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Anaemia Flashcards

1
Q

General features of anaemia

A
  • fatigue, weakness
  • pallor due to vasoconstriction and blood redistribution
  • dyspnoea (in severe anaemia can –> incipient cardiorespiratory failure)
  • Palpitations and tachycardia
  • Headaches and tinnitus
  • chest pain (exacerbation of angina)
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2
Q

Causes of microcytic anaemia

A
  • Iron deficiency anaemia
  • Anaemia of chronic disease
  • Thalassaemia
  • Sideroblastic anaemia
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3
Q

Causes of iron deficiency anaemia

A
  • Inadequate intake
  • impaired absorption
  • increased loss
  • increased requirements
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4
Q

symptoms of iron deficiency anaemia

A
  • Nail flattening and koilonychia
  • glossitis
  • angular stomatitis
  • dysphagia due to oesophageal web and gastritis
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5
Q

Blood tests results in iron deficiency anaemia

A
  • low MCHC (hypochromic)
  • low serum iron
  • low ferritin
  • TIBC high
  • transferrin saturation low
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6
Q

What would a blood film show in iron deficiency anaemia?

A
  • small, pale red cells
  • anisopoikilocytosis (variance in size and shape)
  • pencil cells
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7
Q

What is anaemia of chronic disease?

A

Defects in iron utilisation, mainly caused by inflammation as cytokines trigger changes in intracellular iron metabolism

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8
Q

Blood test results in anaemia of chronic disease?

A

(anaemia is rarely severe)

  • MCV/MCHC low or normal
  • high ferritin
  • low serum iron
  • TIBC may be low
  • transferrin saturation low
  • reticulocutes low for the degree of anaemia
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9
Q

What does alpha thalassaemia major involve?

A
  • Deletion of all 4 genes so no synthesis of alpha chains
  • Hb-Barts hydrops syndrome
  • death occurs in utero as HbF and HbA both need alpha chains
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10
Q

What does alpha thalassaemia traits involve?

A
  • deletion of 1 gene –> slight lowering of MCV and MCHC
  • deletion of 2 genes minimally lowers Hb with a raised red cell count, polychormasia and microcytosis
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11
Q

What does ß thalassaemia major involve?

A
  • No functional ß chains are produced - apperent between 3 - 6 months when [HbF] decreases
  • Severe anaemia, failure to thrive, hepatosplenomegaly
  • Compensatory bone marrow expansion –> skull bossing and maxillary enlargement (hair on end appearance)
  • Blood film shows hypochromic microcytic red cells, target cells, tear drop cells and nucleated red cells
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12
Q

What does ß thalassaemia intermedia involve?

A
  • Some ß chains produced **?
  • patients present later, 2 - 5y/o
  • Mild anaemia, moderate bone changes and normal growth
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13
Q

What does ß thalassaemia trait involve?

A
  • Usually asymptomatic
  • Mild anaemia with microcytic hypochromic red cells
  • often confused with iron deficiency

Investigations: Hb electrophoresis and hb analysis

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14
Q

Holistic treatment of ß thalassaemias?

A
  • Education on risk of acute events
  • genetic counselling
  • traits and intermedia often don’t need treatment
  • Major requires regular transfusions (2 - 4 weeks, splenectomy can reduce frequency) with chelation therapy
  • stem cell transplant
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15
Q

What is sideroblastic anaemia?

A
  • defects in haem synthesis and other pathways needed for erythroblast mitochondria
  • this lead to decreased Hb production and abnormal iron metabolism
  • this leads to accumulation of iron arranged in a ring in the nucleated immature erythroblasts

Symtoms of iron overload: cardiac or hepatic dysfunction

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16
Q

Blood results in sideroblastic anaemia?

A
  • variably severe anaemia
  • usually microcytic hypochromic but can sometimes be dimorphic (depends on cause)
  • systemic iron overload may be present

peripheral blood smear would show ring sideroblasts with perinuclear ring of iron granules

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17
Q

management of sideroblastic anaemia?

A
  • control anaemia - transfusion if severe
  • prevent organ damage from iron overload
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18
Q

Causes of normocytic anaemia?

A
  • Haemolytic anaemia
  • Hereditary normocytic anaemia
  • acquired normocytic anaemia (autoimmune)
  • Aplastic anaemia
  • Sickle cell anaemia
  • combined microcytic and macrocytic anaemias
  • anaemia of chronic disease
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19
Q

What happens in haemolytic anaemia?

A

Increased destruction of RBCs can be:

  • Intravascular - RBC destruction due to complement-mediated lysis or direct RBC trauma - schistocytes
  • Extravascular - accelerated RBC destruction by the reticuloendothelial system, due to immune targeting by antibodies (phagocytosis occurs in the spleen and liver) - spherocytes
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20
Q

What are the types of hereditary normocytic anaemia?

A
  • Membrane defects
  • Metabolism defects
  • Abnormal Hb
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21
Q

Hereditary normocytic anaemia: what are the types caused by membrane defects?

A
  • hereditary spherocytosis - RBCs lose membrane and become spherica, can’t pass through spleen so die early
  • Hereditary elliptocytosis - similar to HS but elliptical RBCs
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22
Q

Hereditary normocytic anaemia: what are the types caused by metabolism defects?

A
  • G6PD deficiency - needed to reduce NADPH - only source of it is in red cells so leaves them susceptible to oxidative stress
  • Pyruvate kinase deficiency - red cells become rigid as a result of reduce ATP formation
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23
Q

Hereditary normocytic anaemia: what are the types caused by abnormal Hb?

A
  • Thalassaemia
  • Sickle cell anaemia
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24
Q

What are the types of acquired normocytic anaemias?

A
  • Autoimmune
  • Alloimmune
  • Drug-induced
  • Secondary
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25
Q

Acquired normocytic anaemias: what happens when it is autoimmune?

A
  • Caused by antibody production against self cells
  • Characterised by DAT (coomb’s test)
  • Warm autoimmune
    • Ab reacts with RBCs more strongly at 37oC
    • red cells usually coated with IgG
  • Cold autoimmune
    • reacts more strongly with RBCs at 4oC
    • red cells usually coated in IgM
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26
Q

Acquired normocytic anaemias: what happens when it is alloimmune?

A
  • Transfusion of incompatible blood –> acute haemolytic transfusion reaction
  • Haemolytic disease of newborn: if mother is RhD- but baby is + the mother will make antibodies against RhD which can cross the placenta - injections of anti-D given to avoid this
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27
Q

Why would a secondary acquired normocytic anaemia occur?

A

Red cell survival can be shortened in many systemic disorders e.g. malaria, burns, hypersplenism, mechanical heart valve

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28
Q

Symptoms of acquired normocytic anaemias

A
  • jaundice
  • splenomegaly
  • dark urine
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29
Q

Blood test results in acquired normocytic anaemias

A
  • Reticulocytosis
  • elevated serum bilirubin (unconjugated)
  • elevated LDH
  • low haptoglobin (binds to free Hb - low levels means more free Hb)
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30
Q

Blood film results in acquired normocytic anaemias

A
  • Hereditary spherocytosis - spherocytes
  • Hereditary elliptocytosis - elliptical RBCs
  • G6PD deficiency - Heinz bodies (oxidised, denatured Hb)
  • may see schistocytes
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31
Q

Management of acquired normocytic anaemia?

A
  • removal of cause
  • oral corticosteroids
  • rituximab
  • splenectomy
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32
Q

aplastic anaemia

A
  • pancytopenia due to aplasia of the bone marrow (failure)
  • substantial decrease in no. of haematopoietic pluripotent stem cells due to:
    • a fault in remaining ones
    • or an immune reaction against them
  • makes them unable to divide and differentiate to populate the bone marrow
33
Q

Types of primary aplastic anaemia?

A
  • Congenital
    • Fanconi and non-fanconi
  • Acquired
    • Idiopathic acquired is the most common type
34
Q

Causes of secondary aplastic anaemia?

A
  • Ionising radiation - radiotherapy, nuclear power stations
  • Chemicals - benzene, TNT, insecticides, hair dye
  • Drugs - busulphan, cyclophosphamide, anthracyclines, sulphonamides, gold
  • Infection - viral hepatitis
35
Q

Symptoms of aplastic anaemia

A
  • recurrent infection (esp mouth and throat)
  • bleeding/easy bruising (bleeding gums, epistaxis, menorrhagia)
36
Q

Investigation results of aplastic anaemia

A
  • Anaemia is normally normocytic and normochromic
  • Leucopenia (selectively granulocytes then lymphocytes in severe)
  • Thrombocytopenia
  • Reticulocytopenia
  • No abnormal cells in blood film
  • Bone marrow - hypoplasia and loss of haematopoietic tissue and replacement with fat (>75%)
37
Q

Management of aplastic anaemia

A
  • Removal of any known cause
  • Supportive care
    • blood products must be filtered and irradiated
    • blood transfusion, platelet concentrates
    • prevention and treatment of infection
  • Immunosuppressive therapy
    • antilymphocyte globulin, corticosteroids, cyclosporin
  • Allogenic stem cell transplant
38
Q

What is sickle cell anaemia?

A
  • Group of Hb disorders where the sickle ß-globin gene inherited HbS is insoluble and forms crystals when exposed to low [O2]
  • Deoxygenated Hb polymerises into long fibres - red cells sickle and may block large vessels or part of the microcirculation –> infarcts of various organs
  • AS recessive
39
Q

What happens during a severe sickle cell crisis?

A
  • Painful vaso-occlusive crises - precipitated by infection, acidosis, dehydration or deoxygenation –> infarcts of varous organs
  • Visceral sequestration crises: sickling within organs and pooling of blood
  • aplastic crises: may occur due to parvovirus or folate deficiency - sudden fall of Hb usually requiring transfusion
  • Haemolytic crises: increased rate of haemolysis with a fall in Hb but increase in reticulocytes - usually accompanies a painful crisis
    *
40
Q

Treatment of sickle cell anaemia

A
  • avoid precipitating factors
  • vaccinations: pneumococcal, Hib and men. and penicillin to reduce infection rate
  • Hydroxycarbamide is patients with recurring complications
  • Transfusions sometimes given as prophylaxis in those having frequent crises
  • Stem cell transplantation indicated in severe cases
  • Crises treatment: rest, warmth, rehydration, Abx if infection is present and analgesia
41
Q

Investigations of sickle cell anaemia

A
  • DNA based arrays show replacement of ßsubunits with HbS
  • Haematuria is the worst and most common symptom - maybe due to minor infarcts of the renal papillae

*Caution needed with anaesthesia, pregnancy and high altitudes

42
Q

Causes of macrocytic anaemia?

A
  • B12/Folate deficiency
  • Pernicious anaemia
  • Pregnancy
  • Liver disease
43
Q

what is megaloblastic anaemia?

A
  • Caused by B12 or folate deficiency
  • defective DNA synthesis
  • causes delayed maturation of the nucleus of RBCs
  • RBCs die in the bone marrow or enter the bloodstream as enlarged, misshapen cells with reduced survival time.
44
Q

Why does folate deficiency occur?

A
  • Insufficient dietary intake (found in greens, legumes)
  • Increased demand e.g. in pregnancy, lactation
  • Intestinal malabsorption occurs in small intestine disorders such as coeliac
  • Drugs and toxins - alcoholism, sulfathalazine, mtx, trimethoprim, anti-convulsants
45
Q

Symptoms of folate deficiency?

A

weight loss and headaches

46
Q

Investigation findings in folate deficiency

A
  • Blood tests:
    • high MCV and MCHC can precede anaemia by a few weeks
    • low serum folate
  • Blood film:
    • hypersegmented neutrophils
47
Q

Management of folate deficiency

A

Folic acid 1mg PO OD

* ensure no B12 deficiency

48
Q

Causes of B12 deficiency

A
  • Dietary intake (contained in dairy products)
  • stores in the liver last for years so dietary deficiency leads on to chronic deficiency
  • can be due to malabsorption (e.g. gastric band)
49
Q

symptoms of B12 deficiency

A

Weight loss, headaches and paraesthesia

50
Q

Investigation findings in B12 deficiency

A
  • Blood tests - low serum B12
  • Blood film - hypersegmented neutrophils
51
Q

What is pernicious anaemia?

A
  • autoimmune disorder - most common cause of B12 deficiency
  • B12 binds to IF (produced by parietal cells) and is then absorbed in the terminal ileum.
  • Deficiency arises from reduced secretion of IF
52
Q

Investigation findings in pernicious anaemia

A

Blood tests

  • positive for intrinsic factor antibodies
    and/or
  • positive antiparietal cell antibody
53
Q

Management of pernicious anaemia

A

Hydroxycobalamin 1mg IM every 3 months (after initial loading every 2 weeks)

54
Q

How to remember causes of microcytic anaemia

A

TAILS

thalassaemia
anaemia of chronic disease
iron deficiency anaemia
lead poisoning
sideroblastic anaemia

55
Q

pathophysiology of thalassaemia

A
  • Normally Hb has 2 alpha and 2 beta chains
  • Here unequal synthesis of these chains creates abnormal Hb which damages the cell membrane and deforms the cell
  • abnormal cells are destroyed mostly by BM, some by spleen
56
Q

Beta thalassaemia trait?

A
  • autosomal recessive condition, significant microcytosis disproportionate to the anaemia
  • heterozygous defect, so usually asymptomatic
  • Hb electrophoresis shows raised HbA2 and HbF (HbA2 lacks the beta chain)
57
Q

Beta thalassaemia major signs and symptoms?

A
  • Homozygous defect - total lack of beta chains, defect on chromosome 7
  • starts aged 3 - 6 months (when HbF levels decline)
  • Severe anaemia - abnormal RBCs get destroyed
  • Marrow expansion –> skull bossing, maxillary enlargement
  • Jaundice
  • Extramedullary erythropoiesis
  • Failure to thrive
58
Q

Beta thalassaemia major investigations and management?

A
  • FBC - microcytic anaemia
  • Hb electrophoresis - v. increased HbF, increased HbA2
  • Blood film - target cells, nucleated RBC
    Management:
  • Lifelong transfusions
  • SE: Haemochromatosis (Fe overload)
  • SC desferrioxamine - decrease risk of Fe overload
  • Bone marrow transplant may be curative
59
Q

Alpha thalassaemia

A

Trait (mild) asymptomatic
–/aa or a-/a-
Blood film: hypochromic microcytes

HbH disease (less mild)
moderate anaemia - may need transfusions
some haemolysis, can lead to HSM and jaundice

Hb Barts –/–
Incompatible with life, causes death in the womb
Hydrops fetalis

60
Q

How to remember causes of macrocytic anaemia?

A

FATRBC

  • *F**oetus
  • *A**lcohol
  • *T**hyroid (hypo)
  • *R**eticulocytosis
  • *B**12/Folate megaloblastic
  • *C**irrhosis
61
Q

Once classified as macrocytic, what next?

A

Blood film to check where megaloblastic

Megaloblastic anaemia (decreased B12/folate) = hypersegmented neutrophil

Alcohol/liver disease = target cells

62
Q

After blood film, what to order in macrocytic anaemia?

A
  • Megaloblastic anaemia - serum B12, red cell folate
  • LFT
    • bilirubin slightly elevated in B12/folate deficiency as megaloblasts get destroyed and release it
    • also may show liver disease causing macrocytosis
  • TFT-screen thyroid
63
Q

what might a bone marrow biopsy show in macrocytic anaemia?

A

megaloblastic erythropoiesis

64
Q

Folate:
source?
storage duration?
absorption site?
deficiency caused by?

A

sources: greens, nuts, liver
stores last 4 months
absorbed in proximal jejunum

deficiency caused by decreased intake/absorption and increased loss/usage
Also caused by ethanol, phenytoin, methotrexate

65
Q

Folate deficiency treatment

A
  • Assess and treat underlying cause
  • Give B12 first unless levels are normal
  • Folate 5mg/d PO
66
Q

B12 deficiency

Sources?
Storage duration?
Absorption site?

Causes of deficiency

A

Sources: meat, fish, dairy
Stores last for 4 years
Absorbed in terminal ileum, requires intrinsic factor made by gastric parietal cells
Used in DNA and myelin synthesis

Deficiency caused by vegan diet (rare due to food fortification), reduced absorption (GI: pernicious anaemia, Crohn’s, terminal ileum resection, gastrectomy)

67
Q

Features of B12 deficient macrocytic anaemia

A
  • glossitis
  • skin - lemon tinge (pallor + v. mild jaundice)
  • neurological symptoms
    • paraesthesia, peripheral neuropathy, distal sensory loss
    • gait disturbance
      • degeneration of spinal cord (severe)
68
Q

Investigations of B12 deficiency macrocytic anaemia

A
  • FBC - microcytic anaemia, decreased WCC and platelets if severe, bone marrow failure leads to pancytopenia if too deficient
    • intrinsic factor/anti-parietal cell antibodies (pernicious)
69
Q

B12 deficiency management

A

if dietary deficiency - oral cyanocobalamin

if malabsorption - IM hydroxycobalamin

70
Q

what is pernicious anaemia?

A
  • Autoimmune disease, autoantibodies against parietal cells or IF –> reduced acid production and decreased IF
    • Usually >40y/o, increased incidence in blood group A
    • Associations with thyroid disease, vitiligo, Addison’s, decreased HPT, 3x risk of gastric adenocarcinoma
71
Q

What is haemolytic anaemia?

A

Active destruction of red blood cells which leads to anaemia.

Can be extravascular (occurs in spleen) or intravascular

72
Q

What does the haemolysis screen involve?

A
  • Increased unconjugated bilirubin (released in RBC degradation)
  • Increased reticulocyte count (baby RBCs)
  • Schistocytes (fragmented RBCs) in intravascular haemolysis
  • Haemoglobinuria in intravascular haemolysis
73
Q

Causes of acquired haemolysis

A
  • Immune - warm (IgG), cold (IgM)
  • Mechanical - MAHA’s, mechanical heart valve
  • Infection (malaria)
  • Burns
  • PNH
74
Q

hereditary causes of haemolysis

A
  • enzyme - G6PD, pyruvate kinsase deficiency
  • membrane - spherocytosis, elliptocytosis
  • haemoglobin - sickle cell, thalassaemia
75
Q

Autoimmune haemolytic anaemias: warm?

A
  • IgG, binds at 37degrees
  • Extravascular haemolysis
  • Ix - DAT/Coomb’s tests - positive
  • Aetiology: idiopathic, SLE, RA, Evan’s
  • Treatment - immunosuppression, splenectomy
76
Q

Autoimmune haemolytic anaemias: cold?

A
  • IgM, binds at 4degrees
  • Intravascular haemolysis
  • Ix: DAT/Coomb’s tests +ve for complement
  • Aetiology: idiopathic, mycoplasma
  • Treatment: keep warm, rituximab
77
Q

What is hereditary spherocytosis?

A

Autosomal dominant defect of RBC membrane, producing large spherocytes whcih get trapped and haemolysed in spleen (extravascular)

Features: splenomegaly, jaundice, pigment gallstones

78
Q

Complications of aplastic crisis?

A

Aplastic crisis
Even transient bone marrow suppression (e.g. parvovirus) causes problems due to patient’s high rate of RBC destruction/short RBC lifespan

Megaloblastic crisis

79
Q

What are reticulocytes? When do they increase?

A
  • Precursors to RBCs
  • Rise during anaemia as body tries to fix low oxygenation by increasing no. of RBCs
  • Increased in blood loss and haemolytic anaemia
  • low in Iron deficiency anaemia as BM does not have the iron to create red cells

Tan Haematology (5 decks)

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