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Cardiovascular 2 > Haemoglobinopathies & Anaemia > Flashcards

Haemoglobinopathies & Anaemia Flashcards

1
Q

Polycythaemia

A

Also known as erythrocytosis, means having a high concentration of red blood cells in your blood. This makes the blood thicker and less able to travel through blood vessels and organs.

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2
Q

Na+ and Cl- are found where?

A

Na+ and Cl- are found as extracellular ions.

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3
Q

K+ is found where?

A

K+ is found as an intracellular ion.

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4
Q

Blood is made up of 55% plasma, what makes up plasma?

A

Plasma is:

92% water

7% proteins (albumin, globulin and fibrinogen)

1% solutes (Na, K, Cl)

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5
Q

Blood is 45% formed elements what are the 3 formed elements that make up blood?

A

Erythrocytes

Leukocytes

Thrombocytes

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6
Q

Explain how haem and iron in rbc’s are recycled.

A

Haem:

  1. Haem is converted into biliverdin.
  2. Biliverdin is then converted to bilirubin which is excreted in bile by the liver.
  3. Excreted as stercobilin in stool.

Iron:

  1. Iron is transported in the blood by the protein transferrin.
  2. Iron is then stored by the protein ferritin in the liver.
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7
Q

Explain the erythrocyte life cycle.

A
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8
Q

Define anaemia.

A

A deficiency in the number of erythrocytes or their haemoglobin content.

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9
Q

What are the two different ways anaemia can occur?

A

You’re either losing rbc’s too quickly, (haemorrhage, haemolysis) or you’re not making them quick enough (Fe3+, B12, Folate, Aplastic).

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10
Q

What are the symptoms of anaemia?

A

As the principle function of erythrocytes is to deliver oxygen to peripheral metabolising tissues, any deficiency results in an oxygen delivery deficiency, producing a wide range of hypoxic symptoms.

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11
Q

What are the serious symptoms of anaemia?

A

Fainting

Red eyes

Chest Pain - Angina

Heart attack

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12
Q

How are different anaemias classified?

A

There are numerous ways of classifying different anaemias, the most common is by the size of the erythrocytes, termed mean cell volume (MCV) -This may be either microcytic, normocytic or macrocytic:

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13
Q

How does microcytic anaemia occur?

A

In microcytic anaemia the RBC’s cannot acquire the sufficient amount of haemaglobin to be released from the bone marrow and therefore undergo extra cell divisions before release producing cells with a smaller MCV.

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14
Q

How does macrocytic anaemia occur?

A

In macrocytic anaemia, B12 or folate deficiencies result in an inability to synthesize new bases to form DNA. This leads to fewer cell divisions (of fully haemaglobinised RBC) and therefore cells with a larger MCV.

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15
Q

Sideroblastic anaemia

A

Sideroblastic anaemia is the failure to incorporate iron in to haeme despite it being available.

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16
Q

In cases of microcytic anaemia, what must be assumed until proven otherwise?

A

That the cause of the microcytic anaemia is iron deficiency (Fe3+).

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17
Q

What can cause normocytic anaemia?

A

Chronic disease e.g CKD, chronic liver disease, rheumatoid arthritis etc.

Active haemorrhage

↑fluid states

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18
Q

What can cause macrocytic anaemia?

A

B12 deficiency

Folate deficiency

Alcoholism

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19
Q

Commonest cause of anaemia?

A

Fe3+ deficiency

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20
Q

What are the commonest causes of Fe3+ deficiency anaemia?

A

Commonest causes of Fe3+ anaemia are menorrhagia and GI bleed (think colorectal cancer in anyone over 50).

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21
Q

What on the hands can be a sign of anaemia?

A

Koilonychia

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22
Q

Anisocytosis

A

Anisocytosis is a medical term meaning that a patient’s red blood cells are of unequal size. This is commonly found in anemia and other blood conditions.

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23
Q

Reticulocytes

A

Pre-mature rbc’s

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24
Q

What does a high reticulocyte count in a patient that is anaemic imply?

A

A high correced reticulocyte count (>3) in a patient who is anaemic implies that there is an appropriate response to the anaemia from the bone marrow (ie it is releasing RBC’s prematurely to cover for the deficit).

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25
Q

What does a low reticulocyte count in a patient that is anaemic imply?

A

A low corrected reticulocyte count implies that either the bone marrow has not had time to respond (ie acute haemorrhage) or that the bone marrow is failing to respond appropriately and implies that there is a problem with the bone marrow (ie aplastic anaemia).

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26
Q

Reticulocytosis is a common finding in what patients?

A

Reticulocytosis is a common finding in patients with autoimmune haemolytic anaemias.

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27
Q

Megaloblastic anemia

A

Megaloblastic anemia is a condition in which the bone marrow produces unusually large, structurally abnormal, immature red blood cells (megaloblasts).

(Macrocytic)

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28
Q

What causes megaloblastic anemia?

A

Vitamin B12 and Folate deficiency

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29
Q

What can cause vitamin B12 deficiency?

A

B12 deficiency may be due dietary deficiency, gastric factors, pernicious anaemia or small bowel disease (ie IBD or coeliac disease of the terminal ileum). Dietary deficiency is extremely rare as little B12 is required. Gastric factors include total gastrectomy.

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30
Q

Pernicious anaemia

A

Pernicious anaemia is an autoimmune disorder which causes gastric mucosa atrophy and destruction of parietal cells (and therefore intrinsic factor which is necessary for B12 absorption).

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31
Q

What is the treatment for macrocytic anaemia?

A

Folic acid supplements

B12: Hydroxycobalamin supplements

32
Q

What haemoglobinopathies can cause anaemia?

A

Sickle cell anaemia

Thalassaemia

33
Q

What type of inheritance is seen in sicke cell anaemia?

A

Autosomal recessive

34
Q

Read how sickle cell anaemia presents.

A

1) Vaso-occlusive crisis (most common) - Plugging of small vessels in bone produces severe bone pain. Affects areas of active marrow and causes a systemic response (tachycardia, sweating and fever) & may lead to sickle chest syndrome
2) Aplastic crisis – Infection of adult sicklers with erythrovirus 19 causes a severe erythrocyte aplasia, characterised by a low Hb and a low reticulocyte count
3) Sequestration crisis – Thrombosis of venous outflow from an organ causes severe pain and loss of function. The most common site is the spleen, causing massive splenomegaly - Other presentations include: CVA, cholelithiasis, avascular necrosis, haematuria, dactilytis, leg ulcer.

35
Q

Thalassaemia

A

Either no or too little production of haemoglobin. This can make people very anaemic (tired, short of breath and pale).

36
Q

What are the two types of Thalassaemia?

A

Alpha

Beta

37
Q

Haemolysis

A

The rupture or destruction of red blood cells.

  • Premature RBC destruction overloads pathways for haemaglobin breakdown, causing a modest rise in unconjugated bilirubin and occasionally jaundice
38
Q

What is haptoglobin?

A

In blood plasma, haptoglobin binds to free hemoglobin, compared to hemopexin that binds to free heme.

39
Q

How are haptoglobin levels affected in haemolytic anaemia?

A

As free haemaglobin is toxic, it is quickly bound to haptoglobin for transportation to the liver (resulting in a fall in haptoglobin levels).

40
Q

What is haemosiderin?

A

Renal tubular cells absorb the Hb, degrade it and store it as haemosiderin.

41
Q

Explain what haemosiderinuria is and what it is a sign of.

A

Renal tubular cells absorb the Hb, degrade it and store it as haemosiderin. When these cells are subsequently sloughed off, they give rise to haemosiderinuria.

A sign always indicative of intravascular haemolysis

42
Q

Where are worn out rbc’s phagocytosed by macrophages in the body?

A

Physiological RBC destruction occurs extravascularly in the reticulo-endothelial cells of the spleen and liver.

43
Q

What symptoms and signs does haemolytic anaemia cause?

A

1) Jaundice
2) ↑ Bilirubin
3) ↑ Reticulocytes
4) ↑ Lactate dehydrogenase (LDH)
5) ↓ Haptoglobins
6) ↑Urinary urobilinogen and +ve urinary haemosiderin

44
Q

Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

A

X-linked recessive hereditary form of haemolytic anaemia.

Deficiency of enzyme responsible for protection against oxidative stress - Pt’s exhibit haemolytic anaemia in response to infection, drugs & food.

  • Common triggers include broad beans, aspirin and antimalarials.
45
Q

Pyruvate Kinase Deficiency

A

Second commonest erythrocyte enzyme defect.

  • Deficiency of ATP production causing a decreased affinity for O2 and a chronic haemolytic anaemia.
46
Q

Aplastic anaemia

A
  • Total bone marrow failure effects all cell-lineage.
  • May be caused by infection (virus), drugs (chloramphenicol, carbimazole).
47
Q

How does aplastic anaemia present?

A

Presents with symptoms associated with anaemia, leukopenia and thrombocytopaenia.

  • deficiency in all the components of blood.
48
Q

Carbimazole is linked to what type of anaemia?

A

Aplastic anaemia

  • Note: carbimazole is used to treat thyrotoxicosis.
49
Q

Myelodysplastic syndrome (MDS)

A

Malignant infiltration of the bone marrow by immature bloods cells.

Prevents normal function of the BM leading to anaemia, leukopenia and thrombocytopaenia and massive splenomegaly.

50
Q

What can myelodysplastic syndrome (MDS) transform into?

A

AML - acute myeloid lymphoma

51
Q

Polycythaemia

A

Polycythaemia. Polycythaemia, also known as erythrocytosis, means having a high concentration of red blood cells in your blood. This makes the blood thicker and less able to travel through blood vessels and organs.

52
Q

Primary polycythaemia

A

Primary polycythaemia is due to inappropriate over-production of erythrocytes.

53
Q

What is secondary polycythaemia caused by?

A

Secondary polycythaemia is due to an increase in erythropoeitin production (smoking, high altitude, renal hypoxaemia, Epo dosing).

54
Q

How does polycythaemia present?

A

Polycythaemia may have arterial thromboses, pruritis which is worse after a hot bath, gout and hepatosplenomegaly, Budd-Chiari syndrome.

55
Q

Granulocytes =

A

Granulocytes = Basophils, eosinophils and neutrophils.

56
Q

Relative polycythaemia

A

Relative polycythemia is an apparent rise of the erythrocyte level in the blood; however, the underlying cause is reduced blood plasma.

Relative polycythemia is often caused by loss of body fluids, such as through burns, dehydration, and stress.

57
Q

What can cause excess epo production leading to secondary polycythaemia?

A
  1. EPO secreting tumours: liver cancer (hepatocellularcarcinoma), kidney cancer (renal cell carcinoma), adrenal adenoma (adenocarcinomas), hemangioblastomas and uterine fibroids.
  2. Hypoxemia: COPD, right-left cardiac shunts, sleep apnea, massive obesity, high altitudes.
58
Q

What does neutrophilia indicate?

A

Neutrophilia: Indicates acute bacterial or fungal infection (e.g appendicitis or cholecystitis).

59
Q

Eosinophilia indicates?

A

Eosinophilia: Indicates a parasitic infection or allergy (e.g intestinal worm).

60
Q

Basophilia indicates?

A

Basophilia: Indicates histamine (e.g allergic reaction).

61
Q

Lyphocytosis indicates?

A

Lymphocytosis: Indicates acute viral infection (e.g EBV, bordatella pertussis, ALL, CLL).

62
Q

Monocytosis indicates?

A

Monocytosis: Indicates chronic inflammation (e.g TB, IBD, RA).

63
Q

High levels of macrophages indicate?

A

General mediator in infection.

64
Q

Leukaemia

A

A malignancy of bone marrow stem cells. It may arise from either lymphoid or myeloid stem cells. - It can

65
Q

What are the symptoms of leukaemia?

A
  • Lethargy/fatigue
  • Easy bruising
  • Epistaxis
  • Recurrent infections

Symptoms arise from affected stem cell hindering function of other stem cells in the bone marrow resulting in anaemia, thrombocytopaenia, infection and from infiltration; non-tender lymphadenopathy, hepatosplenomegaly orchidomegaly, meningism.

66
Q

What are the different types of leukaemia?

A

Lymphoid:

Acute lymphoblastic leukemia (ALL)

Chronic lymphoblastic leukemia (CLL)

Myeloid:

Acute myelogenous leukemia (AML)

Chronic myelogenous leukemia (CML)

67
Q

Lymphoma

A

Malignancy of the lymphatic system (specifically lymphocytes) including the lymph nodes. It is divided histologically in to Hodgkin’s lymphoma (rare) and non-Hodgkins lymphoma (10x more common).

68
Q

What are the symptoms of lymphoma?

A
  • Night sweats
  • Fever
  • Weight loss
  • Pruritis
69
Q

What are the clinical signs of lymphoma?

A

Painless lymphadenopathy - generalised or regional often affecting cervical chain.

70
Q

What is multiple myeloma?

A

Malignancy of plasma cells characterised by increased monoclonal antibodies.

71
Q

How can the symptoms of multiple myeloma be explained?

A

All of the symptoms can be explained by the increased production of one type of antibody (usually IgG (2/3) or IgA (1/3). Increased antibodies release interleukin1 which overexpresses RANKL stimulating osteoclasts = bone pain and hypercalcaemia and multiple osteolytic skull lesions (pepper-pot skull). This overactivity suppresses haematopoeitic stem cells causing a normocytic anaemia. These paraproteins (light chains) also clog up the kidneys (amyloid) causing renal failure.

72
Q

Thrombocytosis vs thrombopaenia

A

Conditions where the body makes too many platelets (thrombocytosis) or too few (thrombocytopaenia).

73
Q

Ready summary of thrombocytosis.

A
  • May be primary (essential thrombocytosis) or secondary (reactive).
  • Essential thrombocytosis is a form of myeloproliferative disorder.
  • Reactive thrombocytosis is in response to infection or inflammation.
  • Usually asymptomatic but may present with symptoms of acute thrombus (PE/MI)
74
Q

Read summary of thrombopaenia.

A

Usually asymptomatic (incidental finding).

Can present with easy bruising and bleeding.

Decreased production: Myelodysplastic syndrome, aplastic anaemia, sepsis

75
Q

Haptoglobin

A

Haptoglobin is a protein produced by the liver. Its purpose is to find and attach itself to free haemoglobin in the blood. This forms a complex that is rapidly removed from the circulation by the liver and the iron is then recycled.

76
Q
A

Cardiovascular 2 (20 decks)

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