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Phase 2a > Haematology > Flashcards

Haematology Flashcards

1
Q

What percentage of blood do RBC make up?

A

45%

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2
Q

Anaemia?

A

Decreased level of Hb or RBC in the blood below the reference for age and sex of the individual meaning that there is a decreased amount of O2 to tissue and organs

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3
Q

In what circumstances would a patient present with decreased Hb but increased RCM?

A

3rd Trimester of pregnancy

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4
Q

Average mcv?

A

800 - 90 fl

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5
Q

Hb ranges for M and F?

A

M - 135 - 175 g/L

F - 115 - 160 g/L

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6
Q

What are the physiological compensatory mechanisms for anaemia?

A

Increase HR

Increase oxygen dissociation

Increased red cell production

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7
Q

What are the pathological consequences of anaemia?

A

myocardial and fatty liver changes

aggravate angina and claudication

skin and nail atrophic changes

CNS changes (cortex and basal ganglia)

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8
Q

what is a key measure of anaemia?

A

Reticulocytes - baby RBC

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9
Q

Microcytic anaemia in relation to MCV and Hb?

A

Decreased MCV and Decreased Hb

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10
Q

Normocytic anaemia in relation to MCV and Hb?

A

Decreased Hb and normal MCV because the Hb produced are normal but there is a low number of them

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11
Q

Macrocytic anaemia in relation to MCV and Hb?

A

Increased MCV and decreased Hb

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12
Q

The greatest cause of microcytic anaemia?

A

Iron deficiency

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13
Q

Aside from iron deficiency, give other causes of microcytic anaemia?

A

Chronic diseases such as renal failure and Thalassamia

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14
Q

What can cause normocytic anaemia?

A

Acute blood loss

Chronic disease

Combined haematinic deficiency

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15
Q

What is a combined haematinic deficiency?

A

Deficiency in iron, B12 and folate

micro and macro cancel each other out to try to look normal

  • consider malabsorption
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16
Q

Causes of Macrocytic anaemia?

FATRBCMD

A

Folate deficiency

Alcohol abuse

Thyroid - hypo

Reticulocytosis

B12 deficiency

Cirrhosis

Myelodysplasia

Drugs

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17
Q

What investigations would be used to determine decreased B12?

A

Antibodies for intrinsic factor, coeliac, parietal cells

Schilling test - see how well a patient can absorb nuclear radioactive B12

Bone marrow biopsy + haematology referral

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18
Q

Growth factors that stimulate blood cells production?

A

Erythropoietin

Thrombopoietin

G-CSF

Interleukin-5 (eosinophils)

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19
Q

What is tumour necrosis factor?

A

They are pro-inflammatory cytokines released by activated macrophages and they can suppress erythropoiesis

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20
Q

Dilutional anaemia?

A
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21
Q

What are the clinical features of anaemia dependant on?

A

Severity and speed of anaemia

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22
Q

Why may someone with anaemia be asymptomatic?

A

The gradual decrease in Hb allows for haemodynamic compensation and enhancement of CO2 carrying capacity - faster HR, Increased O2 dissociation and increased RC production

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23
Q

Non-specific symptoms of anaemia?

A

fatigue

Faint

Breathless

Exacerbation of angina and claudication

Tachycardia

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24
Q

What medication has been associated with macrocytic anaemia?

A

Azathioprine - immunosuppressant that suppresses the production of RBC in BM

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25
Q

what is a hyperchromatic blood film associated with?

A

The centre of the RBC is pale - >1/3

Iron deficiency microcytic anaemia

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26
Q

What are the 2 forms of dietary iron?

A

Non-haem - diet - ferric to ferrous then dissolved and absorbed

Haem - Hb and myoglobin found in red and organ meat

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27
Q

What is the degradation product of ferritin?

A

Haemosiderin

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28
Q

Causes of iron loss?

A

Menstruation

GI bleed

Increased demand (growth and pregnancy)

Poor intake

Malabsorption

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29
Q

What is the difference that would be seen between iron deficiency and anaemia of chronic disease?

A

ID - Decreased serum iron and greater total iron-binding capacity

CD - Decreased serum iron and decreased total iron-binding capacity

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30
Q

Signs of microcytic anaemia?

A

Koilonychia

Atrophic Glostitis - bumpy tongue

Angular stomatitis - cold sores around the mouth

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31
Q
A
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32
Q

Investigations for microcytic anaemia?

A

Blood cultures + film - small and hyperchromatic

D serum ferritin

D serium iron but high total iron binding capacity (Transferrin saturation <19%)

Increased transferrin receptor number

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33
Q

Management of iron deficiency microcytic anaemia?

A

Oral iron - ferrous sulphate to increase reticulocytes and Hb

Parenteral iron - IM or IV for those intolerant to oral iron or malabsorption

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34
Q

How does chronic disease ead to microcytic anaemia?

A

Decreased serum iron and decreased total iron binding capacity

Hepcidin is released in response to inflammation and it binds to export proteins (ferroportin) of partial cells and degrades them, iron is also trapped in macrophages and liver cells - so decreased iron in circulation

Hepcidin is produced by the liver

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35
Q

Difference between megaloblastic and non-megalobastic macrocyticanaemia?

A

Megaloblastic - Defective DNA synthesis of RBC causing them to have delayed nuclear maturation in relation to their cytoplasm - B12

Non-megaloblastic - No DNA impairment of RBC production but they are still large - cirrhosis, hypothyroidism

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36
Q

The absorption process of B12?

A

Liberated from protein complexes by gastric acid and pepsin to vit B12

Bound to intrinsic factor b=to be absorbed in the terminal ileum

stored in the liver

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37
Q

Causes of Decreased B12

A

Vegan

Pernicious anaemia (autoimmune against parietal cells)

Coeliac

Tapeworm

Achlorhydria - no gastric acid or HCL

Nitrous oxide - inactivates B12

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38
Q

Signs of macrocytic anaemia?

A

Glossitis

Angular stomatitis

Mild jaundice

Weakness, ataxia, paresthesia

Visual disturbances

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39
Q

Difference between B12 and folate deficiency?

A

Folate deficiency doesn’t cause neuropathy

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40
Q

Investigations to confirm low folate?

A

Red cell folate and if not a dietary cause, look into small bowel disease

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41
Q

if a patient has megaloblastic anaemia, why do you not give them folic acid?

A

Folic acid could aggravate neuropathy caused by B12 deficiency

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42
Q

Investigations done to confirm macrocytic anaemia?

A

Blood cultures and film - hypersegmented + large

Decreased serum B12

Serum antibodies - parietal, coeliac and intrinsic factor

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43
Q

Management of B12 deficiency anaemia?

A

Hydroxocobalamin or oral B12

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44
Q

Drug that can cause folate deficiency?

A

Phenytoin - antiseizure

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45
Q

Causes of folate deficiency?

A

poor intake

malabsorption

Excessive usage (pregnancy, lactation, renal dialysis)

Drugs - phenytoin

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46
Q

What is aplastic anaemia?

A

Pancytopenia with hypercellularity - deficiency of all cell elements of blood with aplasia of bone marrow

Anaemia caused by bone marrow failure - you get a reduction in blood cell components and the ones made are faulty or trigger an immune reaction

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47
Q

Causes of aplastic anaemia?

A

pregnancy

newborn

alcohol excess

liver disease

hypothyroidism

Drugs - hydroxycarbamide (chemo therapy)

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48
Q

Clinical features of aplastic anaemia?

A

Bruising

Bleeding gums

epistaxis (nose bleeds) - acute haemorrhages of nostril, nasal cavity or nasopharynx

INCREASED RISK OF INFECTION DUE TO DECREASED NEUTROPPILS

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49
Q

Investigations of aplastic anaemia?

A

Blood count - pancytopenia + low reticulocytes

Bone marrow exam - hypercellular and increased fat space

Bone marrow trephine biopsy

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50
Q

Management of aplastic anaemia?

A

Stop causative agent

Blood and platelet transfusion

Bone marrow transplant

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51
Q

What is haemolytic anaemia?

A

Increased destruction of RBC and decreased circulating life span

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52
Q

What is the compensatory mechanism of haemolytic anaemia?

A

BM releases more reticulocytes

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53
Q

Intravascular causes of haemolytic anaemia?

A

Acquired - autoimmune or non-autoimmune

Inherited - membrane disorders, Hb abnormalities or metabolic disorders

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54
Q

examples of inherited membrane disorders than can cause haemolytic anaemia?

A

spherocytosis

Elliptocytosis

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55
Q

Examples of Hb abnormalities that can cause haemolytic anaemia?

A

Thalassaemia

Sickel cell

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56
Q

What metabolic defect can cause haemolytic anaemia?

A

Glucose-6-phosphate deficiency

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57
Q

What factors suggest haemolytic anaemia?

A

Increase plasma Hb and decreased RBC

Increased Hb binding to haptoglobin so decreased haptoglobin in total

+ve schumm test - Detect levels of methemalbumin which is haem and albumin

Haemosiderin

Haemoglobinuria

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58
Q

What are inherited haemolytic anaemias?

A

Anaemia caused by defects in components of mature blood cells e.g. membrane defects, Hb abnormalities or metabolic defects in the machinery of the RBC

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59
Q

Hereditary spherocytosis?

A

Decreased spectrin which is a structural protein of RBC - increased Na permeability

RBC is spherical, rigid and less deformable than a normal RBC

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60
Q

Clinical features of spherocytosis?

A

Asymptomatic

Jaundice, splenomegaly

Pigmented gall stones

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61
Q

Investigations of hereditary spherocytosis?

A

Increased reticulocytes + anaemia

Sphrocytes on blood film

Haemolysis - increased biliverdin

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62
Q

Management of hereditary spherocytosis?

A

Splenectomy after childhood

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63
Q

What is hereditary elliptocytosis?

A

RBC are elliptical in shape rather than standard biconcave

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64
Q

What is thalassaemia?

A

A group of disorders arising from 1 or multiple gene defects resulting in a reduced rate of production of 1 or more globin chains

Alpha and beta thalassaemia

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65
Q

Beta thalassaemia?

A

Little to no beta chain production so alpha bind with delta or gamma chains

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66
Q

What are the 3 forms of beta thalassaemia?

A

BT minor - trait - anaemia absent

BT intermedia - moderate anaemia - splenomegaly, leg ulcers, gall stones

BT major - severe anaemia in the first year of life, failure to thrive and recurrent infections

Hypertrophy of infective bone

Thalassaemic facies - large maxilla, parietal and frontal bone

Hepatosplenomegaly

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67
Q

Investigations used to diagnose thalassaemia?

A

Blood film - hyperchromatic microcytic

Increased reticulocytes

Nucleated red cells in peripheral circulation

Hb electrophoresis - Increased Hbf and decreased HbA

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68
Q

Management of beta thalassaemia?

A

Blood transfusion alongside iron-chelating agents (acerbic acid) to prevent iron overload

Long term folic acid

Bone marrow transplant

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69
Q

Alpha thalassaemia?

A

4x alpha genes per cell and sometimes some or all of them get deleted - manifestation of symptoms is dependant on how many genes have been deleted

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70
Q

Hb barts?

A

Complete absence of all alpha chains in a RBC

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71
Q

what is sickle cell anaemia?

A

family of Hb disorders where the sickle b-globin gene is inherited

Valine or lysine replaced by glutamic acid

In low O2 environments, Hb becomes insoluble and polymerised - rigid sickle shape = premature haemolysis and obstruction of the microcirculation

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72
Q

Exacerbating factors of sickle cell

A

infection, cold, hypoxia, dehydration and acidosis

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73
Q

How do the clinical features of sickle cell differ between child and adults?

A

The effects of vascular occlusion

Child - pain in hands and feet

Adult - pain in long bone, ribs, spine and pelvis (avascular necrosis of bone)

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74
Q

Other clinical features of sickle cell anaemia?

A

Enlarged spleen

Bone marrow aplasia

infection

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75
Q

Long term effects of sickle cell anaemia?

A

deformed bones

splenic atrophy

renal ischaemia

stroke

pigmented gall stones

ulcers

Acute chest syndrome

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76
Q

Investigations of sickle cell?

A

Heel prick and check if from high-risk area

Blood count - high reticulocytes with high Hb

Blood film - Sickled erythrocytes

Hb electrophoresis - HbSS

77
Q

Management of sickle cell?

A

Avoid precipitating factors

Non-opiate adjuvant analgesics - P, NSAIDS

Blood transfusion

Hydroxycarbamide - increases HbF and is used in crisis

78
Q

What is the sickle cell trait?

A

Normal blood count and film but symptoms come in extreme situation e.g. flying in a non-pressurised plane

79
Q

Treatment of emergency sickle crisis?

A

analgesia - morphine and non-opioid adjuvant analgesia

Laxative

Anti-pruritic

Antiemetics - vomit

Anxiolytic - reduce anxiety

Antibiotic

80
Q

How does glucose 6 dehydrogenase deficiency result in haemolytic anaemia?

A

G6PD - maintains levels of glutathione which protects the RBC against antioxidant injury and without it they get injured by antioxidants

81
Q

Complications of G6PD?

A

Jaundice

Haemolytic anaemia

Acute haemolysis

82
Q

Investigations to confirm G6PD deficiency?

A

G6PD in RBC

83
Q

What to avoid if you have G6PD?

A

Flava beans

Drugs such as quinolones

84
Q

Management of G6PD?

A

Remove causative agents and sometimes transfusion

85
Q

Differences between the 2 types of autoimmune acquired haemolytic anaemia?

A

WARM AHA - IgG, autoimmune disorders 2º cause, management is high dose steroids, splenectomy or immunosuppressants

COLD - IgM, 2º cause is infection and management is to avoid cold exposure

86
Q

What is autoimmune haemolytic anaemia?

A

Antigens on the surface of the patients RBC trigger an immunological response and get destroyed by autoimmune antibodies

Dependant on body temp

Antibodies attach to RBC causing extravascular haemolysis (spleen) or intravascular haemolysis (activation of complement)

87
Q

What is the direct antiglobulin coombs test?

A

antibodies or complement attached to surface RBC - react with antiserum or monoclonal antibodies against the attached immunoglobulins or C3d

+ve - agglutination

88
Q

Can drugs cause autoimmune haemolysis?

A

Yes

They can alter the structure of cell membrane resulting in intravascular haemolysis

They can alter the membrane of red blood cell making it antigenic - extravascular haemolysis

89
Q

What are the 2 different types of non-autoimmune acquired haemolysis?

A

Paroxysmal nocturnal haemoglobinuria

Mechanical haemolytic anaemia

90
Q

what is Paroxysmal nocturnal haemoglobinuria?

A

Dark urine at night and in the morning on waking

Deficiency in GPI anchor (CD59, CD55) which teethers proteins to the cell membrane of RBC so the RBC are more sensitive to haemolytic action of complement

intravascular haemolysis, venous thrombosis and bone marrow aplasia

91
Q

Treatment of Paroxysmal nocturnal haemoglobinuria?

A

Blood transfusion

Eculizumab - binds to complement preventing haemolysis

Bone marrow transplant

92
Q

What is mechanical haemolytic anaemia?

A

RBC injured through physical trauma in circulation e.g.

Mechanical valves

prolonged marching (RBC damaged in the feet)

Microangiopathic haemolysis - fragmentation of RBC in abnormal microcirculation causing malignant hypertension

93
Q

What is a major complication of DVT?

A

Pulmonary embolism

94
Q

Investigations of DVT?

A

Wells score >2 - ask questions of factors that make you more prone to DVT

D-dimer - degradation product of fibrin - negative score is more helpful than a positive because positive can be caused by a range of different things

Thrombophilia

Underlying malignancy

Hommans test - tilt the leg

95
Q

Describe virchow’s triad and explain the pathophysiology of thrombus development?

A

Hypercoagulability

Stasis of blood flow

Endothelial injury

96
Q

Risk factors for DVT?

A

Pregnancy - obstruction

Increased age

Hypertension

Surgery

Synthetic oestrogen

Obesity

Immobile

97
Q

Signs of DVT?

A

warm and tender calf

Asymmetrical calf

Pitting oedema

98
Q

Difference between signs/symptoms of arterial thrombosis and venous thrombosis?

A

Arterial

signs - decreased pulse, pale, cold finders and hands, dizziness

Symptoms - pins and needles, cold, numb, muscle pain in the affected area

Venous

Signs - swollen, red, warm to touch, tender

Symptoms - calf pain, tender

99
Q

6 P’s of limb iscahemia?

A

Pain

Pulseless

Paraesthesia

Paralysis

Pallor

Perishingly cold

100
Q

MOA warfarin?

A

Inhibits Vit K reductase (oxidised to reduced) so gamma carboxyglutamic acid component of 10 9 7 2 isn’t made so inhibiting the cascade

(glutamic acid, O2 and CO2)

101
Q

Heparin MOA?

A

Binds to the enzyme inhibitor antithrombin 3 causing antithrombin to be activated

Inhibits thrombin and factor 10

102
Q

What is used to monitor the effect of warfarin?

A

INR - international normalised ratio - a measure of how long it takes for a blood clot to form (measure extrinsic pathway)

APTT - Activated partial thromboplastin time - Measure of the intrinsic pathway

103
Q

Oral anticoagulation that can be used for DVT?

A

Warfarin

Heparin

Fondaparinux, apixaban

104
Q

What is the difference between lymphoblastic leukeaemia and myeloid leukaemia?

A

Lymphoblastic - malignancy of lymphoid tissue affecting B and T lymphocytes

Myeloid -proliferation of cells derived from the myeloblast e.g. eosinophil, basophil, neutrophil and monocytes

105
Q

What is the commonest cancer in childhood?

A

Acute lymphoblastic leukaemia - malignancy of lymphoid tissue affecting B and T lymphocytes by arresting matruation =

uncontrolled proliferation of immature cells

Marrow failure

Tissue infiltration

106
Q

Signs of tissue infiltration (Acute lymphoblastic leukaemia)?

A

Lymphadenopathy

Hepatospenomegaly

Orchidomegaly (testes)

107
Q

What can be used to classify acute lymphoblastic leukaemia?

A

Morphological

Immunological

Cytogenic

108
Q

Signs of marrow failure?

A

Anaemia

Infections

Bleeding

109
Q

Investigations sued to diagnose acute lymphoblatic leukaemia?

A

BC + blood film (decreased WCC)

CXR + CT - lymphadenopathy

Lumber puncture - CNS involvement

110
Q

Management of acute lymphoblatic leukaemia?

A

Blood transfusion

Fluids

PICC line (Hickmans line)

Allopurinal - to prevent tumour lysis syndrome

Chemo

Marrow transplant

Thiopurine - immunosupressant

111
Q

What is haemtological remission?

A

No evidence of leukaemia in blood and normal/recovering blood count and <5% blasts in a normal regenerting marrow

112
Q

What is acute myeloid leukaemia?

A

Neoplastic proliferation of blast cells derived from marrow myeloid cells - immune cells eosinophils, neutrophils, basophills and monocytes

113
Q

What are myelodysplatic syndromes?

A

Disorders that manifest as a result of marrow failure with life threatening risk of infection and bleeding

114
Q

Complications of acute myeloid leukaemia?

A

Infection

Septicaemia

Fever

Increased plasma urate

Leukostasis - elevated blast cell count

115
Q

Signs and symtpoms of acute yeloid leukaemia?

A

Marrow failure

Infiltration

116
Q

Diagnosis of acute myeloid leukaemia?

A

Decreased WCC

Decreased blast cells peripherally

Aur rods - red staining needle like rods found in a bone marrow biopsy due to abnormal fusion of granules (cytoplasma of myeloblasts)

Peripheral blood film showing monoblasts

117
Q

What is chronic myeloid leukaemia?

A

Uncontrolled cloncal proliferation of myeloid cells (they are all the same type of cell whereas acute is all the cells derived from the myeloblast)

118
Q

What is present in >80% of patients with chronic myeloid leukaemia?

A

Philadelphia chromosome

Mutation of chromosome 22 - affecting activity of tyrosine kinase

119
Q

Symptoms of chronic myeloid leukaemia?

A

Decreased weight

Tired + fever + sweating

Abdo pain

Gout

120
Q

Management of chronic myeloid leukaemia?

A

Imatinib - inhibits tyrosine kinase

Hydroxycarbamide

Allogeneic stem cell transplantation

121
Q

Signs of chronic myeloid leukaemia?

A

Spleenomegaly

Hepatosplenomegaly

Anaemia

Bruising

122
Q

Investigations to confirm chronic myeloid lukaemia?

A

Increased WBC

Decreased Hb

Increased Urate

Bone marrow hypercellular

Philadelphia gene - (cytogenetics)

Numerous granulocytic cells at different stages of differentiation

123
Q

What is the hallmark of chronic lymphocytic leukaemia?

A

Progressive accumulation of a malignant clone of functionally incompetant B cells

124
Q

Signs of chronic lymphocyti leukaemia?

A

Enlarged rubbery non-tender noders

Splenomealy

Hepatomegaly

125
Q

Symptoms of chronic lymphocytic leukaemia?

A

usually found on routine bloods

Anaemia, prone to infection, sweats and anorexia

126
Q

Complications of chronic lymphocytic leukaemia?

A

Autoimmune haemolysis

Infection

Marrow failure

127
Q

Treatment of chronic lymphocytic leukameia?

A

Fludarabine + Rituximab + Cyclophosphamide - anticancer drugs

Steroids

Radiotheraphy

Stem cell transplant

Blood transfusion

128
Q

What is the difference between leukamia and lymphoma?

A

Leukaemia - affects blood cells and bone marrow

Lymphoma - affects lymph nodes as solid tumour proliferations of lymphocytes accumulate in lymph nodes and cause lymphadenopathy

129
Q

Hallmark of hodgkins lymphoma?

A

Reed-sternberg cells - abnormal lymphocytes that have more than 1 nucleaus

130
Q

Lymph nodes affected in hodgkins lymphoma?

A

Cervial - supraclavicular - mediastinal

Through contagious spread from LN to LN

131
Q

Risk factors for hodgkins lymphoma?

A

EBV

Benzene

Hisotry of infectious mononucleosis

An affected sibling

Post transplant

132
Q

Symptoms of Hodgkins lymphoma?

A

Enalrged non-tender rubbery lymph nodes

Fever, weight loss, night swetas

Alcohol indiced LN pain

If large enough, the mass can obstructt the SVC

133
Q

Signs of hodgkins lymphoma?

A

Localised and painless lymphadenopathy

Spleno or hepatomegaly

Cachexia - wasting of the body due to severe chronic illness

Anaemia

134
Q

Investigations done to confirm hodkins lymphoma?

A

Tissue diagnosis - Core needle biopsy of lymph node

Bloods - FBC, Film, LFT,Hb, Urate - RS cells, LDH

Imaging - CXR, PET/CT

135
Q

What is LDH?

A

Lactate dehydrogenase enxyme is used to convert sugars into energy for cells to use - increased needed for the proliferation of lymphocytes

136
Q

How does EBV cause HL?

A

By activating nuclear kappa light chain which is a transcription factor - genes that promote proliferation

137
Q

What is staging process is used to stage hodgkins lymphoma?

A

An Arbor staging

1 - confined to single lymph node

2 - 2 or more lymph nodes at the same side of the diaphragm

3 - involvement of both sides of the diaphragm

4- Extralymphatic tissue involement - liver and bone marrow

138
Q

Differnce between type A and type B hodgkins lymphoma?

A

A - no symptoms other than itching

B - Unexplained fever, weight loss >10% and drentching sweat and extranodal extention - breaking the confines of the capsule and affecting surrounding tissue

139
Q

Management of hodgkins lymphoma?

A

1, 2, A - radiotherapy and short course chemo

3, 4, B - radiotherapy and long course Chemo

ABVD

140
Q

ABVD?

A

Adriomycin

Bleomycin

Vinblastine

Dacarbazine

141
Q

What is brentuximab?

A

Monoclonal antibody against CD30 used alongside chemo and stem cell transplant for relapse of hodgkins lymphoma

142
Q

Sideeffects of radio and chemo therapy?

A

IHD, NHL, Hypothyroidism, Lund fibrosis

MYelosupression, alopecia, nausea , infertility, NHL

143
Q

Index used to deteramin the prognosis of hidkins lymphoma?

A

Hasenclaver index

144
Q

Non-hodgkins lymphomas?

A

Any tumours of lymph nodes without reed sterberg cells - mostly derived from B cells

145
Q

Types of aggresive NHL?

A

Diffuse large B bell lymphoma

Burkitt’s - CT 9/14 - MYC genes overexpressed - increased cell growth and metabolism

Mantle cell - CT 14/11 - BCL1 overexpression activating cyclin D! - cell growth

146
Q

Difference of extranodal involvement of burkitt’s lymphoma between african and non-african countries?

A

Africa - Jaw and EBV association

Non-african - Abdomen and no EBV association

147
Q

Types of indolent NHL?

A

Follicular - overexpression of BCLR which normally prevents cell death

Marginal zone - Mucosa associated lymphoid tissue - lining of the stomach of those with chronic inflammation

Lyphoplasmacytic - BM, LN,Spleen - neoplastic cells produce immunoglobuin M proteins causing the blood to become thick (waldenstroms macroglobulinaemia)

148
Q

What are the 2 different types of T cell lymphomas?

A

Adult T cell lymphoma - incorparates it’s DNA into T cell DNA

Mycosis fungoides (cutaneous T cell lymphoma) - Sezary sydtome which is a genrealised red rash and blood film looks cerebriform - nucleaus looks like a brian

149
Q

Signs and symptoms of NHL?

A

Lymphadenopathy (painless)

Fever, sweating and weightloss

Extranodal involvement -

GI - bowel obstruction

BM - fatigue, anaemia, bruising and infections

SC - spinal chord compression - loss of sensation in the legs

150
Q

Diagnosis of NHL?

A

CT

LN core needle biopsy

151
Q

Management of NHL?

A

Chemo and radio

Rituximab - kills CD20 +ve cells by antibody directed cytotoxicity and apoptosis

Rituximab, Cyclophosphamide, Hydroxydaunorubicin (adriomycin), Vincristine, Prednisolone

152
Q

Myeloma?

A

Abnormal proliferation of plasma cells resulting in hypersecretiond of Ig and Ig fragments leading to the destruction of ograns

Mostly IgG - causes a decrease in other immunoglobulins

153
Q

Diagnosis of Myeloma?

A

Urine contains Bence jones protein (Ig light chains of kappa or lambda types filtered throguh the kidneys)

154
Q

Blood film appearance of myeloma?

A

IL6 and CD138

Ms plasma cell

Do you want to see my perinuclear halo?

I’ve lots of RER to show

I have vacules with Ig and my cytoplasm is dark blue

We can do rouleaux before my alarm goes off showing my clockface chromatin

Ms PC occupies 10% of the room and knows most men

155
Q

Clinical features of myeloma?

A

I-CRAB

Infections - marrow infiltration

Calcium elevation - increased osteoclast activity from signalling myeloma cells

Renal insufficency - bence jones proteins have a toxic and inflammatory effect on the kidneys

Normocytic nochromatic Anaemia

Bone lesions

156
Q

Investigations done to confirm myeloma?

A

BLOODS - rouleaux, normocytic anaemia, increased calcium

BONE MARROW BIOPSY - >10% cell infiltration

SERUM AND URINE ELECTROPHORESIS

XRAY - bone lesions e.g. pepper pots skull

157
Q

Management of myeloma?

A

Analgesia

Biphosphonates - inhibit osteoclastic activity, verterbral fractures and pain

Surgery to fix vertebral collapse

local radiotherapy

Transfusions + erythropoisesis

Rehydration

Broad spec antibiotics or regular IV immunoglobulins

158
Q
A
159
Q

What is paraproteinaemia?

A

The presence of circulating immunoglobulins produced by a single clone of plasma cells

Monoclonal M bands on serum electrophoresis

160
Q

Types of paraproteinaemias?

A

Multiple myeloma

Waldenstroms macroglobulinaemia - lymphoplasmacytic NHL which produces IgM paraproteins

Primary amyloidosis

Monoclonal gammaopathy of uncertain significance - not causes by a known condition

Paraproteinaemia in lymphoma or leukaemia

Heavy chain disease- neoplastic cells produce free Ig heavy chains - alpha chains can infilttrate the bowel wall causing malabsorption

161
Q

Amyloidosis?

A

Extracellular deposits of amyloid proteins which are resistant to degredation leading to organ failure - T2DM, altzheimers

162
Q

Differnce between primary and secondary amyloidosis?

A

Primary - proliferation of plasma cells with amyloidic genetic monoclonal immunoglobulins and has many systemic effects (macroglossia)

Secondary -derived from serum amyloid A (acute phas eproteins) reflecting chronic inflammation in rheumatioid arthiritis

163
Q

Familial amyloidosis?

A

Mutations of transport proteins produced by the liver (transthyretin) c neuropathy

Found in serum and CNF and transports thyroxine and retinal binding protein bound to retinal

164
Q

What are myeloproliferative disorders?

A

Disorders caused by clonal proliferation of haematopoietic myeloid stem cells in the bone marrow

165
Q

List the blood cells and associated conditions?

A

RBC - polycythemia

WBC - Chronic myeloid leukaemia

Platelets - essential thrombocythemia

Fibroblasts - myelofibrosis

166
Q

What is the difference between reactive and absolute polycythemia?

A

Reactive - Caused by decreased plasma volume making it look like the RBC mass has increased - dehydration, burns, stress, hypovolaemia

Absolute - (primary vera) increase in RBC mass due to spontaneous proliferation of 1 pluripotent stem cell in bone marrow - they proliferate without even needing EPO

(Secondary) due to hypoxia (high altitude, smoking) or inappropriate EPO secretion from a renal or hepatocellular carcinoma

167
Q

Clinical features of polycythemia?

A

Can be asymptomatic and found on routine bloods

HYPERVISCOCITY - dizziness, headache, tinnitus and visual disturbance

Itching after a hot bath

Erythromelalgia - blocked perfusion to hands causing vascular peripheral pain and inflammation

Splenomegaly

Gout

arterial or venous thrombosis

168
Q

Investigations used to diagnose polycythemia?

A

FBC

Hypercellular bone marrow

Increased RC mass

Erythropoietin levels

Splenomegaly

169
Q

Management of polycythemia?

A

if they are >60 + previous thrombus - hydroxycarbamide (increases fetal haemoglobin and decreases RBC count and platelets)

Alpha interferon - inhibits the growth of abnormal clones of stem cells

Aspirin

170
Q

Essential thrombocythemia?

A

Clonal proliferation of megakaryocytes resulting in increased platelets resulting in microvascular occlusions and AV thrombosis

171
Q

Symptoms of thrombocythemia?

A

Headache, atypical chest pain, light-headed, erythromelalgia

172
Q

Management of essential thrombocythemia?

A

Aspirin

Hydroxycarbamide

173
Q

Myelofibrosis?

A

Extensive scarring of bone marrow. So much scarring meaning that cells are not able to develop properly

Hyperplasia of megakaryocytes which produce platelet-derived growth factor leads to intense fibrosis of the bone marrow

174
Q

Blood film of a patient with myelofibrosis?

A

Tear drop RBC

Decreased Hb

Nucleated RBC

175
Q

Management of myelofibrosis?

A

Allogeneic stem cell transplant

176
Q

MOA Heparin?

A

Activates Antithrombin 3

Inhibits 10a and thrombin

177
Q

MOA tPa or streptokinase?

A

activate plasminogen to become plasmin

178
Q

MOA warfarin?

A

Vitamin K reductase inhibitor so the vitamin K dependant clotting factors (1972) don’t have their gamma carboxyglutamic acid component and so they don’t function

179
Q

3 main factors that stop bleeding?

A

Vasoconstriction

Platelet plug

Coagulation cascade

180
Q

Signs of vascular and platelet disorders?

A

Prolonged bleeding from cuts

Bleeding into the skin (bruising and purpura)

Bleeding from mucous membranes (epistaxis and bleeding gums)

181
Q

What are some vascular defects that can result in bleeding disorders?

A

poor vascular integrity means bleeding into tissue

Ehlers-danlos - low collagen

Scurvy - low vit C means low capillary integrity

Steroids

Osler-weber- rendu - abnormal blood vessel formation in skin, mucous membranes, the gut and even organs

182
Q

What is the difference between Haemophilia A and haemophilia B?

A

A - factor 8 deficiency and B is a factor 9 deficiency

183
Q

Signs of thrombophilia?

A

Arthropathy - bleeding in the joint

Haematoma - bleeding outside of blood vessels

increases PT time and decreased factor 8

184
Q

Why should people with haemophilia avoid IM injections and NSAIDS?

A

They slow down blood clotting time and increase the risk of gut bleeding - the decreased activity of platelets

185
Q

Management of Haemophilia A?

A

Minor - desmopressin - increases the release of clotting factor 8

Major - recombinant factor 8 to 50% the normal level

186
Q

Management of Haemophilia B?

A

Recombinant factor 9

187
Q

Acquired haemophilia?

A

unusual susceptibility to bleed mostly due to hypercoagulability

Slow or irregular blood clotting caused by a defect in the system of coagulation

Autoantibodies against factor 8

188
Q

Other causes of bleeding disorders?

A

Liver disease

Malabsorption

Anticoagulant medication

189
Q
A

Phase 2a (7 decks)

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