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CC2 - exam preparation > Haematology > Flashcards

Haematology Flashcards

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1
Q

What medication is used to prevent tumor lysis syndrome?

A

The history above is clearly that of tumour lysis syndrome- person with lymphoma being started on chemotherapy with high potassium, high phosphate, and low calcium. The most commonly used prophylaxis is allopurinol (either IV or oral), with rasburicase an alternative.

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2
Q

Where does haematopoisesis occur?

A

Yolk sac, liver, spleen and ultimately bone marrow. Changes from a red marrow to a yellow marrow.

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3
Q

Where is normal haematopoisesis restricted to?

A

Vertebrae, pelvis, sternum, ribs, clavicles, skull, upper humerous and proximal femora

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4
Q

Enumerate the satages of red blood cell production/ erythropoiesis.

A
  1. Haemapouetic stem cell
  2. Proerythroblast
  3. Erythroblast (aka normoblast). Becomes more pink as gain haemaglobin. Nucleus condenses
  4. Reticulocyte. Loss of remaining ribsoomal material. 3 days in bone marrow and 1 day in blood
  5. Erythrocyte (-life span 120 days)
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5
Q

Outline some of the key structural features of RBCs.

A

In the membrane there are cytoskeletal component such as the alpha and beta spectrin which bind a protein called ankyrin.

Spherocytosis (hereditary) is caused by ankyrin mutation, whereas elliptosytosis (hereditary) is caused by a glycophorin C mutation.

In the membrane there is RhD, and ABO antigens

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6
Q

What are haptoglobins?

A

These are acute phase proteins that rise on inflammation.

They bind haem from haemolysed cells.

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7
Q

How long do neutrophils spend in the blood?

A

6-10 hours on average

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8
Q

Where is trephine taken from?

A

Posterior iliac crest

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9
Q

How is Vitamin B12 replacement carried out?

A

Vitamin B12 deficiency is managed with a loading and maintenance regime. To begin with, 1mg of hydroxocobalamin is administered IM 3 times a week for two weeks. Maintenance involves hydroxocobalamin 1mg IM every 3 months. If the deficiency is not thought to be diet-related, then this maintenance treatment continues lifelong.

Folate replacement is an appropriate therapy for this patient. However, folate should never be replaced before vitamin B12 due to the risk of precipitating subacute combined degeneration of the cord. (Think BeFore: B before F to help you remember).

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10
Q

Where is beta thalassemia most common?

A

Meditteranean, South East Asia and Saudia Arabia

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11
Q

How is iron deficiency treated?

A

200mg of ferrous sulphate 3 times a day for 3-6 months

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12
Q

What are heinz bodies?

A

Denatured haemaglobin chains

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13
Q

Alpha-Thalassemia is a gene deletion related to the production of the alpha-Hb protein. What happens when one is missing all 4 genes for alpha-Th?

A

Hydrops fetalis

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14
Q

How do red blood cells, frozen plasma and platelets get stored? (clue temp and duration)

A

RBCs stored at 4 degrees celcius for 35 days

Plasma -33 for 35 weeks

Platelets 22 degrees celcius for 5 days

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15
Q

What is myelofibrosis?

A

Fibrosis of bone marrow either pirmary or secondary. Primary is without and secondary is with essential thormbocythemia and polycythemia

More than 55 in 100 people (more than 55%) with myelofibrosis have a change in a gene called JAK2. The JAK2 gene makes a protein that controls how many blood cells the stem cells make.

Up to 35 in 100 people (up to 35%) have a change in the CALR gene.

When the JAK2 or CALR gene becomes mutated your bone marrow may not function correctly. This means scar tissue can build up in your bone marrow.

Some people with myelofibrosis have a history of essential thrombocythaemia or polycythaemia vera.

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16
Q

What is the main treatnent for essential thrombocythemia?

A

Hydroxycarbamide 500mg

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17
Q

How is immune thrombocytopenia purpura treated?

A

Elthrombopag

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18
Q

How are some anaemias treated?

A

EPO -

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19
Q

What are haptoglobins a sign of?

A

Haptoglobins bind free haemagloboin and like bilirubin can be a sign of haemolytic anaemia.

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20
Q

What is the cause of aplastic anaemia?

A

researchers believe that the disorder may result from the body’s own immune system causing damage to bone marrow stem cells. Certain environmental or health conditions are also associated with aplastic anemia and can trigger the disorder.

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21
Q
A
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22
Q

What are the main mutations in myeloproliefrative disease?

A

JAK2 and CARL

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23
Q
A
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24
Q

What is myelofibrosis?

A

Has poor prognosis and involves scarring of bone marrow. Can involve extramedullary haematopoiesis.

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25
26
What are the signs of multiple myeloma?
Calcium: hypercalcaemia occurs as a result of increased osteoclast activity. Renal: renal damage caused by monoclonal chains deposition in the tubule. Anaemia: bone marrow crowding suppresses erythropoiesis causing anaemia. Bleeding: the bone marrow crowding also causes thrombocytopenia. Bones: bone marrow infiltration creates lytic bone lesions Infection: reduction in the production of normal immunoglobulins causes increased susceptibility to infection
27
What are B symptoms?
Drenched at night from sweats - change pyjamas. Fever above 37.5 degrees Weight loss by 10% Pruitis Weakness
28
What is a unique feature of Hodgkins lymphoma that is not so important these days?
Swelling post drinking alcohol
29
Which regions are most dangerous for lymphadenopathy?
Lower neck. Neck divided into 6 regions. Abdomen
30
What are some key features of leukemias and lymphomas?
Anaemia, thrombocytopenia and infections Lymphadenopathy, hepatosplenomegaly
31
What test is done to take a biopsy of a lymph node?
Core needle biopsy
32
What blood tests are important in leukemia/ lymphoma?
Blood film
33
What would you see in a patient with APML?
Auer rods Butterfly shaped nuclei
34
What is the molecular change in PML-RARA?
15:17 translocation
35
What is the treatment for APL?
ATRA with arsenic trioxide
36
Describe a normal blood film.
37
What is the most common leukemia?
CLL
38
What might you see on a CLL blood film?
Smudge cells
39
What is overexpressed in CLL?
BCL-2
40
What are some treatments for Hogkins?
ABVD BEACOP Brentuxamab vedotin
41
What is odd about lymphoma?
Normal cell counts Painless swellings B symptoms
42
What are features of leukemias?
Bleeding Bruising Gum changes Tiredness
43
What is normal Hb range in male and females?
135-180g/L (male) 115-160 g/L (female)
44
What is normal range for platelets?
150-450 x 10^9 /L
45
What is the normal range for WBC?
4.0-11 x 10^9/L wbc
46
What does the PT test assess?
Extrinsic pathway ( factor VII and TF)
47
What does APTT assess?
Intrisnic pathway factors 8,9, 11, 12
48
What is leucocytosis?
49
What is polychromasia?
Polychromasia is a variation in staining of erythrocytes with the Wright's stain because of the presence of young erythrocytes. These young cells have a diffuse bluish color, blue color, indicating residual RNA. They will appear as reticulocytes with methylene blue staining.Literally a range of color
50
Which biochemical test is most important for hereditary spherocytosis?
Bilirubin
51
Which cell would be increased in those with CHRONIC asthma?
Eisonophils
52
In which condition do you see hypochormic pencil cells?
Iron deficient anaemia
53
How would you treat this?
Iron tablets (oral first) and colonoscopy
54
What was a common feature of haemophilia in the royal family?
Bruising, collection of fluid in legs, banging into things. Joint bleeding
55
What type of bleeding is more common with VW disease?
Mucocutaneous
56
What factor is associated with VWF?
Factor 8 (so raised APTT). Do not confuse with haemophilia A.
57
What are some key features of VW disease?
Epitaxis Menorrhagia
58
In which condition would you see painless cervical and mediastinal lymphadenopathy?
Hodgkins lymphoma
59
What is the survival for Hodgkins after 5 years when having chemo?
80% survival
60
What are the features of multiple myeloma?
high Calcium Renal impairment due to multiple myeloma Anaemia Bone lesions Secondary infections
61
What is the test for infective mononucleosis?
Paul Bunnell test
62
What are the features of infective mononucleosis?
Caused by EBV Swinging fever Enlarged cervical lymph nodes Splenomegaly Paul Bunnell test
63
What would a blood film look like for glandular fever?
64
What is a term for low blood cells?
Pancytopenia
65
What are the features of DIC?
Increased APTT, PT, TT decreased platelets increased schitocytes
66
What does HELLP stand for?
Haemolysis elevated liver low platelets
67
What is another name for DIC?
Consumption coagulopathy
68
What are the causes of DIC?
Sepsis, trauma, malignancy, obstetric complications
69
What happens in DIC?
Triggers like sepsis (proteins like LPS) cause pro-thrombotic activity instead of fibrinolysis. Without this balance there is too much clotting. DIC happens when the balance between clots and degradation of clots is tipped towards clots. Risk to key organs.
70
How is DIC treated?
there may be benefits in using continuous infusion unfractionated heparin (UFH) due to its short half-life and reversibility.
71
What features are seen in DIC?
High APTT, PT and TT, fibrinogen Low platelets, fibrin
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76
What is this a picture of?
The Jamshidi needle is a trephine needle for performing bone marrow biopsy, whereby a cylindrical sample of tissue, a core biopsy specimen,
77
What are some clinical features of beta thalassemia?
78
What are four main treatments for beta thalassemia?
Transfusions (life long) Iron overload - deferoxamine vitamin D replacement Endocrine support
79
Name a medication used in iron overload
Deferoxamine
80
What are the types of anaemia?
81
What does MAHA stand for?
Microangiopathic hemolytic anemia
82
What cells are produced in MAHA?
83
What is poikilocytsosis?
Cell shape
84
What is anisocytosis?
Cell size
85
What are the following red blood cell shapes represented of? (i) spherocytes (ii) bite cells (iii) howell jolly bodies (iv) codocytes/ target cells (v) roleaux formation (vi) pencil cells
Answers (i) spherocytes (fragments) - MAHA (ii) bite cells - Glycogen 6 phosphate dehydrogenase deficiency (iii) howell jolly bodies - post splenectomy (iv) codocytes/ target cells - LCAT (cholesterol) (v) roleaux formation - multiple myeloma (vi) pencil cells - iron deficiency
86
Features of B12 deficiency
Macroglossia and SACD (subacute combined degeneration of spinal cord)
87
What is a sequestration crisis?
A sequestration crisis is when **blood pools in organs due to sickling occurring,** and this can cause **worsening of anaemia due to loss of blood in the vasculature**, as well as an **increase in reticulocytes** as the body tries to compensate for the loss in blood. There is no occlusion of any vessels, and therefore this is different from a vaso-occlusive crisis. Therefore, due to the blood results, this is the most likely diagnosis in this patient.
88
What is Richter's transformation?
New B-symptoms in CLL -\> Richter's transformation
89
What is a glucose 6 phosphate dehydrogenase deficiency excacerbated by?
Glucose-6-phosphate dehydrogenase deficiency is an X linked disorder affecting red cell enzymes. It results in a reduced ability of the red cells to respond to oxidative stress. Therefore, red cells have a shorter life span and are more susceptible to haemolysis, particularly in response to drugs (e.g. nitrofurantoin), infection, acidosis and certain dietary agents (e.g. fava beans).
90
What is transfusion threshold?
The transfusion threshold for patients without ACS is 70 g/L
91
What increases the risk of transfusion anaphylaxis?
IgA deficiency increases the risk of anaphylactic blood transfusion reactions
92
What are the features of a transfusion reaction?
G raft vs. Host disease O verload T hrombocytopaenia A lloimmunization B lood pressure unstable A cute haemolytic reaction D elayed haemolytic reaction U rticaria N eutrophilia I nfection T ransfusion associated lung injury
93
What are TACO and TRAIL?
Transfusion Associated Circularory Overload: Hypertension, raised jugular venous pulse, afebrile, S3 present. Transfusion Related Acute Lung Injury: Hypotension, pyrexia, normal/unchanged JVP
94
What are the symptoms of polycythaemia vera?
Symptoms - • headache • dizziness • blurred vision dyspnoea • "full" feeling in the head Signs - • plethora • conjunctival suffusion • engorged retinal vessels • tendency to thrombosis - DVT, MI, CVA • erythromelalgia • tendency to bleeding - epistaxis, GI bleeding, CVA • hypertension – specifically • Acquagenic pruritis • splenomegaly
95
Name 4 myeloproliferative diseases.
Polycythaemia, essential thrombocythemia, CML, myelofibrosis
96
What is the role of B12?
Homocysteine is an amino acid. Vitamins B12, B6 and folate interact with homocysteine and create other proteins that your body needs. Typically, very little homocysteine stays in your blood.
97
What is the test for B12 defieicny?
98
How is B12 absorbed?
Passive • \<1% absorbed • Duodenum and ileum • Active • Haptocorrins (saliva) • Intrinsic factor (stomach) • Cubulin receptor (ileum) • Transcobalamin (circulation) • Enterohepatic circulation
99
1. Name four myeloproliferative diseases. 2. What are some symptoms of polycythaemia vera? 3. What are the symptoms of essential thrombocythemia? 4. What type of drug is hydroxycarbamide? 5. What is anagrelide? 6. What is a test for B12 deficiency? 7. How much B12 is absorbed passively? 8. What is the treatment for B12 replacement? 9. What are some signs of haemolysis? What type of antibodies are warm? What type are cold?
1. Polycythaemia, essential thrombocythemia, CML, myelofibrosis 2. Erythromelalgia, headaches, aquagenic pruitis, plethora 3. Neurological Erythyromyelgia, acroparasthesia, digital ischaemia, visual disturbance 4. Ribonucleotide reductase inhibitor 5. For treating ET 6. Schilling test 7. 1% 8. 3 doses a week/ 2 week s =6 in total. Followed by 3 months 9. Unconjugated bilirubin and LDH, haptoglobins 10. IgG and IgM
100
101
What is the main mutation in polycythaemia vera?
JAK2 (V617F)
102
What is the treatment for multiple myeloma?
(CRAB) BAR Bisphosphonates Resuscination analgesia
103
How is myeloma treated?
R.A.B Resus Analgesia Bisphonates
104
What is the difference between DAT and non-DAT?
DAT uses red blood cells whereas indirect uses just the antibodies.
105
What is key with the management of Warfarin?
The management of such patients depends on the INR and the presence of major or minor bleeding: If there is major bleeding, regardless of INR: stop warfarin, commence IV vitamin K and IV prothrombin complex concentrate (factors II, VII, IX and X) If INR \> 8.0 with minor bleeding or no bleeding: give 1-5 mg vitamin K. Stop warfarin, restart once INR \< 5.0. The INR should be rechecked in 24 hours and if still high then repeat vitamin K INR 5.0-8.0, with no bleeding or minor bleeding: stop warfarin and recommence when INR \< 5.0 If there is bleeding at therapeutic levels then the possibility of an underlying cause should be investigated. The decision to stop or continue warfarin should be considered carefully.
106
What drugs effect warfarin?
Fluconazole and metronidzole
107
108
What do these cell show?
Infective mononucleosis
109
What does this show?
Idiopathic Aplastic anaemia
110
What does this show?
DIC
111
What drugs can cause G6PD haemolysis?
Sulfazalazine, sulphonureus, sulphonamide
112
What are some risks of transfusion?
Blood product transfusion complications may be broadly classified into the following: ## Footnote immunological: acute haemolytic, non-haemolytic febrile, allergic/anaphylaxis infective transfusion-related acute lung injury (TRALI) transfusion-associated circulatory overload (TACO) other: hyperkalaemia, iron overload, clotting

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