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Yr3 Revision: Condensed Notes > Haem I > Flashcards

Haem I Flashcards

1
Q

What is essential thrombocytosis and what investigations / results help to differ it from secondary thrombocytosis? [2]

A

Essential:
- Dysregulated megakaryocyte proliferation
- Platelet count consistently high

Secondary:
- Triggered by infection, trauma, bleeding, hyposplenism
- Transiently raised platelet count

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2
Q

Describe how you manage essential thrombocytosis (depending if they are low or high risk)

A

Low risk = > 40 OR platelet count < 1500, no hx of thrombosis or haemorrhage. no CV risk
- Aspirin alone

High risk: > 60 OR DM/HTN; platelet count > 1500; Hx of thrombosis or haemorrhage, CV risk
- Hydroxycarbamide and aspirin

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3
Q

How do you differentiate between an aplastic and sequestration crisis in SCA? [1]

A

Aplastic: reduced reticulocytes

Seq. crisis: increased reticulocytes

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4
Q

What is TPO? [1]
Where is it produced? [1]
What does it regulate ? [1]

A

Thrombopoietin (TPO):
TPO is produced by the liver and essential for the control of platelet production

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5
Q

Name 5 drugs which cause macrocytic anaemia?

A

Azathioprine
Methotrexate
Fluorouracil
Phenobarbital
Mercaptopurine
Trimethoprim

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6
Q

What are specific signs associated with anaemia of iron deficiency? [4]

A
  • Koilonychia (spoon shaped nails)
  • Angular stomatitis (inflammation of corners of mouth)
  • Restless legs syndrome
  • Hair loss
  • Post-cricoid webs
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7
Q

What do raised ferritin levels indicate? [4]

A

Inflammation (e.g., infection or cancer)
Liver disease
Iron supplements
Haemochromatosis

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8
Q

Describe the management of IDA [3]

A

1ST LINE
- oral iron replacement (ferrous sulfate)
- 200 mg once daily

2ND LINE
- intravenous iron replacement

3rd LINE:
- Blood transfusion

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9
Q

Describe how Fe tests would help to diagnose ACD [3]

A

The clinical presentation of ACD is generally that of the underlying disorder

Serum ferritin:
- Normal or raised (due to release during inflammation)

Serum iron:
- Low

TIBC:
- Low

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10
Q

What are specific signs associated with anaemia of vit. B12 deficiency? [7]

A
  • Glossitis
  • Positive Rombergs test & neurological impairment - posterior column degeneration
  • Decreased vibration sense - posterior column degeneration
  • Ataxia - posterior column degeneration
  • Hyperpigmentation of nails
  • Petechiae: generally a late sign of vitamin B12 deficiency.
  • Optic neuropathy
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11
Q

Describe the clinical features of pernicious anaemia

A

Peripheral neuropathy, with numbness or paraesthesia (pins and needles)
mild jaundice: combined with pallor results in a ‘lemon tinge’
Loss of vibration sense
Loss of proprioception
Visual changes
Mood and cognitive changes

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12
Q

Describe the treatment regime for pernicious anaemia

A

Intramuscular hydroxocobalamin is initially given to all patients with B12 deficiency, depending on symptoms:

No neurological symptoms
- 3 times weekly for two weeks

Neurological symptoms
- alternate days until there is no further improvement in symptoms

MAINTENANCE:

Pernicious anaemia
– 2-3 monthly injections for life of intramuscular hydroxocobalamin

Diet-related:
- oral cyanocobalamin or twice-yearly injections

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13
Q

Which investigations are used to diagnose folate deficiency? [1]

A

Red cell folate is a better measure of levels than serum folate, since levels are affected even with a short period of deficiency.

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14
Q

How do you treat folate deficiency? [1]

A

Folic acid is usually given as a once daily oral dose of 5 mg for up to four months.

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15
Q

What advice should you give pregnant women regarding folic acid intake? [1]

Folate deficiency causes an increased risk of which pathology? [1]

A

all women should take 400mcg of folic acid until the 12th week of pregnancy

Risk of neural tube defects

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16
Q

Define what is meant by: [3]

  • Hereditary spherocytosis
  • Hereditary elliptocytosis
A

Hereditary spherocytosis
- Autosomal dominant familial condition; autoantibodies produced against proteins in RBC membrane mean it causes a spherical shape when they pass through the spleen
- Can still perform adequately despite reduced SA
- Fragile and easily lysed

Hereditary elliptocytosis:
- Autosomal dominant familial condition; autoantibodies produced against proteins in RBC membrane mean it causes an ellipse shape
- Can still perform adequately despite reduced SA
- Fragile and easily lysed

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17
Q

Describe a patient with hereditary spherocytosis may present [4]

A
  • Jaundice at birth
  • However the onset of jaundice can be delayed for many years and some
    patients may go through life with no symptoms and are detected only during
    family studies
  • May eventually develop anaemia
  • Splenomegaly
  • Ulcers on the leg
  • Chronic haemolysis leads to the formation of gall stones
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18
Q

What investigations would indicate either hereditary spherocytosis or hereditary elliptocytosis? [3]

A
  • Raised mean corpuscular haemoglobin concentration (MCHC) on a full blood count
  • Raised reticulocyte count due to rapid turnover of red blood cells
  • Spherocytes / elpitocytes on a blood film
  • EMA testing and cryohaemolysis test if results unequivocal from blood film
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19
Q

What is the treatment of hereditary spherocytosis or hereditary elliptocytosis? [5]

A
  • Folate supplementation- indicated in moderate to severely affected individuals or those who are pregnant to avoid megaloblastic anaemia due to the relative folate deficiency that occurs when the rate of RBC production increases to compensate for the increased rate of haemolysis.
  • Blood transfusions when required
  • Splenectomy.
  • Erythropoietin may reduce the need for transfusions in young infants until they can mount an adequate hematopoietic response to the haemolysis.
  • Gallbladder removal (cholecystectomy) may be required if gallstones are a problem.
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20
Q

Describe the presentation of G6PD deficiency [4]

How can you diagnose this pathology? [1]

A

G6PD deficiency presents with:
- jaundice (often in the neonatal period)
- gallstones
- anaemia
- splenomegaly
- Heinz bodies on a blood film.

Diagnosis can be made by doing a G6PD enzyme assay.

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21
Q

Describe how you would treat a patient with hereditary spherocytosis in:

  • neonates [2]
  • infants (>28 days old), children, and adults [5]
A

Neonates:
- 1st line: supportive care +/- red blood cell transfusions
- 2nd line: folic acid supplementation

infants (>28 days old), children, and adults
- 1st line: supportive care +/- red blood cell transfusions
- 2nd line: folic acid supplementation
- 3rd line: splenectomy with pre-op vaccination regimen
- Consider: cholecystectomy or cholecystostomy
- Plus: post-splenectomy antibiotic pneumococcal prophylaxis

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22
Q

In the diagnosis of aplastic anemia, which parameter is typically reduced in the peripheral blood count?

A. Reticulocyte count
B. White blood cell count
C. Platelet count
D. Hematocrit

A

In the diagnosis of aplastic anemia, which parameter is typically reduced in the peripheral blood count?

A. Reticulocyte count
B. White blood cell count
C. Platelet count
D. Hematocrit

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23
Q
A

Mycoplasma pneumoniae

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24
Q
A

SCA

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25
Hyposplenism
26
**Hyposplenism**
27
Hyposplenism
28
Hyposplenism
29
Autoimmune haemolytic anaemia is which type of hypersensitivity reaction? Type I Type II Type III Type IV
**Type II**
30
State 4 acute clinical presentations of SCA [6]
* Vaso-occlusive crises * Acute chest syndrome * Pulmonary hypertension * Anaemia * Priapism * Splenic sequestration crisis
31
Describe the management for an acute chest syndrome presentation [6]
1st line: - **Oxygen** & **Incentive spirometry** using a machine that encourages effective and deep breathing, **prevents atelectasis** Plus: - **Analgesia** Plus: - **broad-spectrum antibiotics** (because bacterial pneumonia cannot always be ruled out) Consider: - **Antihistamine** (many opioids cause pruritus, which should be managed with an oral antihistamine) Consider: - **Blood transfusion** Consider: - **Hydration** | BMJ BP
32
Describe the management for an vaso-occlusive crisis presentation [6]
1st line: - **Analgesia** Plus: - **Supportive care** Plus: - **broad-spectrum antibiotics** Consider: - **Antihistamine** Consider: - **Blood transfusion** Consider: - **Hydration**
33
Describe the following chronic complications of SCA [4] * **Anaemia** * **Infections** * **Leg ulcers** * **Growth retardation** and delayed puberty
**Anaemia**: - **chronic haemolytic anaemia** is a constant feature in sickle-cell patients, characterized by pallor, fatigue, and exertional dyspnoea. **Infections**: - **Functional asplenia** due to **recurrent splenic infarctions** increases susceptibility to encapsulated bacterial infections, such as **Streptococcus pneumoniae and Haemophilus influenzae.** **Leg ulcers:** - **Chronic venous insufficiency** resulting from **vaso-occlusion** can lead to **non-healing leg ulcers,** predominantly around the medial malleoli. **Growth retardation** and delayed puberty: - due to **chronic** **hypoxia** and **undernutrition** related to **increased metabolic demands** from their condition.
34
Describe the following chronic complications of SCA [4] * **Ocular** complications * **Skeletal** complications * **Renal** complications * **Cardiopulmonary** complications * **Neurological** complications
**Ocular complications**: - Retinal vessel occlusions may cause **proliferative retinopathy**, **vitreous** **haemorrhage** or **retinal** **detachment** leading to vision loss. **Skeletal** **complications**: - **Chronic** **bone** **infarcts** can result in **avascular necrosis of the femoral** and **humeral** **heads**. - **Marrow hyperplasia** may cause **osteopenia** and **pathologic fractures**. **Renal** **complications**: - Repeated renal medullary ischemia predisposes patients to **papillary necrosis** and **renal tubular dysfunction** - can manifest as **nocturia, polyuria, proteinuria** or even **chronic kidney disease.** **Cardiopulmonary** **complications**: - **pulmonary hypertension** and eventually **right-sided heart failure** (cor pulmonale). **Neurological** **complications**: - **ischemic** or **hemorrhagic** **strokes** - **Silent cerebral infarcts** are also common findings on neuroimaging studies.
35
Describe the long-term managment of SCA [7]
1ST LINE: - **supportive care + prevention of complications**: e.g. **pneumococcal** **immunisation, antibiotic prophylaxis** with **penicillin** in children under 5 years of age, nutritional counselling CONSIDER * **hydroxycarbamide**: works by stimulating the production of fetal haemoglobin (HbF). CONSIDER * **L-glutamine**: CONSIDER * **crizanlizumab**: is a monoclonal antibody that targets **P-selectin**. P-selectin is an adhesion molecule found on endothelial cells on the inside walls of blood vessels and platelets. Prevents red blood cells from sticking to the blood vessel wall and reduces the frequency of vaso-occlusive crises. CONSIDER * **voxelotor** CONSIDER * **repeated blood transfusions** 2ND LINE - **haematopoietic stem cell transplantation**
36
Which drug used in SCA management targets P-selectin, preventing sticking of RBC to vessel walls * L-glutamine * hydroxycarbamide * crizanlizumab * voxelotor
Which drug used in SCA management targets P-selectin, preventing sticking of RBC to vessel walls * L-glutamine * hydroxycarbamide * **crizanlizumab** * voxelotor
37
According to NICE guidelines, which laboratory test is considered the gold standard for confirming the diagnosis of sickle cell anemia? a) Complete Blood Count (CBC) b) Hemoglobin Electrophoresis c) Serum Ferritin d) Coagulation Profile
According to NICE guidelines, which laboratory test is considered the gold standard for confirming the diagnosis of sickle cell anemia? a) Complete Blood Count (CBC) **b) Hemoglobin Electrophoresis** c) Serum Ferritin d) Coagulation Profile
38
What is the inheritance patten of thalassaemias? [1]
Both alpha and beta conditions are **autosomal recessive**
39
Describe the different types of alpha thalassaemias [4]
**Alpha thalassemia minima:** - 1 defective alpha subunit - Silent carriers: slightly reduced / normal MCV but no clinical symptoms - Hypochromic and microcytic anaemia **Alpha thalassemia minor:** - 2 defective alpha subunits - Hypochromic and microcyticnaemia - The remaining two alpha genes produce nearly normal levels of RBCs **HbH disease:** - 3 defective alpha subunits - Excess beta chains causes RBC membrane damage and intramedullary haemolysis AND does not release oxygen to tissues, causing increased RBC production **Hydrops fetalis, Bart's hydrops (γ4)**: - 4 defective alpha subunits - Extreme affinity to O2: incompatible with life
40
What is the management of thallassemia major? [4]
Management involves; - **regular transfusions** - **iron chelation** - **splenectomy** - A **bone** **marrow** **transplant** can be curative.
41
Write down the genotype of Beta thalassaemia minor, intermedia and major [3]
**Minor:** (b+/b OR b0/b) **Intermedia**: (b+/b+) **Major**: (b+/b0 OR b0/b0) ## Footnote *Mutation leading to absent production (0)* *Mutation leading to reduced production (+)*
42
Describe the bone deformities that can occur in B.T major [4]
**Unusual** **bone** **formation**: * **Facial deformities**: frontal bossing, maxilla overgrowth, prominence of upper incisors, ‘chipmunk’ facies, dental malocculsion. * **Body habitus changes**: typically short limbs due to early fusion of epiphyses. Skull, pelvis, ribs and spinal changes may be seen * **Osteopaenia/osteoporosis** * **Bone pain**
43
Describe how beta thalassaemia interacts with Fe levels in the body [2]
**Ineffective erythropoiesis** leads to an **increase in iron absorption** from the **gastrointestinal** **tract** that is **compounded by regular blood transfusions**.
44
Describe the diagnostic testing used to confirm the presence of beta thalassaemia [2]
**Haemoglobin analysis**: - completed using **haemoglobin electrophoresis or high-performance liquid chromatography (HPLC)**. Electrophoresis causes different types of haemoglobin to separate into bands. HPLC is an alternative method of determining the types of haemoglobin in blood. - Patients with beta thalassaemia will have an **increased proportion of HbA2 and HbF** due to the **absence of beta globin chains**. Even in beta thalassaemia minor, there will be an elevation in HbA2. **Genetic testing:** - DNA testing provides a **definite** and **precise** **diagnosis** of beta **thalassaemia**. It is able to determine the type of mutation present. ## Footnote *Compared to alpha thal: just genetic testing*
45
When does screening for beta thalassaemia occur? [1] What result would indicate B.T? [1]
**Antenatal screening** is offered to all pregnant women within the UK. It involves concurrent assessment of different haemoglobinopathies (i.e. thalassaemia and haemoglobin variants) at **10 weeks gestation.** In beta thalassaemia, the level of HbA2 is quantified. **Levels of HbA2 >3.5%** is suggestive of being a beta thalassaemia carrier and further analysis of the father is required to determine the risk of beta thalassaemia in the fetus.
46
Describe the managment of beta-thalassaemia intermedia: non-transfusion-dependent [3]
1ST LINE: - **Transfusions at times of symptomatic anaemia** *Patients with beta-thalassaemia intermedia do not usually require regular transfusions (non-transfusion-dependent thalassaemia). They are able to grow and develop at a nearly normal rate despite the moderate anaemia.* *Occasionally, patients become severely anaemic and develop symptoms as a result. **This usually occurs at times of major stress to the body**, such as perioperatively, or during a serious illness or infection.* CONSIDER - **iron monitoring + chelation** with **desferrioxamine or deferasirox** PLUS - **genetic counselling**
47
Describe the managment of beta-thalassaemia intermedia: transfusion-dependent AND beta-thalassaemia major [4]
1ST LINE: - **Regular transfusions** PLUS - **iron monitoring + chelation** with **desferrioxamine or deferasirox** PLUS - **genetic counselling** CONSIDER - **Splenectomy** CONSIDER - **Assessment for stem cell transplantation**
48
Vaccinations agaisnt with pathogens are recommended in splenectomy patients? [4]
Vaccinations against **S. pneumoniae, N. meningitidis, H. influenzae type b and influenza virus** are strongly recommended.
49
Name some pathologies that cause hyposplenism [5]
**sickle cell anaemia** (chronic damage to the spleen results in atrophy) **coeliac disease** **dermatitis herpetiformis** **essential thrombocythaemia** **ulcerative colitis**.
50
What are 4 blood film findings of hyposplenism [4]
Blood film: features of hyposplenism include **Howell-Jolly bodies, Pappenheimer bodies, target cells and irregular contracted red blood cells.**
51
In alpha thalassemia, the Hemoglobin H (HbH) disease results from the deletion of three alpha-globin genes. What is the recommended treatment for patients with HbH disease, according to NICE? a) Blood transfusion b) Hydroxyurea c) Folic Acid supplementation d) Hematopoietic stem cell transplantation
**c) Folic Acid supplementation**
52
NICE recommends screening for alpha thalassemia in newborns. What is the primary screening test used for this purpose? a) Hemoglobin electrophoresis b) Complete Blood Count (CBC) c) DNA analysis d) Serum Ferritin
NICE recommends screening for alpha thalassemia in newborns. What is the primary screening test used for this purpose? a) Hemoglobin electrophoresis b) Complete Blood Count (CBC) **c) DNA analysis** d) Serum Ferritin
53
According to NICE guidelines, which diagnostic test is recommended for confirming the diagnosis of beta thalassemia major? a) Complete Blood Count (CBC) b) Hemoglobin electrophoresis c) Serum Ferritin d) Molecular genetic testing
**d) Molecular genetic testing**
54
subtertian malaria (fever **< every 48hrs**) is caused by Plasmodium falciparum Plasmodium vivax Plasmodium ovale Plasmodium malariae Plasmodium knowlesi
**Plasmodium falciparum**
55
quartan malaria (fever spikes every 72 hours is caused by Plasmodium falciparum Plasmodium vivax Plasmodium ovale Plasmodium malariae Plasmodium knowlesi
**Plasmodium malariae**
56
State 3 signs of malaria [3]
**Pallor** (anaemia) **Splenomegaly** **Jaundice** (due to haemolysis) **TOM TIP**: The most characteristic symptom of malaria is the fever, which spikes very high every 48 hours. In someone with an unexplained fever, consider whether they have travelled somewhere with malaria present. Even exposure several years ago may be relevant, as P. vivax and P. ovale can lie dormant for up to 4 years.
57
What determines if malaria is uncomplicated or complicated? [3]
Criteria for **uncomplicated** malaria includes: * **Parasitaemia < 2%** * **No** **schizonts** on film * **No clinical complications**
58
Describe the management plan of uncomplicated P. falciparum malaria [2]
**First-line options:** * **Riamet** (**Artemether** + **lumefantrine**): four tablets orally at 0, 8, 24, 36, 48 and 60 hours, OR * **Malarone** **(atorvaquone + progunail)**: 4 tablets daily for 3 days **Second-line options:** * **Quinine sulphate** (600 mg TDS) + **Doxycycline** (200 mg OD): 7 days, OR * **Quinine sulphate** (600 mg TDS) + **Clindamycin** (450 mg TDS): 7 days
59
Describe the management plan of complicated P. falciparum malaria [3]
**First-line options:** * **Artesunate**: 2.4 mg/kg intravenous at 0, 12, 24 hours then once daily. Always follow local guidelines on prescribing. * If unavailable, may require **quinine** initially until **artesunate** is acquired. **Second-line options:** * **Quinine dihydrochloride**: Requires dose adjustment in renal and hepatic impairment and cardiac monitoring duration administration. Follow local guidelines. **Follow-on therapy (once improved and able to tolerate oral tablets):** * **Riamet (Artemether + lumefantrine)**: four tablets orally at 0, 8, 24, 36, 48 and 60 hours, OR * **Doxcycline**: 200 mg once daily for 7 days, OR * **Clindamycin**: 450 mg three times a day for 7 days (preferred in pregnancy) ## Footnote **TOM TIP**: Remember artesunate and quinine as treatment options for your exams, as these are the most likely to be relevant. Remember that Plasmodium falciparum is the most common and severe cause, and these patients should be admitted for artesunate treatment and monitoring for complications.
60
Describe the treatment of of non-falciparum malaria or mixed [1]
**Non-falciparum malaria** should be discussed with **microbiology and local guidelines followed**. Patients are usually able to be managed as **outpatients**. Treatment is usually with **chloroquine**, but this depends on resistance patterns. **Follow-on treatment** is typically required for P. vivax and P. ovale.
61
Explain which genetic disorder you need to consider when treating malaria?
**Glucose-6-phosphate deficiency (G6PD)** *(red cell lysis when erythrocytes are put under oxidative stress)* because **antimalarials**, can induce red cell haemolysis. Specifically: * **Primaquine** (definite risk of haemolysis, G6PD needs to be excluded before use) * **Chloroquine** (possible risk of haemolysis, acceptable in acute malaria) * **Quinine** (possible risk of haemolysis, acceptable in acute malaria)
62
Describe the complications of malaria
Severe malaria is **invariably fatal** if left untreated. Also: **Cerebral malaria** (mortality of 50%) ARDS DIC AKI Severe anaemia Septic shock Splenic rupture Multi-organ failure Death
63
Which medications can be used as prophylaxis for malaria [4]
**Proguanil** with **atovaquone** (Malarone) **Doxycycline** **Mefloquine** (risk of psychiatric side effects) **Chloroquine** with **proguanil** (less often used due to high resistance)
64
Name 3 side effects of doxycyline treatment [3]
**diarrhoea** **thrush** **skin sensitivity** to sunlight
65
Which of the following is the most effective form of malaria prophylaxis Doxycycline Proguanil with atovaquone (Malarone) Mefloquine Chloroquine with proguanil
Which of the following is the most effective form of malaria prophylaxis Doxycycline **Proguanil with atovaquone (Malarone)** Mefloquine Chloroquine with proguanil
66
Which of the following has a risk of pysc side effects Doxycycline Proguanil with atovaquone (Malarone) Mefloquine Chloroquine with proguanil
**Mefloquine**: causes anxiety, depression and abnormal dreams.
67
Vaccinations against **S. pneumoniae, N. meningitidis, H. influenzae type b and influenza virus** are strongly recommended for asplenic patients. Which of the above is the most likely infection for asplenia? [1]
**S. pneumoniae**

Yr3 Revision: Condensed Notes (51 decks)

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