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Haematology Flashcards

1
Q

Abnormal FBC for neutrophils?

A

High = neutrophilia, caused by acute bacterial infection
Low = neutropenia, caused by myeloma and lymphoma

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2
Q

Abnormal FBC for lymphocytes

A

High = lymphocytosis, caused by chronic infection
Low = lymphocytopenia

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3
Q

Abnormal FBC for platelets

A

High = thrombocytosis/thrombocythaemia
Low = thrombocytopenia

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4
Q

FBC values for eosinophils and monocytes

A
  • Eosinophils - elevated in parasitic infection
  • Monocytes - elevated in myelodysplastic syndrome
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5
Q

Splenectomy
What does spleen do so what are risks of splecetomy?
what age to wait after till?

A

-To remove spleen
- Wait till after 6 y/o
- Spleen plays defensive role vs encapsulated bacteria
- So wait due to risk of sepsis

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6
Q

What are haematinimics?

A
  • Nutrients needed for haematopoesis (B12, folate, Fe)
  • Deficiencies cause angular stomatitis and glossitis
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7
Q

What is angular stomatitis and glossitis?

A

-It affects one or both corners of your mouth and causes irritated, cracked sores. Although painful, angular cheilitis usually isn’t serious.
- Inflammation of the tongue

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8
Q

Blast vs cyte

A

Blast - immature cell, eg: myeloblast
Cyte - mature cell, eg: myelocyte

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9
Q

Thrombosis chart?

A

Thrombus = arterial or venous

Arterial (atherogenesis inside arteries) can lead to ischaemia in organs/ tissues
—–>cvd —>MI
—–>cerebrovascular—> I. stroke + TIA
—->Peripheral = pvd

Venous (Virchov’s triad) leading to occulded venous drainage
—–>DVT
—–> PE from DVT thrombus emboli

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10
Q

Types of haemostasis

A

Primary: Initiation and formation of platelet plug - platelet activation
Secondary: Formation of the fibrin clot - coagulation cascade

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11
Q

PT/INR

A

pt = prothrombin time

  • Work out INR using equation: Patient PT/Reference PT
  • Normal INR: 0.8 - 1.2

INR could be high due to:
- Anticoagulants (eg: warfarin usually INR of 2-3)
- Liver disease
- Vit K deficiency
- DIC (Disseminated intravascular coagulation)

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12
Q

What is prothrombin time? (PT)

A
  • Coagulation speed through extrinsic pathway
  • Normally 10-13.5s
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13
Q

What is activated partial thromboplastin time? (APTT)

A
  • Coagulation speed through intrinsic pathway
  • Usually 35-45s
  • Patient on heparin may be 60-80
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14
Q

Other values measured for coagulation

A
  • Bleeding time
  • Thrombin time
  • Fibrinogen -> fibrin time (usually 12-14s)
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15
Q

What are myelodysplastic syndromes?

A
  • Bone marrow cells fail to make adequate numbers of healthy blood cells
  • Abnormal cells crowd out healthy normal cells
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16
Q

Diagnostic pathway for thrombocytosis?

A

if the platelet count is >450x10^9
order a blood film, iron status, acute phase reactants(CRP, ESR)

Iron deficincy = treat and then repeat blood count

acute phase response = reactive thrombocytosis

Normal = repeat blood count
if persistent unexplained thrombocytosis then investigate by bone marrow aspiration and biopsy, cytogenetics, molecular genetics( JAK2 etc)

Then diagnose

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17
Q

Most common causes of splenomegaly

A
  • Infection
  • Liver disease
  • Autoimmune conditions (eg: SLE, rheumatoid arthritis)

Once excluded these: myeloproliferative neoplasms and lymphomas

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18
Q

What is budd chiari?

A
  • Thrombus in hepatic artery
  • Increasing pressure
  • Leading to enlarged liver and spleen
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19
Q

Reasons for decrease in RBCs

A

Decreased production
- Iron, folate or B12 deficiency
- Bone marrow failure

Increased loss
- Bleeding
- Haemolysis

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20
Q

Symptoms of post thrombotic syndrome

A
  • Skin hyperpigmentation
  • Venous ulcers due to poor circulation in the leg
  • Leg swelling
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21
Q

What to do if someone is at high risk for DVT

A

Ultrasound scan and give anticoags while waiting for results

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22
Q

HB structures

A

95% - HbA
- 2x alpha
- 2x beta

5% - HbA2
- 2x alpha
- 2x delta

Fetal - Fetal
- 2x alpha
- 2x gamma

Sickle cell
- 2x alpha
- 2x HbS

HbH ( alpha thalassemia in which moderately severe anemia develops due to reduced formation of alpha globin chains.)
- 4 x beta

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23
Q

What to give in suspected neutropaenic sepsis and what is it?

A

(can happen when you have a low level of neutrophils and an infection at the same time.)

  • One of the piperacillin (broad spec antibiotics) with tazobactam
  • Do blood cultures
  • IV fluids and oxygen
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24
Q

Affects of warfarin and heparin on APTT and PT

A
  • Heparin increases APTT
  • Warfarin increases PT
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25
Q

What does ferritin increase in?

A

Infection - as it is an acute phase reactant

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26
Q

What is aplastic anaemia?

A

Pancytopaenia (deficiency of all three cellular components of the blood) with hypocellular bone marrow

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27
Q

signs of essential thrombrocytosis /thrombocythemia?

A
  • Splenomegaly
  • Erythromelalgia (red or blue discolouration in peripheries with burning sensation)
  • Livedoreticularis - a skin condition caused by small blood clots that develop inside the blood vessels of the skin
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28
Q

Diagnosis of thrombocytosis/Essential thrombocythaemia

A
  • Ferritin 221, Serum iron 15, % iron saturation 23% normal to exclude anaemia
  • Blood film: Platelet anisocytosis (Platelets with size variation)
  • BCR::ABL1 no rearrangement on FISH- means unlikely to be leukaemia
  • JAK2V617F mutated- mutated in myleproliferative diseases
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29
Q

Causes of macrocytosis
(ddrrhab)

A
  • Folate or B12 deficiency
  • Reticulocytosis
  • Raised Igs
  • Hypothyroidism
  • Alcohol
  • Bone marrow failure, esp. myelodysplastic syndromes
  • Drugs, eg: methotrexate (inhibits folate metabolism), hydroxyurea
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30
Q

What percentage of patients with DVT get post-thrombotic syndrome?

A

30%

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31
Q

MCV for different types of anaemia

A

<80 = microcytic
80-95 = normocytic
>95 = macrocytic

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32
Q

When would you consider transfusions for anaemia?

A

Hb <70g/L
or
Hb <80g/L + cardiac comorbidity

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33
Q

General symptoms of anaemia

A
  • Fatigue
  • Headache
  • Dizziness
  • Dyspnoea (especially on exertion)- difficult or laboured breathing.
  • Chest pain
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34
Q

General signs of anaemia

A
  • Tachycardia + hypotension
  • Skin pallor
  • Conjunctiva pallor (reds of eye pale)
  • Intermittent claudication- muscle pain due to lack of oxygen that’s triggered by activity and relieved by rest
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35
Q

What does full blood count check for

A
  • RBCs
  • Neutrophils
  • Lymphocytes -
  • Platelets
  • Eosinophils
  • Monocytes
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35
Q

What are megaloblasts?

A
  • Hypersegmented nucleated neutrophils with 6+ lobes
  • Less mature DNA = less compacted around histones
  • So more lobes = more immature
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35
Q

Signs specific to B12 deficiency anaemia

A
  • Angular stomatitis + glossitis
  • Lemon-yellow skin
  • Neurological symptoms
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36
Q

Ideal haemoglobin (g/L) and mean corpuscular volume values (femtolitres)

A

Haemoglobin:
- Women: 120-165g/L
- Men: 130-180g/L
anything less than the lower value is classed as anaemia
MCV:
- 80-100 femtolitres

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36
Q

How long do folate and B12 deficiency anaemia take to develop?

A
  • Folate: months
  • B12: years (more common in older patients)
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37
Q

Causes of B12 deficiency anaemia

A
  • Pernicious anaemia (autoimmune, most common)
  • Gastrectomy
  • Malnutrition
  • Intestinal problems such as Crohn’s and celiac disease
  • Chronic nitrous oxide use
  • Oral contraceptives
  • Vegan (cool people basically😎)
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38
Q

Normal metabolism of B12

A
  • B12 binds to transcobalamin 1 in saliva (protects against stomach acid)
  • Bind to intrinsic factor in duodenum
  • Absorbed as B12-IF complex in terminal ileum
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38
Q

Pathophysiology of pernicious anaemia

A
  • Anti parietal and intrinsic factor antibodies = low IF
  • Low B12-IF complexes
  • Less B12 absorption
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39
Q

Neurological symptoms of B12 deficiency

A
  • Demyelination (DDx for folate deficiency)
  • Symmetrical parathesia- abnormal sensation, typically tingling or pricking caused chiefly by pressure on or damage to peripheral nerves)
  • Muscle weakness
  • Altered mental state
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39
Q

Diagnosis of B12 deficiency

A
  • FBC + blood film (Macrocytic and megaloblasts present)
  • Low serum B12
  • Anti parietal and anti IF antibodies (specific in pernicious anaemia)
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39
Q

Treatment for B12 deficiency

A
  • Dietary advice
  • B12 supplements (PO hydroxycobalamin)
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39
Q

Aetiology of folate deficiency anaemia

A
  • Malnutrition
  • Malabsorption
  • Pregnancy
  • Trimethoprim + methotrexate (dihydrofolate reductase inhibitors)
  • Alcohol
  • Bacterial overgrowth
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39
Q

Symptom of folate deficiency anaemia

A

Angular stomatitis

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39
Q

Diagnosis of folate deficiency anaemia

A
  • FBC + blood film = macrocytic + megaloblasts
  • Low serum folate
  • Could have concomitant B12 deficiency
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39
Q

Treatment for folate deficiency

A
  • Dietary advice (leafy greans, brown rice)
  • Don’t replace folate without checking B12
  • Folate supplements (if concomitant with B12 def., replace B12 first as giving folate first depleted B12)
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39
Q

Treatment for folate deficiency during pregnancy

A
  • Prophylactic folate 400mg for first 12 weeks
  • Ensure baby develops ok
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39
Q

Main complication of folate deficiency

A

Chronic Kidney Disease
CKD

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39
Q

How much folate is needed daily?

A

0.1-0.2mg/day is required as there are minimal body stores- only lasts a few months

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39
Q

Where is folate absorbed?

A

In the proximal jejunum

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39
Q

Causes of non-megaloblastic anaemia

A
  • Alcohol - toxic to RBC and depletes folate and B1
  • Hypothyroidism - inteference w/ EPO, multifactorial
  • Liver disease - liver decompensated cirrhosis
  • NAFLD (Non-alcoholic fatty liver disease)
  • CKD
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40
Q

Types of microcytic anaemia

A

Thalassaemia
Anaemia of chronic disease
Iron deficiency
Lead poisoning
Sideroblastic

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41
Q

Signs specific to iron deficiency anaemia

A
  • Koilonchia (spoon nails)
  • Angular stomatitis
  • Atrophic glossitis
  • Brittle hair and nails
  • Subconjunctival pallor
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42
Q

Iron deficiency anaemia

A
  • Non-inherited Fe deficiency, impairing Hb synthesis
  • Most common anaemia worldwide
  • More common in females
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43
Q

Aetiology of iron deficiency anaemia

A
  • Infants: malnutrition, prolonged breastfeeding
  • Children: malnutrition, malabsorption
  • Adults: malnutrition, malabsorption, menorrhagia, pregnancy, hookworm
  • Elderly (60+): rare, red flag for colon cancer bleeding
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44
Q

NICE recommendation for iron deficiency anaemia age 60+

A

Urgent endoscopy in case of GI malignancy

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45
Q

What causes malabsorption of iron?

A

Conditions that result in inflammation of the duodenum and jejunum
- Coeliac
- IBD
- Crohn’s disease
Medications that reduce stomach acid (eg: PPI)
- Because acid is needed to keep iron as the soluble Fe2+

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46
Q

Hookworm as a cause of iron deficiency anaemia

A
  • Most common cause worldwide
  • Results in GI blood loss
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47
Q

Normal iron function

A
  • Absorbed
  • Circulated bound to transferrin
  • Stored as ferritin or incorporated into Hb
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48
Q

Diagnosis of iron deficiency anaemia

A
  • FBC = microcytic
  • Blood film - pencil cells (smol) platelet aggregates
  • Iron studies
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49
Q

What would a blood film of iron deficiency anaemia show?

A
  • Small, hypochromic (pale) RBCs
  • Target cells - non-specific Bull’s eye pattern
  • Howell Jolly bodies - non-specific nucleated RBCs
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50
Q

Fe studies in iron deficiency anaemia

A
  • Serum Fe: Low
  • Ferritin: Low
  • Transferrin saturation: Low
  • Total iron binding capacity (and transferrin): High
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51
Q

Treatment for iron deficiency anaemia

A
  • Blood transfusion - immediate correction but need to treat underlying cause
  • Iron infusion
  • Oral iron
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52
Q

Iron infusion

A
  • eg: cosmofer
  • Small risk of anaphylaxis
  • CI during sepsis
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53
Q

Oral iron

A
  • Ferrous sulphate 200mg 3x a day
  • SE: constipation, black stools, GI upset
  • If poorly tolerated, consider ferrous gluconate
  • Unsuitable where malabsorption is the cause of the deficiency
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54
Q

What is sideroblastic aneamia?

A
  • Defective Hb synthesis within mitochondria
  • Often X inherited ALA synthetase deficiency
  • High Fe but not used in Hb synthesis, trapped in mitochondria!
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55
Q

Diagnosis of sideroblastic anaemia

A
  • FBC + blood film
  • Fe studies
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56
Q

Fe studies for sideroblastic anaemia

A
  • Serum Fe: High
  • Ferritin: High
  • Transferrin saturation: High
  • Total iron binding capacity (and transferrin): Low
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57
Q

FBC and blood film for sideroblastic anaemia

A
  • Microcytic
  • Ringed sideroblasts (immature RBC)
  • Basophilic stippling (increased basophilic granules)
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58
Q

Signs specific to thalassaemia

A

Bone deformities

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59
Q

What is thalassaemia?-what kinds of genetic haemoglobinopath?

A
  • Autosomal recessive haemoglobinopathy
  • A type of haemolytic anaemia
  • Defective alpha-globin chain = alpha thalassaemia
  • Defective beta-globin chain = beta thalassaemia
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60
Q

Pathophysiology of thalassaemia

A
  • RBCs are more fragile and break down more easily
  • Spleen collect all the destroyed RBCs, resulting in splenomegaly
  • Bone marrow expands to produce extra RBCs -> susceptibility to fractures, pronounced forehead and molar eminence
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61
Q

Where is thalassaemia prevalent?

A

Where malaria is as it is protective from it (like sickle cell)

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62
Q

Alpha thalassaemia

A
  • Less common
  • 4 gene deletions on chromosome 16
  • Associated with HbH
  • Can cause death in utero if severe
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63
Q

Beta thalassaemia

A
  • More common
  • 2 gene mutations in chromosome 2
  • Normal Hb isoforms, just depletion of beta chains
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64
Q

Presentation of thalassaemia

A
  • Failure to thrive
  • Hepatospenomegaly
  • Gallstones
  • Chipmunk face
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65
Q

Diagnosis of thalassaemia

A
  • FBC + blood film
  • Hb electrophoresis - diagnostic
  • Xray - “hair on end” skull
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66
Q

FBC and blood film in thalassaemia

A
  • Hypochromic (pale) RBCs
  • Target cells
  • Microcytic anaemia with high reticulocytes
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67
Q

Treatment for thalassaemia

A
  • Regular transfusion
  • Iron chelation to remove excess iron in body due to blood transfusions
  • Splenectomy
  • Ascorbic acid (vit C)
  • Bone marrow transplant (curative)
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68
Q

Iron chelation

A
  • Prevents Fe overload from transfusions
  • Desfemoxamine
  • SE: deafness, cataracts
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69
Q

Signs specific to haemolytic anaemia

A
  • Prehepatic jaundice
  • Dark urine
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70
Q

Types of haemolysis

A
  • Intravascular - marked by haptoglobin (attaches itself to certain hb so if low then anaemia)
  • Extravascular - @ spleen
  • Can be both
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71
Q

What is autoimmune haemolytic anaemia precipiated by

A

Precipitated by temperature (warm most common but idiopathic)

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72
Q

Pathophysiology of autoimmune haemolytic anaemia

A
  • IgM autoantibodies activate compliment system by binding to cell surface of RBCs
  • Intra/extravascular haemolysis
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73
Q

Special test for autoimmune haemolytic anaemia

A
  • Direct coombs +ve
  • An abnormal (positive) direct Coombs test means you have antibodies that act against your red blood cells.
  • Agglutination of RBCs with coombs reagent-the antibodies in the Coombs reagent bind to the antibodies attached to the erythrocytes, causing agglutination.
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74
Q

What conditions is autoimmune haemolytic anaemia secondary to?

A
  • Leukaemia
  • Lymphomas
  • SLE -Systemic lupus erythematosus - an autoimmune disease in which the immune system attacks its own tissues, causing widespread inflammation and tissue damage in the affected organs
  • Any sort of infections (eg: EBV)
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75
Q

What is G6PDH deficiency

A

(glucose-6-phosphate dehydrogenase). This enzyme helps red blood cells work properly. A lack of this enzyme can cause hemolytic anemia.
- X linked recessive enzymopathy
- Causes 1/2 lifespan + RBC degeneration

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76
Q

What is G6PDH?

A
  • Glucose-6-phosphate dehydrogenase
  • Protects RBCs from vasoxidative damage
  • Involved in glutathione synthesis (protects from ROS like H2O2)
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77
Q

Factors that can precipitate G6PDH deficiency

A
  • Naphthelene (in moth balls (pesticide))
  • Antimalarials, eg: quinine
  • Aspirin
  • Fava beans (contain glucosides that can be oxidised into ROS)
  • Nitrofurantoin- Nitrofurantoin is an antibacterial medication of the nitrofuran class used to treat urinary tract infections
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78
Q

G6PDH attack

A

Rapid anaemia + jaundice (intravascular haemolysis)

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79
Q

Diagnosis of G6PDH deficiency

A

FBC + blood film
- Normal inbetween attacks
- Attack: normocytic, normochromic, increased reticulocytes, heinz bodies and bite cells

Low G6PDH levels

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80
Q

Treatment for G6PDH deficiency

A
  • Avoid precipitants
  • Blood transfusions when attacks ensue
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81
Q

Signs of G6PDH deficiency

A
  • Neonatal or intermittent jaundice
  • Anaemia
  • Gallstones
  • Splenomegaly
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82
Q

Risk factors for G6PDH deficiency

A
  • X-linked recessive (men)
  • West African, Mediterranean, Asian
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83
Q

Symptoms of G6PDH deficiency

A
  • Fatigue
  • Palpitations
  • Dyspnoea
  • Pallor
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84
Q

What is hereditary spherocytosis?

A
  • Autosomal dominant membranopathy
  • Instead of being shaped like a disk, the cells are round like a sphere. These red blood cells (called spherocytes) are more fragile than disk-shaped RBCs
  • Common in Northern Europe and America
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85
Q

Pathophysiology of hereditary spherocytosis

A
  • Deficiency in structural membrane protein spectrin
  • Increased splenic recycling (extravascular haemolysis)
  • Makes RBCs more spherical and rigid
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86
Q

Presentation of hereditary spherocytosis

A
  • General anaemia
  • Neonatal jaundice
  • Splenomegaly
  • Gall stones (50%)
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87
Q

Treatment for hereditory spherocytosis

A

Splenectomy

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88
Q

Treatment of neonatal jaundice in hereditory spherocytosis

A
  • Treated with phototherapy
  • Risk of kernicterus if untreated (bilirubin accumulates in basal ganglia, CNS dysfunction, death)
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89
Q

Diagnosis of hereditary spherocytosis

A
  • FBC and blood film: Normocytic, normochromic, spherocytes, increased reticulocytes
  • Direct coombs -ve
  • Eosin-5-maleimide (EMA) test
  • Cryohaemolysis
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90
Q

Types of malaria

A
  • Plasmodium falciparum (most severe and dangerous, 75% in the UK)
  • Plasmodium vivax
  • Plasmodium ovale
  • Plasmodium malariae
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91
Q

Pathophysiology of malaria

A
  • Spread by female anopheles mosquitos
  • When mosquito bites human, sporozoites are injected
  • Travel to liver
  • Mature into merozoites which enter blood and infect RBCs
  • Merozoites replicate and RBCs rupture after 48 hours, cause a systemic infection
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92
Q

In what forms of malaria can sporozoites lie dormant in the liver for years?

A
  • P.vivax and P.ovale
  • Lie dormant as hypnozoites
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93
Q

Presentation of malaria

A
  • Blackwater fever (malarial haemoglobinuria)-a severe form of malaria in which blood cells are rapidly destroyed, resulting in dark urine.
  • Massive hepatosplenomegaly
  • Pallor
  • Jaundice
  • Myalgia (muscle ache and pain)
  • Headache
  • Vomiting
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94
Q

Diagnosis of malaria

A
  • Malaria blood film
  • 3 samples over 3 consecutive days
95
Q

Oral treatments for malaria

A
  • Quinine sulphate
  • Doxycycline
96
Q

IV treatment for malaria

A
  • Artesunate (most effective but not licensed)
  • Quinine dihydrochloride
97
Q

Complications of P.Falciparum

A

Cerebral malaria
Reduced GCS
AKI
Seizures
Haemolytic anaemia (severe)

DIC
Oedema
Multi-organ failure and death

98
Q

Antimalarials

A
  • 90% effective at preventing infections
    Options:
  • Proguanil and atovaquone (malarone)
  • Mefloquine
  • Doxycycline
99
Q

Proguanil nad atovaquone (malarone) antimalarial

A
  • Most expensive
  • Best side effect profile
100
Q

Mefloquinne antimalarial side effects

A
  • Bad dreams
  • Rarely psychotic disorders or seizures
101
Q

Doxycycline antimalarial side effects

A
  • Diarrhoea and thrush (as it’s a broad spectrum antibiotic)
  • Makes patients sensitive to the sun, causing a rash and sunburn
102
Q

Malaria in children

A
  • Convulsions (60-80%)
  • High ICP, hypoglycaemia
  • Rule out meningitis (examination and CSF analysis if no high ICP)
103
Q

What is sickle cell anaemia?

A
  • Autosomal recessive haemoglobinopathy affecting beta-globin chains
  • Commonest in africa for antimalarial properties (vs plasmoduium falciparum)
  • HbS variant
104
Q

Pathophysiology of sickle cell anaemia

A
  • Glutamic acid -> valine on 6th codon of beta-globin on chromosome 11
  • Causes irreversible RBC sickling
  • RBC more fragile so less efficient
  • Bone marrow focuses more on reticulocytes, decreasing other cell lines (eg: causes neutropaenia)
  • Intra+extravascular haemolysis
105
Q

Presentation of sickle cell anaemia

A

General anaemia symptoms + prehepatic jaundice

106
Q

What are sickle cell complications (crises) precipitated by?

A
  • Cold
  • Hypoxia
  • Acidosis
  • Dehydration
  • Exertion
  • Stress
107
Q

Types of sickle cell crises

A
  • Vaso-occlusive (aka painful crisis)
  • Splenic sequestration
  • Acute chest syndrome
  • Aplastic crisis (from parvovirus)
  • Osteomyelitis
108
Q

Another common complication of sickle cell anaemia

A
  • Osteomyelitis - an inflammation or swelling of bone tissue that is usually the result of an infection
  • Usually due to s.aureus, but in those patients = salmonella
109
Q

Diagnosis of sickle cell anaemia

A
  • Sickle solubility test
  • Newborn heel prick test
  • Antenatal: Molecular genetics
  • Hb electrophoresis - diagnostic when above 90% HbS (number 4 has sickle cell)
110
Q

FBC and blood film in sickle cell anaemia

A
  • Normocytic normochromic
  • Increased reticulocytes
  • Sickled RBC
  • Howell Jolly bodies
111
Q

Last resort treatment for sickle cell anaemia

A

Bone marrow transplant

112
Q

Long term treatment for sickle cell anaemia

A
  • Avoid precipitants
  • Drugs - hydroxycarbamide (aka hydroxyurea) to increase HbF levels, folic acid supplements
  • Transfusion + Fe chelation
113
Q

Vaso-occlusive crisis

A
  • Sickel shaped RBCs clog capillaries, causing distal ischaemia
  • Dehydration and raised haematocrit
  • Can cause priapism in men (treated with aspiration of blood from the penis)
114
Q

Treatment for acute complicated attacks

A
  • Low threshold for hospital admission
  • Treat any infection
  • Keep warm
  • IV fluids
  • Simple analgeisa
115
Q

Splenic sequestration crisis

A
  • RBCs block blood flow within the spleen - can cause autosplenectomy
  • Splenomegaly
  • Can lead to severe anaemia and circulatory collapse (hypovolaemic shock)
  • Splenectomy used in cases of recurrent crisis
116
Q

Aplastic crisis

A
  • Temporary loss of the creation of new RBCs
  • Most commonly triggered by infection with parvovirus B19
  • Management is supportive with blood transfusions
  • Usually resolves spontaneously within a week
117
Q

Acute chest syndrome sickle cell crisis

A
  • Caused by pulmonary vessel vaso-occlusion
  • Fever or resp symptoms with new infiltrates seen on Xr
  • Can be due to infection or non-infective causes
  • Medical emergency with high mortality
118
Q

Treatment for acute chest syndrome

A

Exchange blood transfusion - sickle cell blood replaced w healthy blood

119
Q

Chronic complications of sickle cell anaemia

A
  • Avasular necrosis of joints
  • Silent CNS infarcts
  • Nephropathy
  • ED
120
Q

What to give patients with sickle cell painful crisis?

A
  • IV fluids
  • Analagaesia - NSAIDs
  • Oxygen if low
  • Broad spectrum antibiotics due to neutropaenia
121
Q

Haemolytic vs non-haemolytic anaemia

A

Failing bone marrow in non-haemolytic

122
Q

Myelophthisic process in non-haemolytic anaemia

A

Bone marrow infiltrated with something else (eg: tumour cells)

123
Q

Pathophysiology of aplastic anaemia

A

Pancytopenia where bone marrow fails and stops making haematopoetic stem cells

124
Q

Cause of aplastic anaemia

A
  • Idiopathic
  • Could be infection (EBV, parovirus B19) or congenital
125
Q

Diagnosis of aplastic anaemia

A
  • FBC = normocytic anaemia with decreased reticulocytes
  • Bone marrow biopsy = hypocellularity
126
Q

Complication of aplastic anaemia

A
  • Increased infection risk (neutropenia)
  • Treat with broad spectrum antibiotic and bone marrow transplant
127
Q

Signs specific to CKD

A
  • Oedema
  • Hypertension
  • Excoriations on the skin (conscious repetitive picking of skin that leads to skin lesions and significant distress or functional impairment)
128
Q

Pathophysiology of CKD anaemia

A
  • Occurs in chronic diseases
  • Decreased bone marrow stimulation for production of erythropoetin
129
Q

Diagnosis of CKD anaemia

A
  • Normocytic and normochromic
  • Decreased reticulocytes due to low erythropoetin
130
Q

What is antithrombin 3 deficiency

A
  • Inherited (or aquired in nephrotic syndrome)
  • Antithrombin 3 inhibits factor Xa by binding to its co-factor heparin
131
Q

Pathophysiology of DIC- Disseminated intravascular coagulation

A
  • Tissue damage causes release and activation of tissue factor
  • Widespread activation of coagulation cascade and therefore platelet activation (crisis)
  • Platelets unnecessarily consumed + microthrombose in small blood vessels
  • Tissue plasminogen activator activated, leads to increased fibrinolysis - clotting removed
  • Lack of systemic platelets -> increased bleed risk
132
Q

Aetiology of DIC

A
  • Trauma
  • Sepsis (eg: meningococcal meningitis)
  • Malignancy
133
Q

Diagnosis of DIC

A
  • Low platelets
  • Low fibrinogen
  • Increased D-dimer
  • Long PT and APTT
134
Q

Presentation of DIC

A
  • Bleeding (epistaxis, bruising, rash, GI bleeding)
  • Acute resp distress syndrome
135
Q

Treatment for DIC

A
  • Treat underlying cause
  • Fresh frozen plasma to replace clotting factor
  • Cryoprecipitate to replace fibrinogen
  • Platelet transfusion
136
Q

How does DIC cause infection

A

High clotting factors uses up platelets so systemic defences are down

137
Q

Epidemiology of haemophilia?

A

X linked recessive clotting factor deficiency so way more common in males

138
Q

Types of haemophilia

A

A - Factor 8 deficiency, most common
B - Factor 9 deficiency, also known as Christmas disease🎄
C - Factor 11 deficiency, very rare

139
Q

Presentation of haemophilia

A
  • Spontaneous bleeds
  • Haemarthrosis (bleeding into joint)
  • V. easy bruising
  • Epistaxis🤧
140
Q

When do most cases of haemophilia present?

A
  • In neonates or early childhood
  • Intercranial haemorrhage, haematomas, cord bleeding
141
Q

Diagnosis of haemophilia

A

Diagnosis of haemophilia
- Normal PT, long APTT (as only intrinsic pathway is affected)
- Low CF assay (which factor depends on type)

142
Q

Treatment for haemophilia A and B

A

A - high intensity IV factor 8 + desmopressin (releases F8 stored in vessel walls) every two days
B - IV factor 9

143
Q

Risk factors for aquired haemophilia

A
  • Age >60
  • IBD
  • Diabetes
  • Pregnancy/ postnatal
  • Malignancy
144
Q

Pathophysiology of Von Willebrand disease

A
  • Autosomal dominant mutation of VWF gene on chromosome 12
  • Defect in quality or quantity of VWF
  • VWF is responsible for the basis of the platelet plug -> more spontaneous bleeding and bruising
  • Most common inherited bleeding disorder
145
Q

Presentation of Von Willebrand disease

A

Mucocutaneous bleeding:
- Epistaxis
- GI bleeds
- Mennhoragia
- Bleeding gums with brushing 🪥
- Heavy bleeding during surgery

146
Q

Diagnosis of Von Willebrand disease

A
  • Normal PT, long APTT
  • Normal factor 8/9 assay
  • Low VWF
147
Q

Treatment of Von Willebrand disease

A
  • Desmopressin (releases VWF from endothelial Weibel palade bodies)
  • IV factor 8 can also be infused for 2 weeks in severe cases
  • Tranexemic acid (antifibrinolytic)
148
Q

Treatment for glandular fever?

A
  • Supportive therapy (fluids, analgesia)
  • Avoid contact sports for 6 months eels to prevent splenic rupture
149
Q

What’s EBV associated with?

A

Epstein-Barr Virus
-mono
Many conditions inc:
- Hodgkin’s lymphoma
- Burkitt’s lymphoma
- Nasopharyngeal carcinoma

150
Q

Incidence and spread of EBV

A
  • 15-24 years old
  • Spread via saliva or bodily fluids
151
Q

Symptoms of EBV
and how long is treatment?

A

Varied mild symptoms:
- Fever
- Tonsilitis
- Hepatosplenomegaly
- Cough

Self limiting 2-4 weeks

152
Q

Diagnosis of EBV

A

(Not with a green viral swab like most viruses)

  • FBC = Atyptical lymphocytes on blood film
  • Serology = EBV Igs with clotted sample
  • ELISA TEST
153
Q

Pathophysiology of haemolytics uraemic syndrome

A
  • Paediatric condition
  • Following infection with shiga-toxin producing bacteria like E.Coli/Shigella (typically 5 days after gastroenteritis)
  • Microvascular clot formation, deposition of platelets and fibrin in small vessels
154
Q

Presentation of haemolytic uraemic syndrome

A
  • Mostly self limiting but presents as medical emergency
  • AKI (oliguria, haematuria, hypertension) -> uraemia
  • Hameolytic anaemia
  • Thrombocytopaenia
155
Q

Diagnosis of haemolytic uraemic syndrome

A
  • FBC
  • Blood smear
  • Differentiate from TTP (Thrombotic Thrombocytopenia Purpura) with ADAMTS13 testing
156
Q

FBC in haemolytic uraemic syndrome

A
  • Thrombocytopaenia
  • Anaemia
  • High reticulocytes
  • High LDH
  • High bilirubin
157
Q

Treatment of haemolytic uraemic syndrome

A
  • Supportive fluids
  • Antibiotics
158
Q

Types of HIV

A
  • HIV-1 - more common, most virulent
  • HIV-2 - less common, less virulent
159
Q

Transmission of HIV

A
  • Retrovirus
  • Sexual transmission
  • Sharing needles
160
Q

HIV RNA copies vs CD4 count

A

CD4 starts high, drops drastically after a stage, goes up again by stage 2 then dramatic drop baso 0 by group 3 then gone by stage 4

peaks at stage 1 then drops by stage 2, then increases, gets to AIDS by stage 4 then death

1 and 2 weeks then after years 3 and 4

  1. CD4+ dip then ‘set point’
  2. Clinical latency (years)
  3. Symptoms
  4. AIDS (CD4+ <200/mm3)
161
Q

Treatment for HIV

A

HAART - highly active antiretroviral therapy
- 3 drugs <, reverse transciptase inhibitors
- Aim is to maintain CD4 count and decrease HIV RNA copies

162
Q

Risk factors of HIV

A
  • Sharing needles, needle stick injury
  • MSM - unprotected anal sex
163
Q

Pathophysiology of HIV

A
  • HIB gp120 binds to CD4 on TH
  • Endocytoses RNA + enzymes
  • Reverse transcriptase RNA -> DNA
  • Integrase; viras DNA integrated into host’s
  • Protein synthesis
  • Viral proteins + RNA exocytose and take part of CD4+ cell CSM
  • Increased viral copies, decreased CD4+ (TH cells)
164
Q

AIDS definining conditions

A
  • CMV (eg: collitis -> owl eyes)
  • Pneomocystis jirovecci pneumonia
  • Kaposi sarcoma
  • Cryptosporadium (fungal) infection
  • TB
  • Toxiplasmosis
  • Lymphomas
165
Q

Diagnosis of HIV

A
  • History + Anti HIV Ig, p24 Ab (Elisa testing)
  • Monitor progression - HIV RNA copies + CD4 count
166
Q

Pathophysiology of leukaemia

A
  • Neoplastic proliferation of WBC line (myeloblasts or lymphoblasts)
  • Lose ability to differentiate but maintain ability to replicate
  • Decreased production of other haematopoetic cells (functional pancytopaenia)
167
Q

General signs of leukaemia

A
  • Thrombocytopaenia - bleeding and bruising
  • Leukopaenia - infections
  • Anaemia symptoms
  • Hepatosplenomegaly
  • Fatigue
  • Failure to thrive (children)
168
Q

Testing for leukaemia

A
  • FBC (shows pancytopaenia)
  • Blood film
  • Bone marrow biopsy - diagnostic
  • Imaging (CT/CxR)
  • Genetic testing
169
Q

Blood films for leukaemia

A
  • ALL - increased lymphoblasts
  • AML - increased lymphoblasts with auer rods, myeloperoxidase positive
  • CLL - smudge cells (Richter’s transformation)
170
Q

Leukaemia bone marrow biopsies

A

ALL - lymphoblasts ≥20%
AML - myeloblasts ≥20%
CLL - pancytopaenia (except lymphocytosis)
CML - increased granulocytes, blast cell percentage shows severity (diff card)

171
Q

Chromosomal translocations in leukaemia

A

ALL - mostly t(12:21)
AML - t(15:17)
CLL - multifactorial
CML - t(9:22) (Philadelphia chromosome)

172
Q

What do tyrosine kinases do

A

Increase cell proliferation

173
Q

What is hypogammaglobulinaemia

A
  • Low conc. of immunoglobulins present in CLL
  • This is because B cells proliferate but don’t differentiate to plasma cells (which are responsible for producing Igs)
174
Q

What to give with chemo

A

✨allopurinol✨
- Chemo releases uric acid from cells which can accumulate in kidneys
- This would cause tumour lysis syndrome
- Allopurinol prevents it

175
Q

Acute lymphoblastic anaemia (ALL)

A
  • Neoplastic proliferation of lymphoblasts, mostly B
  • Terminal deoxynucleotidyl transferase expressed
  • Patients typically under 6 (75%) and over 50
  • Associated with Down’s syndrome
176
Q

Presentation of ALL

A
  • General leukaemia symptoms
  • Lymphadenopathy
  • CNS infiltration -> headaches, CNS palsies
177
Q

Philadelphia chromosome in ALL

A
  • 30% in adults 3-5% in children
  • Worse prognosis
178
Q

Treatment of ALL

A
  • Chemotherapy
  • Typically good prognosis
179
Q

Presentation of AML

A
  • General leukaemia symptoms
  • Gum hypertrophy
180
Q

Acute myelogenous leukaemia (AML)

A
  • Neoblastic proliferation of myeloblasts
  • Present mostly in the elderly (over 65)
  • Rapid progression if not treated asap
  • 3 year survival 20%, 5 year survival 15%
181
Q

AML + DIC

A
  • Common subtype of AML
  • Acute premyelocytic leukaemia (APML)
182
Q

Treatment of AML

A
  • Chemo + tretinoin (used for APML)
  • Transfusion for anemia
  • Abx (antibiotix) prophylaxis for neutropaenia
  • Last resort: bone marrow transplant
183
Q

Aetiology of AML

A
  • Down’s syndrome
  • Radiation
184
Q

Chronic lymphocytic leukaemia (CLL)

A
  • Neoplastic proliferation of lymphocytes, mostly B
  • Most common leukaemia in adults
  • Over 70 year olds, men typically
  • 5 year survival of 75%
185
Q

Presentation of CLL

A
  • General anaemia symptoms
  • Often asymptomatic
  • Rubbery non-tender lymphadenopathy
  • Might have night sweats and weight loss
186
Q

Treatment of CLL

A
  • Watch and wait in early stages
  • Monoclonal antibodies (rituximab)
  • Bruton kinase inhibitors (ibrutinib)
  • Palliative (if v old)
  • IV Ig for hypogammaglobulinaemia
187
Q

Complicaton of CLL

A

Richter transformations🥲
- B cells massively accumulate in lymph nodes
- Mahoosive lymphadenopathy and transformation from CLL to aggressive lymphoma
- #oops

188
Q

Presentation of CML

A
  • General leukaemia
  • Massive hepatosplenomegaly when with malaria
189
Q

Treatment for CML

A

Chemo + imantinib (tyrosine kinase inhibitor)

190
Q

Severity of CML based on blast cell % in bone marrow biopsy

A
  1. <10% - Chronic (best)
  2. 10-19% - Accelerated
  3. ≥20% - Blast crisis (worst, could progress to AML, poorer prognosis - often happens when CML is untreated or diagnosed late)
191
Q

Chronic myelogenous leukaemia (CML)

A
  • Neoplastic myelocyte proliferation - mainly neutrophils
  • Adults around 60
  • BCR - ABL gene fusion causing tyrosine kinase to be irreversibly switched on
192
Q

What are B symptoms of lymphomas?

A
  • Fever
  • Night sweats
  • Unintentional weight loss
193
Q

Ages susceptible to lymphomas

A

Hodgkin: Bimodal distribution, with peaks in early 20s and in 70s
Non-Hodkin: Over the age of 40

194
Q

Diagnosis of lymphoma

A
  • Lymph node biopsy
  • PET-CT/MRI chest/abdo/pelvis for staging
  • Performance status score to establish treatment
195
Q

How to distinguish between Hodgkin and non-Hodgkin lymphoma?

A

Lymph node biopsy (core needle/excision needle)

  • Hodgkin’s shows Reed-Sternberg cells🦉
  • Abnormally large B cells with multiple nuclei and nucleoli
  • These aren’t present in Non-Hodgkin lymphoma, but you can still use the biopsy to determine NHL subtype, eg: Burkitt’s shows starry sky🌌
196
Q

Ann Arbor staging in both types of lymphoma

A
  1. One region of lymph nodes
  2. 2 or more lymph nodes on same side of the diaphragm
  3. Lymph nodes on both sides of the diaphragm
  4. Extranodal organ spread

also:
a - no B symptoms
b - B symptoms

197
Q

Pathophysiology of Hodgkin lymphoma

A

Proliferation of lymphocytes in lymph nodes, typically in the cervical, axillary or inguinal lymph nodes

198
Q

Risk factors of Hodgkin lymphoma

A
  • HIV
  • EBV (gladular fever)
  • Autoimmune conditions
  • Family history
199
Q

Presentation of Hodgkin lymphoma?

A
  • B symptoms (Hodgkin is a high grade B cell lymphoma)
  • Non-tender rubbery lymphadenopathy which becomes painful after drinking alcohol
200
Q

Other possible symptoms of Hodgkin lymphoma

A
  • Fatigue
  • Itching
  • Cough
  • Dyspnoea
  • Abdo pain
  • Recurrent infections
201
Q

Popcorn cells🍿

A
  • Seen in nodular lymphocyte predominant Hodgkin’s
  • (a subtype of Hodgkin lymphoma)
  • They are a variant of Reed-Sternberg cells
202
Q

Treatment of Hodgkin lymphoma

A
  • ABVD immunochemotherapy (adriamycin, bleomycin, vinblastine, dacarbazine)
  • +/- radiotherapy
  • Marrow transplant
203
Q

Side effects of chemo for Hodgkin lymphoma

A
  • Alopecia
  • N+V
  • Myelosuppression
  • BM failure
  • Infection
  • Infertility
  • Leukaemia
204
Q

Side effects of radiotherapy for Hodgkin lymphoma treatment

A
  • Cancer
  • Damage to tissues
  • Hypothyroidism
205
Q

Febrile neutropaenia

A
  • Massive risk in patients with recent/high dose chemo (or on carbimazole)
  • Fever, tachycardia, swears, rigors, tachypnoea
  • Treated with immediate broad spectrum antibiotics
206
Q

Diagnosis of Hodgkin lymphoma

A
  • FBC: Anaemia, high ESR
  • High LDH
  • CXR - Wide mediastinum
  • Blood film - Reed Sternberg cells
207
Q

Types of Non-Hodgkin lymphoma

A
  • Low grade - Follicular
  • High grade - Diffuse large B cells
  • Very high grade - Burkitt’s
208
Q

Cells affected in Non-Hodgkin lymphoma

A
  • B cells (90%)
  • T cells (10%)
  • NK cells (<1%)
209
Q

MALT lymphoma

A
  • Affects mucosal-associated lymphoid tissue
  • Associated with H.pylori infection or eye chlamydia infection
210
Q

Other risk factors for Non-Hodgkin lymphoma

A
  • Hep B or C infection
  • Exposure to trichloroethyline (pesticide)
  • Immunosuppression
211
Q

Presentation of Non-Hodgkin lymphoma

A
  • B symptoms
  • Non-tender rubbery lymphadenopathy, not affected by alcohol
  • Can get hepatosplenomegaly
212
Q

Treatment of low-grade lymphoma

A
  • Radiotherapy
  • R-CHOP Chemotherapy
    Rituximab (targets CD20 on B-cells)
    Cyclophosphamide
    Hydroxy-daunorubicin
    Oncovin (brand name for vincristine)
    Prednisolone
213
Q

Indolent lymphoma

A
  • Slow growing and advanced on presentation
  • “Incurable”
  • High on Ann Arbour scale
  • Median survival 9-12 years variable across and within subtypes
214
Q

Aetiology of indolent lymphoma

A
  • Primary and secondary immunodeficiency
  • Infection
  • Autoimmune disease
215
Q

Burkitt lymphoma

A
  • Associated with EBV, malaria and HIV
  • Often causes massive jaw lymphadenopathy in children
216
Q

Two types of non-Hodgkin lymphomas

A
  • Aggressive - quick onset but can be treated
  • Indolent - worse prognosis
217
Q

Pathophysiology of multiple myeloma

A
  • Neoplastic monoclonal proliferation of a plasma cell
  • Results in large quantities of a single type of antibody/monoclonal paraprotein being produced (55% IgG, 20% IgA)
218
Q

Monoclonal gammopathy of undetermined significance (MGUS)

A
  • Too much Ig released by abnormal plasma cells
  • <10% BM plasma cells
  • Asymptomatic
  • 1% of cases develop into myeloma
219
Q

Smouldering myeloma

A
  • Progression of MGUS with higher levels of antibodies
  • Premalignant, more likely to progress to myeloma than MGUS
  • Waldenstrom’s macroglobulinaemia is a type of smouldering myeloma with excessive IgM specifically
220
Q

Epidemiology of myeloma

A
  • Around 70 years old
  • Afro-Carribeans
  • Male
  • Obesity
  • 1% of cancers are myeloma
221
Q

Presentation of multiple myeloma (🦀)

A

HyperCalcaemia
Renal failure
Anaemia
Bone lesions

222
Q

What are anaemia and bone lesions caused by in multiple myeloma?

A

Bone marrow failure

223
Q

What are hypercalcaemia and bone lesions caused by in multiple myeloma?

A

Increased osteoclast bone resorption due to cytokines released by plasma and stromal cells

224
Q

What is renal failure caused by in multiple myeloma?

A
  • Hypercalcaemia -> calcium oxalase renal stones
  • Immunoglobulin light chain deposition - Bence Jones protein in pee
225
Q

Testing for multiple myeloma

A
  • FBC and blood film
  • U+E = renal failure
  • Bone profile = hypocalcaemia and increased ALP
  • Serum electrophoresis
  • Urine dipstick - Bence Jones protein
  • Xr
226
Q

FBC and blood film for multiple myeloma

A
  • Normocytic normochromic anaemia
  • Raised ESR
  • Rouleaux formation in blood film (aggregation of RBCs)
227
Q

Serum electrophorisis in multiple myeloma

A
  • Ig paraprotein ‘M spike’
  • Hypergammaglobulaemia for that specific Ig
228
Q

X-ray for multiple myeloma

A
  • Pepper pot skull
  • Osteolytic lesions -> “punched out holes”
229
Q

Confirmatory diagnosis of multiple myeloma

A
  • Bone marrow biopsy required - >10% plasma cells
  • NICE recommends full body MRI (if not, then CT or Xray in that order of preference)
230
Q

Treatment of multiple myeloma

A
  • Bisphosphonates (eg: alendronate) to decrease octeoclastic activit and increase osteoblastic activity - protects bones

Older patients:
- Anti-myeloma chemotherapy
- Consider BM stem cell transplant

231
Q

Plasma viscocity in multiple myeloma

A
  • Increased due to large amounts of immunoglobulins in blood
    Complications:
  • Easy bruising and bleeding
  • Reduced or loss of sight due to vascular disease in the eye
  • Purple discolouration of extremities
  • Heart failure
232
Q

Supportive treatment for multiple myeloma

A
  • Bisphosphonates
  • Blood transfusion/EPO injection
  • Antibiotics and pain-killers as needed
  • GCSF to boost neutrophils
  • Radiotherapy
  • Kyphoplasty occasionslly indicated
  • Psychological support
233
Q

What is multiple myeloma characterised by?

A
  • Monoclonal protein in serum or urine
  • Lytic bone lesions/ CRAB end organ damage
  • Excess plasma cells in bone marrow
234
Q

Classic features of myeloma

A
  • Osteoporosis
  • Nephrotic syndrome
  • Hypercalcaemia
  • Thrombocytopaenia
235
Q

What is the precursor condition to developing multiple myeloma?

A

Mammyloid gammopathy of undetermined significance

236
Q

Pathophysiology of polycythaemia vera

A
  • A high concentration of erythrocytes in the blood
  • Due to JAK2V617 mutation
  • The affected bone marrow can also produce excessive platelets and white blood cells
237
Q

Relative vs absolute polycythaemia

A

Relative:
- Normal number of erythrocytes, reduction in plasma
- Causes include obesity, dehydration execessive alcohol and increased erythropoetin

Absolute:
- Increased number of erythrocytes
- Primary and secondary

238
Q

Primary vs secondary polycythaemia

A

Primary: Polycythaemia vera - abnormality in the bone marrow
Secondary: Disease outside the bone marrow causing overstimulation of the bone marrow

239
Q

General symptoms of polycythaemia

A
  • Erythromalagia (fig 1)
  • Reddish plethoric complexion
  • Blurred vision
  • Headache + dizziness

(features nonspeifically related to hyperviscosity)

240
Q

Symptoms unique to polycythaemia vera

A
  • Itchy after a bath (contact with warm water) 🛁
  • Hepatosplenomegaly
241
Q

Diagnosis of polycythaemia vera

A
  • FBC = high WCC, high platelets, high RBCs
  • High Hb
  • Genetic tests JAK2V617 positive
242
Q

Treatment of polycythaemia vera

A

Non-curative, main aim is to maintain a normal blood count

Low-risk:
- Venesection regularly (often used solely)
- Low dose aspirin

High risk:
- Consider chemotherapy (hydroxycarbamide) for patients at high risk of thrombus (60+, background of thrombosis)

243
Q

What haematocrit is concerning in polycythaemia vera?

A

> 45%

244
Q

Differential diagnoses of abnormal or prolonged bleeding

A
  • Thrombocytopaenia (low platelets)
  • Haemophilia A and B
  • Von Willebrand disease
  • Disseminated intravascular coagulation (secondary to sepsis)
245
Q

Ge symptoms of thrombocytopaenia (all of the symptoms of ITP)

A
  • Purpuric rash
  • Easy bleeds
  • Mennhoragia
246
Q

Heparin-induced thrombocytopenia

A
  • Antibodies bind to heparin after administration -> prothrombotic state
  • Onset of emboli
  • Platelets <50%, D-dimer high
  • Treatment: stop heparin, anticoagulate
247
Q

Pathophysiology of ITP

A
  • Autoimmune
  • Antibodies (usually IgG) created against platelet, causing platelet destruction
  • Specifically, Gpiib/iiia on platelets are destroyed
248
Q

Types of ITP (immune thrombocytopenic purpura)

A

Type 1: Children 2-6, post viral infection, acute + severe
Type 2: Adult women w malignancy, HIV or other autoimmune condition. Chronic + mild

249
Q

Diagnosis of ITP

A
  • FBC - raised WCC, low Hb, low platelets
  • Increased bone marrow megakaryocytes (due to negative feedback)
250
Q

Treatment of ITP

A

First line: Prednisolone + IV IgG
this decreases splenic platelet destruction
Second line: Splenoctomy

251
Q

Pathophysiology of TTP (Thrombotic Thrombocytopenia Purpura)

A
  • ADAMTS13 (VWF cleaving protein) deficiency
  • VWF remain as multimers and aggregate at endothelial injury sites
  • Incidence same as Type 2 ITP
252
Q

Symptoms unique to TTP

A
  • AKI
  • Fever
  • Haemolytic anaemia
  • Neurologicla symptoms
253
Q

Diagnosis of TTP

A
  • Same FBC as ITP
  • Raised billirubin and creatinine
  • Blood smear: schistocytes
  • RBCs; microangiopathic haemolytic anaemia
  • Genetic testing shows low ADAMTS13
254
Q

Treatment of TTP

A

First line: Plasmapharesis
Second line: Prednisolone + rituximab

255
Q

Pathophysiology of tumour lysis syndrome

A
  • Collection of metabolic disturbances occuring with rapid destruction of neoplastic cells following chemotherapy
  • More common in aggressive treatment of haem malignancies
256
Q

Signs of tumour lysis syndrome

A
  • Hyperuricaemia, hyperkalaemia, hyperphosphataemia
  • Hypocalcaemia
  • AKI
257
Q
A

Haroon flashcards (10 decks)

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