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Integrated Med > Haematology > Flashcards

Haematology Flashcards

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

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2
Q

What is erythropoiesis

A

RBC formation

  • stimulated by EPO
    Lifespan = 120d
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3
Q

Define agranulocytosis

A

Absence of circulating neutrophils

Side effect of carbimazole

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4
Q

Causes of neutrophilia (>10)

A

Bacterial infection
Corticosteroids
Neoplasia / CML
Myeloproliferative disorder
Bleeding / burns
Inflammation
Smoking

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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

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6
Q

Causes of lymphocytosis (>5)

A

Viral infection
CLL and lymphoma
Chronic infection
Septic shock / MI / trauma
Smoking
Raised BMI / metabolic syndrome

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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

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8
Q

Define anaemia

A

Hb <130 in men
Hb <115 in women

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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

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10
Q

General signs / symptoms of anaemia

A

Fatigue / weakness
Pallor (conjunctival)
SOB / tachycardia / dizziness

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11
Q

Specific signs / symptoms of anaemia

A

Koilonychia : IDA
Jaundice : haemolytic anaemia
Leg ulcers: sickle cell disease
Tingling fingers / toes : B12 deficiency

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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

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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

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14
Q

DD for microcytic anaemia (TAILS)

A

Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

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15
Q

DD for macrocytic anaemia (ABCDEF)

A

Alcohol and liver disease
B12 deficiency
Compensatory reticulocytosis
Drugs
Endocrine
Folate deficiency

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16
Q

DD for normocytic anaemia (CHARMD)

A

Chronic disease
Haemolysis
Acute blood loss
Renal anaemia
Marrow disorder
Deficiencies combined

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17
Q

Causes of IDA

A

Inadequate intake
Malabsorption - coeliac, gastrectomy
Increased requirements - pregnancy
Chronic blood loss - menorrhagia / GI bleed

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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

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19
Q

Foods to avoid in IDA

A

Excess cows milk (only 10% iron is absorbed)
Tannin (tea) - inhibits Fe absorption

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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

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21
Q

Management of IDA

A
  1. Determine cause: thorough hx and exam
  2. Treat underlying cause
  3. Dietary advice
  4. Oral iron supplements eg ferrous sulphate / ferrous fumurate
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22
Q

What is B12 and folate

A

B12 = coenzyme needed for folate conversion
Folate = needed for RBC synthesis

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23
Q

What is B12 and folate

A

B12 = coenzyme needed for folate conversion
Folate = needed for RBC synthesis

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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

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25
Q

Causes of folate deficiency

A

Low dietary intake
Malabsorption (duodenum / jejunum) eg coeliac / jejunal resection
Increased requirements - pregnancy, haemolytic anaemia

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26
Q

Diagnosis for B12 & folate deficiency

A

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

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27
Q

Management of B12 and folate deficiency

A

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

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28
Q

What is important to remember if B12 and folate deficiency both present

A

Must replace B12 first to avoid subacute combined degeneration of the spinal cord

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29
Q

What is RBC aplasia

A

Failure of RBC synthesis

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30
Q

Causes of RBC aplasia

A

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

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31
Q

Clinical features of haemolytic anaemia

A

Anaemia (Normo/macrocytic)
Mild splenogmegaly
Jaundice

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32
Q

Investigations for haemolytic anaemia

A

FBC: decreased Hb
Blood film: increased reticulocytes
Increased unconjugated Bilirubin
Increased LDH
Decreased haptoglobin

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33
Q

Extrinsic causes of haemolytic anaemias

A
  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)
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34
Q

Causes of microangiopathic anaemia

A

Malignant HTN / pre-eclampsia
HUS, DIC, vasculitis, TTP, post BM transplant
Prosthetic heart valve

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35
Q

Intrinsic causes of haemolytic anaemias

A

Hb alpha or beta chain abnormality (thalassaemia, SCD)
RBC membrane abnormality (hereditary spherocytosis)
Enzyme defects (G6PD deficiency, pyruvate kinase deficiency)

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36
Q

What is hereditary spherocytosis

A

Auto dominant or de novo mutations of RBC membrane proteins

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37
Q

Pathogenesis of hereditary spherocytosis

A

Abnormal membrane can’t deform well -> can’t pass through the spleen -> RBC destroyed in the spleen

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38
Q

Clinical presentation of hereditary spherocytosis

A

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

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39
Q

Diagnosis of hereditary spherocytosis

A

FBC and iron studies - normocytic anaemia
Blood film - spherical RBCs with no central pallor + some microspherocytes

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40
Q

Pathogenesis of pyruvate kinase deficiency

A

Low levels of PK enzyme result in failure of RBCs to produce ATP -> RBCs haemolysed prematurely

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41
Q

Clinical presentation of pyruvate kinase deficiency

A

Chronic haemolytic anaemia - varies greatly in severity

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42
Q

Diagnosis of pyruvate kinase deficiency

A

FBC and iron studies - normocytic anaemia
PK activity levels - reduced
Direct Coombs test - negative

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43
Q

Management of hereditary spherocytosis and pyruvate kinase deficiency

A

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

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44
Q

What is a G6PD deficiency

A

X linked mutation in G6PD gene (enzyme involved in preventing oxidative damage to RBCs)

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45
Q

Risk factors for G6PD deficiency

A

African
Mediterranean
Middle Eastern

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46
Q

Clinical presentation of G6PD deficiency

A

Asymptomatic between haemolytic episodes

Neonatal jaundice - usually within first 3d
Chronic haemolysis
Acute intravascular haemolysis - fever, pallor, malaise, dark urine

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47
Q

Diagnosis of G6PD deficiency

A

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

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48
Q

Management of G6PD deficiency

A

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

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49
Q

Pathogenesis of paroxysmal nocturnal haemoglobinuria (PNH)

A

A rare acquired disorder of haematopoietic stem cells which causes production of defective RBCs
Defective cells are haemolysed by the body’s complement system

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50
Q

Symptoms of PNH

A

Episodic haemoglobinuria (dark urine)
Anaemia (SOB, chest pain, fatigue)

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51
Q

Complications of PNH

A

Thromboses: increased risk of blood clots
Pancytopenia : increased risk of infection and bleeding + anaemia

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52
Q

Management of PNH

A
  1. Symptomatic eg iron replacement for anaemia
  2. Folate replacement for ongoing haemolysis
  3. +/- anticoagulation : thrombosis prophylaxis
  4. +/- monoclonal antibodies with block complement
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53
Q

What is the difference between B-thalassaemia major and B-thalassaemia minor

A

Major: both genes abnormal: no HbA = very severe anemia
Minor: 1 gene abnormal = almost normal Hb with marked low MCV

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54
Q

Clinical features of B-thalassaemia major

A

Severe anaemia - transfusion dependent
Jaundice and pallor
Hepatosplenomegaly
Characteristic facies (frontal bossing)

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55
Q

Management of beta thalassaemia

A

Lifelong monthly blood transfusions
Iron chelation
BM transplant = only cure

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56
Q

What are the types of alpha thalassaemia

A
  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
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57
Q

What are ways of investigating thalassaemias

A
  1. FBC and iron studies : microcytic hypochromic anaemia
  2. Blood film : ‘target cells or nucleated RBC
  3. Hb HPLC: proportions of HbA, HbF, HbA2
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58
Q

Cause of sickle cell disease

A

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

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59
Q

Pathogenesis of sickle cell disease

A

Sickle shaped RBCs have decreased lifespan -> get stuck and occlude vessels causing infarction

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60
Q

Prognosis of sickle cell disease

A

Most severe form of= 50% die by 40y from complications

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61
Q

Long term problems of sickle cell disease

A

Short stature / delayed puberty
Stroke and neuro damage
Heart failure and renal dysfunction
Pigment gallstones

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62
Q

Clinical presentation of sickle cell disease

A

Moderate anaemia : 60-100g/L
Jaundice
Infection : increased susceptibility pneumococcus and H influenzae
Splenomegaly
Painful vaso occlusive crises
Acute anaemia (aplastic crisis)

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63
Q

Diagnosis of sickle cell disease

A
  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
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64
Q

Triggers of vaso occlusive crises

A

Cold
Infection
Dehydration
Hypoxia / altitude
Drugs

65
Q

Triggers of vaso occlusive crises

A

Cold
Infection
Dehydration
Hypoxia / altitude
Drugs

66
Q

Management of sickle cell disease

A
  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
Q

Management of vaso occlusive crises

A
  1. ABCDE
  2. Analgesia: IV/SC morphine + paracetamol and ibuprofen
  3. FBC, reticulocytes, infection screen
  4. Cross match and transfusion
  5. Enoxaparin
68
Q

What is pancytopenia

A

Combination of anaemia, leukopenia and thrombocytopenia

69
Q

Aetiology of pancytopenia

A
  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
Q

Common differentials of pancytopenia

A

Acute leukaemia
Multiple myeloma
Hypersplenism - cirrhosis, PVT
Felty’s syndrome
Chemotherapy / radiotherapy
B12 / folate deficiency
Some non Hodgkin lymphoma
Myelodysplasia

71
Q

Less common differentials of pancytopenia

A

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
Q

Investigations for pancytopenia

A
  1. History
  2. Examination: petechiae / purpura, lymphadenopathy, abdo mass
  3. Blood tests
  4. Bone marrow aspirate and biopsy
  5. Imaging
73
Q

What is a myeloproliferative disorder

A

Bone marrow disorders with differentiated myeloid cell subtype expansion in blood

Known as myeloproliferative neoplasms

74
Q

What is polycythaemia rubra Vera

A

High RBCs

Causes: headaches, fatigue, blurred vision, pruritis, HTN

Tx: reduce clot risk

5% develop AML

75
Q

What is essential thrombocythaemia

A

High platelets

Causes: abdo pain, mottled skin, stroke, burning hands / feet

Tx: reduce stroke risk

5% develop AML

76
Q

What is chronic myeloid leukaemia

A

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
Q

What is primary myelofibrosis

A

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
Q

What is primary myelofibrosis

A

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
Q

What is multiple myeloma

A

Malignant clonal proliferation of plasma cells (due to B cell mutations) -> excess secretion on one type of paraprotein / light chain

Median age = 70y

80
Q

What causes hypercalcaemia

A

Increased osteoclast activity -> fluids + bisphosponates

81
Q

Clinical presentation of multiple myeloma (CRABBI)

A

HyperCalcaemia
Renal dysfunction
Anaemia
Bleeding
Bone pain / lesions
Infection

82
Q

Investigations for multiple myeloma

A
  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
Q

Management of multiple myeloma

A

Mx = palliative

Several lines of chemo + immuno-modulators agents
+ bone protection with bisphosphonates
+ infection prophylaxis
+ radiotherapy for pain in isolates sites

84
Q

Emergency presentations of multiple myeloma

A

Pathological vertebral fracture
Bone pain
Acute renal failure
Spinal cord compression
Hyperviscosity
Hypercalcaemia

85
Q

Poor prognostic factors of acute lymphoblastic leukaemia

A

Age <1 or >10
WBC: >50x10^9L
CNS involvement
Cytogenetic abnormalities in tumour cells
Persisting blast cells after initial chemo

86
Q

Pathogenesis of acute lymphoblastic leukaemia

A

Rapidly proliferating lymphocytes stem cells = accumulation of immature lymphoblast cells (can be a B cell or a T cell)

87
Q

Peak age of acute lymphoblastic leukaemia

A

2-5y

88
Q

Symptoms of acute lymphoblastic and acute myeloid leukaemia

A

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
Q

Management of leukaemia

A
  1. Pre-chemo: dexamethasone
  2. Chemotherapy: induce -> intensify -> maintain
  3. Concurrent treatment : intrathecal chemo
90
Q

Management of acute myeloid leukaemia

A
  1. Pre-chemo : hydroxyurea
  2. Intensive chemo : 3-4 cycles
  3. Risk stratification for further Tx : further chemo or stem cell transplant
91
Q

Prognosis of acute lymphoblastic leukaemia

A

Children: 90% cure rate
Adults: 35-40% 5y survival

92
Q

Acute myeloid leukaemia prognosis

A

Depends on age, genetics and response

93
Q

Pathogenesis of chronic lymphocytic leukaemia

A

Accumulation of apoptosis-resistant B cells

94
Q

Symptoms of chronic lymphocytic leukaemia

A

Often asymptomatic

Anaemia : lethargy, pallor, SOB
Neutropenia: recurrent infections
Thrombocytopenia: bruising, petechiae
B symptoms: malaise, night sweats, weight loss

95
Q

Investigations for chronic lymphocytic leukaemia

A

FBC: dec Hb, dec plts, inc WCC, inc lymphocytes

Peripheral blood film
Flow cytometry on blood to identify CLL cells
Bone marrow biopsy

96
Q

Investigations for chronic lymphocytic leukaemia

A

FBC: dec Hb, dec plts, inc WCC, inc lymphocytes

Peripheral blood film
Flow cytometry on blood to identify CLL cells
Bone marrow biopsy

97
Q

Management of chronic lymphocytic leukaemia

A

If no Sx: watch and wait
B Sx: chemo, targeted therapy

98
Q

Management of chronic lymphocytic leukaemia

A

If no Sx: watch and wait
B Sx: chemo, targeted therapy

99
Q

Prognosis for chronic lymphocytic leukaemia

A

85% 5y survival
A few transform into aggressive lymphoma = very bad prognosis

100
Q

Pathogenesis of chronic myeloid leukaemia

A

Increased production of myeloid progenitors
-> 95% have Philadelphia chromosome

101
Q

Symptoms of chronic myeloid leukaemia

A

Insidious onset
No BM failure
Tiredness / lethargy
Massive splenomegaly: abdo discomfort

102
Q

Investigations for chronic myeloid leukaemia

A
  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
Q

Management of chronic myeloid leukaemia

A
  1. Tyrosine kinase inhibitors : imatinib

-> monitor response
-> some develop resistance to TKIs and need BMT

104
Q

Prognosis of chronic myeloid leukaemia

A

70% 5y survival

Without treatment can transform into AML = very bad prognosis

105
Q

What is the difference between Hodgkin’s lymphoma and non hodgkins

A

Hodgkin’s has reed sternberg cells (10%)
Non Hodgkin’s doesn’t (90%)

106
Q

Aetiology of Hodgkin’s lymphoma

A

Reed sternberg cells (mutated B cell)

-> 50% associated with EBV

107
Q

Peak age of Hodgkin’s lymphoma

A

15-35 and 60

108
Q

Peak age of Hodgkin’s lymphoma

A

15-35 and 60

109
Q

Symptoms of Hodgkin’s lymphoma

A

Painless lymphadenopathy (usually cervical)
SVC / bronchi obstruction
Becomes painful with alcohol

B symptoms in 40%

110
Q

Investigations for hodgkins and non Hodgkin’s lymphoma

A

FBC and film
Lymph node biopsy / excision = diagnostic
Bone marrow biopsy
CT or FDG-PET- stage and grade

111
Q

Management of hodgkins and non Hodgkin’s lymphoma

A

B cell: immunochemotherapy +/- radiotherapy

T cell: immunochemotherapy or just chemotherapy

112
Q

Types of primary haemostasis

A
  1. Vasoconstriction - slows blood for plt adhesion
  2. Platelet adhesion then activation then aggregation
  3. Haemostatic plug- platelets + fibrinogen = soft plug
113
Q

Clinical presentation of impaired primary haemostasis

A

Mucocutaneous bleeds: bruising, petechiae, epistaxis, menorrhagia, gum bleeds

Intra op / immediate post op bleeding

114
Q

Causes of impaired primary haemostasis

A

Impaired vWF = platelets can’t bind endothelium

Impaired platelet function : congenital, medications

Reduced platelet number: thrombocytopenia

115
Q

Causes of thrombocytopenia

A

Increased destruction: sepsis, immune thrombocytopenia

Decreased production: alcohol, leukaemia, aplastic anaemia

Other: congenital, Hypersplenism

116
Q

What is secondary haemostasis

A

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
Q

What is secondary haemostasis

A

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
Q

Clinical presentation of impaired secondary haemostasis

A

Muscular / soft tissue bleeds : extensive bruising
Haemarthrosis
Delayed post op bleeding and poor wound healing

119
Q

Clinical presentation of impaired secondary haemostasis

A

Muscular / soft tissue bleeds : extensive bruising
Haemarthrosis
Delayed post op bleeding and poor wound healing

120
Q

Causes of impaired secondary haemostasis

A

Congenital: haemophilia
Acquired: anticoagulant drugs, dilution post transfusion, liver dysfunction, DIC

121
Q

What is disseminated intravascular coagulation

A

Acquired syndrome with inappropriate systemic activation of coagulation pathways

122
Q

Pathogenesis of disseminated intravascular coagulation

A

Consumption of CFs and plts -> if severe can cause organ dysfunction, infarction and bleeding

123
Q

Causes of disseminated intravascular coagulation

A

Infection
Trauma
Burns
Malignancy
Severe liver failure
Obstetric complications
ABO mismatch transfusion

124
Q

Presentation of disseminated intravascular coagulation

A

Bruising
Excessive bleeding
Renal failure

125
Q

Investigations for disseminated intravascular coagulation

A

FBC: low plts
Coag screen: inc PT and APTT
Inc d dimers and dec fibrinogen

126
Q

Management of disseminated intravascular coagulation

A

Aggressive resuscitation
Treat cause
Support with FFP / cryoprecipitate only if active bleeding

127
Q

What is haemophilia

A

X linked recessive coagulation disorder

128
Q

Clinical features of haemophilia

A

Mild: bleed after surgery
Moderate: bleed after minor trauma
Severe: spontaneous joint / muscle bleeds

129
Q

Investigations for haemophilia

A

FBC, blood film, LFTs
Coag screen: inc APTT and normal bleed time
CF assays : low factor VIII or IX

130
Q

Investigations for haemophilia

A

FBC, blood film, LFTs
Coag screen: inc APTT and normal bleed time
CF assays : low factor VIII or IX

131
Q

Management of haemophilia

A

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
Q

Definition of massive haemorrhage

A

Loss of one blood volume in 24h period
Loss of 50% of circulating blood volume in 3h
150ml/min

133
Q

What is blood group determined by

A

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
Q

What are alternatives to blood transfusion

A

Cell savers - recycle and clean patients own blood during operations

Iron infusion or B12/folate replacement

EPO

135
Q

Indications for massive blood transfusion

A

Trauma
Ruptured AAA
GI bleed
Cardiac surgery

136
Q

Complications of massive blood transfusion

A

Dilution of platelets and clotting factors

Triad: acidosis, hypothermia, coagulopathy

137
Q

What medication can be used to reverse rivaroxaban and apixaban

A

Andexanet alfa

138
Q

When does myelodysplastic syndrome arise

A

5 years post chemotherapy / radiotherapy

139
Q

What can Myelodysplasia progress to

A

Acute myeloid leukaemia

140
Q

What is the difference between acute and chronic leukaemia

A

Acute is rapidly progressing
Chronic is slowly progressing

141
Q

What is ALL associated with

A

Down’s syndrome
Sometimes Philadelphia chromosome (more common in CML)

142
Q

What is richters transformation

A

When CLL transforms into high grade B cell lymphoma (rare)

143
Q

Describe the chronic phase of CML

A

Asymptomatic
- incidental finding of a raised WCC
Can last several years

144
Q

What is the accelerated phase of CML

A

Abnormal blast cells take up a high proportion (10-20%) of the bone marrow and blood cells

  • anaemia
  • thrombocytopenia
  • immunodeficiency
145
Q

What is the blast phase of CML

A

Over 20% of the blast cells in the blood
Severe symptoms
Pancytopenia often fatal

146
Q

What is CML associated with

A

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
Q

Characteristic finding in AML on blood film

A

Auer rods
High proportion of blast cells

148
Q

Characteristic finding in AML on blood film

A

Auer rods
High proportion of blast cells

149
Q

What electrolyte imbalances does tumour lysis syndrome result in

A

High Uric acid
High K+
High phosphate
Low Ca

150
Q

What causes anaphylaxis during a blood transfusion

A

Patients with an IgA deficiency who have anti-IgA antibodies

151
Q

What causes anaphylaxis during a blood transfusion

A

Patients with an IgA deficiency who have anti-IgA antibodies

152
Q

Features and management of non haemolytic febrile reaction (in blood transfusion)

A

Fever, chills

Slow / stop transfusion
Paracetamol
Monitor

153
Q

Features and management of minor allergic reaction during blood transfusion

A

Pruritus, urticaria

Temporarily stop transfusion
Antihistamine
Monitor

154
Q

Features and management of anaphylaxis in blood transfusion

A

Hypotension
Dyspnoea
Wheezing
Angioedema

Stop transfusion, IM adrenaline, oxygen, fluids

155
Q

Features and management of acute haemolytic reaction in blood transfusion

A

Fever, abdo pain, hypotension

Stop transfusion, send blood for direct Coombs test, repeat typing and cross matching
Fluid resuscitation

156
Q

Features and management of acute haemolytic reaction in blood transfusion

A

Fever, abdo pain, hypotension

Stop transfusion, send blood for direct Coombs test, repeat typing and cross matching
Fluid resuscitation

157
Q

Features and management of transfusion associated circulatory overload

A

Pulmonary oedema, HTN

Slow / stop transfusion
Consider IV loop diuretic

158
Q

Features and management of transfusion related acute lung injury (in blood transfusion)

A

Hypoxia, pulmonary infiltrates on CXR, fever, hypotension

Stop transfusion
Oxygen and supportive care

Integrated Med (6 decks)

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