Haematology Flashcards
(158 cards)
1
Q
What is haematopoiesis
A
Production of blood cells and platelets
Adults: occurs in red bone marrow
Children: occurs in all bones
Fetus: occurs in liver and spleen
2
Q
What is erythropoiesis
A
RBC formation
- stimulated by EPO
Lifespan = 120d
3
Q
Define agranulocytosis
A
Absence of circulating neutrophils
Side effect of carbimazole
4
Q
Causes of neutrophilia (>10)
A
Bacterial infection
Corticosteroids
Neoplasia / CML
Myeloproliferative disorder
Bleeding / burns
Inflammation
Smoking
5
Q
Causes of neutropenia (<1.5)
A
Viral infection
Cytotoxic drugs
Bone barrow failure
Severe sepsis
Autoimmune (SLE)
Hypersplenism (felty’s)
B12 / folate deficiency
6
Q
Causes of lymphocytosis (>5)
A
Viral infection
CLL and lymphoma
Chronic infection
Septic shock / MI / trauma
Smoking
Raised BMI / metabolic syndrome
7
Q
What are the types of white cell (5)
A
Neutrophil - chemotaxis and phagocytosis
Lymphocytes - cell mediated immunity (T cells, B cells, natural killer cells)
Eosinophil - against parasite and allergies
Basophil - release histamine in inflammation
Monocyte - precursor of macrophages
8
Q
Define anaemia
A
Hb <130 in men
Hb <115 in women
9
Q
Causes of anaemia
A
Decreased RBC production eg IDA, BM disorders, cytotoxic drugs / chemotherapy , CKD, aplastic anaemia
Increased RBC destruction eg SCD, thalassaemias
Blood loss eg vWD, meckel’s diverticulum
10
Q
General signs / symptoms of anaemia
A
Fatigue / weakness
Pallor (conjunctival)
SOB / tachycardia / dizziness
11
Q
Specific signs / symptoms of anaemia
A
Koilonychia : IDA
Jaundice : haemolytic anaemia
Leg ulcers: sickle cell disease
Tingling fingers / toes : B12 deficiency
12
Q
Investigations for suspected anaemia
A
FBC: MCV
Iron studies: serum iron and ferritin
Blood film: size, shape, colour of red cells
Serum bilirubin: high in haemolysis
Hb HPLC or Hb electrophoresis
13
Q
What are the types of haemaglobin (fetal and adult)
A
Fetal (HbF): 2a chains + 2y chains -> higher O2 affinity
Adult (HbA) 2a chains + 2B chains -> lower O2 affinity
14
Q
DD for microcytic anaemia (TAILS)
A
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia
15
Q
DD for macrocytic anaemia (ABCDEF)
A
Alcohol and liver disease
B12 deficiency
Compensatory reticulocytosis
Drugs
Endocrine
Folate deficiency
16
Q
DD for normocytic anaemia (CHARMD)
A
Chronic disease
Haemolysis
Acute blood loss
Renal anaemia
Marrow disorder
Deficiencies combined
17
Q
Causes of IDA
A
Inadequate intake
Malabsorption - coeliac, gastrectomy
Increased requirements - pregnancy
Chronic blood loss - menorrhagia / GI bleed
18
Q
High Fe foods recommended for IDA
A
Red meat, liver
Pulses, beans, peas
Leafy greens veg
Oily fish
Fortified cereals
Dried fruit / nuts
19
Q
Foods to avoid in IDA
A
Excess cows milk (only 10% iron is absorbed)
Tannin (tea) - inhibits Fe absorption
20
Q
Diagnosis for IDA
A
FBC: decreased MCV = microcytic
Iron studies: decreased serum iron and decreased serum ferritin
Blood film: abnormally shaped, small hypochromic RBCs
21
Q
Management of IDA
A
- Determine cause: thorough hx and exam
- Treat underlying cause
- Dietary advice
- Oral iron supplements eg ferrous sulphate / ferrous fumurate
22
Q
What is B12 and folate
A
B12 = coenzyme needed for folate conversion
Folate = needed for RBC synthesis
23
Q
What is B12 and folate
A
B12 = coenzyme needed for folate conversion
Folate = needed for RBC synthesis
24
Q
Causes of B12 deficiency
A
Low dietary intake - vegan / vegetarian
Malabsorption - terminal ileum eg crohn’s, gastrectomy
Low intrinsic factor - eg pernicious anaemia
25
Causes of folate deficiency
Low dietary intake
Malabsorption (duodenum / jejunum) eg coeliac / jejunal resection
Increased requirements - pregnancy, haemolytic anaemia
26
Diagnosis for B12 & folate deficiency
FBC: increased MCV = macrocytic
Blood film: hypersegmented neutrophils and tear drop cells
Iron and B12 studies : decreased B12, decreased serum folate, decreased cobalamin
Intrinsic factor antibodies
27
Management of B12 and folate deficiency
If not pernicious anaemia, dietary advice : B12=eggs, fortified cereals, dairy. Folate = broccoli, peas, brown rice
IM B12 and folic acid supplements
If pernicious anaemia: lifelong IM B12 replacement
28
What is important to remember if B12 and folate deficiency both present
Must replace B12 first to avoid subacute combined degeneration of the spinal cord
29
What is RBC aplasia
Failure of RBC synthesis
30
Causes of RBC aplasia
Diamond black anaemia = rare, congenital -> raised MCV +/- short stature, abnormal thumbs
Transient erythroblastopenia = triggered by viral infection in children
Parvovirus B19 = infects young RBCs - only causes RBC aplasia in children / adults with inherited haemolytic anaemia
31
Clinical features of haemolytic anaemia
Anaemia (Normo/macrocytic)
Mild splenogmegaly
Jaundice
32
Investigations for haemolytic anaemia
FBC: decreased Hb
Blood film: increased reticulocytes
Increased unconjugated Bilirubin
Increased LDH
Decreased haptoglobin
33
Extrinsic causes of haemolytic anaemias
1. Autoimmune haemolysis -> autoantibodies causes extra vascular haemolysis and spherocytosis
2. Microangiopathic anaemia -> mechanical haemolysis triggered by physical trauma to RBCs in circulation by abnormal microcirculation with deposition on fibrin strands
3. Infection (malaria, CMV, E. coli)
4. Drugs (nitrofurantoin, penicillin, quinine)
34
Causes of microangiopathic anaemia
Malignant HTN / pre-eclampsia
HUS, DIC, vasculitis, TTP, post BM transplant
Prosthetic heart valve
35
Intrinsic causes of haemolytic anaemias
Hb alpha or beta chain abnormality (thalassaemia, SCD)
RBC membrane abnormality (hereditary spherocytosis)
Enzyme defects (G6PD deficiency, pyruvate kinase deficiency)
36
What is hereditary spherocytosis
Auto dominant or de novo mutations of RBC membrane proteins
37
Pathogenesis of hereditary spherocytosis
Abnormal membrane can’t deform well -> can’t pass through the spleen -> RBC destroyed in the spleen
38
Clinical presentation of hereditary spherocytosis
Jaundice - intermittent
Anaemia - mild
Splenomegaly - due to RBC destruction in spleen
Gallstones - persistent high bilirubin precipitates into stones
Aplastic crisis - new RBCs not made fast enough
39
Diagnosis of hereditary spherocytosis
FBC and iron studies - normocytic anaemia
Blood film - spherical RBCs with no central pallor + some microspherocytes
40
Pathogenesis of pyruvate kinase deficiency
Low levels of PK enzyme result in failure of RBCs to produce ATP -> RBCs haemolysed prematurely
41
Clinical presentation of pyruvate kinase deficiency
Chronic haemolytic anaemia - varies greatly in severity
42
Diagnosis of pyruvate kinase deficiency
FBC and iron studies - normocytic anaemia
PK activity levels - reduced
Direct Coombs test - negative
43
Management of hereditary spherocytosis and pyruvate kinase deficiency
Oral folic acid - increased need as increased RBC synthesis
Tx for aplastic crisis due to parvovirus - transfusions until resolves
Splenectomy - if severe anaemia when young / poor growth / troublesome symptoms
44
What is a G6PD deficiency
X linked mutation in G6PD gene (enzyme involved in preventing oxidative damage to RBCs)
45
Risk factors for G6PD deficiency
African
Mediterranean
Middle Eastern
46
Clinical presentation of G6PD deficiency
Asymptomatic between haemolytic episodes
Neonatal jaundice - usually within first 3d
Chronic haemolysis
Acute intravascular haemolysis - fever, pallor, malaise, dark urine
47
Diagnosis of G6PD deficiency
FBC: decreased Hb, increased reticulocytes
Unconjugated bilirubin: raised
LDH: raised
Blood film: Heinz body inclusions (only seen during haemolytic crisis)
G6PD activity: reduced (only check when stable)
Direct Coombs test : negative
48
Management of G6PD deficiency
Safety net for signs of acute haemolysis
Advise on foods / drugs to avoid: quinine, sulphonamides, nitrofurantoin, high dose aspirin, fava beans
+/- blood transfusion during haemolytic crisis
49
Pathogenesis of paroxysmal nocturnal haemoglobinuria (PNH)
A rare acquired disorder of haematopoietic stem cells which causes production of defective RBCs
Defective cells are haemolysed by the body’s complement system
50
Symptoms of PNH
Episodic haemoglobinuria (dark urine)
Anaemia (SOB, chest pain, fatigue)
51
Complications of PNH
Thromboses: increased risk of blood clots
Pancytopenia : increased risk of infection and bleeding + anaemia
52
Management of PNH
1. Symptomatic eg iron replacement for anaemia
2. Folate replacement for ongoing haemolysis
3. +/- anticoagulation : thrombosis prophylaxis
4. +/- monoclonal antibodies with block complement
53
What is the difference between B-thalassaemia major and B-thalassaemia minor
Major: both genes abnormal: no HbA = very severe anemia
Minor: 1 gene abnormal = almost normal Hb with marked low MCV
54
Clinical features of B-thalassaemia major
Severe anaemia - transfusion dependent
Jaundice and pallor
Hepatosplenomegaly
Characteristic facies (frontal bossing)
55
Management of beta thalassaemia
Lifelong monthly blood transfusions
Iron chelation
BM transplant = only cure
56
What are the types of alpha thalassaemia
1. Deletion of all 4 a globin genes: a-thalassaemia major / Hb Bart’s hydrops fetalis = death in utero
2. Deletion of 3 a-globin genes : HbH disease = mild / moderate anaemia - may need transfusions
3. Deletion of 1-2 a globin genes: asymptomatic - marked low MCV
57
What are ways of investigating thalassaemias
1. FBC and iron studies : microcytic hypochromic anaemia
2. Blood film : ‘target cells or nucleated RBC
3. Hb HPLC: proportions of HbA, HbF, HbA2
58
Cause of sickle cell disease
Mutation in B-globin gene which produces an abnormal HbS chain rather than HbA
If low O2, infection or acidosis, it polymerises and bends RBC into a sickle shape
59
Pathogenesis of sickle cell disease
Sickle shaped RBCs have decreased lifespan -> get stuck and occlude vessels causing infarction
60
Prognosis of sickle cell disease
Most severe form of= 50% die by 40y from complications
61
Long term problems of sickle cell disease
Short stature / delayed puberty
Stroke and neuro damage
Heart failure and renal dysfunction
Pigment gallstones
62
Clinical presentation of sickle cell disease
Moderate anaemia : 60-100g/L
Jaundice
Infection : increased susceptibility pneumococcus and H influenzae
Splenomegaly
Painful vaso occlusive crises
Acute anaemia (aplastic crisis)
63
Diagnosis of sickle cell disease
1. Screening: pregnant women and neonates
2. FBC and iron studies
3. Blood film: sickle cells and target cells
4. LFTs and bilirubin
5. Hb HPLC = HbS +/- HbA
64
Triggers of vaso occlusive crises
Cold
Infection
Dehydration
Hypoxia / altitude
Drugs
65
Triggers of vaso occlusive crises
Cold
Infection
Dehydration
Hypoxia / altitude
Drugs
66
Management of sickle cell disease
1. Infection prophylaxis: vaccines + daily PO penicillin
2. Daily folate supplementation
3. Decreased risk vaso occlusive crises
4. Treat vaso occlusive crises
5. Hydroxycarbamide to increased HbF
6. Blood transfusion
7. BM transplant
8. New treatments
67
Management of vaso occlusive crises
1. ABCDE
2. Analgesia: IV/SC morphine + paracetamol and ibuprofen
3. FBC, reticulocytes, infection screen
4. Cross match and transfusion
5. Enoxaparin
68
What is pancytopenia
Combination of anaemia, leukopenia and thrombocytopenia
69
Aetiology of pancytopenia
1. Reduced blood cell production (bone marrow failure)
2. Increased blood cell destruction (immune mediated)
3. Increased blood cell sequestration (in big spleen / liver)
70
Common differentials of pancytopenia
Acute leukaemia
Multiple myeloma
Hypersplenism - cirrhosis, PVT
Felty’s syndrome
Chemotherapy / radiotherapy
B12 / folate deficiency
Some non Hodgkin lymphoma
Myelodysplasia
71
Less common differentials of pancytopenia
Myelofibrosis
Aplastic anaemia
Haemophagocytic lymphohistocytosis
Drug induced
Autoimmune
Paroxysmal nocturnal haemoglobinuria
Parovirus in haemolytic anaemia
Transfusion related GVHD
Hairy cell leukaemia
Non haematological malignancy
Infection
72
Investigations for pancytopenia
1. History
2. Examination: petechiae / purpura, lymphadenopathy, abdo mass
3. Blood tests
4. Bone marrow aspirate and biopsy
5. Imaging
73
What is a myeloproliferative disorder
Bone marrow disorders with differentiated myeloid cell subtype expansion in blood
Known as myeloproliferative neoplasms
74
What is polycythaemia rubra Vera
High RBCs
Causes: headaches, fatigue, blurred vision, pruritis, HTN
Tx: reduce clot risk
5% develop AML
75
What is essential thrombocythaemia
High platelets
Causes: abdo pain, mottled skin, stroke, burning hands / feet
Tx: reduce stroke risk
5% develop AML
76
What is chronic myeloid leukaemia
WBC very high
Plts high
Anaemia
Causes: infection, fatigue, bruises, bone pain, abdo pain, night sweats, weight loss
Tx: tablets which target the BCR-ABL gene - cures most patients
BM transplant if targeted
77
What is primary myelofibrosis
Scar tissue prevents cell production
Causes: fatigue, SOB, bruises, abdo pain, bone pain, night sweats, weight loss, gout
Tx: blood transfusions / EPO
Low dose aspirin
Allopurinol (gout)
JAK2 inhibitors
Chemo + stem cell transplant if young
78
What is primary myelofibrosis
Scar tissue prevents cell production
Causes: fatigue, SOB, bruises, abdo pain, bone pain, night sweats, weight loss, gout
Tx: blood transfusions / EPO
Low dose aspirin
Allopurinol (gout)
JAK2 inhibitors
Chemo + stem cell transplant if young
79
What is multiple myeloma
Malignant clonal proliferation of plasma cells (due to B cell mutations) -> excess secretion on one type of paraprotein / light chain
Median age = 70y
80
What causes hypercalcaemia
Increased osteoclast activity -> fluids + bisphosponates
81
Clinical presentation of multiple myeloma (CRABBI)
HyperCalcaemia
Renal dysfunction
Anaemia
Bleeding
Bone pain / lesions
Infection
82
Investigations for multiple myeloma
1. Bloods : decreased Hb, WCC, plts
Raised ESR
2. Bone profile : raised Ca
3. Serum electrophoresis : serum free light chains / paraprotein
4. X-ray : lytic lesions
5. MRI / PET scan
6. BM biopsy
83
Management of multiple myeloma
Mx = palliative
Several lines of chemo + immuno-modulators agents
+ bone protection with bisphosphonates
+ infection prophylaxis
+ radiotherapy for pain in isolates sites
84
Emergency presentations of multiple myeloma
Pathological vertebral fracture
Bone pain
Acute renal failure
Spinal cord compression
Hyperviscosity
Hypercalcaemia
85
Poor prognostic factors of acute lymphoblastic leukaemia
Age <1 or >10
WBC: >50x10^9L
CNS involvement
Cytogenetic abnormalities in tumour cells
Persisting blast cells after initial chemo
86
Pathogenesis of acute lymphoblastic leukaemia
Rapidly proliferating lymphocytes stem cells = accumulation of immature lymphoblast cells (can be a B cell or a T cell)
87
Peak age of acute lymphoblastic leukaemia
2-5y
88
Symptoms of acute lymphoblastic and acute myeloid leukaemia
Rapid onset (1-2w)
Anaemia : lethargy, pallor, SOB
Neutropenia: sore throat, recurrent fevers, infections
Thrombocytopenia : bruising, petechiae, nose bleeds
End organ failure: lymphadenopathy, splenomegaly
General: fatigue, malaise, anorexia, bone pain
89
Management of leukaemia
1. Pre-chemo: dexamethasone
2. Chemotherapy: induce -> intensify -> maintain
3. Concurrent treatment : intrathecal chemo
90
Management of acute myeloid leukaemia
1. Pre-chemo : hydroxyurea
2. Intensive chemo : 3-4 cycles
3. Risk stratification for further Tx : further chemo or stem cell transplant
91
Prognosis of acute lymphoblastic leukaemia
Children: 90% cure rate
Adults: 35-40% 5y survival
92
Acute myeloid leukaemia prognosis
Depends on age, genetics and response
93
Pathogenesis of chronic lymphocytic leukaemia
Accumulation of apoptosis-resistant B cells
94
Symptoms of chronic lymphocytic leukaemia
Often asymptomatic
Anaemia : lethargy, pallor, SOB
Neutropenia: recurrent infections
Thrombocytopenia: bruising, petechiae
B symptoms: malaise, night sweats, weight loss
95
Investigations for chronic lymphocytic leukaemia
FBC: dec Hb, dec plts, inc WCC, inc lymphocytes
Peripheral blood film
Flow cytometry on blood to identify CLL cells
Bone marrow biopsy
96
Investigations for chronic lymphocytic leukaemia
FBC: dec Hb, dec plts, inc WCC, inc lymphocytes
Peripheral blood film
Flow cytometry on blood to identify CLL cells
Bone marrow biopsy
97
Management of chronic lymphocytic leukaemia
If no Sx: watch and wait
B Sx: chemo, targeted therapy
98
Management of chronic lymphocytic leukaemia
If no Sx: watch and wait
B Sx: chemo, targeted therapy
99
Prognosis for chronic lymphocytic leukaemia
85% 5y survival
A few transform into aggressive lymphoma = very bad prognosis
100
Pathogenesis of chronic myeloid leukaemia
Increased production of myeloid progenitors
-> 95% have Philadelphia chromosome
101
Symptoms of chronic myeloid leukaemia
Insidious onset
No BM failure
Tiredness / lethargy
Massive splenomegaly: abdo discomfort
102
Investigations for chronic myeloid leukaemia
1. FBC: inc plts, inc WCC, inc neutrophils, inc basophils
2. Peripheral blood film : mature myeloid cells
3. BM biopsy
4. Cytogenetics : Philadelphia
5. Molecular assessment
103
Management of chronic myeloid leukaemia
1. Tyrosine kinase inhibitors : imatinib
-> monitor response
-> some develop resistance to TKIs and need BMT
104
Prognosis of chronic myeloid leukaemia
70% 5y survival
Without treatment can transform into AML = very bad prognosis
105
What is the difference between Hodgkin’s lymphoma and non hodgkins
Hodgkin’s has reed sternberg cells (10%)
Non Hodgkin’s doesn’t (90%)
106
Aetiology of Hodgkin’s lymphoma
Reed sternberg cells (mutated B cell)
-> 50% associated with EBV
107
Peak age of Hodgkin’s lymphoma
15-35 and 60
108
Peak age of Hodgkin’s lymphoma
15-35 and 60
109
Symptoms of Hodgkin’s lymphoma
Painless lymphadenopathy (usually cervical)
SVC / bronchi obstruction
Becomes painful with alcohol
B symptoms in 40%
110
Investigations for hodgkins and non Hodgkin’s lymphoma
FBC and film
Lymph node biopsy / excision = diagnostic
Bone marrow biopsy
CT or FDG-PET- stage and grade
111
Management of hodgkins and non Hodgkin’s lymphoma
B cell: immunochemotherapy +/- radiotherapy
T cell: immunochemotherapy or just chemotherapy
112
Types of primary haemostasis
1. Vasoconstriction - slows blood for plt adhesion
2. Platelet adhesion then activation then aggregation
3. Haemostatic plug- platelets + fibrinogen = soft plug
113
Clinical presentation of impaired primary haemostasis
Mucocutaneous bleeds: bruising, petechiae, epistaxis, menorrhagia, gum bleeds
Intra op / immediate post op bleeding
114
Causes of impaired primary haemostasis
Impaired vWF = platelets can’t bind endothelium
Impaired platelet function : congenital, medications
Reduced platelet number: thrombocytopenia
115
Causes of thrombocytopenia
Increased destruction: sepsis, immune thrombocytopenia
Decreased production: alcohol, leukaemia, aplastic anaemia
Other: congenital, Hypersplenism
116
What is secondary haemostasis
After initial platelet aggregation, the platelet membrane flips and provides a surface for binding of coagulation factors
This stabilises the fibrinogen plug
Activates clotting cascade : chain reaction resulting in cleaving of fibrinogen -> fibrin
117
What is secondary haemostasis
After initial platelet aggregation, the platelet membrane flips and provides a surface for binding of coagulation factors
This stabilises the fibrinogen plug
Activates clotting cascade : chain reaction resulting in cleaving of fibrinogen -> fibrin
118
Clinical presentation of impaired secondary haemostasis
Muscular / soft tissue bleeds : extensive bruising
Haemarthrosis
Delayed post op bleeding and poor wound healing
119
Clinical presentation of impaired secondary haemostasis
Muscular / soft tissue bleeds : extensive bruising
Haemarthrosis
Delayed post op bleeding and poor wound healing
120
Causes of impaired secondary haemostasis
Congenital: haemophilia
Acquired: anticoagulant drugs, dilution post transfusion, liver dysfunction, DIC
121
What is disseminated intravascular coagulation
Acquired syndrome with inappropriate systemic activation of coagulation pathways
122
Pathogenesis of disseminated intravascular coagulation
Consumption of CFs and plts -> if severe can cause organ dysfunction, infarction and bleeding
123
Causes of disseminated intravascular coagulation
Infection
Trauma
Burns
Malignancy
Severe liver failure
Obstetric complications
ABO mismatch transfusion
124
Presentation of disseminated intravascular coagulation
Bruising
Excessive bleeding
Renal failure
125
Investigations for disseminated intravascular coagulation
FBC: low plts
Coag screen: inc PT and APTT
Inc d dimers and dec fibrinogen
126
Management of disseminated intravascular coagulation
Aggressive resuscitation
Treat cause
Support with FFP / cryoprecipitate only if active bleeding
127
What is haemophilia
X linked recessive coagulation disorder
128
Clinical features of haemophilia
Mild: bleed after surgery
Moderate: bleed after minor trauma
Severe: spontaneous joint / muscle bleeds
129
Investigations for haemophilia
FBC, blood film, LFTs
Coag screen: inc APTT and normal bleed time
CF assays : low factor VIII or IX
130
Investigations for haemophilia
FBC, blood film, LFTs
Coag screen: inc APTT and normal bleed time
CF assays : low factor VIII or IX
131
Management of haemophilia
Avoid NSAIDs and IM injections
For any acute bleeding: elevation, compression and TXA
IV recombinant factor VIII/ IX
For mild disease: desmopressin = anti diuretic that causes secretion of factor VIII and vWF into plasma
For severe disease or before major surgery : regular prophylactic IV factor
132
Definition of massive haemorrhage
Loss of one blood volume in 24h period
Loss of 50% of circulating blood volume in 3h
150ml/min
133
What is blood group determined by
Antigens on RBCs - encoded by A, B and O genes on chromosome 9
From 3 months old we produce natural IgM antibodies against the A or B antigens that we dont have
134
What are alternatives to blood transfusion
Cell savers - recycle and clean patients own blood during operations
Iron infusion or B12/folate replacement
EPO
135
Indications for massive blood transfusion
Trauma
Ruptured AAA
GI bleed
Cardiac surgery
136
Complications of massive blood transfusion
Dilution of platelets and clotting factors
Triad: acidosis, hypothermia, coagulopathy
137
What medication can be used to reverse rivaroxaban and apixaban
Andexanet alfa
138
When does myelodysplastic syndrome arise
5 years post chemotherapy / radiotherapy
139
What can Myelodysplasia progress to
Acute myeloid leukaemia
140
What is the difference between acute and chronic leukaemia
Acute is rapidly progressing
Chronic is slowly progressing
141
What is ALL associated with
Down’s syndrome
Sometimes Philadelphia chromosome (more common in CML)
142
What is richters transformation
When CLL transforms into high grade B cell lymphoma (rare)
143
Describe the chronic phase of CML
Asymptomatic
- incidental finding of a raised WCC
Can last several years
144
What is the accelerated phase of CML
Abnormal blast cells take up a high proportion (10-20%) of the bone marrow and blood cells
- anaemia
- thrombocytopenia
- immunodeficiency
145
What is the blast phase of CML
Over 20% of the blast cells in the blood
Severe symptoms
Pancytopenia often fatal
146
What is CML associated with
Philadelphia chromosome (abnormal chromosome 22 due to a swap of genetic material between section of chromosome 9 and 22)
Abnormal gene sequence called BCR-ABL1 - codes for abnormal TK enzyme that drives proliferation of abnormal cells
147
Characteristic finding in AML on blood film
Auer rods
High proportion of blast cells
148
Characteristic finding in AML on blood film
Auer rods
High proportion of blast cells
149
What electrolyte imbalances does tumour lysis syndrome result in
High Uric acid
High K+
High phosphate
Low Ca
150
What causes anaphylaxis during a blood transfusion
Patients with an IgA deficiency who have anti-IgA antibodies
151
What causes anaphylaxis during a blood transfusion
Patients with an IgA deficiency who have anti-IgA antibodies
152
Features and management of non haemolytic febrile reaction (in blood transfusion)
Fever, chills
Slow / stop transfusion
Paracetamol
Monitor
153
Features and management of minor allergic reaction during blood transfusion
Pruritus, urticaria
Temporarily stop transfusion
Antihistamine
Monitor
154
Features and management of anaphylaxis in blood transfusion
Hypotension
Dyspnoea
Wheezing
Angioedema
Stop transfusion, IM adrenaline, oxygen, fluids
155
Features and management of acute haemolytic reaction in blood transfusion
Fever, abdo pain, hypotension
Stop transfusion, send blood for direct Coombs test, repeat typing and cross matching
Fluid resuscitation
156
Features and management of acute haemolytic reaction in blood transfusion
Fever, abdo pain, hypotension
Stop transfusion, send blood for direct Coombs test, repeat typing and cross matching
Fluid resuscitation
157
Features and management of transfusion associated circulatory overload
Pulmonary oedema, HTN
Slow / stop transfusion
Consider IV loop diuretic
158
Features and management of transfusion related acute lung injury (in blood transfusion)
Hypoxia, pulmonary infiltrates on CXR, fever, hypotension
Stop transfusion
Oxygen and supportive care
