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haematology Flashcards

1
Q

Anaemia: define

A

Hb below certain levels
children 12-14 and women above 15 = <120g/L in blood

men above 15 = <130g/L

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2
Q

Anaemia: Signs and Symptoms?

A

Symptoms:

  1. Fatigue.
  2. Faintness.
  3. Breathlessness.
  4. Reduced exercise tolerance.

Signs

  1. Pale skin and mucous membranes.
  2. Tachycardia.
  3. Bounding pulse.
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3
Q

Anaemia: investigations?

A
  1. Blood tests: FBC and blood film.
  2. Biopsies.
  3. Reticulocyte count. - - If production is the issue then the reticulocyte count will be low
    - If removal is the issue then the reticulocyte count will be high
  4. B12 levels./FOLATE LEVELS!!
  5. Serum ferritin.
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4
Q

Anaemia: MCV?`

A

The various types of anaemia are classified by Mean Corpuscular Volume (MCV) which is essentially the average volume of RBC’s or basically their size

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5
Q

Anaemia: treatment?

A

Treat the underlying cause e.g. if iron deficient give ferrous sulphate.

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6
Q

Microcytic Anaemia: define

A

low MCV <80 fL

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7
Q

Microcytic Anaemia: aetiology

A
  1. Iron deficiency. commonest cause!
  2. Anaemia of chronic disease.
  3. Thalassaemia.!!!!!!!!!!!!!
  4. sideroblastic anaemia
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8
Q

Microcytic anaemia on a blood film?

A

Blood film = rbc = microcytic and PALE (Hypochromic (pale))

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9
Q

Normocytic anaemia: define

A

normal MCV - 80-100 fL

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10
Q

Normocytic anaemia: aetiology?

A
  1. Acute blood loss.
  2. Anaemia of chronic disease. (can also be microcytic!!)
  3. Combined hematinic deficiency. things like B12/folate/iron etc theyre baso nutrients required for formation of rbc
  • Endocrine disorders such as hypopituitarism, hypothyroidism and
    hypoadrenalism!!
  • Renal failure!!
  • Pregnancy!!
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11
Q

What kind of anaemia is seen in patients with multiple myeloma?

A

Normochromic normocytic.

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12
Q

Macrocytic anaemia: define

A

high MCV

> 100 fL

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13
Q

Macrocytic anaemia: aetiology?

A

Megablastic anaemia = due to:

  • Vit B12 deficiency
  • Folate deficiency
  • Drug induced

Non-megaloblastic anaemia = due to:

  • alcohol abuse
  • Hypothyroidism
  • Pregnancy
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14
Q

Macrocytic anaemia: 2 types? describe the cells

A
  • megaloblastic - bc of delayed nuclear maturation - large immature rbc called megaloblasts and hypersegmented neutrophils
  • non-megaloblastic - just large mature rbc
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15
Q

What kind of anaemia could methotrexate cause?

A

Macrocytic due to folate deficiency.

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16
Q

iron-deficiency anaemia: define?

A

Iron deficiency anaemia is a condition where a lack of iron in the body leads to a reduction in the number of red blood cells.

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17
Q

iron-deficiency anaemia: aetiology?

A
  1. Blood loss. (eg also menorrhagia)
  2. Poor absorption. (eg coeliac disease)
  3. Decreased intake in diet.
  4. Hook worm! (results in GI blood loss) !!
  5. Breastfeeding, low iron in breast milk.
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18
Q

iron-deficiency anaemia: treatment?

A

Oral iron e.g. Ferrous sulphate tablets.
- Side effects; nausea, abdominal discomfort, diarrhoea/constipation
and black stools

• Can give FERROUS GLUCONATE if side effects are bad

  • Parenteral iron e.g IV iron or deep intramuscular iron in extreme cases e.g.
    severe malabsorption
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19
Q

iron-deficiency anaemia: signs and symptoms?

State two features with regards to red blood cell appearance that would make you think a patient had anaemia due to iron deficiency.

A
  1. Koilonychia. spoon shaped nails
  2. Brittle hair and nails.
  3. Atrophic glossitis. Atrophy of the papillae of the tongue
  4. Tiredness, reduced exercise tolerance.
  5. SOB.
  6. Angular stomatitis/cheilosis - ulceration of the corners of the mouth
  7. Hypochromia (pale).
  8. Microcytosis.
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20
Q

folate deificiency: Give 4 causes of folate deficiency.

A
  1. Dietary.
  2. Malabsorption.
  3. Increased requirement e.g. in pregnancy.
  4. Folate antagonists e.g. methotrexate
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21
Q

Haemolytic anaemia: define

A

Increased destruction of rbc’s

→ intravascular haemolysis and extravascular haemolysis (destruction of rbc’s outside and inside of the vasculature)

The premature breakdown of RBCs, BEFORE their normal lifespan of around 120 days

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22
Q

Haemolytic anaemia: pathophysiology

what happens when rbc’s destroyed

A

When rbc are destroyed → they release lactate dehydrogenase

The Hb is also broken down into → globin, unconjugated bilirubin and iron

Usually body can clear up al these products of rbc destruction BUT in haemolytic anaemia - You have an OVERWHELMING amount of rbc destruction - SO you have free haemoglobin in circulation

Haptoglobins help clear up these free haemoglobins

BUT INTRAVASCULAR HAEMOLYSIS is when:

  • When RBCs are rapidly destroyed in circulation, haemoglobin is liberated
  • This is initially bound to HAPTOGLOBULIN but these soon become saturated
  • Excess free plasma haemoglobin is filtered by the renal glomerulus and enters the urine, although small amounts are reabsorbed by the renal
    tubules
  • In renal tubular cells, haemoglobin is broken down and becomes deposited in the cells as HAEMOSIDERIN
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23
Q

Haemolytic anaemia: signs?

A
  1. Pallor.
  2. Jaundice.
  3. Splenomegaly.
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24
Q

Haemolytic anaemia: investigations?

A
  1. lactase dehydrogenase increased bc rbc release it when they’re destroyed
  2. Reticulocyte count increased bc body is trying to compensate by producing more rbc
  3. Bilirubin increased (so pt can present w jaundice)
  4. LOW haptoglobin bc they are bound to Hb and are being cleared up by the body
  5. Blood film - RBCs can be either NORMOCYTIC or if there are many young RBC’s (which are larger) due to excessive destruction of old RBCs then MACROCYTIC
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25
Q

Haemolytic anaemia: aetiology?

A
  1. sickle cell anaemia
  2. alpha thalassaemia
  3. beta thalassaemia
  4. malaria
  5. G6PDeficiency
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26
Q

Sickle cell anaemia: define and inheritance pattern and pathophysiology?

What can precipitate sickling in sickle cell anaemia?

A

????????????

A haemoglobin disorder of quality. HbS polymerises -> sickle shaped RBC.

Autosomal recessive. Sickle cell disease is homozygous SS.

Trauma, cold, stress, exercise.

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27
Q

sickle cell anaemia: epidemiology?

A
  • african descent - but also in inda, middle east and south europe
  • AUTOSOMAL RECESSIVE
    1 in 4 chance of disease
  • 50% chance of being a carrier
  • 1 in 4 chance of being disease free
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28
Q

sickle cell anaemia:
symptoms and signs for:

Heterozygous sickle cell trait?

Homozygous Sickle cell anaemia?

Long-term problems?

A

Heterozygous sickle cell trait?
none
Carriage offer protection against FALCIPARUM MALARIA!!

Homozygous Sickle cell anaemia?
- vaso-occlusive crisis - eg pulmonary etc or pain in hands (dactylitis) baso the microvasculature - clots forming there- and in long bones in adults
also can = pul hypertension
anaemia

long-term = growth and development problems, things like cardiomegaly, neurological things

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29
Q

sickle cell anaemia: investigations? gs?

A
  • Blood count:
    • Level of Hb is in the range of 60-80 g/L
    • RAISED RETICULOCYTE COUNT
    • Sickle cell disease is haemolytic, there is increased degradation of RBC’s. Production therefore increases in order to keep up with degradation and so reticulocyte count is raised.
  • Blood films:
    • Sickled erythrocytes shown
  • Sickle solubility test will be POSITIVE

GOLD STANDARD/CONFIRMS DX!!! - HB ELECTROPHORESIS!!!!
• Confirms diagnosis!!!!!!!!
• Shows 80-95% HbS and absent HbA
• Aim for diagnosis at birth (cord blood) to aid prompt pneumococcal
prophylaxis

reticulocyte count raised BS Sickle cell disease is haemolytic, there is increased degradation of RBC’s. Production therefore increases in order to keep up with degradation and so reticulocyte count is raised.

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30
Q

sickle cell anaemia: management?

A

?????????
FOLIC ACID to all haemolysis patients!!!!
1. Transfusion.
2. Hydroxycarbamide. — to prevent painful crises in people with sickle cell anaemia?

  1. Stem cell transplant.
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31
Q

What is the advantage of being a carrier of sickle cell disease?

A

Carriage offers protection against falciparum malaria.

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32
Q

Glucose 6 Phosphate deficiency: define and pathophysiology?

A

????????????
Glucose-6-phosphate dehydrogenase deficiency is an inborn error of metabolism that predisposes to red blood cell breakdown

G6PD protects cells against oxidative damage.

G6PD deficiency = decreased levels of reduced glutathione → increased susceptibility to oxidative stress
↓ G6PD → ↓ reduced NADPH → ↓ reduced glutathione → increased red cell susceptibility to oxidative stress

G6PD is vital for a reaction that is necessary for RBC’s by providing a NADPH which is used with glutathione to PROTECT the RBC from OXIDATIVE DAMAGE from compounds such as hydrogen peroxide

  • This inherited enzyme deficiency thus results in reduced RBC lifespan due to oxidant damage
  • Gene for G6PD is localised to chromosome Xq28 near the factor VIII gene
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33
Q

sickle cell anaemia: acute vs chronic complications?

A

ACUTE
1. Very painful crisis.

2. Stroke in children.
3. Cognitive impairment.
4. Infections.

CHRONIC
1. Renal impairment.

  1. Pulmonary hypertension.
  2. Joint damage.
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34
Q

What is the significance of parvovirus for someone with sickle cell disease?

A

Parvovirus is a common infection in children. It leads to decreased RBC production and can cause a dramatic drop in Hb in patients who already have a reduced RBC lifespan. This can be dangerous for someone with sickle cell.

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35
Q

G6PD: signs and symptoms?

A 
????????
Crises characterised by:
1. Haemolysis.
2. Jaundice.
3. Anaemia.
**Fatigue, palpitations, pallor, SOB**!!
Most are asymptomatic but may get oxidative crisis due to reduction in glutathione production, this is precipitated by:
- Made worse by **ingesting fava beans**
- **gallstones** are common
- **splenomegaly** may be present
- In attacks:
    - Rapid anaemia
    - Jaundice
    - Chronic haemolytic anaemia
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36
Q

G6PD: investigations?

A
  • Blood count is normal between attacks
  • Blood film during attack:
    • Heinz bodieson blood films.
    • Bite cells (cells with an indentation in the membrane)Bite and blister cellsmay also be seen
    • Irregularly contracted cells
    • Reticulocytosis - increased reticulocytes
  • G6PD enzyme levels!!!! - will be LOW (but note, immediately after attack the test may be normal since the oldest RBCs with the least G6PD activity are destroyed selectively - so results may show falsely high concentration of G6PD)
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37
Q

G6PD: management?

A
  • Stop offending drugs and or fava beans
  • Blood transfusion may be lifesaving
  • Splenectomy is not usually helpful
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38
Q

Malaria: P. falciparum define and pathophysiology

A

Unique as it causes cerebral malaria - Fatal infection.

The parasite matures in RBCs → ‘knobs’ on RBC surface → bind to receptors on endothelial cells in capillaries & venules → bind to non-infected RBCs = ‘Rosetting’ → sequestration in small vessels (including brain, lung) → microcirculation obstructed: tissue hypoxia

Unique cerebral malaria, fatal infection. Parasites mature in RBC’s, RBC’s collect in small vessels and cause blockage of cerebro-microvasculature = hypoxia!

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39
Q

Malaria: what do - some species do once they get inside the liver?

which species??

A

Some species [P. Ovale, P. Vivax] lay dormant in the liver & relaspse by reactivating months later] -

Not eradicated by most conventional anti-malarial treatments NEED TO GIVE THEM CHLOROQUINE AND PRIMAQUINE TO FULLY ERADICATE!!!!
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40
Q

Malaria: complicated vs uncomplicated?

A

Complicated = Vascular occlusion , drowsiness, increased ICP -> seizures/coma

Uncomplicated = Fever with sweats and chills

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41
Q

Malaria: investigations?
gold standard?

other??

A

Diagnosis via blood film (thick and thin) w/ light microscopy

Giemsa stain blood smear

Thick and thin blood smears

(If negative, 2 more films should be sent within 48 hours)

  • 3 separate films (different times) required to rule out completely1st smear may be +ve in 95% cases.Thick: sensitive but low resolution, tell you if you have malaria.Thin: identify morphological features & quantification of parasitaemia. Identification of species on thin film: Trophozoite most commonly used and rapid antigen test ——tell you species and parasite count.

OTHER
Travel history

Rapid diagnostic tests (detect parasitic antigens)

PCR

FBC, LFTs, U&Es, blood gases, blood culture

CXR, lumbar puncture

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42
Q

Malaria: symptoms and signs?

when do they occur?

A

Symptoms occur from 6 days post infection
BUT Vivax and Ovale commonly present 6 months post infection

  • Febrile paroxysm (10-12 hours of intense rigors, chilss -> high fever, profuse diaphoresis)
  • haemolytic anaemia - bc malaria parasites infect rbc - at the end of that infection cycle, red blood cell ruptures - process lowers the amount of red blood cells and can in a severe stage cause severe anemia.

SIGNS - - Splenomegaly,

  • hypovolaemia
  • AKI
  • and nephrotic syndrome
  • Anaemia - (haemolytic anaemia) - malaria parasites infect rbc - at the end of that infection cycle, red blood cell ruptures - process lowers the amount of red blood cells and can in a severe stage cause severe anemia.
  • Jaundice
  • Hepatosplenomegaly
  • Black water Fever - darkened urine - bc of all the haemolysis (baso excreting Hb out of kidneys)

SYMPTOMS

  • Fever,
  • chills,
  • malaise,
  • abdo pain,
  • diarrhoea,
  • vomiting,
  • headache,
  • cough,
  • shortness of breath
  • fits
  • confusion
  • Fever, haemolysis, chills, sweats, headaches etc.

Non-specific features:

  • Anaemia
  • Low platelets
  • Hyperbilirubinaemia
  • Mildly raised transaminases (ALT, AST)
43
Q

Malaria: treatment

in non-falciparum

in Uncomplicated falciparum malaria

‘Complicated’ P. falciparum malaria

A

Artemisinin combination therapy (ACT)
OR → PO Chloroquine

Artemisinin combination therapy (ACT)
→ PO Quinine (SULPHATE) & Doxycycline as 2nd agent

IV Artesunate
→ THEN Artemisinin combination therapy (ACT) OR give IV Quinine (sulphate)

44
Q

Thalassaemia: define

A

A haemoglobin disorder of quantity. There is reduced synthesis of one or more globin chains with leading to a reduction in Hb -> anaemia.

45
Q

alpha vs beta thalassaemia: define?

and aetiology for both?

A

Alpha Thalassaemia = Reduced A chain synthesis

Beta Thalassaemia = Reduced B chain synthesis
They have very few beta chains, alpha chains are in excess

Alpha Thalassaemia = gene deletions
Beta Thalassaemia = - Caused by over 200 genetic defects

46
Q

Beta thalassaemia: clinical classification of beta thalassaemia?

A
  1. Thalassaemia major.
  2. Thalassaemia intermedia.
  3. Thalassaemia carrier/heterozygote.
47
Q

Aplastic anaemia: define?

A

When bone marrow stem cells are damaged -> pancytopenia.

48
Q

aplastic anaemia: investigations?

A
  • Blood count:
    • Pancocytopenia with low reticulocyte count
  • Bone marrow examination:
    • Hypocellular marrow with increased fat spaces
49
Q

pernicious anaemia: define and pathophysiology?

A

Type of megaloblastic anaemia which causes b12 deficiency

is an AUTOIMMUNE DISORDER in which the parietal cells of the stomach are attacked (THUS PARIETAL CELL LOSS) resulting in atrophic gastritis

and the loss of intrinsic factor production

And thus vitamin B12 malabsorption

(bit more in notion)

50
Q

pernicious anaemia: symptoms and signs?

A

Onset is insidious with progressively increasing symptoms of anaemia e.g.

fatigue, headache, pallor, dyspnoea, anorexia, tachycardia and palpitations

Red sore tongue (glossitis) and angular stomatitis/cheilosis (ulceration of the corners of the mouth) may be present

lemon-yellow skin colour due to the combination of pallor and mild jaundice caused by excess breakdown of haemoglobin (due to fact that
body will try to remove defective large RBCs)

51
Q

pernicious anaemia: investigations?

A

BLOODS
- intrinsic factor antibodies, although found in only 50% of patients with pernicious anaemia are SPECIFIC for DIAGNOSIS

- **Blood count & film:**
• Typical of megaloblastic anaemia
• RBC’s are MACROCYTIC
• Peripheral film shows oval macrocytes (large RBC’s) with hypersegmented neutrophil polymorphs with six or more lobes in the
nucleus
  • Serum bilirubin may be raised as a result of ineffective erythropoiesis
    resulting in increased RBC breakdown
  • Serum B12 is low
  • Hb is low
  • Reticulocyte count is LOW
  • Intrinsic factor antibodies - DIAGNOSTIC but lower sensitivity i.e. not
    present in all patients
52
Q

Beta thalassaemia: Thalassaemia major - management? and the side-effects?

key presentations?

A

major - blood transfusion dependant - chelation agents (deferoxamine to avoid iron overload) splenectomy when splenomegaly causes excessive

relies on regular transfusions
There is a risk of iron overload from the regular trasnfusions. Excess iron will be deposited in various organs e.g. the liver and spleen and cause fibrosis.

These patients usually present very young due to having severe anaemia and so a failure to feed/thrive.

53
Q

Beta thalassaemia: Thalassaemia minor - carrier/heterozygote

- clinical manifestations?

A

often asymptomatic

54
Q

Beta thalassaemia: beta Thalassaemia major - investigations?

A
  1. Raised reticulocyte count.

2. Microcytic anaemia.

55
Q

HIV: define

also define serodifferent and seroconversion?

A

HIV infection is caused by a retrovirus that infects and replicates in human lymphocytes and macrophages,

eroding the integrity of the human immune system over a number of years,

culminating in immune deficiency and a susceptibility to a series of opportunistic and other infections as well as the development of certain malignancies.

SERODIFFERENT =
When a couple is serodifferent, this meansone person is living with HIVand the other person is HIV-negative.

SEROCONVERSION
Seroconversion isa sign that the immune system is reacting to the presence of the virus in the body.
It’s also the point at which the body produces antibodies to HIV.
→ Once seroconversion has happened, an HIV test will detect antibodies and give a positive result.

56
Q

HIV: epidemiology

A
  • 37.7 million people across the globe with HIV in 2020.
  • Every day 4000new HIV infections (adults and children)(2020)
  • 60%are in sub-Saharan Africa
  • 10%are among children under 15 years of age
  • Newly infected people going down
  • Those living with HIV going up
57
Q

HIV: aetiology?

A

Virus

Most people are infected
- through sexual contact; unprotected anal or vaginal sex

  • before birth or during delivery (35% chance risk of HIV being passed down to child from pregnant mother)
  • during breastfeeding,
  • or when sharing contaminated needles and syringes -
    • receiving unsafe injections, blood transfusions and tissue transplantation, and medical procedures that involve unsterile cutting or piercing; and
    • experiencing accidental percutaneous needle stick injuries, including among health workers
  • having another sexually transmitted infection (STI) such as syphilis, herpes, chlamydia, gonorrhoea and bacterial vaginosis;
58
Q

HIV: pathophysiology?

A

Retrovirus = RNA virus which uses a reverse transcriptase (RTA) to make a DNA copy that becomes integrated into DNA of host cell

  1. HIV attaches to and penetrates host T cells via CD4+ molecules and chemokine receptors
  2. After attachment, HIV RNA and several HIV-encoded enzymes are released into the host cell.
  3. Viral replication requires that reverse transcriptase (an RNA-dependent DNA polymerase) copy HIV RNA, producing proviral DNA; this copying mechanism is prone to errors, resulting in frequent mutations and thus new HIV genotypes.
  4. viral DNA enters the host cell’s nucleus and is integrated into the host DNA in a process that involves integrase, another HIV enzyme.
  5. viral HIV DNA can be transcribed to HIV RNA and translated to HIV proteins
  6. HIV proteins are assembled into HIV virions at the host cell inner membrane and budded from the cell surface within an envelop of modified human cell membrane
59
Q

HIV: investigations?

differential diagnosis?

A

Diagnosis is confirmed:
- HIV antibody test

If seeing recurrent respiratory infections best to test for HIV to rule it out and or detect it EARLY

should be considered in any patient with unusual or recurrent serious infections without another cause, especially in those with risk factors for HIV infection.

60
Q

HIV: management?

A

Acute - newly confirmed infection:

  • Start antiretroviral therapy regardless of CD4 count(500< = normal but **<200 = bad)
    And select antiretroviral therapy regimen (INSTI-based regimen etc) and supportive care (counselling, prophylaxis of opportunistic infections, vaccinations)
  • *Ongoing** - virological or immunological treatment failure:
  • Re-assessment of antiretroviral therapy!!!!!Virological failure is defined as the inability to achieve or maintain suppression of viral replication to an HIV RNA level <200 copies/mL
    Immunological failure is the failure to achieve and maintain an adequate CD4 response despite virological suppression

U = U (Undetectable = untransmittable)

Without treatment,HIV infection progresses to AIDS in approximately 10 years, with death following within three years after onset of AIDS. With appropriate treatment, a 20-year-old with HIV infection can expect to live to reach 71 years of age.

61
Q

AIDs: define

A
  • Advanced HIV disease or AIDS– if untreated or non-responsive to therapy.Said to have AIDS if develop:
    • Opportunistic infections such as pneumocystis pneumonia, cytomegalovirus (CMV) retinitis, and cryptococcal meningitis.
    • Certain malignancies such as Kaposi’s sarcoma, non-Hodgkin’s lymphoma, and carcinoma of the cervix.
    • any HIV antibody test would be reliable at this advanced stage of HIV and the CD4 count is likely to be less than 200 cells per mL
62
Q

HIV: prevention?`

A

PreP - for partners - daily dosing and event-based dosing

Effectiveness has been demonstrated in MSM (men who have sex with men), heterosexual serodifferent couples, and injecting drug users

PEP - 28 days Combination Antiretroviral Therapy -

treatment that can stop an HIV infection after the virus has entered a person’s body.

It must be taken within 72 hours of exposure

63
Q

Leukaemia: define?

pathophysiology?

A

A malignant proliferation of haemopoietic stem cells

rapidly proliferating immature blast
blood cells in the bone marrow that are non functional i.e. defective

the bone marrow is not able to make as many normal functioning cells resulting in fewer functioning blood cells in the blood resulting in the symptoms of leukaemia

soon too many wbc myeloblasts in bone marrow - that they SPILL INTO BLOOD
if build-up of myeloblasts = acute myeloid leukaemia

if build-up of lymphoblasts = acute lymphoblastic leukaemia

————-thus too little platelets made = thromocytopenia = bleeding
or too little rbc made = anaemia = fatigue
or too little neutrophils = neutropenia = infection

64
Q

Name 4 sub-types of leukaemia.

and define all.

A
  1. AML - acute myeloid leukaemia.
  2. CML - chronic myeloid leukaemia - uncontrolled clonal proliferation of basophils, eosinophils and neutrophils.
  3. ALL - acute lymphoblastic leukaemia - uncontrolled proliferation of immature lymphoblast cells.
  4. CLL - chronic lymphoblastic leukaemia
    Proliferation of B lymphocytes leads to the accumulation of mature B cells that have escaped apoptosis.
65
Q

AML: risk factors?

A
  • Associated with radiation (ionising)**
  • and syndromes such as Down’s (trisomy 21)
  • Preceding haematological disorders.
  • Prior chemotherapy
66
Q

Leukaemia: signs and symptoms?

A
  1. Anaemia. - - breathlessness, fatigue, angina and claudication
    - There is pallor and cardiac flow murmur
  2. Infection. - - infections
    - There is fever and mouth ulcers
  3. Bleeding.- bleeding and bruising
    (first three bc of bone marrow failure)
  4. Hepatomegaly. Because of tissue infiltration.
  5. Splenomegaly. Because of tissue infiltration.

can be Gum hypertrophy

67
Q

Leukaemia: investigations?

A
  1. Blood film.
  2. Bone marrow biopsy.
  3. Lymph node biopsy.
  4. Immunophenotyping.
  5. Cytogenetics.
    (microscopy
68
Q

CML: risk factors?

A

More than 80% have the Philadelphia chromosome

which forms a fusion gene BCR/ABL on chromosome 22,

which has tyrosine kinase activity - stimulates cell division

69
Q

Leukaemia: gen. epidemiology?

A
  • ALL is mainly seen in childhood- most common between 2-4 yo - commonest cancer in childhood
  • CLL is a disease of the elderly - most common leukaemia - occurs mainly in later life
  • AML is commonest acute leukaemia of adults
  • CML- almost exclusively a disease of adults - most often 40-60yrs - slight male predominance
70
Q

Leukaemia: management?
general?

then for CML?? and why??
and CLL???

A
  • Blood and platelet transfusions
  • Neutropenia may lead to deadly infections - treat with prophylactic antivirals, antibacterial and antifungals
  • ALLOPURINOL (prevents tumour lysis syndrome)
  • IV fluids - insert Hickman line (permanent cannula into main vessel, tunnelled
    under sub-cutaneous fat so harder for infection to arise) so can easily take
    blood for testing and administer drugs and fluids
  • Chemotherapy
  • Marrow transplantation

for CML!!!!!! -

  • *ORAL IMATINIB -** specific BCR/ABL tyrosine kinase inhibitor BC more than 80% have philadelphia chormosome which has tyrose kinase activity
  • Stem cell transplant

For CLL!!!!
also HUMAN IV IMMUNOGLOBULINS

71
Q

Lymphoma: define

A

A malignant growth of WBC’s predominantly in the lymph nodes.

disorders caused by malignant proliferations of lymphocytes
accumulate in the lymph nodes causing LYMPHADENOPATHY (enlarged lymph nodes),

72
Q

Lymphoma: risk factors?

But also aetiology kinda

A
  1. Primary immunodeficiency.
  2. Secondary immunodeficiency e.g. HIV.
  3. Infection e.g. EBV!!!!!!, HTLV-1.
  4. Autoimmune disorders e.g. RA.

and FHx!!!!

73
Q

Lymphoma: pathophysiology?

A

There is impaired immunosurveillance and infected B cells escape regulation and proliferate. (This is just a theory).

74
Q

Lymphoma: general symptoms?

symptoms specific to hodgkins and non-hodgkins?????????????????????????

A
  1. Enlarged lymph nodes in arm/neck.
  2. Symptoms of compression syndromes.
  3. General systemic ‘B’ symptoms e.g. weight loss, night sweats, malaise.
  4. Liver and spleen enlargement.

hodgkin’s:

  1. Painless lymphadenopathy.
  2. Presence of ‘B’ symptoms e.g. night sweats, weight loss.

Non-hodgkin’s:
Nodal disease - superficial lymphadenopathy - pancytopenia (anaemia, infection)

75
Q

Lymphoma: investigations?

A

literally same as leukaemia!!

  1. Blood film.
  2. Bone marrow biopsy.
  3. Lymph node biopsy.
  4. Immunophenotyping.
  5. Cytogenetics.

(Raised lactose dehydrogenase = worse prognosis BC increased cell turnover and thus cell proliferation)

76
Q

What is needed for diagnosis of Hodgkins lymphoma?

A

Presence of Reed-sternberg cells.

77
Q

Hodgkin’s lymphoma: ann arbor classification?

A

I - Confined to single lymph node region

II - Involvement of two or more nodal areas on the same side of the diaphragm

III - Involvement of nodes on both sides
of the diaphragm

IV - Spread beyond the lymph nodes e.g. liver or bone marrow

  • Each Stage is either A or B:
    Stage A: no systemic symptoms other than pruritus (severe itching of skin)Stage B: presence of B symptoms such as fever, weight loss and night sweats
78
Q

Hodgkin’s lymphoma: management for stage 1-2A (<3 areas involved)?

Stages II-A to IV-B (with more than 3 areas involved):

A

ABVD
short course combination chemotherapy
followed by radiotherapy.

Longer course of ABVD

79
Q

Hodgkin’s lymphoma: complications of management?

GOT TO CARD 30 LYMPH

A
  1. Secondary malignancies - (radiotherapy may increase this risk)
  2. IHD. (radiotherapy may increase this risk)
  3. Infertility. (Chemo can cause this)
  4. Nausea. (Chemo)
  5. Alopecia. (Chemo)
80
Q

Non-Hodgkin’s lymphoma: describe grades

A
  • Low grade e.g. Follicular Lymphoma
    • Slow growing
    • Usually advanced at presentation
    • Incurable
    • Median survival 9-11 baso 10 years
  • High grade e.g. Diffuse Large B Cell Lymphoma
    • Example is Diffuse Large B-cell Lymphoma
    • Usually has nodal presentation
    • 1/3 cases have extranodal involvement
      Aggressive. Nodal presentation, patient unwell. Often curable.

Very high grade e.g. Burkitt’s Lymphoma

81
Q

Non-Hodgkin’s lymphoma: management for low grade vs high grade?

A

Low grade: If symptomless - do nothing. ——> Radiotherapy, combination chemotherapy and mAb may be used if symptomatic.

High grade:

  • Early: short course chemotherapy and radiotherapy. R-CHOP regimen
  • Advanced: combination chemotherapy and mAb.

R-CHOP regimen:
• R - RITUXIMAB (monoclonal antibody - minimal side effects)
• C - CYCLOPHOSPHAMIDE
• H - HYDROXY-DAUNORUBICIN
• O - VINCRISTINE (Oncovin brand name)
• P - PREDNISOLONE

rituximab =

  • Monoclonal antibody
  • Targets CD20 expressed on cell surface B cells
  • Chimeric mouse/human protein
  • Minimal side effects
82
Q

Hodgkin’s Lymphoma: epidemiology?

A
  • MALE predominance
  • Majority of cases occur in TEENAGERS e.g. 13-19 and ELDERLY e.g. over 65
  • 2 peaks of incidence; teenagers and elderly
83
Q

Myeloma: define

A

Malignancy of plasma cells leading to progressive bone marrow failure. It is associated with production of characteristic paraprotein, bone disease and renal failure.

84
Q

Myeloma: investigations?
—– in order to diagnose myeloma there MUST BE evidence of …?

What would you expect to see on the blood film taken from someone with myeloma?

A
  1. Blood film. Rouleaux formation (aggregations of RBC’s).
  2. Bone marrow aspirate and trephine biopsy. - Increased plasma cells.
  3. Electrophoresis. - Monoclonal protein band.
  4. X-ray.
  5. CT scan.
  6. MRI scan.
  7. Chromosomal abnormalities.
  8. Urine - (Immunoglobulin light chains with kappa or lamda lineage.)

must be evidence of mono-clonality
baso when =
Abnormal proliferation of a single clone of plasma cell leading to immunoglobulin secretion and causing organ dysfunction especially to the kidney.

on blood film:
Rouleaux formation (aggregations of RBC's).
85
Q

Myeloma: symptoms?

and signs?

A
  1. Tiredness.
  2. Bone/back pain.
  3. Infections.

CRAB!
1. Calcium is elevated.

  1. Renal failure.
  2. Anaemia. normochromic and normocytic!!!!
  3. Bone lesions.
86
Q

Symptomatic myeloma: management?

A

Chemotherapy, analgesia and bisphosphonates.
(VAD OR CTD CHEMO!!!)

Radiotherapy and bone marrow transplant can also be done

87
Q

Suggest 3 ways in which multiple myeloma can lead to AKI.

A
  1. Deposition of light chain.
  2. Hypercalcaemia.
  3. Hyperuricaemia.
88
Q

In extreme cases, patients with myeloma can present with blurred vision, gangrene and bleeding. What is the pathology behind this?

A

Paraproteins form aggregates in the blood and change the viscosity.

89
Q

Polycythaemia: define:

A

increased rbc levels due to overproduction by bone marrow

baso having a high concentration of red blood cells in your blood/increase in haematocrit

90
Q

Polycythaemia: divided into?

A

Primary cause:
Polycythaemia rubra vera - over reactive bone marrow.

Secondary Causes:

  1. Heavy smoking.
  2. Lung disease.
  3. Cyanotic heart disease.
  4. High altitude.
91
Q

Polycythaemia: investigations?

What 3 blood test values would be increased in someone with polycythaemia?

A
  • Blood count:
    • Raised white cell count (WCC) and increased platelets = distinguishes PV from other secondary causes
    • Raised Hb (major criteria)
    • raised haematocrit

Presence of JAK2 mutation on genetic screen (major criteria)

Bone marrow biopsy showing FIBROSIS

  1. Hb.
  2. RCC.
  3. PCV.
92
Q

Polycythaemia: symptoms?

A
  1. Itching. (after a hot bath)
  2. Headache.
  3. Dizziness.
  4. Visual disturbance. /blurred vision
  5. tired
  6. sweating
  7. Plethoric complexion (flushed skin)
  8. hepatosplenomegaly - due to extramedullary haemopoiesis - bc excess rbc build up in speen
  9. Gout and kidney stones
  10. more prone to blood clots
93
Q

Polycythaemia: management?

A
  • Venesection:
    • Aims to lower PCV and platelet count
    • Removal of 400-500mL of blood weekly to relieve symptoms

no cure

chemo

94
Q

polycythaemia: aetiology/pathophysiology?

A

normally kidneys produce erythropoietin which binds to haematopoietic stem cell AND ACTIVATES JAK2 GENE → and causes cell to divide and produces more RBC

BUT this mutation = the haematopoietic stem cells DIVIDE even in the absence of erythropoietin

and they become the predominant haematopoietic stem cells →» and HAEMATOCRIT INCREASES!

these haematopoietic stem cells start to die out - and SCAR TISSUE FORMS - THUS bone marrow can no longer produce rbcs

→ = ANAEMIA (low hb/rbc levels)

95
Q

VWD: define and Pathophysiology?

A

the most commoninheritedcause ofabnormal bleeding(haemophilia)

involve a deficiency, absence or malfunctioning of aglycoproteincalledVWF

96
Q

Tumour Lysis Syndrome: define

A

Break down of malignant cells ->

content release -> metabolic disturbances; can cause hyperuricaemia, hyperkalaemia, hypocalcaemia.

97
Q

Tumour Lysis Syndrome: risk factors?

A

—High tumour burden
—Pre-existing renal impairment
—inc age

—high grade disease

98
Q

Tumour Lysis Syndrome: management?

A
  • Aggressive hydration
  • ALLOPURINOL
    (xanthine oxidase inhibitor) or RASBURICASE (recombinant urate oxidase) both work to reduce uric acid production
  • Monitor electrolytes
99
Q

Thrombocytopenia: define

2 types?

A

This is a deficiency of platelets in the blood
<150000/ml^3
Can either be due to decreased production or increased destruction

ITP - immune thrombocytopenic purpura -

TTP - thrombotic thrombocytopenic purpura

100
Q

Thrombocytopenia: 2 causes?

A
  1. Production failure e.g. marrow suppression, marrow failure.
  2. Increased removal e.g. immune response (ITP), consumption (DIC), splenomegaly.
101
Q

ITP: define and Pathophysiology?

Aetiology?

A

due to immune destruction of platelets

Baso antibodies are produced against platelets - and they get destroyed by body ——> thus = purpura (red or purple spots on the skin caused by bleeding underneath skin)

The antibody-coated platelets are removed after they bind to Fc receptors on macrophages

IgG antibodies form to platelets and megakaryocytes

Aetiology: viral infections
Malignancy

102
Q

ITP: signs and symptoms?

A
  • Sudden self-limiting purpura (red or purple spots on the skin caused by bleeding underneath skin)
  • Easy bruising
  • easy bleeding
  • TKI
  • Epistaxis (nose bleed)
  • Menorrhagia (heavy menstruation)
  • Gum bleeding
103
Q

ITP: investigations?

management?

A
  • Platelet autoantibodies are detected in about 60-70% of patients, and are
    presumed to be present, although not detectable in the remaining patients;
    the antibodies often have specificity for platelet membrane glycoproteins
    IIb/IIIa and/or Ib
  • Bone marrow examination:
    • Shows thrombocytopenia with increased or normal megakaryocytes in
    the marrow
MANAGEMENT:
- First line:
    - Corticosteroids e.g. PREDNISOLONE
    - IV immunoglobulin e.g. IV IgG - raises platelet count more rapidly than
    steroids - thus useful for surgery
104
Q

TTP: define and Pathophysiology ?

A

Widespread adhesion and aggregation of platelets leads to microvascular thrombosis and thus consumption of platelets and thus profound thrombocytopenia

Baso when tiny blood clots develop throughout thesmall vesselsof the body using up platelets and causing thrombocytopenia, bleeding under the skin and other systemic issues. It affect the small vessels so it is described as amicroangiopathy.

blood clots develop due to a problem with a specific protein calledADAMTS13

This protein normallyinactivates von Willebrand factorand reducesplatelet adhesionto vessel walls andclot formation. A shortage in this protein leads to von Willebrand factor overactivity and the formation of blood clots in small vessels. This causes platelets to be used up leading to thrombocytopenia. The blood clots in the small vessels break up red blood cells, leading tohaemolytic anaemia.
ANAEMIA + THROMBOCYTOPENIA + AKI!!!

med 2a (13 decks)

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