CR EOYS5 Flashcards

1
Q

A patient’s investigations reveal pancytopenia and macrocytosis. His peripheral smear shows hyper-segmented neutrophils. Serological tests reveal positive anti intrinsic factor antibodies. What other biochemical derangements might be observed in this patient?

A. Increased plasma metanephrines
B. Elevated ferritin levels
C. Increased urinary 5-hydroxytryptamine
D. Elevated plasma homocysteine

A

A patient’s investigations reveal pancytopenia and macrocytosis. His peripheral smear shows hyper-segmented neutrophils. Serological tests reveal positive anti intrinsic factor antibodies. What other biochemical derangements might be observed in this patient?

D. Elevated plasma homocysteine

This patient likely has megaloblastic anemia due to a deficiency of vitamin B12. Vitamin B12 is an essential co-factor involved in two important enzymes. One of these enzymes is methionine synthase, which converts homocysteine to methionine. The deficiency of cobalamin will lead to the impaired functioning of this enzyme. This will result in an accumulation of homocysteine which can be detected in the blood.

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2
Q

Which is the most common?

ASD
VSD
PDA
Coarctation of the aorta

A

Which is the most common?

ASD
VSD
PDA
Coarctation of the aorta

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3
Q

Explain what bundle branch block is [1]

Which part of ECG can see bundle branch block occur in? [1]

A

Explain what bundle branch block is [1]
Disruption to the electrical signal that causes your heart to beat [0.5]
Causes altered pathways for depolarisation [0.5]

Which part of ECG can see bundle branch block occur in? [1]
Prolongs QRS

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4
Q

Which views of the heart are seen by each type of ECG lead on a standard 12-lead ECG? [4]

A
  • *S**eptal: V1, V2
  • *A**nterior: V3, V4
  • *L**ateral: V5, V6, AVL, I
  • *I**nferior: II, III, AVF

AVR: neutral

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5
Q

What is concentric hypertrophy characterised by visually? [1]

How does concentric hypertrophy occur? [1]

When does concentric hypertrophy occur? [2]

What does concentric hypertrophy cause to happen and why? [1]

A

What is concentric hypertrophy characterised by visually? [1]
- Increased wall thickness / reduced lumen of ventricle

How does concentric hypertrophy occur? [1]
- New sarcomeres produced

When does concentric hypertrophy occur? [1]

  • Aortic stenosis
  • Chronic hypertension

What does concentric hypertrophy cause to happen and why? [1]
- Decreases pre-load due to reduction in compliance (stiff)

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6
Q

Eccentric hypertrophy:

  • Caused by? [4]
  • Characterised by? [2]
A

Eccentric hypertrophy:

Caused by? [1]

  • Aortic and mitral regurgitation
  • Systolic dysfunction (loss of cardiac inotrophy
  • -Volume overload (hypervolaemia due to ventricular or renal failure)
  • Alcohol / cocaine

Characterised by? [2]
- Chamber dilation - lumen gets bigger, wall gets smaller: cant contract properly

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7
Q

Which of the following forms the right ventricle?

Truncus ateriosus
Bulbus cordis
Sinus venosus
Primitive ventricle
Primitive atria
A

Which of the following forms the right ventricle?

Truncus ateriosus
**Bulbus cordis**
Sinus venosus
Primitive ventricle
Primitive atria
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8
Q

Which of the following forms the ventricular outflow tracts?

Truncus ateriosus
Bulbus cordis
Sinus venosus
Primitive ventricle
Primitive atria
A

Which of the following forms the ventricular outflow tracts?

Truncus ateriosus
**Bulbus cordis**
Sinus venosus
Primitive ventricle
Primitive atria
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9
Q

Label this xx

A
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10
Q

Tetralogy of Fallot is comprimised of which 4 heart defects/

A
  1. Ventricular septal defect
  2. Overriding Aorta: aorta is positioned above the VSD
  3. Pulmonary stenosis (narrowing)
  4. Right ventricular hypertrophy
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11
Q

What is persistant truncus arteriosus? [2]

A

Spiral structure doesn’t form in truncus arteriosus: one common outflow tract. Both L & R ventricle pump into same outflow tract [1]

A large VSD (ventricular septal defect) below the truncal valve allows mixing of right and left ventricular blood [1]

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12
Q

Which structures of the heart are formed from the bulbus cordis?

A

The smooth outfow of the left and right ventricles. The muscular right ventricle. The muscular intraventricular septum.

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13
Q

Which complications are associated with a patent truncus arteriosus?

A

A large ventricular septal defect. Progressive heart failure. Degree of cyanosis is variable.

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14
Q

How would you ID acute myeloid leukemia? (AML)

A
  • A heterogeneous population of myeloblasts with cells ranging from small to medium-sized to large. Note presence of a few maturing myeloid elements.
  • Large myeloblasts with prominent nucleoli. Maturing myeloid elements i.e. neutrophils or eosinophils.
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15
Q

How would you ID chronic lymphocytic leukemia (CLL) from PBS and BMS?

A
  • PBS: Mature-appearing lymphocytes with high nuclear to cytoplasmic ratios, with scant agranular cytoplasm and homogeneously condensed chromatin without nucleoli. Characteristic “soccer ball’ chromatin pattern. Numerous smudge cells
  • BMS: tissue is displaced by nodular and interstitial aggregates of clonal B cells.
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16
Q

How would you ID chronic myeloid leukemia (CML) from PBS and BMS?

A

PBS: > 100K white blood cells with neutrophilia, significant increase in metamyelocytes and myelocytes, also basophilia and eosinophilia

BMS: increased granulocyte precursors, basophils, eosinophils and occasionally monocytes

Normal erythroid compartment, variable pseudo Gaucher cells and sea blue histiocytes, increased reticulin fibres

17
Q

Describe the process of migration of neutrophils through

A

Process of migration:
- chemokines picked up by receptors on the neutrophils
– causes intercellular signals to get more & more neutrophils
- cause selectins (which are present on endothelial cells) and attach – endo. cells open and and squeeze through to allow enter area of inflammation

18
Q

What is shown here?

A

Kerley B Lines

These are thin lines, often of no more than a couple of centimetres often found towards the base and peripheral regions of the lung where the interstitial has thickened. They lie at 90o to the pleura. On the image, some of these lines are present towards the base of the right lung.

19
Q

What condition is shown here?

A

Upper Lobe Blood Diversion

Due to the increased pressures, blood is pushed upwards creating a ‘stag antler’ appearance. Blood is diverted as fluid is more likely to build up lower down due to gravity and cause relative hypoxia and vasoconstriction, thus the blood is diverted to the upper zones.

In the midline of this film, we can also see several sternal sutures.

20
Q

Explain the mechanism of iron absorbtion and transport in the body for haem iron and non haem iron

A

Haem iron

  1. Haem iron – (highly bioavailable) absorbed through DMT1
  2. Fe removed from Haem. Can then be stored as ferritin OR can exit cell through Ferroportin

Non-haem iron:

  1. Mostly in the form of Fe3+, but only Fe2+ can be absorbed by the enterocyte. Enzyme reductase: Fe3+ à Fe2+
  2. Enters via DMT1
  3. Fe removed from Haem. Can then be stored as ferritin OR can exit cell through Ferroportin

Then transferrin transports Fe3+ around body

21
Q

What is the first committed cell in erythropeoisis? [1]

What is the pathway from Haematopoietic stem cell (HPSCs) - to erythrocyte? [1]

A

What is the first committed cell in erythropeoisis? [1]
Proerythroblast

Haematopoietic stem cell (HPSCs) –> common myeloid progenitor cell –> (CMPC)Proerythroblast –> erythroblast –> reticulocyte – > erythocyte

The proerythroblast develops into an (early) erythroblast. The erythroblast then undergoes a sequence of changes where its nucleus progressively shrinks and its cytoplasm becomes filled with haemoglobin (not stained). When full of haemoglobin it is called a normoblast. The normoblast then expels its nucleus and becomes a reticulocyte. Most reticulocytes stay in the marrow and mature into erythrocytes but some may be released into the blood, especially after haemorrhage. Reticulocytes can transport oxygen, just not as efficiently as mature erythrocytes. They can mature into adult RBCs in the circulation

22
Q

Explain the mechanism of how Fe is recylced during rbc breakdown?

A

Explain the mechanism of how Fe is recylced during rbc breakdown?

splenic macrophages break down old rbc, via action of haem oxygenase.
Fe3+
enters circulation carried ontransferrinto be re-used and exits viaferroportin

23
Q

What are specific signs associated with anaemia of iron deficiency? [2]
What are specific signs associated with anaemia of vit. B12 deficiency? [1]
What are specific signs associated with anaemia of thelessasmia? [1]

A

What are specific signs associated with iron deficiency? [2]

  • Koilonychia (spoon shaped nails)
  • Angular stomatitis (inflammation of corners of mouth)

What are specific signs associated with anaemia of vit. B12 deficiency? [1]
Glossitis (inflammation of tongue)

What are specific signs associated with anaemia of thelessasmia? [1]
abnormal bone facial development

24
Q

What is anaemia of inflammation / chronic disease caused by? [3]

Why will iron supplementation not help in patients with this condition?

A

What is anaemia of inflammation?
IL-6 released and causes:
* more hepcidin: which blocks release of Fe2+ out of via ferroportin
* blocks production of EPO (& therefore rbc production)
* inhibits production of rbc in bone marrow

Why will iron supplementation not help in patients with this condition?
Problem is with iron being absorbed in gut and iron getting out of macrophages, along with depressed erythropoietin release and erythropoiesis caused by inflammatory cytokines – need to treat cause inflammation

25
Q

Explain what elevated erythrocyte Sedimentation Rate test indicates [1]

A

Elevated sedimentation rate (ESR) indicates inflammation – can be secondary to infection, auto-immune illness – different labs will have slightly different normal ranges

  • Erythrocytes have negative surface charge*
  • Ensures that they electrostatically repel each other and do not stick together, particularly in capillaries.*
  • Inflammatory cytokines or bacteria in blood increase amount of (positively charged) fibrinogen in plasma*
  • Excess fibrinogen binds to red cell membrane and reduces its negative charge, causing RBCs to adhere*
  • Therefore, rate of sedimentation increases as clumps of RBCs fall down through test tube more quickly.*
26
Q

Explain how B12 [1] and folate deficiencies [1] would affect the NS?

Which pathology would B12 deficiency lead to if left untreated? [1]

A
  • Folate deficiency: neural tube defects
  • B12 deficiency: demylination

if not treated can lead to: subacute combined degeneration of the cord

Degeneration of posterior and lateral columns of spinal cord: TEST FOR WITH ROMBERG SIGN

27
Q

How do you diagnose megaloblastic anaemia from a PBS? [1]

What is elevated for when evaluating megaloblastic anaemia bloods? [3]

What should you also test for alongside a full blood count for megaloblastic anaemia? [2]

A

Diagnostic cell on smear for megaloblastic anaemia is hyper-segmented neutrophils: 6 or more lobes

Also elevated:
* MCV
* serum ferritin (bc is a type of inflammation)
* plasma lactate dehydrogenase

What should you also test for alongside a full blood count for megaloblastic anaemia? [2]
* vit. B12 level
* serum folate lvls / rbc folate levels

(but Serum vitamin B12 blood test can be unreliable so repeat if borderline , a low ”normal” B12 level does not rule out B12 deficiency!

If levels indeterminate check plasma total homocysteine (tHcy) and/or plasma methylmalonic acid (MMA)

28
Q

Treatment for folate deficiency? [1]
Treatment for vitamin B12 deficiency:
- pernicious anaemia? [1]
- alternative causes? [1]

A

Folate: oral folic acid

B12:
- for pernicious anaemia: hydroxycobalamine IM (NOT ORAL). be aware of hypokalaemia due to increased K+ requirement as erypoesis increase back to normal
- other: Oral cyanocobalamine

29
Q

What is haemolytic anaemia? [1]

What are the classifications? [2] State all of each

What are the key lab findings to show haemolytic anaemia? [3]

A

What is haemolytic anaemia? [1]
abnormal breakdown reduces lifespan of rbc

What are the two types? [2]

Inherited (defetive gene):
* .glycolysis defect
* pentose p pathway (generates NADPH - which involved in anti-oxidants for rbc)
* membrane protein defects
* SCA

Acquired:
* heat damage
* mech damage
* antibody damage

What are the key lab findings to show haemolytic anaemia? [3]_
Raised: reticulocytes, bilirubin (breakdown of haem) & LDH

30
Q

what are symptoms of haemolytic anaemia? [3]

A

Will depend on time frame of haemolysis:

Jaundice
- more rbc being produced, more bilirubin is made as a product of more haem breakdown.
- This can lead to pigment gallstones High bilirubin causes yellowish discolouration of skin & sclera of eye

Splenomegaly
- extramedullary hematopoiesis (“making blood” outside bone marrow) in red pulp spleen.

Massive sudden haemolysis
- e.g. from incompatible blood transfusion can cause cardiac arrest due to lack of oxygen delivery to tissues & hyperkalaemia due to release of intracellular contents) Urine dark colour due to conjugated bilirubin

31
Q

What is pernicious anaemia caused by?

A

Autoimmune antibodies to parietal cells (sensitive) or IF (specific) – destroy or inactivate IF: SO:
Decreased or absent Intrinsic factor (IF) causes progressive exhaustion of B12 reserves.

32
Q

How can folate mask B12 defiency?

A

Elevated intake of folic acid alleviates anaemia caused by B12 deficiency by providing a continual supply of active folate (otherwise B12 needed to regenerate active folate via methionine synthase). BUT irreversible neurological sequelae B12 deficiency may still occur!

33
Q

Name & explain 2 inherited defects that cause haemolyitc anaemia, arising from enzymes? [2]

A

Pyruvate Kinase Deficiency:
- Causes deficiency in pyruvate kinase in erythrocytes: final step in glycolysis
- Less energy for ATPase pump – cells lose K+ & H20, dehydrate and are destroyed - haemolysis

Pentose Phosphate Pathway: Glucose-6-phosphate dehydrogenase (G6PD) deficiency:
- NADPH required to keep glutathione in reduced state – major cellular anti-oxidant
- Pentose phosphate pathway is only source of reduced glutathione in RBC – so RBCs very affected when exposed to oxidative stress
- Increased oxidative stress from infection or drugs (examples, sulfa drugs, primaquine) or certain foods (one example, fava beans) will cause haemolysis

34
Q

Haemolytic anaemias:

Explain how can get aquired damage to RBC?

A

Microangiopathic haemolytic anaemia: from mechanical damage:

  • stress from passing through heart valves
  • cells snag on fibrin strands
  • heat damage
  • osmotic damage (drowing in freshwater)

ALL TYPES OF INTRAVASCULAR HAEMOLYSIS

35
Q

GO OVER THE CYCLE in lecture

A
36
Q

Label A

A

Heinz Bodies