Haematology Flashcards
1
Q
What is leukaemia?
A
Cancer of WBCs in bone marrow
2
Q
What is AML?
A
Clonal expansion of myeloid blasts in bone marrow, peripheral blood or extramedullary tissues.
3
Q
List 3 RF for AML?
A
Previous haem disorder
Pre radiation or chemo exposure
Exposure to Benzenes (Paint, petrol, rubber)
4
Q
What may be seen on Blood film in someone with AML?
A
Auer Rods
5
Q
What are the Sx of AML?
A
*Anaemia: pallor, lethargy, weakness
*Neutropenia + freq infxns
- although WBC counts
* Thrombocytopenia: bleeding
* Splenomegaly
* Bone pain
GUM HYPERTROPHY
Leukaemia skin cuts
6
Q
What is the mx of AML?
A
Blood and platelet transfusion
Chemo (> drugs- Cytarabine, Daunorubicin)
Bone marrow transplant
Stem cell transplant
7
Q
What is CML?
A
Malignant clonal disorder of myeloid stem cells arising due to the t9;22
mutation (Philadelphia chromosome) resulting in the BCR-ABL fusion gene (Activates Tyrosine Kinase)
8
Q
What results may be present on a blood film in CML?
A
Elevated granulocytes (Basophils, and eosinophils)
9
Q
What is the Tx of CML?
A
Imatinib- Tyrosine Kinase Inhibitor
10
Q
What other tx options are available for CML other than the 1st line?
A
Hydroxy urea
Interferon alpha
11
Q
What is the epidemiology of CML?
A
Male aged 65-74
12
Q
What is CLL?
A
A lymphoproliferative disorder in which monoclonal B lymphocytes are predominantly found in peripheral blood
13
Q
What are the sx of CLL?
A
Often asymptomatic
Enlarged non tender rubbery lymph nodes
Systemic sx
Bleeding
14
Q
What will be present on the blood film of pt with CLL?
A
Smudge/smear cells
15
Q
What staging system is used for CLL staging?
A
Binet staging
16
Q
What is the mx of CLL?
A
Watch and wait
Chemo (Rituximab)
BM transplant
17
Q
What is Richter Transformation?
A
A serious complication of CLL and is often fatal. It is characterised by the sudden transformation of the CLL into a significantly more aggressive form of large cell lymphoma.
18
Q
What is the sx of Richter transformation?
A
Sudden and dramatic increase in the size of lymph nodes characterised by usually painless areas of swelling in the neck, axilla, abdomen (spleen) or groin.
Patients also often experience a dramatic unexplained weight loss, fevers and night sweats
19
Q
What is ALL?
A
clonal expansion of lymphoblasts
20
Q
What other conditions is ALL associated with?
A
Down’s syndrome
Klinefelter’s syndrome
Fanconi Anaemia
21
Q
What are the sx of ALL?
A
Hepatosplenomegaly
Fever
Lymphadenopathy
Bleeding/petechial rash
Anaemia
Bone pain
Meningeal infiltration
Mediastinal mass- svc obstruction
22
Q
What is the histology appearance of Burkitt’s lymphoma?
A
‘starry sky’ appearance of highly proliferative cells with basophilic cytoplasm.
23
Q
What metabolic abnormalities are seen in Tumour lysis syndrome?
A
Hyperkalaemia, Hyperphosphataemia, Hyperuricaemia, Hypocalcaemia
24
Q
What is the most common childhood malignancy?
A
ALL
25
What is Hodgkin's lymphoma
Uncontrolled proliferation of B-lymphocytes characterised by the presence of Reed Sternberg cells
26
What is Hodgkin's lymphoma associated with?
EBV and HIV
27
What sx are present in Hodgkins?
Asymmetrical painless lymph nodes (cervical>mediastinal)- painful when drinking alcohol
B sx: Fever, Night sweats, WL
Hepatosplenomegaly
28
What Ix findings are common in Hodgkin's Lymphoma?
Reed-Sternberg cells (owl eyes)
Eosinophilia
High LDH
Normocytic anaemia
29
Which subtype of Hodgkin's carries the best prognosis?
Lymphocyte predominant
30
Which subtype of Hodgkin's carries the worst prognosis?
Lymphocyte depleted
31
List 5 RF of non-Hodgkin's lymphoma?
Hx of viral illness (EBV)
FHx
Elderly and White
Hx of chemo or radiation
Immunodeficient
32
What is the mx of Hodgkin's Lymphoma?
Chemotherapy (ABVD regimine)
Adriamycin (Doxorubicin)
Bleomycin
Vinblastine
Dacarbazine
33
List the sx present in non-Hodgkin's?
Painless lymphadenopathy (non-tender, rubbery, asymmetrical)
B symptoms
Extranodal sx - Gastric, Bone marrow, Skin, CNS
34
What is the GS Ix for Hodgkin's and non-Hodgkin's lymphoma?
Excisional node biopsy
35
What is the mx for non-Hodgkin's lymphoma?
Chemotherapy (R-CHOP regimine)
Rituximab – a monoclonal antibody that targets CD20 on B-cells.
Cyclophosphamide
Hydroxydaunorubicin (Doxorubicin)
Oncovin (Vincristine) –
Prednisone
36
What model is used for in the staging of lymphoma?
Lugano staging (modified Ann Arbour w/ PET)
37
What is the most common inherited thrombophilia?
Factor V Leiden deficiency (Activated Protein C resistance)
38
List the causes of microcytic anaemia?
TAILS
Thalassaemia
Anaemia of chronic disease
IDA
Lead poisoning
Sideroblastic anaemia
39
List the causes of normocytic anaemia?
(AAAHH)
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
40
List the causes of megaloblastic anaemia?
Vit B12 deficiency
Folate deficiency
41
List the causes of normoblastic anaemia?
Alcohol
Reticulocytes
Hypothyroidism
Liver disease
Drugs- e.g. azathioprine
42
What is sideroblastic anaemia?
In which RBC cannot form haem in the mitochondria > deposits of iron in mitochondria > ring sideroblasts
43
What is the Ix for sideroblastic anaemia?
FBC- hypochromic microcytic anaemia
Iron studies-Raised iron, ferritin and transferrin
Blood film- Basophillic stippling of RBC
44
What would the result of iron studies be in sideroblastic anaemia?
Raised iron, ferritin and transferrin
45
What may be seen on a blood film in sideroblastic anaemia?
Basophillic stippling of RBC
46
What is the mx of sideroblastic anaemia?
Supportive- treat cause
+/- Pyridoxine (B6)
47
What are the sx for lead poisoning?
Abdo pain
Peripheral neuropathy (mainly motor)
Neuropsychiatric sx
Fatigue
Constipation
*Blue line in gum margins*
48
What may be seen on blood film in lead poisoning?
Basophillic stippling of RBC
49
What is the lifespan of RBCs?
110-120 days
50
What is the lifespan of platelets?
7-10 days
51
List 3 causes of IDA?
Inadequate intake of dietary iron
Impaired absorption
Increased iron requirements
Idiopathic
Blood loss
52
List the sx of IDA?
Dyspnoea
Pallor
Fatigue
Palpitations
Hair loss
Pica- abnormal craving of non-food substances
53
List the signs of IDA?
Koilonychia (spooning of nails)
Atrophic glossitis
Angular stomatis
54
List the result of iron studies in IDA?
Ferritin- low
TIBC- high
Transferrin- High
55
What will be seen on bloodfilm in IDA?
Hypochromic microcytic RBC
Target cells
Poikilocytes
56
What is the 1t line mx of IDA?
Oral ferrous sulphate + lifestyle advice
57
What antibody is associated with cold autoimmune haemolytic anaemia
IgM
58
What antibody is associated with warm autoimmune haemolytic anaemia
IgG
59
List the 3 broad categories of hereditary haemolytic anaemia?
Inherited RBC defects-hereditary spherocytosis, elliptocytosis
Enzyme deficiencies- G6PD
Abnormal Hb production-Sickle cell anaemia, Thalassemia
60
What is aplastic anaemia?
Defined by pancytopenia (low RBC, WBC, Platelets) with hypocellular marrow with no abnormal cells
61
List the causes of aplastic anaemia?
Idiopathic
Congenital- Fanconis anaemia
Parvovirus B19
Drugs-gold, NSAIDs, Phenytoin, chloramphenicol
62
What is the GS ix for aplastic anaemia?
Bone marrow biopsy
63
What is pernicious anaemia?
autoimmune disorder affecting the gastric mucosa that results in vitamin B12 deficiency
64
List the causes of b12 deficiency?
Pernicious anaemia
Decreased in take of vitb12- vegans
Diminished gastric breakdown of B12- Medicines, PPIs, H2 antagonists
Malabsorption from G1 tract- crohns, coeliacs
65
Where is B12 absorbed?
terminal ileum
66
How is vit b12 absorbed?
Ingested> vitb12 binds to intrinsic factor secreted by parietal cells from gastric fundus > vitb12 complex travels through small intestine where it is absorbed in terminal ileum
67
Which cells release intrinsic factor?
Parietal cells in gastric fundus
68
List the sx of B12 deficiency?
Anaemia-pallor, fatigue
Neurological- paraesthesia, numbness, ataxia, peripheral neuropathy, memory loss, poor concentration
Mild jaundice
Glossitis
69
What is the Ix of choice in pernicious anaemia?
Intrinsic factor antibody
70
What is sickle cell disease?
An autosomal recessive single gene defect in the beta chain of Hb which results in production of sickle cell Hb (HbS)
71
What amino acid change is seen in sickle cell disease?
Valine replaces glutamic acid on Chr 11
72
What is the gold standard investigation for sickle cell anaemia?
Hb electrophoresis
73
What is the treatment for acute sickle crisis?
Oxygen
Iv morphine
IV fluids
+/- penicillin
74
List 3 chronic complications of sickle cell disease?
Renal impairment
Pulmonary hypertension
Joint damage
75
List 3 acute complications of sickle cell disease?
Vaso-occlusive crisis
Aplastic crisis
Stroke
76
What drug may you give someone to prevent painful sickle crises?
Hydroxycarbamide
77
What can precipitate sickling?
Trauma
Stress
Cold
Exercise
78
What is the general mx of sickle cell disease?
Avoid triggers- dehydrations, stress etc
Ensure vaccinated
Hydroxycarbamide
79
What is the mode of inheritance of G6PD?
X linked recessive
80
What haematological disease are Mediterranean people most likely to have?
G6PD
| Thallesemia
81
What histological feature is seen in G6PD?
Heinz bodies and bite cells
82
What are the triggers for G6PD?
```
Fava beans
Red wine
Soy products
Henna
Drugs (NSAIDs, primaquine, notrofurantoin)
```
83
What is the pathophysiology behind G6PD deficiency?
Low G6PD — low amounts of reduced gluthathione — therefore oxidative damage to RBC
84
List the features and management of a non-haemolytic febrile reaction following blood transfusion?
Fever and chills
mx- slow/stop transfusion + paracetamol + monitor
85
List the features and management of a minor allergic reaction following blood transfusion?
Pruritus, Urticaria
Temporarily stop transfusion + Antihistamine + monitor
86
List the features and management of an anaphylactic reaction following blood transfusion?
Hypotension, Dyspnoea, Wheezing, Angioedema
mx- Stop transfusion + IM Adrenaline + ABC support
87
List the features and management of a acute haemolytic reaction following blood transfusion?
ABO incompatibility
Fever, Abdominal pain, Hypotension
Stop transfusion + check blood product + send blood for direct coombs test and repeat cross matching
88
List the features and management of a Transfusion Associated Circulatory Overload
(TACO) reaction following blood transfusion?
Excessive rate of transfusion, pre-existing heart failure
Pulmonary oedema, HTN
Slow/stop transfusion + Consider IV loop diuretics
89
List the features and management of a Transfusion related acute lung injury (TRALI) reaction following blood transfusion?
Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood
Hypoxia, Pulmonary infiltrates on CXR, fever, hypotension
90
What transfusion product is at most risk of contamination?
Platelets are stored at room temperature, which increases the risk of bacterial proliferation. Common contaminants include Staphylococcus epidermidis and Bacillus cereus.
91
What is acute chest syndrome (background of sickle cell)?
vaso-occlusion within the pulmonary microvasculature → infarction in the lung parenchyma
dyspnoea, chest pain, pulmonary infiltrates on chest x-ray, low pO2
92
WHat is the mx of acute chest syndrome?
Pain relief
respiratory support e.g. oxygen therapy
Antibiotics- if infecion
transfusion: improves oxygenation
93
List the features seen on bloodfilm in hyposplenism?
Howell Jowell bodies
Siderocytes
94
What is the 2nd line mx of CML?
Hydroxyurea
95
What will be seen on blood film in myelofibrosis?
Tear drop poikilocytes
96
Myelofibrosis can be secondary to what other condition?
approximately 6-10% of patients with polycythaemia vera develop myelofibrosis due to marrow exhaustion.
97
What is the MOA of dabigatran?
Direct thrombin inhibitor
98
What is the reversal agent of dabigatran?
Idarucizumab
99
What is the MOA of rivaroxaban/apixaban?
Direct factor Xa inhibitor
100
What is multiple myeloma?
a haematological malignancy characterised by plasma cell proliferation.
101
What is the reversal agent of apixaban/rivaroxaban?
Andexanet alfa
102
What are the features of myeloma?
CRABBI
Calcium- hypercalcaemia
Renal-
Anaemia
Bleeding
Bones- lytic lesions leading to pathological fractures
Infection
103
What are the ix for multiple myeloma?
peripheral blood film: rouleaux formation
Protein electrophoresis- Raised igA/igG- in urine Bence Jones Protien
104
What is GS Ix for multiple myeloma?
Bone marrow aspiration- plasma cells significantly raised
105
What is the transfusion threshold for patients without ACS?
70g/L
106
What is the transfusion threshold for patients with ACS?
80g/L
107
What is acute intermittent porphyria?
a rare autosomal dominant condition caused by a defect in porphobilinogen deaminase, an enzyme involved in the biosynthesis of haem.
108
What are the features of acute intermittent porphyria?
abdominal: abdominal pain, vomiting
neurological: motor neuropathy
psychiatric: e.g. depression
hypertension and tachycardia common
109
What is the classical symptomatic feature of acute intermittent porphyria?
classically urine turns deep red on standing
110
What is the mx of acute intermittent porphyria?
avoiding triggers
acute attacks
--IV haematin/haem arginate
--IV glucose should be used if haematin/haem arginate is not immediately available
111
What is the inheritance pattern for haemophillia?
X-linked recessive
112
What is the pathophysiology of haemophilia A?
Factor 8 deficiency results in prolonged APTT
113
What is the pathophysiology of haemophilia B?
aka christmas disease
Factor 9 deficiency
114
What are the clinical features of haemophilia?
Easy bruising
Haematoma formation
Haemarthroses (often spontaneous) - pain, swelling and decreased ROM
115
What are the coagulation studies in haemophilia?
Prolonged APTT (Intrinsic pathway affected F8/9/11)
Normal bleeding time
Normal PT/INR
116
What is the mx of multiple myeloma?
Chemotherapy - cyclophosphamide, doxorubicin etc.
Steroids - e.g. dexamethasone
Bisphosphonates - reduce bone pain/disease
Stem cell transplant
Transfusions for anaemia/ thrombocytopenia etc.
117
What is the most common inherited bleeding disorder?
Von willerbrands disease
118
What is the inheritance pattern of VWDs?
Autosomal dominant
119
What is the function of VWF?
VWF facilitates binding of platelets to subendothelial collagen in the platelet adhesion phase of primary haemostasis
VWF also carries factor VIII
120
What are the clinical features of VWD?
Frequent epistaxis
Menorrhagia and IDA
Gingival bleeding
Petechiae
121
What are the result of coagulation studies in VWF?
Increased APTT (slight)
Reduced Factor 8 activity
Prolonged bleeding time
122
What is the Ix of choice to diagnose VWD?
PFA-100 test (>95% sensitive) - platelet function analysis
123
