Blood and lymphatic disordersdisorders Flashcards

1
Q

What is the basic blood test performed on most patients in the ED?

A

A Complete Blood Count (CBC) also called a Full Blood Count (FBC)

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2
Q

What sample is needed for a Complete Blood Count?

A

A venous sample taken from a new venopuncture

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3
Q

What does a Complete Blood Count test for?

A

The count and characteristics of all formed elements including WBC, RBCs haematocrit, haemoglobin and platelets

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4
Q

What is the normal white blood cell count, and what is considered high and low?

A

Normal: 5000-1000
<5000: Leukopenia
>10,000: Leucocytosis

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5
Q

What is the normal red blood cell count, and what is considered high and low?

A

Normal: 4-5.5 million
<4 million: Anaemia
>5.5 million: Polycythaemia

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6
Q

What is the normal Hb count in men and women?

A

Women: 12-16 g/dl

Men: 13 - 18 g/dl

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7
Q

What are PT and INR tests?

A

A prothrombin time (PT) test measures how long it takes for a clot to form in a blood sample. An INR (international normalized ratio) is a type of calculation based on PT test results.

INR is normally used for patients on warfarin long term to aid treatment/dosage levels to keep them within the desired theraputic range

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8
Q

Why might an INR test be used to diagnose liver problems?

A

Liver issues will lead to vitamin K issues, affecting the INR.

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9
Q

What tests can be done to test patients’ clotting time?

A

PT/INR
PTT/aPTT

Patients suspected to have a clotting disorder will have both a PT and PTT ordered

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10
Q

What is a PTT and an aPTT

A

Partial Thromboplastin Time and activated Partial Thromboplastin Time.

Used for patients on heparin and to diagnose clotting disorders

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11
Q

What does a D-Dimer test essentially measure?

A

Fibrin degredation - looks for strokes, PEs, DVTs etc.

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12
Q

What does a platelet count test help signify?

A

Too much bleeding or too much clotting

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13
Q

What is lymphoma and what causes it?

A

Lymphoma is a broad term for cancer that begins in cells of the lymphatic system. (Malignant neoplasia involving lymphocyte proliferation in lymph nodes)

No specific causes

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14
Q

What are the two main types of lympoma?

A

Hodgkin’s lymphoma
Non-Hodgkin’s Lymphoma

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15
Q

What is Hodgkin’s lymphoma?

A

Anorganised type of lymphoma usually initially starting in one node and speading to adjacent nodes and organs via lymphocytes

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16
Q

What is the defective lymphocyte with Hodgkin’s lymphoma?

A

T lymphocytes

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17
Q

What is the usual first indicator of Hodgkin’s lymphoma?

A

A singular enlarged painless lymph node

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18
Q

Will patients with Hodgkin’s lymphoma have signs of infection or illness?

A

Not usually signs of acute infection, but they will have signs of illness such as weight loss, anaemia, recurrent bouts of fever, fatigue.

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19
Q

What is non-Hodgkin’s lymphoma?

A

Lymphoma similar intially to Hodgkin’s but develops erratically with multiple lymph node involvement and widespread metastases

Non-organised

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20
Q

What is lymphedema?

A

Abnormal collection of lymph fluid in the extremities

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21
Q

What can cause lymphedema?

A

It can be congenital or due to:

Cancer/cancer treatment
Trauma
Surgery
Chronic Venous Insufficiency

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22
Q

What are the main complication of lympedema?

A

Chronic lymphedema can in turn lead to cellulitis and fibrosis

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23
Q

What are blood dyscrasias?

A

Blood disorders

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24
Q

What are the three main subgroups of blood dyscrasia?

A

Clotting disorders (Inc. Disseminated Intravascular Coagulation (DIC))
Neoplastic blood disorders
Anaemias

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25
Q

What are clotting disorders?

A

Problems in the body’s ability to control how the blood clots i.e. too little clotting (bleeding disorders), or too much clotting (thrombophilia)

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26
Q

What is haemophilia?

A

An umbrella term for deficiences in clotting factors, affecting the blood’s ability to clot

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27
Q

How do people get haemophilia?

A

It is usually inherited but can be due to spontaneous gene mutations

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28
Q

How many types of haemophilia are there, and which clotting factor does each one involve?

A

Haemophilia A - deficiency of clotting factor VIII
Haemophilia B (Christmas disease) - deficiency of clotting factor IX
Haemophilia C (Rosenthal’s) - deficiency of clotting factor XI

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29
Q

What is Von Willebrand Disease?

A

A common hereditary disease where there is a deficiency of Von Willebrand factor which causes slower clotting

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30
Q

What are the possible signs of bleeding disorders?

A

Persistent bleeding from gums, repeated nosebleeds
Petechiae – pinpoint rash from bleeding capillaries
Bruises
Haemoptysis
Haematemesis
Blood in faeces

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31
Q

What are the possible signs of thrombophilia?

A

Signs of PE and DVT or MI and strokes

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32
Q

What is Disseminated Intravascular Coagulation (DIC) and how is it dangerous?

A

Disseminated intravascular coagulation (DIC) is a rare but serious condition that causes abnormal blood clotting throughout the body’s blood vessels. Often life-threatening condition involving both excessive bleeding and excessive clotting.

It occurs as a complication of various primary problems which activate coagulation in the microvasculature throughout the body. (May be the result of excessive tissue injury or the release of tissue thromboplastin)
Multiple thrombosis and infarctions occur which consume clotting factors and platelets and stimulate fibrinolysis. This leads to haemorrhage and hypovolaemic shock.

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33
Q

What can cause DIC?

A

Sepsis
Cancer
Major trauma
Serious obstetric complications (e.g. placental abruption)

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34
Q

What are the signs and symptoms of DIC?

A

Low plasma fibrinogen
Thrombocytopenia
Prolonged bleeding time
Haemorrhage
Petechiae
Mucosal bleeding
Vascular occlusions
Respiratory impairment
Neurological effects – seizures, reduced consciousness
Acute renal failure.

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35
Q

What is polycythaemia and its signs/symptoms?

A

An abnormally high number of red blood cells in the blood (increase in erythrocytes, granulocytes and thrombocytes), as a primary disease or secondary condition (usually associated with lung or heart disease or living at high altitude).

Increased blood viscosity
Risk of thrombosis
Increased blood pressure
Cardiac hypertrophy
Spleen and liver enlargement

Fatigue or weakness
Headaches
Dizziness
Shortness of breath
Visual disturbances
Nose bleeds
Bleeding gums
Heavy menstrual periods
Bruising

36
Q

What are neoplastic blood disorders?

A

Over proliferation of certain blood cells e.g. polycythaemia and leukaemia

37
Q

What is leukaemia?

A

A group of neoplastic disorders involving the white blood cells - Hyper production of immature non-functioning leukocytes within the bone marrow, large quantities released into the circulation and they then infiltrate the lymph nodes, spleen, liver, brain and other organs as numbers increase.

38
Q

What is the aetiology of leukaemia?

A

Chronic leukaemia’s are more common in older people
Acute leukaemia’s primarily occur in children and young adults

39
Q

What cell proliferation is present in acute vs chronic leukaemia?

A

Acute leukaemia is characterised by a high proportion of immature cells
Chronic leukaemia has a higher concentration of more mature cells, although their function is typically impaired.

40
Q

How does leukaemia lead to symptoms of aneamia and severe pain?

A

Proliferation of white blood cells is so high that other cells are unable to be produced causing a reduction in RBCs and platelets - leading to signs of anaemia and increased/spontaneous bleeding.

The increased pressure within the bone itself causes severe pain

41
Q

What are the signs and symptoms of leukaemia?

A

Recurrent infections – unresponsive to treatment
Severe haemorrhage
Signs of anaemia
Bone pain
Weight loss
Fever
Spleen, liver, lymph node pain

42
Q

What is anaemia?

A

Anaemia is a term for a number of disorders/diseases characterised by decreased haemoglobin content i.e. a reduction in production or an increase in destruction

43
Q

What is the most common type of anaemia?

A

Iron deficient anaemia

44
Q

How much iron does he body usually have, where?

A

The body contains 4 grams of iron (normally) and 75% of this is in haemoglobin (normally), the rest is stored in reserve in the liver, bone marrow & spleen, plus a little circulating as it’s on it’s journey to the erythroblast.

In women 1ml of blood contains 0.5 milligrams of iron, slightly less in men.

45
Q

What is iron deficient anaemia?

A

Decreased haemoglobin content due to low iron levels

46
Q

How is iron deficient anaemia diagnosed?

A

Complete Blood Count - to look at haemoglobin and haematocrit levels.
Iron level test
Blood smear test - will look at the shape and age of the red cells. If they are small this is termed ‘microcytic’ and pale is termed ‘hypochromic’.

47
Q

What is the term for small RBC appearance on a blood smear test?

A

Microcytic

48
Q

What is the term for pale RBC appearance on a blood smear test?

A

Hypochromic

49
Q

What causes iron defiecient anaemia?

A

Blood loss (usually chronic) – GI bleeds, menstruation++, peptic ulcers, haemorrhoids etc.

Poor intake - pregnancy (in later stages of pregnancy the iron demand of the foetus is high), low iron diet

Absorption problem - coeliac, colitis, bowel surgery etc.

50
Q

What are the signs and symptoms of iron deficient anaemia?

A

Pallor – look conjunctiva, palms, buccal mucosa
Glossitis
Lethargy, fatigue
Irritability – hypoxia
SOB on exertion
Koilonychia
Degenerative changes to hair
Tachycardia, palpitations, syncope
Delayed healing
Menstrual irregularities

51
Q

What glossitis and how can iron deficient anaemia cause it?

A

A smooth tongue

Iron is needed for new epithelial tissue maturing, iron deficient anaemia means a lack of availab;e iron to do this properly

52
Q

What is Koilonychia and how is it related to iron deficient anaemia?

A

Spoon shaped nails, can be a symptom of iron deficient anaemia - the pathophysiology around this has not been proven, but it is assumed to be low levels of O2 in the nail matrix or possibly to do with epithelial tissue maturation in the nail bed

53
Q

How are menstural irregularities related to iron deficient anaemia?

A

Iron deficient anaemia can cause irregular periods. The mechanism is not fully understood but it may be that periods become irregular as the body does not want to let go of any red cells

54
Q

What pernicious anaemia?

A

Vitamin B12 (folate) deficient anaemia - a type of anaemia where the body is not producing enough healthy red cells

55
Q

What causes pernicious anaemia?

A

An autoimmune process where the immune system attacks the parietal cells of the GI mucosa causing a decrease in intrinsic factor production and thereby redued B12 absorption – without vitamin B12 the body cannot make healthy red blood cells.

The exact cause like many automimmune diseases is unknown however:
-There is a possible genetic link.
-It is more common in older people and the parietal cells become less efficient and produce less intrinsic factor.
-It is linked to other autoimmune disorders such as type 1 diabetes, primary Addison’s disease etc.
-People who have had stomach surgery where their parietal cells have been damaged or had a portion removed may also suffer with this

56
Q

What kind of red blood cell is characteristic of pernicious anaemia?

A

Pernicious anaemia is categorised by the presence of many large, immature, nucleated red cells circulating in the blood. (usually only new red cells have a nucleus and as they mature the nucleus disappears)

57
Q

Where is most vitamin B12 absorbed?

A

The ileum

58
Q

What are the signs and symptoms of pernicious anaemia?

A

Similar to that of iron deficient anaemia (pallor, breathlessness, glossitis, fatigue, tachycardia etc.) plus:

Nerve damage (paraesthesia in the extremities, clumsiness)
Stomach problems as the stomach lining will become thinner

59
Q

What is the treatment for pernicious anaemia?

A

IM viatmain B12 injections for life
Blood transfusion idf serious enough
Long term monitoring

60
Q

What is aplastic anaemia?

A

Aplastic anaemia is a serious condition affecting the blood, where the bone marrowand stem cells do not produce enough blood cells. It is also called bone marrowfailure and can happen suddenly (acute) or develop over a period of time (chronic).

AKA “Bone Marrow Failure” (Anaemia + Thrombocytopenia + Leukocytopenia)

61
Q

Where are haematopoietic stem cells found?

A

In the red bone marrow which is in flat bones such as sternum, ribs & pelvis.

62
Q

What can cause aplastic anaemia, can it be prevented?

A

Causes are not well understood.
-It is an autoimmune disorder where the haematopoietic stem cells become targeted for destruction
-There are some genetic links
-A person can get acquired aplastic anaemia due to exposure to toxins such as radiation, some pesticides or medications (e.g. Chloramphenicol)

Currently there is no way to prevent it.

63
Q

What is the peak age of onset of aplastic anaemia?

A

It is most commonly an early childhood disease with another peak in the 20-25 age range

64
Q

What are the signs and symptoms of aplastic anaemia?

A

Most will have same as iron deficient anaemia (pallor, breathlessness, glossitis, fatigue, tachycardia etc.) plus:

Most common sign is unexplained & frequent bruising
Bleeding with extended time for clot formation
Repeated infections

65
Q

What are the treatments for aplastic anaemia?

A

Bone marrow transplant & blood transfusions (often effective)
Immunosuppressive therapy (usually first ine treatment)
Androgens such as danazol or oxymetholone

66
Q

Why are patients with aplastic anaemia given immunosuppressants?

A

To try to slow the immune system destruction of the haematopoietic stem cells.
Might also be anti-rejection medications that are given to transplant patients.

67
Q

Why are patients with aplastic anaemia given androgens?

A

To stimulate erythropoietin production

68
Q

What is Sickle Cell Disease?

A

A group of inherited health conditions that affect the red blood cells. With SCD, the red blood cells have a tendency to go out of shape and become sickle-shaped (like a crescent moon) - instead of their normal disc shape.

69
Q

What is the difference between sickle cell disease and sickle cell trait?

A

To get SCD, you need to have two altered haemoglobin genes, one from each parent. If you only have one of these genes, you will have sickle cell trait, which is very much milder and does not normally cause illness. If you have the trait, the majority of red cells in the blood are normal round shaped cells. Some sickle shaped cells may be present under certain conditions.

70
Q

What is sickle cell anaemia?

A

It is abnormal haemoglobin production caused by an inherited variant of haemoglobin - haemoglobin-S (the normal variant is haemoglobin-A), resulting in a substitution of valine in place of what should be glutamic acid. This causes the HbS to become rigid under low O2 conditions. When deoxygenated the haemoglobin crystalises and alters the shape of the erythrocyte – from a disc to a sickle. HbS is really sensitive to low oxygen conditions.

71
Q

What sort of things can trigger sickle cell disease/crisis?

A

Low O2 conditions (including ones caused internally by vasoconstriction) such as:

High altitude
Infection
Blood loss
Dehydration (think weather changes)
Temperature (weather hot/cold)
Exercise
Stress
Swimming in cold water

72
Q

What is the aetiology of sickle cell trait?

A

Sickle cell trait is found in 1 in 4 West Africans and 1 in 10 Afro-Caribbeans and is also found in people who originate from the Mediterranean, Asia and the Middle East. It is less common in white Europeans

73
Q

Why isn’t sickle cell present from birth?

A

Because in utero the baby relies on foetal haemoglobin, this continues through birth and until the baby is about 6 months old when it begins to rely on its own haemoglobin.

74
Q

What causes sickle cell anaemia?

A

Sickle cell disease, an inherited condition

75
Q

What is the lifespan of affected sickle cell red blood cells?

A

20 days, normal is 120

76
Q

How does sickle cell anaemia cause spleenic issues and what are they?

A

One of the functions of the spleen is to filter out damaged and dead RBCs. Sickle celled RBCs die quicker and are recogised as damaged even before they die. The spleen can deal witha small number of these circulating cells but these abnormal red cells can become stuck in the spleen, if this is in a few areas then the spleen will continue to work but become enlarged as it’s trying to cope with the back up of red cells waiting to get through – this is splenomegaly

Sometimes these abnormally shaped, crystalised cells become trapped in the spleen and block bigger areas of the filtering mechanism. The spleen gets much larger very quickly and many RBCs are held there, not enough for the rest of the body - this is called splenic sequestration and it is a medical emergency.

77
Q

What are signs and symptoms of splenic sequestration?

A

Usually an acute presentation over 1-2 days in a child with sickle cell disease:
Pallor
Fatigue
SoB
Pain in L side abdomen radiating to the R

78
Q

How can splenic sequestration be prevented, and what is the treatment?

A

There is no way to prevent it

Treatment would be blood transfusion and possible splenectomy

79
Q

What mechanism is responsible for most sickle cell disease complications and what complications does it cause?

A

Obstruction in small blood vessels – thrombus formation, infarction, necrosis

Acute chest syndrome
Dactylitis “Hand & Foot Syndrome”
Sickle cell nephropathy
Sickle cell maculopathy (eyes)
Cerebral ischemia
LV dilation
Pulmonary hypertension
Leg ulcers
Gallstones (Excess bilirubin from excess haemolysis)

80
Q

What is acute chest syndrome?

A

Acute chest syndrome is a severe pneumonia-like lung-related complication of sickle cell disease. It occurs due to vaso-occlusion within the pulmonary vasculature of patients with sickle cell disease. This results in deoxygenation of hemoglobin and sickling of erythrocytes, which can cause further vaso-occlusion, ischemia, and endothelial injury. Acute chest syndrome can progress quickly and is the most common cause of death in patients with sickle cell disease.

81
Q

What are the signs and symptoms of acute chest syndrome?

A

Pyrexia
SoB
Low SpO2
Cough
Chest pain
Possible wheezing/pneumonia

82
Q

What are the signs and symptoms of sickle cell anaemia and when do they typically present?

A

Anaemia signs – pallor, fatigue, tachycardia etc.
Hyperbilirubinemia (from excess breakdown of haeme)– jaundice, gallstones
Splenomegaly
Vascular occlusion (crisis) – pain, tissue damage
Occlusion in the lungs – acute chest syndrome (pain, fever)
Heart failure
Frequent infections – reduced healing

Signs do not normally present until after 12 months

83
Q

What are the treatments for sickle cell disease?

A

Analgesia around the clock (usually morphine)
IV fluids
Bed rest
Warm compresses (esp. on extremities)
Remove restrictions (watches/jewellery/tight clothing/BP)
Blood transfusion (if haemolytic crisis i.e. they don’t have enough circulating red cells)
Folic acid supplement to help with RBC production (not iron)

84
Q

Why aren’t haemolytic sickle cell disease patients given iron?

A

The amount of iron in a sickle patient is not usually an issue, if iron was given it could cause iron toxicity where iron collects in the organs and the level becomes toxic.

85
Q

What is Crizanlizumab?

A

A new medication for sickle cell disease - it discourages sickled cells from adhering to the endothelial vessel surface, only some patiets are eligible.

86
Q

What is normal blood pH?

A

7.35-7.45