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Year 3 TCDs > Anemia > Flashcards

Anemia Flashcards

1
Q

What are the 9 general signs of anemia?

A
  1. Fatigue
  2. Weakness
  3. Pale/yellowish skin
  4. Irregular heartbeats
  5. SOB
  6. Dizziness/light-headedness
  7. Chest pain
  8. Cold hands + feet
  9. Headache
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2
Q

In a middle-aged male with weight loss and upper GI symptoms what should be excluded and how can this be done?

A

Malignancy must be excluded

Give iron + refer for urgent gastro-enterology assessment

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3
Q

What values are low in a hypochromic, microcytic anemia?

A

Low MCV and MCH

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4
Q

What blood tests would you perform before a total gastroectomy?

A

FBC to check for anemia
Cross match 2 units of blood
Biochemical profile as baseline in case of any post-op complications

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5
Q

What is Imerslund-Grasbeck disease?

A

A rare condition characterised by vitamin B12 deficiency often causing megaloblastic anemia.

Usually appears in childhood

Other features may include failure to thrive, infections, and neurological damage

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6
Q

What is sickle cell anemia?

A

Sickle cell disease is when the bone marrow makes abnormal shaped red cells (I.e. sickle shaped)

Results when the child inherits the abnormal Hb S gene from BOTH parents (autosomal recessive) making the child Hb SS.

Sickle cell trait + sickle cell disease is prevalent in Africa because it protects people against Plasmodium falciparum malaria, a very common tropical parasite passed to humans through mosquitos. The malarial parasite cannot occupy Hb S so the overall parasitic burden is less.

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7
Q

What would a full blood count show in someone with sickle cell anemia?

A

Anemia, usual hemoglobin range from 6-8 g/dl

Size of RBCs are small (microcytic, low MCV) due to the very small disasters of the sickle shaped RBCs

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8
Q

What is sickle cell crisis?

A

When small blood vessels are occluded by a number of sickle shaped red cells which cause infarction of the tissues. These crises are often precipitated by hypoxia, infection and dehydration but often no cause is found

When this occurs patients need to be admitted to hospital for:

  1. Analgesia
  2. IV fluids
  3. Antibiotics

There may need to be a blood transfusion + in severe cases a red-cell exchange transfusion

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9
Q

What is hemolysis, what are the 3 signs to recognise it, and what is one important cause?

A

Hemolysis is when red blood cells break down.

It can be recognised by: the presence of anemia, which is usually microcytic with elevated reticulocytes (>2%) and jaundice with unconjugated bilirubin

One important cause = blood transfusion reaction

  • Antibodies from patient’s blood reject blood from a donor (recognise it as foreign)
  • This is the basis of cross matching tests for blood in the lab
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10
Q

What are the 2 types of Coomb’s tests done?

A
  1. Indirect antiglobin test
    - To antibody in the patient’s serum
    - Test used to cross match blood for suitability for transfusion
    - A positive test means the tested donor red cells are INCOMPATIBLE and SHOULD NOT be given as a transfusion
  2. Direct antiglobulin test (DAT)
    - This test is done less often
    - Detects antibody to patient’s own serum causing an autoimmune haemolytic anemia
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11
Q

What sort of anemia occurs after acute blood loss?

A

Normochromic, normocytic anemia

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12
Q

Is there always an immediate anemia?

A

No b/c until the concentration has changed (hemoglobin is expressed as a concentration)

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13
Q

List 3 causes of normochromic, normocytic anemia’s

A
  1. Acute blood loss
  2. Anemia of chronic disease or 2º anemia
  3. Anemia of renal failure (deficiency of EPO)
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14
Q

What are the signs of shock + visible loss of volume of blood?

A
  • Pale and clammy
  • Pulse
  • BP
  • Level of consciousness
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15
Q

What do normal RBCs look like?

A

Even size, even shape, and an area of central pallor < 33% of the RBC diameter

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16
Q

what are the 3 humanistic deficiencies?

A
  1. Iron
  2. B12
  3. Folate

Must always find out why the humanistic is deficient; likely cause is often age dependent

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17
Q

What are 3 causes of iron deficiency anemia?

A
  1. Inadequate iron-containing foods in the diet
  2. Malabsorption (celiac disease, milk, tea)
  3. Blood loss- especially from GI tract
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18
Q

What are 3 causes of thalassemia?

A
  1. Beta thalassemia trait - 2 genes so a trait (carrier) or beta thalassemia minor has 1 abnormal beta globin gene and is diagnosed by having a raised HbA2
  2. Beta thalassemia major has 2 abnormal genes and so has no HbA only HbF
  3. Alpha thalassemia has 4 genes:
    - 1 gene deleted = silent
    - 2 genes deleted = alpha thalassemia trait
    - 3 genes deleted = hemoglobin H disease
    - 4 genes deleted = Bart’s Hydrops Fetalis
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19
Q

Name the 4 main causes of iron deficiency anemia

A
  1. Physiological - rapid growth, menarche, pregnancy
  2. Neonatal - prematurity, low birth weight, blood loss (including early cord clamping)
  3. Diet - Cow’s milk is the commonest cause in toddlers but not UK adults
  4. GIT - commonest GIT causes are NSAID drugs but in an older male/post-menopausal female COLONIC + GASTRIC CANCERS use also be considered likely until excluded
    - Hiatus hernia, H. Pylori, Duodenal/gastric ulcers, esophageal varies, Meckel’s diverticulum, Celiac disease, Polyposis coli, Milk enteropathy, Hereditary haemorrhaging telangiectasia, Inflammatory bowel disease
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20
Q

How is Iron deficiency anemia diagnosed?

A
  • Low Hb
  • Low MCV
  • Low MCH

Look at ferritin (although not reliable b/c it is low in periods of rapid growth + is an acute reactant protein = high levels in those with infections + inflammatory conditions i.e. RA, severe skin diseases)

  • Serum iron, transferrin saturation, zinc protoporphyrin
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21
Q

What are the signs and symptoms of iron deficiency?

A
  1. Headache (especially with activity)
  2. Craving for non-food items to eat - ‘Pica’
  3. Sore/smooth tongue
  4. Brittle nails or hair loss
  5. Spoon-shaped nails (Koilonychia)
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22
Q

What is haptocorrin?

A

It’s a vitamin B12-binding protein present in high amounts in different body fluids including human milk.

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23
Q

What is intrinsic factor?

A

Glycoprotein secreted by parietalor chief cells of the gastric mucosa.

It has an important role in the absorption of B12 in the intestine

Failure to produce/utilise intrinsic factor = pernicious anemia

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24
Q

What is the cubulin receptor?

A

Cubulin is a protein that acts as a receptor for intrinsic factor-vitamin B12 complexes

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25
Q

What are causes of acquired B12 deficiency?

A
  1. Nutritional - vegan, poor diet, pregnancy
  2. Malabsorption
    - Gastric -> surgery, pernicious anemia (IF and gastric parietal cell antibodies)
    - Intestine -> ileal resection, fish tapeworm, tropical sprue
  3. Get malabsorption of B12 but not deficiency in Crohn’s, Celiac, Cystic Fibrosis
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26
Q

What are causes of acquired folate deficiency?

A
  1. Nutritional - poor diet, Goat’s milk only
  2. Intestinal - celiac, jejune resection
  3. Excessive requirement - pregnancy, prematurity
  4. Increased turnover - chronic hemolysis, severe skin disease
  5. Drugs - methotrexate, anticonvulsants
  6. Excess loss - dialysis
  7. Miscellaneous - alcohol (beer is a good source of folate)
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27
Q

What are the symptoms of B12 deficiency?

A
  1. Insidious onset
  2. Mild jaundice + anemia
  3. Glossitis (inflammation of tongue)
  4. Angular cheilitis (inflammation of the corners of the mouth)
  5. Neuropathy (peripheral neuropathy, subacute degeneration now the cord, optic neuropathy, dementia)
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28
Q

What are the symptoms of folate deficiency?

A

As for B12 deficiency but more often a sensory peripheral neuropathy only

Deficiency in pre-conception is associated with an increased incidence of neural tube defects in babies

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29
Q

What are other causes of macrocytosis other than megaloblastic anemia?

A
  1. Alcohol
  2. Pregnancy
  3. Drugs - chemotherapy, antiHIV drugs
  4. Liver disease
  5. Raised reticulocyte count
  6. Hypothyroidism
  7. Myelodysplasia, including acquired sideroblastic anemia
  8. Aplastic anemia and red cell aphasia
  9. hypoxia
  10. Myeloma + other paraproteinemias
30
Q

What is the definition of hemolytic anemia?

A

Anemia due to the destruction rather than underproduction of RBCs

31
Q

What investigations would you do for hemolytic anemia?

A
  1. FBC with reticulocytes
  2. Blood film - essential
  3. Bilirubin/lactic dehydrogenase (LDH)

Specific:

  1. Coomb’s tests (DAT)
  2. EMA-binding
  3. Glucose 6 phosphates dehydrogenase level
  4. Hemoglobin indentification (HPLC)
32
Q

Name 3 congenital and 4 acquired causes of hemolytic anemia

A

Congenital:

  1. RBC membrane (hereditary eplliptocytosis, hereditary spherocytosis)
  2. RBC enzyme deficiencies (G6PD, Pyruvate kinase)
  3. RBC hemoglobin disorders (thalassemia, sickle cell disease)

Acquired:

  1. Autoimmune hemolysis (AIHA)
  2. Microangiopathic hemolytic anemia (HUS, TTP, DIC)
  3. Drugs, infections, toxins
  4. Copper deficiency (Wilson’s Disease)
33
Q

What is hereditary spherocytosis?

A
  • Inherited abnormalities of red cell membrane proteins (spectrin, actin)
  • Autosomal dominant
  • Micro-spherocytosis + polychromatic macrocytes
  • Neonatal jaundice
  • Chronic hemolysis
  • Jaundice
  • Gallstones

Diagnosis:

  • Fam history (positive in ~75%)
  • FBC
  • Reticulocyte count
  • Blood film
  • If above not positive, use specific test: EMA-binding

Treatment:

  • Give folic acid regularly
  • Often needs splenectomy
34
Q

What is glucose-6-phosphate dehydrogenase deficiency?

A
  • X-linked disorder (lyonisation = inactivation of an X chromosome)
  • Usually well between attacks
  • Often family history or history of neonatal jaundice
  • Sudden onset of:
    1. Feeling unwell + lack of energy
    2. Become pale and yellow in colour have a backache
    3. Passing dark coloured urine

Avoid:

  • Beans
  • Anti-malarial
  • Aspirin
  • Vitamin K
35
Q

What is the pathophysiology of sickle cell disease?

A
  • Beta globin variant
  • Co-dominant
  • Carriers HbAS normal
  • Usually HbSS
  • Auto-infarction of spleen with increased infection risk

Crises:

  • Painful
  • chest
  • Abdominal
  • Stroke
36
Q

What is the long term management of sickle cell disease?

A

Simple measures can reduce the incidence of painful crises + infections:

  • keep warm
  • keep hydrated
  • Eat well
  • take penicillin + folic acid (prevents pneumonia)
37
Q

What are the complications of sickle cell disorder?

A
  1. Stroke
  2. Increased vulnerability to infection
  3. Acute chest syndrome - lungs suddenly lose their ability to breath in oxygen (often result of an infection)
  4. Pulmonary hypertension - where the blood pressure inside the blood vessels that connects the heart to the lungs becomes dangerously high
38
Q

Why is surgery a problem for patients with sickle cell disease?

A

Propensity to hypoxia:

  1. Pre-existing anemia
  2. Pre-operative dehydration
  3. Anaesthesia
  4. Operative blood loss

Stress of the surgery predisposes to sickle crises:

  1. Fever
  2. Post-op hypoxia
  3. Post-op infection

These predispose to development of:

  1. Painful crises
  2. Acute chest syndrome
39
Q

What drug is used for the long-term management of sickle cell disease?

A

Hydroxyurea

40
Q

What’s another long-term management option for those with sickle cell anemia?

A

Stem cell transplantation

  • Offered for those needing regular transfusions
  • Multiple red cell antibodies
  • Returning to Africa
  • Need a donor
  • Infertility
  • GvHD (graft vs host disease)
41
Q

What is beta thalassemia major?

A
  • Inheritance of 2 abnormal beta thalassemia genes
  • Severe anemia from 4-6mo of age
  • Management is based on long term blood transfusions and iron chelation or stem cell transplantation
  • Don’t start chelation too early - wait til Ferritin > 1000ug/L to avoid neuro + skeletal toxicity
42
Q

Which organs can be affected by iron overload?

A
  1. Liver
  2. Heart
  3. Thyroid
43
Q

What is the name of iron chelation used?

A

Desferasirox (ExJade)

  • Oral dissolvable preparation
  • Once daily dosage
  • Minor GI tract side effects
  • No neuro/skeletal toxicity
  • 24h chelation
  • First line > 6y of age
  • Second line >2y to <6y
  • Expensive
44
Q

Describe autoimmune hemolytic anemia

A
  • 2 basic types: warm + cold depending on the thermal range across which the antibody is active
  • Diagnosis is made by confirming hemolysis as usual but the blood film is usually critical in making the diagnosis
  • Followed by finding a positive direct antiglobulin test (DAT or Coomb’s test)
  • DAT is positive for IgG in WARM + for complement in COLD AIHA
45
Q

What is the difference between warm AIHA and cold AIHA?

A

Warm:

  • Idiopathic
  • Secondary:
    1. Rheumatoid disease (SLE)
    2. Lymphoma
    3. Chronic lymphatic leukaemia
    4. Drugs (cephalosporins)
    5. Ovarian teratoma

Cold:

  • Idiopathic
  • Secondary to:
    1. EBV infection
    2. Mycoplasma pneumonia
    3. Ulcerative colitis
46
Q

What are normal hematocrit levels?

A

Between 35-45% (0.35-0.45)

47
Q

What is the normal values of hemoglobin?

A

14g/dL

48
Q

How are hematocrit and hemoglobin related?

A

Both are either elevated, lowered, or normal

49
Q

What levels of MCV indicate the different anemias ?

A

Low MCV = microcytic anemia
Normal MCV = normocytic anemia
High MCV = macrocytic anemia

50
Q

What does the reticulocyte count indicate?

A

Low levels = decreased production from bone marrow

Increased levels = normal production but destruction in blood stream

51
Q

What 5 values in a blood test do you look at to determine whether someone is anemic?

A
  1. Hematocrit
  2. Hemoglobin
  3. MCV
  4. Reticulocyte count
  5. RBC morphology
52
Q

What is the extracellular control of iron synthesis and absorption, and can cause normocytic anemia?

A

Hepcidin

  • Regulator of iron metabolism
  • Hepcidin inhibits iron transport by binding to the iron export channel FERROPORTIN which is located on the base lateral surface of gut enterocytes and the plasma membrane of reticuloendothelial cells (macrophages)
53
Q

What is the effect of ferroportin?

A

Ferroportin controls the release of iron from the intestinal enterocytes into the blood stream and the release of iron from the marrow macrophages for erythropoiesis.

54
Q

How does hepcidin control the levels of ferroportin? (3)

A

a. An increase in Hepcidin cause Ferroportin to degrade, reducing Ferroportin levels prevents iron release from GI tract enterocytes into the blood stream and also traps iron in the marrow macrophages, thus reducing total iron bio-availability. So when iron is plentiful and the transferrin (TFR) saturation is high, less iron is absorbed and fewer red cells produced in the marrow. In inflammation with high levels of interleukin 6 (IL6), the same mechanism is activated explaining the classical marrow iron findings of the normocytic, normochromic anaemia of chronic disease; with excess iron in the free and marrow particle macrophages but no iron in the erythroblasts (i.e. no siderocytes). This lack of production in anaemia of chronic disease also known as anaemia of inflammation or secondary anaemia) also explains why sometimes this type of anaemia can be hypochromic and is not always normochromic and normocytic i.e. there is a functional iron deficiency.
b. A decrease in Hepcidin has the opposite effect, promoting iron absorption form the gut and releasing iron form the marrow macrophages to assist increased erythropoiesis. So in iron deficiency, a low TFR situation inhibits Hepcidin and Ferroportin increases thus promoting iron absorption and availability.
c. In haemolytic anaemia, there is an increase in the levels of Growth Differentiation Factor 15 (GDF15). High levels of GDF15 promote iron absorption by inhibiting Hepcidin and this explains why patients with chronic haemolysis can become iron overloaded without ingesting excess iron or by having blood transfusions.

55
Q

What can abnormal TMPRSS6 genes cause?

A

Iron-refractory iron deficiency anemia

This gene provides instructions for making a protein called Matriptase-2 which helps regulate iron levels in the body

56
Q

What is IRIDA?

A

Iron-refractory iron deficiency anemia

Rare autosomal recessive iron metabolism disorder characterised by iron deficiency anemia (hypochromic, microcytic) that is often unresponsive to oral iron intake and partially responsive to parenteral iron treatment

57
Q

Shirley Downs (age 26) felt tired all the time and made an appointment to see her GP at the local surgery. Apart from being tired and a bit pale she was generally well. Her GP sent off a full blood count and blood film (see image above).

Results:

Hb: 75.2 g/L (normal 121-151 g/L)

MCV: 62.7 fl (normal 77-94 fl)

MCH: 21.3 pg (normal 27-34 pg)

What is the diagnosis and what should also be tested?

A

MCV and MCH are both low indicating a microcytic anemia. Blood film shows pale centres = hypochromic microcytic anemia associated with iron deficiency

The most likely cause of her iron deficiency is menorrhagia and up to 15% of women with heavy periods have vWD -> test for Von Willebrand’s Disease

58
Q

What are GI red flags for malignancy?

A
  • Weight loss
  • PR bleeding
  • Persistent change in bowel habits
59
Q

Eight months ago Leanne had a baby boy, Jack, who developed neonatal jaundice on day 1 of life and required UV light therapy for 4 days.

When tested at 6 months of age Jack had abnormal EMA-binding.

Leanne and her partner, Mike, underwent testing. Leanne has been perfectly well but Mike has had upper right quadrant abdominal pains on and of for several years.

Leanne had an entirely normal full blood count and blood film.

Mike’s results (also see blood film above):

Hb: 111 g/L (normal 121-166 g/L)

MCV: 96 fl (normal 77-92 fl)

Reticulocytes: 186x109/L (normal 105x109/L

Bilirubin: 41 µmol/L (normal <21 µmol/L)

What is going on? What other condition is a complication of this disease?

A

The diagnosis is likely to be hereditary spherocytosis.

He should have an abdominal ultrasound to exclude gallstones (which are a likely complication of HS)

60
Q

What is true of thalassemias?

A

HbH disease is most likely caused by inherited deletion of 3 of the 4 alpha globin genes

Individuals with beta thalassemia trait have a raised level of HbA2 (this is how carriage of beta thalassemia is diagnosed)

61
Q

When does beta thalassemia major present and how is it treated?

A

Usually presents after 6mo of life

Stem cell transplantation can “cure” it

62
Q

What is the main cause of death in beta thalassemia major?

A

Cardiac iron overload

Due to the often poor compliance with subcutaneous desferrioxamine over the first two decades of life, iron deposition in the heart eventually produces resistant cardiac failure in many patients

63
Q

Prescribe a once only dose of vitamin B12. The patient is due their ONCE every 3 month injection today. The time is now 1pm. The patient has no known drug allergies.

A

Hydroxycobalamin, IM

64
Q

What drug is used to treat sickle-cell disease?

A

Hydroxycarbamide (aka hydroxyurea)

  • Increases hemoglobin
  • Oral
65
Q

What are the consequences of too much iron?

A
  • Diabetes
  • HF, irregular heartbeat, arrhythmia
  • Cirrhosis
  • Delayed puberty
  • Low estrogen/testosterone
  • Hypothyroidism/hypoparathyroidism
66
Q

What are the treatment options for thalassemia?

A
  1. Blood transfusion (monthly)
  2. Chelation therapy (if iron is too high after blood transfusions)
  3. Stem cell/bone marrow transplant (CURE!)
67
Q

When is a blood spot test performed and what does it test for?

A

Done when baby is 5D old

Tests for:

  • Sickle cell disease
  • Cystic fibrosis
  • Congenital hypothyroidism
  • Inherited metabolic diseases:
    1. Phenylketonuria
    2. MCADD
    3. MSUD
    4. IVU
    5. GA1
    6. Homocystinuria
68
Q

What are the screening tests for someone suspected of having thalassemia?

A
  1. Hemoglobin electrophoresis with hemoglobin A2 and F
  2. Full blood count
  3. Iron studies (free erythrocytes protoprophyrin, ferritin, other iron studies)
69
Q

What are the findings from the screening tests for each type of thalassemia?

A

B-thalassemia carrier = high HbA2

B-thalassemia major = No HbA2, only HbF

A-thalassemia with 3 genes deleted (HbH disease) = HbH presence (5-40%)

A-thalassemia with 4 genes deleted (Bart’s Hydrops Fetalis) = formation of gamma-4 tetramers (Hb Bart)
- Have very high O2 affinity = ineffective O2 delivery to tissues

70
Q

What are the clinical features of HbH disease?

A
  • Microcytic hypochromic anemia
  • Splenomegaly
  • Mild jaundice
  • Skeletal changes mainly affecting face
  • Iron hyper-absorption
  • Presence of HbH (5-40%)
  • Hemoglobin H inclusions
71
Q

What are the clinical signs of Bart’s Hydrops Fetalis?

A
  • Neonatal onset
  • Often lethal
  • Generalized oedema, pleural and pericardial effusions, severe hypochromic anemia
  • Hepatomegaly

Year 3 TCDs (24 decks)

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