Haematology Flashcards
1
Q
What are the 4 components of blood?
A
erythrothcytes (red blood cells)
thrombocytes (platelets)
leukocytes (neutrophils, eosinophils, basophils, lymphocytes, monocytes)
plasma (water, proteins, clotting factors, electrocytes, CO2, O2)
2
Q
What is meant by the term haematocrit?
A
volume percentage of red blood cells
3
Q
What is meant by the term haematopoiesis?
A
the production of red blood cells in the bone marrow
4
Q
Haematopoiesis regulation depends on glycoprotein growth factors, which drive the proliferation and differentiation of 5 progenitor cells such as?
A
erythropoietin (production of red blood cells) thrombopoietin (production of platelets) interleukins colony-stimulating factors negative regulators
5
Q
Multipotent haematopoietic stem cells (HSCs) are able to differentiate into?
A
myeloid and lymphoid cell lines
6
Q
Lymphoid progenitor cells give rise to which 3 cell types?
A
- B-lymphocytes
- T-lymphocytes
- natural killer cells
7
Q
B-lymphocytes give rise to which cell types?
A
plasma cells
8
Q
Myeloid progenitor cells give rise to which 3 cell types?
A
- megakaryocyte
- erythroblast
- myeloblast
9
Q
Megakaryocyte cells give rise to which cell types?
A
platelets
10
Q
Erythroblasts give rise to which cell types?
A
- reticulocyte
- red blood cells
11
Q
Myeloblast cells give rise to which 4 cell types?
A
- monocytes
- neutrophils
- basophils
- eosinophils
12
Q
What is the term used to describe the following process?
- low oxygen level in the body due to; hypoxia, decreased O2 availability to the blood, increased O2 demand from the tissues
- kidneys then secrete hormone erythropoietin
- erythropoietin stimulates enhanced erythropoiesis in the bone marrow
- RESULT: increased red blood cell count
A
erythropoiesis
13
Q
What are 2 elements needed for erythropoiesis to take place?
A
- iron
- B12/folate
14
Q
Which cell type is being described?
- biconcave shape
- no nucleus
- rich in haemoglobin (iron-containing protein)
- primary function is gas exchange
- lifespan of 120 days
A
erythrocytes (red blood cells)
15
Q
Each haemoglobin is made of 4 subunits; ??
A
2 alpha globin chains
2 beta globin chains
16
Q
What is the name of the graph that shows the following?
shows how the hemoglobin saturation with oxygen (SO2,), is related to the partial pressure of oxygen in the blood (PO2)
A
oxygen dissociation curve
17
Q
What is the name of the condition being described?
- decreased number of red blood cells, haemoglobin or ability to carry oxygen
- CAUSES; blood loss, impaired red blood cell production, increased red blood cell destruction
- haemoglobin <130g/L (male), <120g/L (female)
A
anaemia
18
Q
What are the following symptoms of?
- eyes: yellowing or in severe cases redness
- skin: paleness, coldness, yellowing
- respiratory: shortness of breath
- muscular: weakness
- fatigue, dizziness, fainting
- heart: low blood pressure, chest pain, increased heart rate
- spleen: enlargement
A
anaemia
19
Q
What are the 3 sub categories of anaemia?
A
- microcytic anaemia
- normocytic anaemia
- macrocytic anaemia
20
Q
Which type of anaemia is being described?
- MCV 80-100 fL
- total haemoglobin and haematocrit reduced, red blood cell size remains normal
reticulocyte count low:
anaemia of chronic disease
aplastic anaemia
reticulocyte count normal:
acute haemorrhage
haemolytic anaemia
A
normocytic anaemia
21
Q
Which type of normocytic anaemia is the following?
- common syndrome in which the anaemia is due to an inflammation mediated reduction in red blood cell production and sometimes red blood cell survival
- caused by: infection, neoplasms, autoimmune reactions and trauma
A
anaemia of chronic disease
22
Q
Which type of normocytic anaemia is the following?
- HSC in bone marrow are damaged - pancytopenia
- bruising, more prone to infections, clotting issues
- caused by: chemicals, drugs, radiation, infection, autoimmune disease
A
aplastic anaemia
23
Q
Which type of normocytic anaemia is the following?
- premature destruction of red blood cells, rate of destruction greater than the rate of production
- jaundice
- caused by: autoantibodies, medications and underlying malignancy
A
haemolytic anaemia
24
Q
Which type of normocytic anaemia is the following?
- as a result of trauma blood loss
- put into 4 classes
A
acute haemorrhage
25
What are the 2 main types of microcytic anaemia (MCV <80 fL)?
- iron deficiency
| - thalassaemia
26
What are 4 predisposing factors of iron deficiency anaemia?
- pregnancy
- Afro Caribbean women
- vegan diet
- obesity
27
What are 3 causes of iron deficiency anaemia?
- decreased intake (inadequate diet, impaired absorption)
- increased requirement (pregnancy, lactation, growth)
- blood loss (GI bleeding, heavy menstrual bleeding)
28
What are 6 symptoms of iron deficiency anaemia?
- fatigue
- koilonychia (spoon nails)
- hair loss
- angular stomatitis
- glossitis
- PICA (start to eat non food items)
29
What are 2 treatment options for iron deficiency anaemia?
- oral supplementation
| - red cell transfusion
30
Which type of microcytic anaemia is the following?
- inherited erythropoiesis disorder - abnormal haemoglobin production
- type depends on which globin chain is abnormal: alpha and beta
- inherited in recessive fashion
- most common in Italian, greek, Middle Eastern, south asian and African ethnicity
thalassemia
31
What are 10 symptoms of thalassaemia?
- lethargy
- enlargement of spleen or liver
- jaundice
- failure to thrive
- low height/weight
- pallor (pale)
- spinal deformity
- large head
- chipmunk faces
- misaligned teeth
32
What are 5 treatment options for thalassaemia?
- genetic counselling
- transfusion if symptomatic
- iron chelation
- splenectomy
- stem cell transplant if only cure
33
What are 4 complications of thalassaemia?
- skin pigmentation
- thrombotic compilations
- cardiovascular complications
- oestopaenia
34
Which type of anaemia is the following?
- inhibition of DNA synthesis during erythropoiesis - cell therefore has prolonged growth phase of cell cycle - large cell
macrocytic anaemia (MCV > 100 fL)
35
What are the 3 main causes of macrocytic anaemia?
- B12/folate deficiency
- hypothyroidism
- alcoholism
36
What are the 2 types of macrocytic anaemia?
megaloblast
and
non-megaloblast
37
Which type of macrocytic anaemia are the following causes of?
- B12 deficiency (pernicious anaemia)
- folate deficiency (eg. dietary insufficiency)
megaloblast
38
Which type of macrocytic anaemia are the following causes of?
- alcohol
- reticulocytosis
- liver disease
- hypothyroidism
non-megaloblast
39
Which type of macrocytic anaemia is the following?
- caused by autosomal recessive gene defect in the beta chain of haemoglobin which results in production of sickle cell haemoglobin
sickle cell anaemia
40
What are 6 symptoms of sickle cell anaemia?
- fever
- jaundice
- bone pain
- pneumonia like symptom
- tachycardia
- maxillary hypertrophy with overbite
41
What are 5 treatment options for sickle cell anaemia?
- blood transfusion
- bone marrow transplant
- antibiotics
- antihistamine
- analgesia
42
What are 5 precipitating factors (trigger) of sickle cell anaemia?
- acidosis
- dehydration
- cold temperatures
- extreme exercise
- stress & infection
43
What are 5 complications of sickle cell anaemia?
- iron overload
- leg ulcers
- blindness
- gallstones
- cardiovascular manifestations
44
What is the definition of haemostasis?
the physiological process that stops bleeding at the site of an injury while maintaining normal blood flow elsewhere in the circulation
45
What are the 3 stages of haemostasis?
1. vascular reaction
2. platelet aggregation (primary haemostasis)
3. coagulation cascade (secondary haemostasis)
46
What stage of haemostasis is the following?
- injury to a blood vessel triggers local sympathetic nervous reaction
- vascular smooth muscle cells constrict
- vasospasm occurs
- blood flow to injured area reduced
- endothelin release causes vasoconstriction
- serotonin, ADP and thromboxane released from recruited platelets to enhance vasoconstriction
- reflex constriction of neighbouring arterioles
vascular reaction
47
Which cell type is the following?
- does not have a nucleus
- derived from megakaryocyte
- circulating lifespan of 10 days
- without injury - do not adhere to each other or endothelium
platelets 'thrombocytes'
48
Which stage of haemostasis is the following?
- platelets adhere to exposed endothelial materials eg. collagen and von Willebrand factor
- vWF in endothelium binds to platelet glycoprotein Gplb
- vWF binding exposes Gpllb/llla sites
- Gpllb and Gpllla link with counterparts on other platelets via vWF
platelet aggregation
49
Which process of haemostasis is the following?
- platelets degranulate releasing adenosine diphosphate (ADP), serotonin and thromboxane A2
- ADP recruits more platelets
- serotonin is a vasoconstrictor
- thromboxane A2 stimulates platelet aggregation and further activation
- platelet plug generated at site of vessel injury
platelet plug formation
50
Which stage of haemostasis is the following?
- 2 pathways result in common pathway
- stepwise progression
- most components are serine proteases
- activated components act as enzyme for next step
- results in generation of fibrin clot
coagulation cascade
51
What are the two pathways that result in common pathway associated with the coagulation cascade?
intrinsic pathway
and
extrinsic pathway
52
Which pathway associated with coagulation cascade is the following?
- factor Xa converts prothrombin to thrombin
- thrombin converts fibrinogen into fibrin
- factor Xllla helps crosslinking of fibrin to stabilise clot
common pathway
53
What are 2 clotting co-factors?
calcium
| vitamin K
54
Which clotting factor is the following?
| - binds to the phospholipids to provide a surface for assembly of coagulation factors
calcium
55
Which clotting factor is the following?
| - required for carboxylation of factors 2, 7, 9, 10 in liver
vitamin K
56
What is the term used for the following process?
- dissolve blood clots during the process of wound healing and prevent blood clots in healthy blood vessels
- tissue plasminogen activator (tPA) produces plasmin which breaks up fibrin
fibrinolysis
57
What category is medication is the following?
- decrease platelet aggregation (inhibit thrombus formation)
- effective in arterial circulation
- used in prevention and management of small vessel disease
- indications: primary and secondary prevention of stroke/heart attack
antiplatelets
58
What are 2 anti platelet medications?
aspirin
and
clopidogrel
59
Which anti platelet medication is the following?
| - COX-inhibitor reduces platelets production of thromboxane
aspirin
60
Which anti platelet medication is the following?
| - inhibits ADP binding to platelet and therefore Gpllb/llla complex
clopidogrel
61
Which category of medication is the following?
- inhibit the coagulation cascade
- prevent and treat thrombus
anti coagulants
62
What are 2 anticoagulants?
warfarin
and
heparin
63
Which anticoagulant medication is the following?
| - vitamin K antagonist (reduced factors 2,7,9,10)
warfarin
64
Which anticoagulant medication is the following?
| - binds to and activates antithrombin > inactivates thrombin and factor Xa
heparin
65
What are 3 pro-coagulant medications?
tranexamic acid
prothrombin complex
vitamin K
66
Which pro-coagulant medication is the following?
- antifibrinolytic MOA > stabilisation of fibrin clot
- commonly used in trauma, tooth extraction, PPH
tranexamic acid
67
Which pro-coagulation medication is the following?
- contains factors 2,7,9,10
- reverse effects of warfarin
- ++ expensive
prothrombin complex
68
Which pro-coagulation medication is the following?
- reverses action of warfarin
- provides vit K for vit K dependent factors
Vitamin K
69
What are 6 coagulopathies (bleeding disorders)?
- haemophilia A&B
- von willebrand disease
- thrombocytopenia
- liver failure
- disseminated intravascular coagulation
- trauma 'triad of death'
70
Which bleeding disorder is the following?
- X-linked recessive inheritance
- A-reduced factor 8
- B-reduced factor 9
- spontaneous bleeding
- heavy bleeding in dental treatment/surgery
haemophilia A&B
71
Which bleeding disorder is the following?
- autosomal dominant
- most common hereditary clotting disorder
- deficiency in vWF
- easy bruising, nose bleeds and bleeding gums
- treat with desmopressin prior to dental procedure
von willebrand disease
72
Which bleeding disorder is the following?
- low levels of platelets
- inherited or acquired condition
- purpura of arms and petechiae in feet, legs and mucous membranes
thrombocytopenia
73
Which bleeding disorder is the following?
| - impaired clotting factor synthesis
liver failure
74
Which bleeding disorder is the following?
- widespread clotting throughout the body
- occlusion of small vessels
- clotting factors and platelet 'used up'
- widespread bleeding & organ failure - high mortality
- causes: cancers, massive tissue injury, sepsis
disseminated intravascular coagulation
75
Which bleeding disorder is the following?
- hypothermia (decreased heart performance)
- acidosis (increased lactic acid in blood)
- coagulopathy (decreased coagulation)
trauma 'lethal triad'
76
Which type of leukaemia is the following pathophysiology of?
- differentiation 'freeze' occurs in myeloid progenitor cells
- myeloblasts do not mature/differentiate
- disruption in control of proliferation leads to uncontrolled clone of immature myeloid cells
- immature myeloblasts secrete inhibitory substances into bone marrow
- development of healthy blood cells in marrow disrupted > anaemia, neutropenia and thrombocytopenia
acute myeloid leukaemia
77
What are 3 environmental risk factors of acute myeloid leukaemia?
- radiation
- benzene (rubber, dye, pesticide production)
- therapy related AML
78
What are 3 genetic risk factors for acute myeloid leukaemia?
- chromosomal translocations
- normal karyotype
- point mutations
79
What is the name of the acute myeloid leukaemia classification system?
French-american-british (FAB)
80
What are 7 presentations of acute myeloid leukaemia?
- pallor
- bruising/petechiae
- fatigue
- gingival enlargement
- dizziness
- bone pain
- abdo pain
81
What are the following risk factors of?
- age >65 years
- previous chemo
- haematological dyspoiesis
- genetic factors
- radiation exposure
- downs syndrome
- benzene exposure
acute myeloid leukaemia
82
What 4 investigations are carried out to diagnose acute myeloid leukaemia?
- full blood count
- peripheral blood smear
- coagulation screen
- bone marrow biopsy
83
What are 6 complications of acute myeloid leukaemia?
- tumour lysis syndrome
- pancytopaenia
- DIC
- CNS leukaemia
- neutropenic sepsis
- leukostasis
84
What are 3 treatment options for acute myeloid leukaemia?
- supportive care (transfusions, treat infections, hydration, hydorxycaramide)
- chemotherapy
- stem cell transplant
85
Which type of leukaemia is the following?
- increased and unregulated growth of myeloid cells in bone marrow and accumulation in the blood -proliferation of mature and precursor granulocytes
- only known risk is exposure to ionising radiation
chronic myeloid leukaemia
86
Which type of leukaemia is the following pathophysiology of?
- pathognomic feature is reciprocal translocation between chromosomes 9 and 22, resulting in an abnormal chromosome 22 termed the Philadelphia chromosome
- BCR gene on chromosome 22 is fused to to ABL gene on chromosome 9, resulting in a BCR-ABL fusion oncogene > p210 BCR-ABL protein
- BCR-ABL is active tyrosine kinase that alters activity of downstream signal transduction proteins > transforming normal haematopoietic stem cells to malignant cells
chronic myeloid leukaemia
87
What are 7 presentations of chronic myeloid leukaemia?
- fatigue
- weight loss
- abdominal pain
- bone pain
- splenomegaly
- chloroma
- fever
88
What are 2 treatment options for chronic myeloid leukaemia?
- targeted therapy with a tyrosine kinase inhibitor (imatinib mesylate, nilotinib, dasatinib, bosutinib)
- stem cell transplant and high dose chemotherapy
89
What are the 3 phases of chronic myeloid leukaemia?
1. chronic phase <5% blasts
2. accelerated phase 10-30% blasts
3. blast crisis >30% blasts
90
What is the group of disorders being described called?
| - group of disorders characterised by overproduction of myeloid cell lines in bone marrow
myeloproliferative disorders
91
Which myeloproliferative disorder is the following?
- rare
- hepatosplenomegaly (accumulation in liver and spleen)
- progressive neutrophilia
- absence of Philadelphia chromosome in diagnostic criteria
- transformation to acute myeloid leukaemia
- survival 6m-20 years
chronic neutrophilic leukaemia
92
Which myeloproliferative disorder is the following?
- primarily erythrocytosis, often thrombocytosis and leukocytosis
- JAK2 V617F somatic mutations > activates EPO receptor signalling
- increased red blood cell mass and/or haematocrit > hyperviscosity
- increased risk of thrombosis
- treat with phlebotomy and aspirin
polycythaemia vera
93
Which myeloproliferative disorder is the following?
- abnormal HSC clone proliferation results in fibrosis/scarring/replacement of bone marrow
- unknown aetiology - associated with JAK 2 mutation
- abnormalities in red blood cells, white blood cells and platelet production
- leukoerythroblastosis and splenomegaly are the clinical hallmarks
primary myelofibrosis
94
Which myeloproliferative disorder is the following?
- dysregulated megakaryocyte proliferation > increased platelet no./size
- thrombosis and bleeding
- half of patients are asymptomatic
- diagnosis of exclusion
- high risk patients treat with aspirin and hydroxycarbamide
essential thrombocythemia
95
Which myeloproliferative disorder is the following?
- very rare MPD
- overproduction of eosinophils
- chronic disease that may progress to AML
- over activation of the oncogene or translocation
- can be treated with Imatinib
chronic eosinophilic leukaemia
96
Which type of leukaemia is the following?
- 75% in children <6 years of age
- malignant clonal disease that develops when lymphoid progenitor cell becomes genetically altered through somatic changes and undergoes uncontrolled proliferation
- early lymphoid precursors replace HSC of bone marrow and infiltrating various body organs
acute lymphocytic leukaemia
97
What are the 4 causes of acute lymphocytic leukaemia?
1. genetic
2. environmental (eg. radiation)
3. folate metabolism
4. viral
98
Which type of leukaemia is the following pathophysiology of?
- classification: B-lymphoblastic leukaemia, T-lymphoblastic leukaemia
- genetic alteration of lymphoid progenitor through somatic changes > uncontrolled proliferation and clonal expansion
- leukaemia blasts infiltrate the bone marrow and other organs
- leukaemia blast cells duplicate most normal lymphoid progenitor cells
- genetic abnormalities:
chromosomal translocations or aneupoloidy are found in 75% of all cases
philadelphia chromosome
Philadelphia-like acute lymphocytic leukaemia (poor prognostic factor)
acute lymphocytic leukaemia
99
What are 5 presentations of acute lymphocytic leukaemia?
1. bruising/petechiae
2. fever
3. lymphadenopathy
4. renal enlargement
5. symptoms of anaemia
100
What are 6 ways acute lymphocytic leukaemia can be diagnosed?
- full blood count
- blood smear: lymphoblasts
- bone marrow biopsy
- lumbar puncture
- CT/MRI brain
- CT thorax/abo/pelvis
101
What are risk factors for acute lymphocytic leukaemia?
- age <6 years
- white
- male
- previous chemo
- family history
102
What are 5 treatment options for acute lymphocytic leukaemia?
1. chemotherapy
2. tyrosine kinase inhibitor
3. prophylactic antimicrobials/antifungals
4. blood transfusions
5. stem cell transplant
103
What are 8 complications of acute lymphocytic leukaemia?
1. infections
2. febrile neutropenia
3. tumour lysis syndrome
4. treatment related alopecia
5. treatment related coagulopathy
6. organ failure
7. CNS toxicity
8. infertility
104
Which type of leukaemia is the following?
- disease of middle aged to older people
- more common in white people
- lymphoproliferative disorder - monoclonal B lymphocytes are predominantly found in peripheral blood
- if found predominantly in lymph nodes = small lymphocytic lymphoma
chronic lymphocytic leukaemia
105
Which type of leukaemia is the following pathophysiology of?
- exact cause unclear > accumulation of multiple genetic events affecting oncogenes and tumour suppressor genes (eg. TP53, NOTCH1, SF3B1, ATM and BIRC3), leading to increased cell survival and resistance to apoptosis
- molecular markers eg. mutated immunoglobulin heavy chain (IgVH)
- cells infiltrate lymphatic system and haematopoietic organs such as liver, spleen and bone marrow
- cells also involved in initiating autoantibody production by normal B cells, leading to autoimmune reactions such as autoimmune haemolytic anaemia
chronic lymphocytic leukaemia
106
What are 6 presentations of chronic lymphocytic leukaemia?
1. symptoms of anaemia
2. lymphadenopathy
3. splenomegaly
4. recurrent infections
5. bruising
6. fever, chills, night sweats
107
What are 3 treatment options for chronic lymphocytic leukaemia?
- observation in early stages
- chemoimmunotherapy
- stem cell transplant
108
What are 5 complications of chronic lymphocytic leukaemia?
- second malignancy
- Richter transformation
- autoimmune haemolytic anaemia
- immune thrombocytopenia purpura
- hypogammaglobulinaemia
109
Which type of leukaemia is the following?
| - malignant neoplasms of lymphatic tissue
lymphoma
110
What is the differentiating factor between Hodgkins lymphoma and non-hodgkins lymphoma?
presence of reed-sternberg cells in Hodgkins lymphoma
111
Which type of lymphoma is the following?
- uncommon haematological malignancy arising from mature B cells > Ig
expression absent
- owl eyed appearance, giant cells
- presence of Hodgkins cells and Reed-Sternberg cells
- can be Epstein-Barr virus related
- PET-CT, essential to determine extent of disease
- biopsy necessary to confirm diagnosis
- treatment: chemotherapy plus radiotherapy
Hodgkins lymphoma
112
Which type of lymphoma are the following clinical presentations?
- most commonly presents with painless cervical and/or supraclavicular lymphadenopathy in a young adult
- fever, night sweats, weight loss
Hodgkins lymphoma
113
Which type of lymphoma is the following?
- incidence increases with age, more common in white men
- malignant lymphoid cells retain qualities of normal counterparts (B cells to produce immunoglobulins; T cells to travel to extra-nodal sites such as skin and CNS)
non-hodgkins lymphoma
114
What are 5 clinical presentations of non-hodgkins lymphoma?
1. night sweats
2. weight loss
3. fever
4. lymphadenopathy
5. jaundice
115
What are 5 investigations to help diagnose non-hodgkins lymphoma?
1. full blood count
2. blood smear
3. lymph node biopsy
4. skin biopsy
5. bone marrow biopsy
116
What are the following risk factors of which type of lymphoma?
- age >50 years
- male
- HIV
- Epstein barr virus
- immunocompromised host
non Hodgkins lymphoma
117
What is a treatment option for non-hodgkin lymphoma?
R-CHOP-21
| rituximab plus cyclophosphamide, doxorubicin, vincristine, prednisolone given for 21 days
118
Which type of leukaemia is the following?
- characterised by terminally differentiated plasma cells, infiltration of the bone marrow by plasma cells and the presence of a monoclonal Ig or Ig fragment in the serum/urine
- usually associated with osteolytic bone disease, anaemia and renal failure
multiple myeloma
119
What are 4 clinical presentations of multiple myeloma?
1. susceptibility to infections - particularly respiratory infections
2. impaired kidney function
3. bone damage and resulting in pain
4. low haemoglobin levels in blood resulting in anaemia
120
What are 4 investigations to help diagnose multiple myeloma?
1. serum/urine electrophoresis (paraprotein spike and Bence-Jones proteins in urine)
2. skeletal survey
3. bone marrow aspirate
4. full blood count
121
What are 3 treatment options for multiple myeloma?
1. autologous HSC transplant
2. bisphosphonates
3. hydration
122
What are 7 oral considerations of haem malignancy?
1. salivary gland hypofunction
2. bleeding
3. gingival enlargement
4. drug-induced osteonecrosis of the jaw
5. TMJ disorders
6. acute periodontal infections
7. neuropathy
