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Haematology Flashcards

1
Q

What are the symptoms of anaemia?

A 
Fatigue
Dyspnoea
Dizziness
Palpitations
Headache
Tinnitus
Anorexia
Angina (if pre-existing coronary disease)
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2
Q

Signs of anaemia?

A

Pallor (+ of conjunctiva)
Tachycardia
Flow murmurs (ejection systolic over apex)
Rare: retinal haemorrhage, cardiac enlargement, heart failure

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3
Q

Causes of microcytic anaemia?

A 
Iron deficiency
Thalassaemia
Sideroblastic
Anaemia of chronic disease (usually normocytic)
Lead poisoning (rare)
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4
Q

Causes of normocytic anaemia?

A 
Chronic disease
Acute blood loss
Bone marrow failure
Renal failure (erythropoietin)
Hypothyroid
Haemolysis
Pregnancy
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5
Q

In a patient with normocytic anaemia who also has low WCC and low platelets, what diagnosis would you suspect?

A

Marrow failure

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6
Q

Causes of macrocytic anaemia?

A 
B12/folate deficiency
Alcohol excess/liver disease
Reticulocytosis (e.g. with haemolysis)
Cytotoxics, e.g. hydroxycarbamide
Myeloma
Marrow infiltration
Hypothyroid
Antifolate drugs, e.g. phenytoin
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7
Q

Causes of iron deficiency anaemia?

A

Blood loss (menorrhagia, GI)
Poor diet
Malabsorption (coeliac)
Hookworm

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8
Q

Signs of iron deficiency anaemia?

A

Koilonychia (spoon-shaped nails)
Atrophic glossitis
Angular stomatitis

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9
Q

Tests in iron deficiency?

A 
FBC
Haematinics
Blood film (target cells, poikilocytes)
Coeliac serology
Gastroscopy + colonoscopy
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10
Q

Treatment for iron deficiency anaemia?

A

Treat the cause
Ferrous sulphate - continue until Hb normal + another 3 months to replenish stores
(then IV iron)

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11
Q

Side effects of ferrous sulphate?

A

Nausea
Abdominal discomfort
Constipation/diarrhoea
Black stools

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12
Q

Pathophysiology behind anaemia of chronic disease?

A

Poor use of iron in erythropoiesis
Cytokines shorten RBC survival
Reduced EPO production + response to it

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13
Q

List some examples of causes of anaemia of chronic disease

A 
Malignancy
RA
Chronic infection
Vasculitis
Renal failure
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14
Q

Management of anaemia of chronic disease?

A

Treat underlying cause

EPO

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15
Q

What is sideroblastic anaemia?

A

Ineffective erythropoiesis and iron loading in the marrow

*think of sideroblastic anaemia whenever microcytic anaemia does not respond to iron

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16
Q

What is haemosiderosis in sideroblastic anaemia?

A

Endocrine, liver and heart damage due to iron deposition

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17
Q

Causes of sideroblastic anaemia?

A 
Congenital (delta-aminolevulinate synthase 2 deficiency)
Idiopathic
Chemo
Anti-TB drugs
Irradiation
Alcohol excess
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18
Q

What test results would be seen in sideroblastic anaemia?

A 
Low Hb
Low MCV
High ferritin
Hypochromic blood film
Sideroblasts in marrow biopsy
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19
Q

Treatment of sideroblastic anaemia?

A

Remove cause
Transfusion
Pyridoxine

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20
Q

Tests in macrocytic anaemia?

A

FBC, haematinics, LFT, TFT
Blood film
Bone marrow biopsy

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21
Q

In what foods is folate found?

A

Liver
Green vegetables
Nuts
Yeast

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22
Q

Maternal folate deficiency causes what problems in the foetus?

A

Neural tube defects

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23
Q

List some causes of folate deficiency

A

Poor diet (elderly, poverty, alcoholics)

Increased demand, e.g. pregnant, increased cell turnover (CA, haemolysis, inflammatory disease, renal dialysis)

Malabsorption (coeliac)

Alcohol

Drugs (anti epileptics, methotrexate, trimethoprim)

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24
Q

Management of folate deficiency?

A

Assess for poor diet
Coeliac serology

Folic acid + B12

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25
Causes of glossitis?
``` B12 deficiency Iron deficiency Contact dermatitis/ food allergy Crohns, coeliac Drugs Alcoholism ```
26
Causes of B12 deficiency?
Diet (vegan, elderly) Malabsorption (PA, crohns, resection, pancreatic insufficiency) Chronic biguanide (metformin) use
27
Food sources of B12?
Meat Fish Dairy products
28
How does absorption of B12 occur?
Intrinsic factor from parietal cells in the stomach bind to B12, allowing it to be absorbed in the terminal ileum
29
What is the alternative name for B12?
Cobalamin
30
What are the clinical features of B12 deficiency?
Lemon tinged skin (pallor + jaundice) Glossitis Anaemia Sx Psych: irritable, dementia, depression, psychosis Parasthesia, peripheral neuropathy Subacute combined degeneration of the spinal cord
31
What is the classical triad of subacute combined degeneration of the spinal cord?
Extensor plantars Absent knee jerks Absent ankle jerks
32
What is the pathology behind pernicious anaemia?
Autoimmune atrophic gastritis Leading to reduced secretion of intrinsic factor from parietal cells Unbound B12 therefore not absorbed
33
What other diseases may be associated with pernicious anaemia?
Autoimmune: thyroid, addison's, vitiligo, hypoparathyroidism | Gastric carcinoma
34
Investigations in pernicious anaemia?
``` FBC (Hb, MCV, wcc, platelets) Haematinics Parietal cell autoantibodies Intrinsic factor antibodies Blood film (hypersegmented neutrophils) Marrow ```
35
Management of B12 deficiency?
Treat cause | B12 IM/ PO
36
Why is there a marked continuing high MCV after initiating B12 treatment for deficiency?
Reticulocytosis
37
Normal lifespan of a RBC?
120 days
38
Haemolysis can occur intravascularly or extravascularly. Explain extravascular haemolysis.
Macrophages in liver, spleen, bone marrow (reticuloendothelial system)
39
Give some investigation findings that would support increased red cell breakdown
Raised bilirubin Low Hb, normal/high MCV High urinary urobilinogen High serum LDH (released from red cells)
40
What blood test finding would suggest increased red cell production?
High MCV (reticulocytosis)
41
What examination finding might suggest extravascular haemolysis?
Splenomegaly
42
Give some important aspects of the history in suspected haemolytic anaemia?
``` FHx Previous anaemia Ethnicity Dark urine Drugs Travel ```
43
Important aspects of the examination in suspected haemolytic anaemia?
Jaundice Hepatosplenomegaly Gallstones (^bili) Leg ulcers
44
Investigations in haemolytic anaemia?
``` FBC LFT (bili) LDH Urinary urobilinogen Haptoglobin Blood film ``` If travel Hx - malaria thick + thin films Extras: osmotic fragility testing direct coombs test Hb electrophoresis
45
What does coombs test identify?
Immune causes of haemolytic anaemia | Identifies RBCs coated with antibody
46
Acquired causes of haemolytic anaemia?
``` Infection Autoimmune Malignancy CLL, lymphoma TTP, HUS Drugs DIC Transfusion Hep B+C Vaccinations Pre-eclampsia ```
47
Hereditary causes of haemolytic anaemia?
Enzyme defect: G6PD deficiency, PKD Membrane defect: hereditary spherocytosis Haemoglobinopathy: sickle cell, thalassaemia
48
How is sickle cell inherited?
Autosomal recessive
49
Pathophysiology of sickle cell?
Abnormal Hb results in deformed RBCs (sickle cells) | These haemolyse readily -> Vaso-occlusive crisis
50
Investigation findings in sickle cell?
FBC: low Hb, ^reticulocytes/MCV Blood film: sickle cells + target cells LFT: ^bili
51
Triggers for vaso-occlusive 'painful' crisis in sickle cell?
Cold Hypoxia Dehydration Infection
52
Presentation of vaso-occlusive 'painful' crisis in sickle cell?
``` Severe pain Dactylitis in <3 yr olds Acute abdomen (mesenteric ischaemia) Stroke, seizure, cognitive defects Avascular necrosis (eg femoral head) Leg ulcers Priapism ```
53
What is a sequestration crisis in sickle cell? Management?
Pooling of blood in spleen + liver > organomegaly, shock, severe anaemia Transfusion
54
What is an aplastic crisis in sickle cell?
Parvovirus B19 infection leads to sudden reduction in marrow production of RBCs
55
Complications of sickle cell?
``` Splenic infarction Poor growth CKD Gallstones Retinal disease Iron overload Lung hypoxia -> Fibrosis -> Pulm HTN ```
56
Management of chronic sickle cell?
``` Analgesia for crises Hydroxycarbamide Abx prophylaxis + immunisations (splenic infarct) Transfusions Bone marrow transplant ```
57
Management of sickle cell crisis?
``` Analgesia Septic screen Fluids O2 Abx if pyrexial Transfusion/ exchange transfusion ```
58
Sx of acute chest syndrome in sickle cell?
``` Wheeze Dyspnoea Pain Fever Cough ```
59
What causes the infiltrates in acute chest syndrome in sickle cell?
Infection Fat embolism from bone marrow
60
Management of acute chest syndrome in sickle cell?
``` O2 Analgesia Abx (cephalosporin + macrolide) Bronchodilators in wheeze Transfusion/ exchange ```
61
Examples of macrolide antibiotics?
Azithromycin Clarithromycin Erythromycin
62
Examples of cephalosporin antibiotics?
Cefuroxime | Ceftriaxone
63
What is thalassaemia?
Reduced production of one globin chain | Leads to haemolysis
64
Where in the world is thalassaemia common?
Med to far East
65
Ix in beta thalassaemia?
``` FBC Haematinics Film Hb electrophoresis MRI (for myocardial siderosis) ```
66
Clinical features of patient with beta thalassaemia?
``` Presents in 1st year of life Severe anaemia Failure to thrive Hepatosplenomegaly Skull deformity Osteopenia ```
67
Management of beta thalassaemia?
Life-long regular transfusions Folate Iron chelators (deferipone + desferrioxamine) Ascorbic acid -> iron excretion Splenectomy Management of endocrine complications (DM, thyroid) Marrow transplant
68
Side effect / risk of regular transfusions in beta thalassaemia, and the consequences of this?
Iron overload Endocrine: pituitary, thyroid, pancreatic failure (DM) Liver disease Cardiac toxicity
69
What are the 3 processes that halt bleeding after injury? (+ therefore the 3 types of bleeding disorder)
Vasoconstriction Platelet plugging Coagulation cascade
70
Bleeding disorders. Platelet/Vascular vs Coagulation disorder. What is the pattern of bleeding in platelet/vascular disorders?
1. Prolonged bleeding from cuts 2. Bleeding into skin (bruising, purpura) 3. Mucous membranes (epistaxis, gums, menorrhagia)
71
What is the bioactive form of folate?
THF (tetrahydrofolate)
72
Why does a B12 deficiency cause a folate deficiency?
Methionine synthase (B12-dependent enzyme) is required for the formation of the bioactive form of folate, tetrahydrofolate (THF)
73
Which tends to be more severe: extravascular, or intravascular haemolysis?
Intravascular
74
What is the difference between intravascular and extravascular haemolysis?
Extravascular: ^RBC breakdown, e.g. by macrophages Intravascular: destruction of RBCs in circulatory system
75
What is multiple myeloma?
Cancer of bone marrow plasma cells (differentiated B lymphocytes)
76
What is the peak incidence for multiple myeloma?
60-70 years
77
What are the clinical features of multiple myeloma?
``` CRAB HyperCalcaemia Renal impairment Anaemia Bone lesions/disease ``` Others: bone disease, lethargy, infection + progressive bone marrow failure, paraprotein production, amyloidosis, macroglossia, CTS, neuropathy, hyperviscosity
78
What are the SYMPTOMS of myeloma that patients present with?
Bone disease: Backache, pathological fractures (bone ribs), vertebral collapse Hypercalcaemia: Moans, groans, bones etc Frequent infections Symptoms of anaemia (fatigue, pale, SOB)
79
What investigation should be carried out for all patients over 50 with back pain?
Serum protein electrophoresis and ESR
80
What Ix should be done for multiple myeloma?
FBC (anaemia) Serum monoclonal proteins (usually IgG/IgA) Urine proteins (Bence Jones proteins) BMBx (bone marrow biopsy - increased plasma cells Blood film (roleaux formation - stacked RBCs) Imaging (XR - lytic lesions - rain-drop skull)
81
What is the term given to the lytic lesions seen on X-ray in multiple myeloma?
'Rain-drop skull'
82
In what condition is 'pepper-pot skull' seen?
Hyperparathyroidism
83
What conditions are urinary Bence Jones proteins seen in?
Multiple myeloma Waldenstrom's macroglobulinaemia
84
What are the diagnostic criteria for multiple myeloma?
One major and one minor OR 3 minor criteria
85
What are the major criteria for diagnosis of multiple myeloma?
Plasmacytoma 30% plasma cells in bone marrow sample ^M protein in blood/urine
86
What are the minor criteria for multiple myeloma diagnosis?
10-30% plasma cells in bone marrow sample Minor ^M protein in blood/urine Osteolytic lesions Low levels of antibodies (not from cancer cells) in blood
87
Give 4 poor prognostic features of multiple myeloma
>2 osteolytic lesions Beta-2 macroglobulin >5.5mg/L Hb <11g/L Albumin <30g/L
88
What are the principles of management of myeloma?
Only treat symptomatic patients For asymptotic MM and MGUS - regular monitoring
89
How is symptomatic myeloma managed?
If candidate for stem cell transplant - induction treatment followed by stem cell transplant If not - intensive drug treatment
90
What drug treatments may be used in the management of multiple myeloma?
Thalidomide or bortezomib based chemo- regimes, alongside dexamethasone Supportive treatment alongside chemo: analgesia, bisphosphonates, blood transfusions/EPO to treat anaemia
91
What is monoclonal gammopathy of undetermined significance?
Asymptomatic premalignant plasma cell proliferative disorder (M protein <3/dL)
92
Give 2 risk factors for MGUS
Male | Agent Orange exposure
93
What will be seen on bone marrow biopsy in a patient with MGUS?
Plasma cells <10%
94
What is lymphoma?
Malignant proliferation of lymphocytes These accumulate in lymph nodes and cause lymphadenopathy (also found in peripheral blood/other organs, e.g. liver, spleen, bone marrow)
95
What are the causes of lymphoma?
Most cases unknown Infection (EBV, HTLV-1, H pylori) Primary immunodeficiency (ataxia telangiectasia, Wiscott-Aldrich syndrome) Secondary immunodeficiency (HIV, transplant patients) Autoimmune disorders
96
What are the symptoms of Hodgkin's lymphoma?
Enlarged, painless, non-tender, 'rubbery' superficial lymph nodes. Asymmetrical. Constitutional 'B' symptoms

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