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Yr 3: CN2 > Haem I > Flashcards

Haem I Flashcards

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1
Q
A
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2
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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3
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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4
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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5
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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6
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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7
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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8
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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9
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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10
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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11
Q
A

Hyposplenism

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12
Q
A

Hyposplenism

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13
Q
A

Hyposplenism

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14
Q

Autoimmune haemolytic anaemia is which type of hypersensitivity reaction?

Type I
Type II
Type III
Type IV

A

Type II

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15
Q

Describe the long-term managment of SCA [7]

A

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

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16
Q

Which drug used in SCA management targets P-selectin, preventing sticking of RBC to vessel walls

  • L-glutamine
  • hydroxycarbamide
  • crizanlizumab
  • voxelotor
A

Which drug used in SCA management targets P-selectin, preventing sticking of RBC to vessel walls

  • L-glutamine
  • hydroxycarbamide
  • crizanlizumab
  • voxelotor
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17
Q

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

A

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

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18
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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19
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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20
Q

What is the inheritance patten of thalassaemias? [1]

A

Both alpha and beta conditions are autosomal recessive

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21
Q

Write down the genotype of Beta thalassaemia minor, intermedia and major [3]

A

Minor: (b+/b OR b0/b)
Intermedia: (b+/b+)
Major: (b+/b0 OR b0/b0)

Mutation leading to absent production (0)
Mutation leading to reduced production (+)

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22
Q

Describe the diagnostic testing used to confirm the presence of beta thalassaemia [2]

A

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.

Compared to alpha thal: just genetic testing

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23
Q

When does screening for beta thalassaemia occur? [1]

What result would indicate B.T? [1]

A

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.

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24
Q

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

A

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

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25
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**
26
subtertian malaria (fever **< every 48hrs**) is caused by Plasmodium falciparum Plasmodium vivax Plasmodium ovale Plasmodium malariae Plasmodium knowlesi
**Plasmodium falciparum**
27
quartan malaria (fever spikes every 72 hours is caused by Plasmodium falciparum Plasmodium vivax Plasmodium ovale Plasmodium malariae Plasmodium knowlesi
**Plasmodium malariae**
28
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.
29
What determines if malaria is uncomplicated or complicated? [3]
Criteria for **uncomplicated** malaria includes: * **Parasitaemia < 2%** * **No** **schizonts** on film * **No clinical complications**
30
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
31
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.
32
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**
33
Which autoimmune conditions increase the liklihood of HL? [3]
* rheumatoid arthritis * systemic lupus erythematosus * sarcoidosis
34
What are the two types of HL? [2]
**Classical Hodgkin’s lymphoma (95%)** **Nodular lymphocyte-predominant Hodgkin’s lymphoma (5%)**
35
Describe the features of **nodular lymphocyte-predominant Hodgkin’s lymphoma (5%)** [4]
* More commonly affects **males** (75%) * **Not** associated with **EBV** * **Absence of Reed-Sternberg (RS) cells**, and is characterised by LP (“popcorn”) cells * Presents with peripheral adenopathy, and is the only type of Hodgkin's lymphoma that affects the **mesenteric lymph nodes**
36
Which of the following types of classical HL is the most common? Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
**Nodular sclerosis**
37
Which of the following types of classical HL has the worst prognosis? Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
Which of the following types of classical HL has the worst prognosis? **Lymphocyte-depleted**
38
Which of the following types of classical HL has the best prognosis? Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
Which of the following types of classical HL has the best prognosis? **Lymphocyte-rich**
39
Which of the following types of classical HL is most associated with HIV patients? Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
**Lymphocyte-depleted**
40
Which of the following types of classical HL is most associated with older patients Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
Which of the following types of classical HL is most associated with older patients **Lymphocyte-rich**
41
Which of the following types of classical HL is most associated with mediastinal lymphadenopathy and bulk nodes? Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
**Nodular sclerosis**
42
Which of the following types of classical HL is most associated with peripheral adenopathy and spleen involvement? Lymphocyte-depleted Nodular sclerosis Lymphocyte-rich Mixed cellularity
**Mixed cellularity**
43
Describe the treatment plan for **Stage I/II classical Hodgkin's lymphoma** [2] and **Stage III/ IV classical Hodgkin's lymphoma** [2]
**Stage I/II classical Hodgkin's lymphoma:** - **Chemotherapy** followed by **radiotherapy** - Main treatment regimen: **ABVD** (doxorubicin, bleomycin, vinblastine, dacarbazine) + **radiotherapy** **Stage III/ IV classical Hodgkin's lymphoma**: - **Chemotherapy** alone: **ABVD or BEACOPP** (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisolone)
44
Describe the treatment plan for **Stage I/ II nodular lymphocyte-predominant Hodgkin's lymphoma**: [1] and **Stage III/ IV nodular lymphocyte-predominant Hodgkin's lymphoma** [2]
**Stage I/ II nodular lymphocyte-predominant Hodgkin's lymphoma**: - **Involved-field** **radiotherapy** alone is the main treatment for this group of patients with excellent clinical benefit. **Stage III/ IV nodular lymphocyte-predominant Hodgkin's lymphoma**: - **R-CHOP** (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone) is administered in symptomatic patients or those with rapid disease progression . - **Regular** **monitoring** is adequate in **asymptomatic** patients.
45
Describe the management plan for HL in refractory disease [2]
**Chemotherapy followed by autologous stem cell transplantation (ASCT)** **Immunotherapeutic agents** can be considered: - **Brentuximab vedotin** - **nivolumab** - **pembrolizumab**
46
Describe the common complcations of HL [6]
Complications of Hodgkin's lymphoma are **usually chemotherapy or radiotherapy related.** **Thyroid**: - Around 50% of the patients who received radiotherapy experience symptoms of **hypothyroidism.** - **thyroid** **cancer** and **hyperthyroidism** also possible **Chemotherapy drugs**,: especially alkylating agents are associated with **secondary malignancies**: - **acute myeloid leukaemia** - **paraneoplastic syndrome** **Cardiac abnormalities**: - **doxorubicin** are at a higher risk of developing **cardiomyopathy** - **acute pericarditis** shortly after receiving radiotherapy - **valvular heart disease** or **coronary heart disease** in the long-term after radiotherapy **Pulmonary toxicity**: - **bleomycin-related pulmonary toxicity** - **Radiation-induced pneumonitis** **Infertility** :( **Infections**: - **Neutropenia** is common in patients taking chemotherapeutic regimens such as **ABVD**, and almost all patients receiving **BEACOPP** are neutropenic.
47
State where the following generally occur: [4] State for the following if they're generally aggressive or slow [4] - ALL - CLL - Lymphomas - Myelomas
ALL: - **Bone marrow** - **Aggressive** CLL: - **Blood** - **Slow** Lymphomas: - **Lymphoid tissue** - **Agrresive (high grade); slow (low grade)** Myelomas: - **BM** - **Slow**
48
What is the name for this skin condition related to NHL? [1]
**Mycosis fungoides**: skin lesions including an eczematous reaction which proceeds to form plaques, tumours, and fungating ulcers.
49
Which of the following are associated with *large abdominal mass and symptoms of bowel obstruction* Lymphoblastic lymphoma Burkitt's lymphoma Adult T-cell leukaemia-lymphoma Anaplastic large cell lymphoma
**Burkitt's lymphoma**
50
Which of the following are associated with *large mediastinal mass, SVC syndrome and cranial nerve palsies* Lymphoblastic lymphoma Burkitt's lymphoma Adult T-cell leukaemia-lymphoma Anaplastic large cell lymphoma
**Lymphoblastic lymphoma**
51
Which of the following are associated with: *fulminating clinical course with skin infiltrates, lymphadenopathy, hepatosplenomegaly, and leukaemia. May also be associated with symptoms of hypercalcemia* Lymphoblastic lymphoma Burkitt's lymphoma Adult T-cell leukaemia-lymphoma Anaplastic large cell lymphoma
**Adult T-cell leukaemia-lymphoma**
52
When would a lumbar puncture be indicated as a biospy in a NHL patient? [1] What is meant by Fluorescence in situ hybridisation (FISH) and when is it used? [1] What further tests would you perform if positive? [1]
**Lumbar puncture:** - In patients with **suspected CNS disease, cytology and flow cytometry** may be required. **Fluorescence in situ hybridisation (FISH)**: - NICE advise using FISH to **identify MYC rearrangement** (**Burkitt lymphoma)** in those with **high-grade B-cell lymphoma**. - If found, further testing to **identify BCL2 and BCL6** **rearrangements** and the immunoglobulin partner should be arranged.
53
What is the commonly used chemotherapy regimen used in NHL? [5] Describe the MoA of each [5]
**R-CHOP**: **Rituximab**: * A **monoclonal antibody** with activity against **CD20** - an antigen found on the surface of B-cells. **Cyclophosphamide**: - An **alkylating** **agent**, **inhibits DNA synthesis** through cross linking of DNA. **Doxorubicin**: - An **anthracycline** that **inhibits** **topoisomerase** **II** leading to **inhibition of DNA and RNA synthesis**. **Vincristine**: - **Inhibits microtubule formation** by binding to **tubulin**. **Prednisolone** - a glucocorticoid steroid.
54
State common side effects of each of **R-CHOP** [5]
**R-CHOP**: **Rituximab**: * infusion reactions * hepatitis B reactivation * mucocutaneous reactions **Cyclophosphamide**: - transitional cell carcinoma of the bladder - bone marrow suppression - infertility. **Doxorubicin**: - cardiomyopathy - myelosuppresion - skin reactions. **Vincristine**: - peripheral neuropathy - bladder atony. **Prednisolone** - steroid effects
55
Which of the following is most likley to have caused bone marrow suppression? Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
Which of the following is most likley to have caused bone marrow suppression? Rituximab **Cyclophosphamide** Doxorubicin Vincristine Prednisolone
56
Which of the following is most likley to have caused peripheral neuropathy? Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
Which of the following is most likley to have caused peripheral neuropathy? **Vincristine**
57
Which of the following is most likley to have caused bladder atony? Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
**Vincristine**
58
Which of the following is most likley to have caused Hep B reactivation Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
**Rituximab**
59
Which of the following is most likley to cause infertility? Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
Which of the following is most likley to cause infertility? Rituximab **Cyclophosphamide** Doxorubicin Vincristine Prednisolone
60
Which of the following is most likley to cause an infusion reaction? Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
**Rituximab**
61
Describe the common presentation of the following types of NHL [3] - Diffuse large B cell lymphoma (DLBCL) [3] - Follicular lymphoma [1] - Burkitt’s lymphoma [1]
**Diffuse large B cell lymphoma (DLBCL):** - **rapidly enlarging mass**, commonly in the **neck**, **abdomen** or **mediastinum** - **Extranodal** is common in **GI tract** - **Disease** in the **mediastinum** may lead to **SVCO** **Follicular lymphoma**: - **insidious manner** with gradually worsening, **painless lymphadenopathy** **Burkitt’s lymphoma:** - rapidly enlarging **tumour** in the **jaw** of a **child** - associated with **EBV**
62
Describe the treatments for the following types of NHL [3] (in both local and advanced stages diseases) - Diffuse large B cell lymphoma (DLBCL) [2] - Follicular lymphoma [2] - Burkitt’s lymphoma [1]
**Diffuse large B cell lymphoma (DLBCL):** * **Limited stage disease**: **R-CHOP**; Combined modality therapy (**CMT**) may be used where **chemoimmunotherapy is combined with radiotherapy.** * **Advanced stage disease**: Treatment may involve **R-CHOP** or other regimens such as (da)-**EPOCH-R.** **Follicular lymphoma:** - **Limited stages**: **local radiotherapy** - **Advanced disease**: **immunotherapy** with **rituximab** if **asymptomatic**. If **symptomatic**: **RCHOP** **Burkitt’s lymphoma** - **Intensive chemotherapy**
63
Any patient suffering from NHL who exhibits neutropenia should be given what treatment? [1]
Any patient with severe neutropenia should be given **antibiotic prophylaxis with chemotherapy**
64
Describe what is meany by tumour lysis syndrome [1] What electrolyte disturbances may this cause? [5]
**Tumour lysis syndrome** commonly occurs after treatment of high-grade lymphomas; when tumor cells **release** their **contents** into the **bloodstream**, either spontaneously or in response to therapy * **hyperuricaemia** * **hyperkalaemia** * **hyperphosphataemia** * **hypocalcaemia** * **acute kidney injury**
65
Which of the following is most likley to cause hypogammaglobulinaemia? Rituximab Cyclophosphamide Doxorubicin Vincristine Prednisolone
Which of the following is most likley to cause hypogammaglobulinaemia? **Rituximab**
66
Which lymph nodes does Burkitt's lymphoma typically present in? [1]
**Abdomen and mesenteric** lymph nodes

Yr 3: CN2 (29 decks)

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