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Year 5 Core Conditions > Haematology > Flashcards

Haematology Flashcards

1
Q

What is DIC?

A

A syndrome characterised by the systemic activation of blood coagulation, which generates intravascular fibrin, leading to thrombosis of small- and medium- sized vessels, and eventually organ dysfunction.
DIC is characterised by evidence of both thrombin and plasmin activation.

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2
Q

What are the risk factors for developing DIC? (12)

A
  1. Infections (especially sepsis)
  2. Malignancy (especially leukaemia’s)
  3. Major trauma including crush syndrome
  4. Incompatible blood transfusion
  5. Transplant rejection
  6. Severe liver disease
  7. Pancreatitis
  8. Heat stroke
  9. Dissecting aortic aneurysm
  10. Complications post-surgery
  11. Recreational drugs
  12. Anti-phospholipid syndrome
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3
Q

How can DIC present?

A

Aside from the obvious features of the underlying condition causing the DIC,

  1. Large bruises
  2. Spontaneous bleeding at venipuncture sites, on the soft palate, legs and site of trauma
  3. Bleeding from at least three unrelated sites is typical:
    - ENT
    - GI
    - Respiratory tract
    - Site of venepuncture or IV fusion
  4. Confusion or disorientation
  5. Fever
  6. Signs of haemorrhage
  7. Signs of ARDS
  8. Signs of thrombosis
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4
Q

What investigation results indicate DIC?

A
  1. Platelet count is typically low, with a downward trend, especially in acute sepsis associated DIC
  2. Fibrin degradation products including D-dimer are elevated. D-dimer is useful in diagnosis and monitoring of DIC. Although not specific, a normal D-dimer excludes DIC as it is highly sensitive.
  3. Prothrombin time (PT) is elevated (prolonged)
  4. Activated partial thromboplastin time (aPTT) elevated (prolonged)
  5. Fibrinogen level low, although this may be normal in more than half of cases
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5
Q

What is acute non-haemolytic reactions during transfusion?

A

Incompatible transfused red cells react with the patient’s own anti-A or anti-B antibodies or other alloantibodies (anti-Rhesus) to red cell antigens. Complement can be activated and may lead to disseminated intravascular coagulation.

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6
Q

What are the chances of ABO incompatibility and mortality when red cells are mistakenly administered?

A
  1. 33% risk of ABO incompatibility

2. 10% risk of mortality with the severest reaction seen in a group O individual receiving group A red cells.

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7
Q

In order for a clot to be formed, what is needed?

A
  1. Platelets
  2. Vessel wall
  3. Clotting factors
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8
Q

What happens with those three elements for a clot to form? (platelets, vessel wall, clotting factors)

A

Platelets stick together and to the vessel wall lining, to plug the hole. A fibrin clot formation then occurs to make the plug more stable.

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9
Q

What mediates the adhesion of platelets to the vessel lining?

A

Von willibrand factor

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10
Q

What are the causes of a prolonged PT (prothrombin time)? (7)

A
  1. Deficiency or inhibition of one or more of factors II, V, VII, X and fibrinogen
  2. Liver disease
  3. Warfarin
  4. Vitamin K deficiency
  5. DIC
  6. Massive blood transfusion
  7. Gross over-heparinisation
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11
Q

What can cause an isolated prolonged prothrombin time? (4)

A
  1. Early liver disease
  2. Early warfarin administration
  3. Early vitamin K deficiency
  4. Factor VII deficiency (rare)
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12
Q

What part of the coagulation pathway is the ‘motor’ and what is the ‘engine’?

A

The prothrombin is the motor as it starts of the initial thrombin burst, and this then triggers the engine as it activates the ‘intrinsic system’

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13
Q

What are the causes of a prolonged activated partial thromboplastin time? (9)

A
  1. Deficiency or inhibiton of one or more of factors II, V, X, VIII, IX, XI, XII, or fibrinogen
  2. Liver disease
  3. Warfarin
  4. Vit K deficiency
  5. DIC
  6. Massive transfusion
  7. Heparin-unfractionated
  8. Lupus anticoagulant
  9. Haemophilia
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14
Q

What are the causes of a prolonged PT AND APTT? (5)

A
  1. Deficiency of II, V, X, fibrinogen
  2. DIC
  3. Vit K deficiency
  4. Liver failure
  5. Warfarin
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15
Q

What are the properties of LMW heparin?

A

LMWH has a higher ratio of of anti-Xa to anti-IIa activity (compared to fractionated heparin) and has a longer half life allowing for once daily administration. Heparin also has a more predictable anticoagulant response; monitoring is not routinely required.

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16
Q

Why would a patient need to monitor heparin levels?

A

If they have renal diease (4 hours post dose as this is the half life)

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17
Q

What can the complications of heparin be?

A
  1. Heparin induced thrombocytopenia (HIT) - drop in platelet count >50% from baseline, usually 5-10 days after starting
  2. Skin/allergic reactions
  3. Bleeding
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18
Q

What can attempt to treat bleeding with unfractionated heparin/LMWH?

A

Protamine sulphate (derived from fish sperm - check patient does not have fish allergy)

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19
Q

How is INR calculated?

A

INR = patient PT/ Control PT

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20
Q

Which drugs potentiate the warfarin effect? (11)

A
  1. Cimetidine
  2. Amiodarone
  3. Sulphinpyrazone
  4. Cotrimoxazole
  5. Erythromycin
  6. Cephalosporins
  7. Ampicillin
  8. NSAIDs
  9. Chlorpromazine
  10. Sulphonylureas
  11. Corticosteroids
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21
Q

Which drugs inhibit factor Xa? (3)

A
  1. Rivaroxaban
  2. Apixaban
  3. Edoxaban
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22
Q

Which drug inhibits thrombin (factor IIa)?

A

Dabigatran

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23
Q

What are the contraindications for using DOACs? (3)

A
  1. Renal impairment
  2. Women of child bearing age
  3. Extremes of body weight
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24
Q

What is aspirin and how does it work?

A

Aspirin is an antiplatelet and works by inactivating platelet cyclooxygenase. There are no reversal agents and its effect last 4-5 days

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25
Q

In addition to aspirin, what are the other antiplatelet drugs?

A
  1. Clopidogrel
  2. Prasugrel
  3. Ticagrelor
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26
Q

What type of drugs are clopidogrel and ticagrelor?

A

P2Y(12) antagonists

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27
Q

What can cause vitamin K deficiency?

A
  1. Obstructive jaundice
  2. Prolonged nutritional deficiency
  3. Broad spectrum antibiotics
  4. Neonates (classical 1-7 days)
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28
Q

What is the treatment for vitamin K deficiency?

A

IV/oral vitamin K 10mg for 3-5 days

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29
Q

Why are patients with liver disease more at risk of bleeding?

A

The liver is essential to the maintenance of normal levels of coagulation factors, components of the fibrinolytic system, and naturally occurring anticoagulants. Hence patients with liver disease invariably have impaired homeostasis.

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30
Q

Summarise the pathogenesis of DIC?

A
  1. Excess thrombin generation
  2. Reduced natural anticoagulant activity
  3. Decreased fibrinolysis
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31
Q

How does DIC lead to both clotting and bleeding?

A

The clotting occurs in the form of thrombosis in microvasculature, leading to organ failure due to fibrin deposition, whereas the bleeding occurs due to using up all the use of platelets and coagulation factors –> thrombocytopenia and coagulation factor deficiency

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32
Q

What would the blood film of a patient with a splenectomy features? (4)

A
  1. Howell-Jolly bodies
  2. Pappenheimer bodies
  3. Target cells
  4. Irregular contracted erythrocytes
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33
Q

When is Hb defined as low for men and women?

A

Men <135g/L

Women <115g/L

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34
Q

What are the symptoms of anaemia? (6)

A
  1. Fatigue
  2. Faint
  3. Breathlessness
  4. Headaches
  5. Palpitations
  6. Tinnitus
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35
Q

What are the signs of anaemia? (4)

A
  1. Pallor
  2. Tachycardia
  3. Systolic flow murmur
  4. Heart failure
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36
Q

What are the causes of microcytic anaemia, MCV <80?

A
  1. Iron deficiency
  2. Sideroblastic anaemia
  3. Thalassaemia
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37
Q

What are the causes of normocytic anaemia? (4)

A
  1. Acute haemorrhage
  2. Anaemia of chronic disease
  3. Aplastic anaemia
  4. Haemolytic anaemia
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38
Q

What are the causes of macrocytic anaemia, MCV>100?

A 
Megaloblastic 
- Vitamin B12 deficiency
- Folate deficiency
Non-megaloblastic
- Pregnancy
- Alcohol
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39
Q

Where is iron absorbed?

A

Duodenum and upper jejunum

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40
Q

What are the causes of iron deficiency anaemia?

A
  1. Chronic blood loss e.g. peptic ulcer, menhorrhagia
  2. Increased requirement e.g. pregnancy
  3. Malabsorption e.g. Crohn’s, coeliac
  4. Malnutrition e.g. poor diet
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41
Q

What are the signs of iron deficiency anaemia? (5)

A
  1. Koilonychia (spooning of the nail bed)
  2. Angular stomatitis
  3. Glossitis
  4. Pallor
  5. Tachycardia
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42
Q

What are the investigations for suspected iron deficiency anaemia?

A
  1. Bloods - reduced serum iron, reduced serum ferritin, reduced MCV, reduced transferring saturation
  2. Peripheral blood film - microcytic, hypochromic RBCs
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43
Q

What are the treatments for iron deficiency anaemia?

A
  1. Treat underlying cause
  2. Ferrous sulphate 200mg TDS
  3. Consider transfusion if symptomatic at rest e.g. dyspnoea, chest pain
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44
Q

What are the side effects of ferous sulphate tablets?

A

GI upset - constipation, diarrhoea, cramps, vomiting

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45
Q

What are the chronic disease that often contribute to anaemia of chronic disease?

A
  1. IBD
  2. Rheumatoid arthritis
  3. CKD
  4. TB
    (chronic inflammation, chronic infection and malignancy)
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46
Q

What is the pathophysiology behind anaemia of chronic disease?

A

Interleukin 6 promotes an increase in liver hepcidin which alters iron metabolism. EPO normally promotes BM to make RBCs but there is a decrease in response to EPO so there is less production of RBCs over time.

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47
Q

What investigations can be carried out when suspecting anaemia of chronic disease? (5)

A
  1. FBC
  2. MCV
  3. Increased ferritin
  4. Decreased reticulocyte count
  5. Treat the underlying cause
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48
Q

What is the cause of aplastic anaemia?

A

Bone marrow failure

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49
Q

What are the causes of aplastic anaemia/bone marrow failure?

A
  1. Idiopathic
  2. Fanconi’s anaemia (inherited)
  3. Post viral e.g. hepatitis
  4. Drugs (NSAIDs, penicillamine)
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50
Q

What investigations may be carried out in suspected aplastic anaemia? (2)

A
  1. Blood count - pancytopenia and reduced reticulocytes

2. Bone marrow biopsy - hypo cellular marrow and no abnormal cell population

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51
Q

What is the treatment for aplastic anaemia? (3)

A
  1. Treat underlying cause
  2. Supportive
  3. Bone marrow transplantation
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52
Q

Where is a bone marrow transplant taken from?

A

Iliac crest

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53
Q

Why does haemolytic anaemia occur?

A

When RBCs are broken down faster than they are produced

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54
Q

What are the hereditary causes of haemolytic anaemia?

A
  1. Enzyme defects - glucose 6 phosphate dehydrogenase deficiency (favism)
  2. Membrane defects e.g. hereditary spherocytosis
  3. Abnormal haemoglobin production e.g. sickle cell anaemia or thalassaemia
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55
Q

What are the acquired causes of haemolytic anaemia? (crap card)

A
  1. Immune mediate

2. Non-immediate mediated

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56
Q

What happens to certain LFTs when there is an increased breakdown of RBCs?

A
  1. Increased bilirubin
  2. Increased urobilinogen
  3. Increased LDH
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57
Q

What would a blood film possibly show (depending on cause) for haemolytic anaemia?

A
  1. Sickle cells
  2. Spherocytes
  3. Ellipocytes
  4. Heinz bodies (G6PDD)
  5. Hypochromic - thalassaemia
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58
Q

What are the clinical features of sickle cell anaemia?

A
  1. Symptoms of anaemia/haemolysis - pallor, fatigue, tachycardia, ‘lemon tinge’ skin (pale + jaundice)
  2. Vaso occlusion - PAIN, chronic renal failure, dactylitis
  3. Crisis - acute chest syndrome, acute abdomen (mesenteric ischaemia), bone infarction
  4. Growth failure
  5. Increased susceptibility to infection (hyposplenism)
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59
Q

Why does sickle cell disease cause the clinical features of sickle cell anaemia?

A

Due to the shape of the RBC, it disrupts blood flow and breaks down - haemolysis, leading to anaemia. They also obstruct small blood capillaries. Also sequester in the spleen and block it, so you get splenomegaly and it doesn’t work as well.

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60
Q

What are the treatments for sickle cell anaemia? (5)

A
  1. Blood exchange/transfusion
  2. Folic acid
  3. Hydroxycarbamide (if frequent and painful crisis)
  4. Antibiotic prophylaxis
  5. Avoid precipitants and stay hydrated
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61
Q

What is an important side effect of hydroxycarbamide to be aware of?

A

WBC suppression

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62
Q

What is acute chest syndrome associated with sickle cell crisis?

A

Pneumonia like syndrome due to sickling in pulmonary vasculature - chest pain, fever, dyspnoea, tachypnoea

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63
Q

What is the treatment for a vaso-occlusive crisis? (ribs, spine, pelvis) (6)

A
  1. Cross match blood
  2. Analgesia - paracetamol, ibuprofen, codeine, morphine IV
  3. Supportive care - O2 + treat cause
  4. IV fluids
  5. Antibiotics
  6. Give blood if Hb or reticulocytes fall quickly
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64
Q

What is the management for acute chest syndrome? (6)

A
  1. CXR, ABG
  2. Oxygen + spirometry
  3. Analgesia
  4. Broad spectrum ABX
  5. Blood transfusion
  6. Fluids
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65
Q

What is megaloblastic anaemia?

A

Unusually large, structurally abnormal RBCs due to defective DNA synthesis

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66
Q

What are the causes of megaloblastic anaemia?

A
  1. B12/folate deficiency

2. Drugs

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67
Q

How can B12 deficiency present?

A

It is macrocytic anaemia with peripheral neuropathy and neuropsych complaints (depression, irritability)

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68
Q

What are the causes of B12 deficiency? (4)

A
  1. Poor diet (not enough meat, fish, dairy)
  2. Malabsorption (pernicious anaemia, Crohn’s, coeliac)
  3. Reduced gastric breakdown of B12 (previous gastric surgery, atrophic gastritis)
  4. Drugs (metformin - decreased B12 absorption, PPI)
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69
Q

What is atrophy gastritis?

A

An autoimmune gastritis with atrophy of parietal cells leading to no intrinsic factor and then B12 isn’t absorbed

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70
Q

Why is B12 not absorbed if there is no intrinsic factor?

A

B12 combines to IF to form a complex, and this travels to the terminal ileum where it binds with transcobalamin and gets absorbed into the blood stream.

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71
Q

Why is B12 so vital for the body - what is its role?

A

B12 helps make functioning RBCs and is involved in DNA synthesis of all cells + myelin sheath

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72
Q

Why is B12 deficiency important to correct - what can it lead to?

A

Subacute degeneration of the spinal cord

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73
Q

What is the classic triad of subacute degeneration of the spinal cord?

A
  1. Upgoing plantars
  2. Loss of knee jerk
  3. Loss of ankle jerk
74
Q

Before subacute degeneration of the spinal cord, what other symptoms present of B12 deficiency? (3)

A
  1. Anaemia symptoms
  2. Paraesthesia, ataxia, loss of vibration sense
  3. Peripheral neuropathy
75
Q

What is the treatment for B12 deficiency?

A

IM hydroxycobalaminv (depends on severity)

76
Q

What is pernicious anaemia?

A

Autoimmune atrophic gastritis - autoantibodies are made against parietal cells so unable to make intrinsic factor and hence unable to absorb B12

77
Q

Pernicious anaemia is associated with what other diseases? (4)

A
  1. Thyroid disease
  2. Vitiligo
  3. DM
  4. Addison’s
78
Q

Pernicious anaemia affects which sex more?

A

Females > males 1.6 times

79
Q

What is there a small risk of associated with pernicious anaemia?

A

Gastric cancer

80
Q

How is folate deficiency difference to B12 deficiency in terms of symptoms?

A

Absence of neurological signs

81
Q

What food substances contain folate?

A
  1. Green veg
  2. Nuts
  3. Liver
82
Q

Where is folate absorbed in the body?

A
  1. Proximal jejunum

2. Duodenum

83
Q

What is folate deficiency caused by?

A
  1. Poor diet (poverty, alcoholics)
  2. Increased demand (pregnancy, renal disease)
  3. Malabsorption (coeliac)
  4. Drugs, alcohol, methotrexate (inhibits synthesis of folic acid)
84
Q

What is found on investigation of bloods for folate deficiency?

A
  1. On FBC - low Hb, increased MCV
  2. Low reticulocyte count
  3. Low serum folate
  4. Blood film - macrocytic RBC and hypersegmented neutrophils
85
Q

What are the four types of leukaemias?

A
  1. Acute lymphoblastic leukaemia (ALL)
  2. Acute myeloid leukaemia (AML)
  3. Chronic lymphocytic leukaemia (CLL)
  4. Chronic myeloid leukaemia (CML)
86
Q

What are the two standard types of lymphoma?

A

Hodgkin’s and Non-Hodgkin’s lymphoma

87
Q

What is the top differential for a 47 year old male, with a 2 week history of sore gums with no dental cause identified, extreme fatigue (unable to get out of bed) and significant weight loss over a month with a loss of appetite, and noticed bruising. White plaques have formed in the mouth making swallowing painful.

A

AML - proliferation of myeloid (immature) cells.

88
Q

Why do people with AML get sore, bleeding gums?

A

Low blood platelets (thrombocytopenia) can lead to bleeding gums, bruising, severe nose bleeds, heavy periods.

89
Q

What medical emergency can occur with AML? ..and what are the symptoms? (5)

A

Leukostasis - the white cells produced in AML are much larger than normal white cells, and can obstruct small vessels, preventing RBCs accessing tissues, leading to:

  1. Headache
  2. Weakness to one side
  3. Slurred speech
  4. Confusion
  5. Sleepiness
90
Q

What are the risk factors for AML? (3)

A
  1. Down syndrome
  2. Bone marrow failure (aplastic anaemia or Fanconi’s)
  3. Smoking
91
Q

What happens in AML?

A

There is a proliferation of immature myeloid cells

92
Q

What is the 5 year survival rate for AML?

A

5 years

93
Q

What is the most common leukaemia in adults?

A

CLL - >40% at >65 years old

94
Q

What are Auer rods which can be found on peripheral blood films?

A

Crystalised granules in myeloid blasts

95
Q

What can be found on a FBC for AML? (4)

A
  1. Increased WCC (leucocytosis)
  2. Neutropenia
  3. Thrombocytopenia
  4. Anaemia
96
Q

What is the management of AML?

A
  1. Hydration - electrolyte abnormalities
  2. Allopurinol - tumour lysis syndrome
  3. Leukoreduction - hydroxycarbamide/leukapheresis
  4. Transfusions - RBC + platelets
  5. Treat infections
  6. Induction chemotherapy - cytarabine and daunorubicin
  7. +/- stem cell transplant at irst remission (allogeneic)
97
Q

What happens in tumour lysis syndrome?

A

Electrolyte and metabolic disturbance due to breakdown of large number of leukaemic cells (hyperuricaemia, hyperphosphateaemia, hyperkalaemia, hypocalcaemia, renal impairment)

98
Q

4 year old girl presents with 2 week history widespread bruising, with hepatosplenomegaly on examination and appears pale. What is the top differential?

A

ALL - acute lymphoblastic leukaemia - proliferation of immature lymphoblastic cells

99
Q

What four things are associated with ALL?

A
  1. Down syndrome
  2. Ionising radiation
  3. Kleinefelter’s
  4. Fanconi anaemia
100
Q

What are the diagnostic features of ALL?

A
  1. Bone marrow blasts >20%

2. Blood smear leukaemic lymphoblasts

101
Q

ALL is the most common childhood leukaemia, what % are aged less than 6 when diagnosed?

A

75%

102
Q

What are the signs/symptoms of ALL? (5)

A
  1. Neck stiffness, headache, photophobia
  2. Symptoms of anaemia
  3. Infections due to low WCC
  4. Bleeding/bruising/petechial rash due to low platelets
  5. Hepatosplenomegaly
103
Q

What is the management of ALL?

A
  1. Hydration - U&Es abnormalities
  2. Allopurinol - tumour lysis syndrome
  3. Prophylactic antimicrobials (aciclovir + fluconazole + ciprofloxacin + co-trimoxazole)
  4. Transfusions - RBC + platelets
  5. BEWARE of neutropenic sepsis
  6. Induction chemotherapy - prednisolone, vincristine, daunorubicin, tyrosine kinase inhibitor (imatinib)
  7. If CNS involvement - intrathecal methotrexate
104
Q

What happens if there is CNS involved with ALL?

A

Signs of meningism, papilloedema, CN palsy. An LP will be performed.

105
Q

Which chromosome and gene is associated with CML?

A

Philadelphia chromosome - BCR-ABL fusion gene

106
Q

What are the symptoms associated with CML? (7)

A
  1. Malaise
  2. Fever
  3. Weight loss
  4. Abdominal discomfort (splenomegaly)
  5. Night sweats
  6. Arthralgia (increased uric acid from cell turnover)
  7. Gout - purine breakdown
107
Q

What are the three stages of CML? (3)

A
  1. Chronic - 90% asymptomatic
  2. Accelerated - more rapidly dividing, makes defective cells (bleeding due to abnormal platelets and splenomegaly)
  3. Blast phase - bone pain and features of acute leukaemia
108
Q

What are the investigations carried out for CML and what do they reveal?

A
  1. FBC - increased WCC, anaemia, platelet count depends on phase
  2. Peripheral blood film - WBC - mature myeloid cells
  3. Bone marrow biopsy - granulocytic hyperplasia
  4. Cytogenetics (FISH) - philadelphia chromosome
109
Q

What is the management for CML?

A
  1. Tyrosine kinase inhibitors e.g. imatinib or dasatinib
  2. Stem cell transplant
  3. High-dose induction chemotherapy
110
Q

What are the side effects associated with use of imatinib?

A
  1. Muscle cramps

2. Heart failure

111
Q

What is the side effect associated with dasatinib?

A

Pleural effusion

112
Q

Does having the philadelphia chromosome suggest a good or bad prognosis?

A

Good - 95% survival

113
Q

What is CLL?

A

An accumulation of mature B-cells that have escaped programmed cell death

114
Q

What are the clinical features of CLL?

A

Normally asymptomatic so picked up on routine testing e.g. anaemia/infection prone
Signs can include lymhadenopathy (enlarged, rubbery, non-tender lymph nodes) and splenomegaly

115
Q

What are the B symptoms associated with CLL? (3)

A
  1. Weight loss
  2. Night sweats
  3. Fever
116
Q

What cells can be seen on blood film associated with CLL?

A

Smear cells

117
Q

What is the management for CLL? (5)

A
  1. Watch and wait
  2. Supportive - transfusions, IV IG
  3. Chemotherapy - rituximab + cyclophosphamide + fludarabide
  4. Stem cell transplant
  5. Radiotherapy for nodes
118
Q

What is the difference between lymphomas and leukaemias?

A

Lymphomas are SOLID, leukaemias are circulating

119
Q

What is a classic history for a lymphoma?

A

28 year old female patient presents to GP with flu like symptoms, noticed some swelling on the left side of the supraclavicular region that becomes tender when she drinks wine

120
Q

Which cells are associated with Hodgkin’s lymphoma?

A

Reed Sternberg cells - binucleate malignant B lymphocytes

121
Q

Which infection increases the risk of Hodgkin’s lymphoma?

A

EBV (glandular fever)

122
Q

What is the staging system used for Hodgkin’s lymphoma?

A

Ann-Arbor

123
Q

What are the nodal symptoms associated with lymphomas? (5)

A
  1. Enlarged
  2. Non-tender
  3. Rubbery
  4. Alcohol induced pain
  5. Mediastinal lymphadenopathy (mass effect –> bronchial obstruction)
124
Q

What are the B symptoms associated with Hodgkin’s lymphoma? (5)

A
  1. Fever
  2. Night sweats
  3. Weight loss
  4. Pruritis
  5. Lethargy
125
Q

What is the treatment for lymphomas?

A

Chemo and radiation

126
Q

What is an example of Non-Hodgkin’s lymphoma and how is it discernable from Hodgkin’s?

A

Non-Hodgkin’s lymphoma is any lymphoma without Reed-Sternberg cells.

127
Q

Name a high grade non-hodgkin’s lymphoma?

A

Burkitt’s lymphoma

128
Q

What is the most common non-hodgkin’s lymphoma?

A

Diffuse large B cell lymphoma

129
Q

What are the causes of non-hodgkin’s lymphoma?

A
  1. Immunodeficiency
  2. HIV
  3. EBV
130
Q

What are the extranodal signs associated with lymphomas?

A
  1. Bone marrow - pancytopenia (fatigue, dyspnoea)
  2. Splenomegaly + hepatomegaly
  3. Dry cough - mediastinal mass/pneumonia
  4. Gut - diarrhoea, vomiting, abdo pain
  5. Bone pain
131
Q

B cell symptoms are more common in which type of lymphoma - HL or NHL?

A

Non H L

132
Q

How does Ann Arbor staging work for lymphoma?

A

Stage 1 - single lymph node group
Stage 2 - multiple on same side diaphragm
Stage 3 - multiple on opposite side diaphragm
Stage 4 - extranodal disease

B - weight loss >10%, fever, night sweats
A - no B symptoms

133
Q

What is the top differential for this case - 72 year old male presents to GP with lower back pain and feeling tired. His wife says he seems a bit more confused. His bloods are checked and he has anaemia, increased calcium and increased creatinine.

A

Multiple myeloma

134
Q

What happens in multiple myeloma?

A

Proliferation of malignant plasma cells –> secretion of Ig

135
Q

What are the signs/symptoms of myeloma?

A 
CRAB 
C - calcium elevated
R - renal failure
A - anaemia
B - bone lesions
136
Q

What type of anaemia occurs in myeloma?

A

Normocytic, normochromic anaemia

137
Q

What screening is performed in suspected myeloma?

A

Serum and urine electrophoresis

  • light chain deposits
  • Bence Jones proteins
138
Q

What is the treatment for myeloma?

A
  1. NSAIDs
  2. Bisphosphonates
  3. EPO transfusions
  4. IV immunoglobulin infusion
  5. Chemo
139
Q

What are the causes of death associated with myeloma?

A
  1. Infection

2. Renal failure

140
Q

What level of calcium is classed as raised?

A

> 2.7

141
Q

What causes the renal impairment in myeloma?

A

Light chain deposits in tubules

142
Q

What can occur with regards to the bony lesions in myeloma?

A

Spinal cord compression - lytic lesions lead to osteoporosis lead to SCC

143
Q

What is the name of the lytic lesions seen in the skull with myeloma?

A

Raindrop skull (mistakenly known as pepper-pot skull)

144
Q

What are the haematological emergencies? (3)

A
  1. Neutropenic sepsis
  2. Sickle cell crisis
  3. Blood transfusion reaction
145
Q

Where are the places sickle cell crisis can occur? (5)

A
  1. Mesentery (mesenteric ischaemia)
  2. Penis (priapsim)
  3. Hands/feet –> dactylitis
  4. CNS –> stroke, seizures
  5. Lungs –>acute chest syndrome
146
Q

In sickle cell crisis, why is there an increase in MCV?

A

Haemolysis of sickled RBCs –> raised reticulocyte count –> reticulocytes are larger than RBCs and their increased number results in apparent increase in MCV

147
Q

Why is there an increase in bilirubin in a sickle cell crisis?

A

Bilirubin is the breakdown product of Hb (as haemolysis is occurring there is more Hb)

148
Q

What is the management of sickle cell crisis after analgesia? (4)

A
  1. IV fluids
  2. Antibiotics
  3. Oxygen
  4. (Crossmatch blood)Blood transfusion
149
Q

What can precipitate a sickle cell crisis? (4)

A
  1. Infection
  2. Cold
  3. Hypoxia
  4. Dehydration
150
Q

What is the systematic way of consent for blood transfusion? (5)

A
  1. Identify patient
  2. Benefits - symptomatic, improved QOL, post-trauma life saving
  3. Risks - allergic reaction, anaphylaxis, transfusion reaction, TACO, infection, iron overload, haemolytic reaction (ABO incompatibility), wrong blood
  4. Alternatives - treat cause, iron, tranexamic acid
  5. Explain no longer able to donate blood
    (to consider: irradiated blood, HLA matched?)
151
Q

What range is normally accepted for a transfusion to be carried out regardless of symptoms?

A

<70/80

152
Q

What are the signs associated with low Hb? (3)

A
  1. Tachycardia
  2. Systolic flow murmur
  3. Pale
153
Q

If someone receiving a blood transfusion starts to spike a temperature and their BP drops to 79/47, what are the possible causes? (3)

A
  1. Acute haemolytic reaction
  2. TACO
  3. Anaphylaxis
154
Q

What do you do if someone spikes a temperature and BP drops during a blood transfusion?

A

Stop the transfusion

155
Q

In the same patient who has had a reaction to their blood transfusion and spiked a temperature, you then notice oozing from the cannula site and from the puncture wound from your first failed attempt at cannulation. What haematological state has occurred?

A

DIC

156
Q

What is DIC?

A

Activation of the coagulation pathway in response to infection –> microvascular thrombosis –> end organ damage. Once coagulation factors used up –> bleeding.

157
Q

What are the causes of DIC?

A
  1. Sepsis
  2. Malignancy
  3. Trauma in obstetric emergencies
158
Q

What is the treatment for DIC?

A
  1. Treat underlying cause

2. Give blood and platelets

159
Q

A 46 year old afrocaribbean lady presents with GP with menorrhagia. She has FBC Hb78, MCV 75, platelets 398 and WCC 8.8. What do the blood results show?
What is the cause?

A

Microcytic anaemia.

Iron deficiency anaemia due to heavy periods

160
Q

What would you expect to see on examination of a patient with microcytic anaemia? (7)

A
  1. Pale conjunctiva
  2. Tachycardia
  3. Tachypnoea
  4. Systolic flow murmur
  5. Glossitis
  6. Angular stomatitis
  7. Koilonychia
161
Q

What are the side effects associated with ferrous sulphate replacement therapy? (3)

A
  1. Black stools
  2. Constipation
  3. Nausea
162
Q

A 23 year old man presents to GP with a lump in his neck which he has had for 6 weeks. It is painless and has a rubbery feel. What is the top differential?

A

Lymphoma

163
Q

What symptoms might the patient with the painless lump on neck have?

A

B symptoms - fever, night sweats, weight loss.

Might also have enlarged lymph nodes elsewhere.

164
Q

What is the staging used in lymphoma?

A

Ann Arbor

165
Q

What is the top differential for the patient with lymphoma presenting to A&E with difficulty breathing and swelling of the face, with distended neck veins?

A

SVC obstruction

166
Q

What is the differential for a 72 yr old man presenting with lower back pain for 3/12, with vertebral fractures on X-ray?

A

Multiple myeloma - (could possibly be prostate cancer with metastases?)

167
Q

What is multiple myeloma?

A

Malignant proliferation of B lymphocytic plasma cells

168
Q

What common immunoglobulin is expressed in multiple myeloma?

A

IgG (2/3rds of cases)

169
Q

What are the common symptoms associated with multiple myeloma?

A
  1. Recurrent infections
  2. Tiredness
  3. Easily bruised and bleeds
  4. Confusion
  5. AKI symptoms? - polyuria/polydipsia
170
Q

What investigations need to be carried out in suspected multiple myeloma? (4)

A
  1. Bloods (usual + calcium, IgG/E/M)
  2. Blood film
  3. Urine + serum electrophoresis
  4. Bence Jones protein
171
Q

Why are people with multiple myeloma prone to bacterial infection? (2)

A
  1. Bone marrow infiltration so neutropenia

2. Lack of functioning immunoglobulins

172
Q

What are the acute complications of multiple myeloma? (3)

A
  1. Spinal cord compression
  2. Hypercalcaemia
  3. AKI
173
Q

What happens in multiple myeloma?

A

Usually plasma cells produce various forms of immunoglobulins, however in myeloma, one particular plasma cell clone begins to replicate in an uncontrolled manner, resulting in one specific type of immunoglobulin being massively overproduced by the large group of identical plasma cell clones. These spikes can be seen on serum and urine electrophoresis.

174
Q

Why does anaemia occur in multiple myeloma?

A

The plasma cell clones accumulate in bone marrow, crowding the normal healthy tissue responsible for producing normal blood cells. This results in anaemia, impaired immune function and low platelets, increasing the risk of bleeding.

175
Q

In myeloma, overcrowding in the bone marrow causes anaemia, but how does kidney damage/AKI occur?

A

The abnormal plasma cells produce a paraprotein. These paraproteins are abnormal immunoglobulin light chains which can cause damage tot he kidneys by forming protein casts in the renal tubules.

176
Q

Why does myeloma cause lytic lesions and hypercalcaemia?

A

The abnormal plasma cells secrete factors which active osteoclasts to break down bone, resulting in these widespread lesions, bone pain and hypercalcaemia

177
Q

Which two immunoglobulins are most common produced in multiple myeloma?

A

IgG 2/3rds

IgA 1/3rd

178
Q

What is the mnemonic used to remember the symptoms of multiple myeloma?

A 
CRAB
C- calcium (raised)
R - renal failure
A - anaemia
B - bone lesions
179
Q

What type of bone pain is commonly experienced in people with myeloma?

A

Common in the spine and ribs, it typically worsens with activity and persistent pain in a particular area should raise suspicions of a pathological fracture.

180
Q

What can be seen on X-ray of the skull in people with myeloma?

A

Rain drop skull - multiple lytic lesions

181
Q

What symptoms occur due to renal failure with multiple myeloma? (6)

A 
Due to uraemia: 
1. N&amp;V
2. Weight loss
3. Lethargy 
Due to raised phosphate:
4. Pruritis 
5. Muscle cramping 
Due to pulmonary oedema secondary to an inability to excrete fluids 
6. SOB
182
Q

Which condition is associated with JAK2 mutation?

A

Polycythaemia vera

Year 5 Core Conditions (25 decks)

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