pathophysiology of DIC
A
- Tissue factor (TF) is present on many cell types and not normally in contact with general circulation but is exposed to after vascular damage
- Upon activation TF binds with coagulation factors that then trigger the extrinsic pathway (Via F7) which subsequently triggers the intrinsic pathway (12 to 11 and 9) of coagulation
91
Q
- What can DIC ?
A
- Sepsis
- Trauma
- Obstetric complications e.g. haemolysis, elevated liver FT, low platelets (HELLP) syndrome
92
Q
- What does a typical blood picture look like in DIC ?
A
- Decreased platelets and fibrinogen
- Increased PT, APTT and fibrinogen degradation products
- Schistocytes due to microangiopathic haemolytic anaemia
93
Q
- What is thrombotic thrombocytopenic purpura ?
A
- A condition where tiny thrombi develop through the small vessels using up platelets. As the problem is in the small vessels it is described as a microangiopathy causing:
- Thrombocytopenia, purpura and tissue ischaemia with end organ damage
94
Q
- What is the pathogenesis of Thrombotic thrombocytopenic purpura ?
A
- Abnormally large and sticky multimers of von Willebrand’s factor cause platelets to clump within vessels
- In TTP there is a deficiency of ADAMTS13 (a metalloprotease enzyme) which breakdowns (‘cleaves’) large multimers of von Willebrand’s factor
- overlaps with haemolytic uraemic syndrome (HUS)
95
Q
- What are features of thrombotic thrombocytopenic purpura ?
A
- Rare – typically adult females
- Fever
- Fluctuating neuro signs (microemboli)
- Microangiopathic haemolytic anaemia
- Thrombocytopenia
- Renal failure
96
Q
- What are potential causes of thrombotic thrombocytopenic purpura ?
A
- Post-infection e.g. urinary, GI
- Pregnancy
- Drugs: ciclosporin, oral contraceptive pill, penicillin, clopidogrel and aciclovir
- Tumours
- SLE
- HIV
97
Q
- How is thrombotic thrombocytopenic purpura treated ?
A
- Guided by haematologist
- This may involve plasma exchange, steroids and rituximab
98
Q
- What is Immune thrombocytopenia purpura ?
A
- A condition where antibodies are created against platelets leading to their destruction and thrombocytopenia
- It is characterised by purpura which are non-blanching lesions caused by bleeding under the skin
99
Q
- How can immune thrombocytopenia purpura be managed ?
A
- Prednisolone (steroids)
- IV immunoglobulins
- Thrombopoietin receptor agonists e.g. avatrombopag
- Rituximab
- Splenectomy
100
Q
- Why is rituximab useful in autoimmune disease ?
A
- B cells produce antibodies
- Reducing B cell numbers reduces inflammation
101
Q
- What is heparin-induced thrombocytopenia ?
A
- The development of antibodies against platelets in response to heparin (usually unfractionated heparin but can also occur with low-molecular-weight heparin)
- Heparin-induced antibodies target protein on platelets called platelet factor 4 (PF4)
102
Q
- What is the pathophysiology of heparin-induced thrombocytopenia ?
A
- HIT antibodies bind to platelets activating the clotting system causing a hypercoagulable state and thrombosis
- They also break down platelets causing thrombocytopenia
- There is then a counterintuitive situation where a patient is on heparin, has a low platelet count, and develops abnormal blood clots
103
Q
- When does heparin-induced thrombocytopenia usually occur ?
A
- The condition typically presents around 5-10 days after starting treatment with heparin
104
Q
- How is heparin-induced thrombocytopenia managed ?
A
- Stopping heparin and using an alternative anticoagulant guided by a specialist e.g. fondaparinux or argatroban
105
Q
- What would the blood results be in terms of prothrombin time, APTT, bleeding time and platelet count for a patient on warfarin ?
A
- PT – prolonged
- APTT, bleeding time and platelet count – normal
106
Q
- What would the blood results be in terms of prothrombin time, APTT, bleeding time and platelet count for a patient on aspirin ?
A
- Prothrombin time, APTT and platelet count normal
- Prolonged bleeding time
107
Q
- What would the blood results be in terms of prothrombin time, APTT, bleeding time and platelet count for heparin ?
A
- Prothrombin time – normal but can be prolonged
- APTT and platelet count – normal
108
Q
- What would the blood results be in terms of prothrombin time, APTT, bleeding time and platelet count for DIC ?
A
- Prothrombin time, APTT, bleeding time – prolonged
- Platelet count – low