Haem I Flashcards
What is essential thrombocytosis and what investigations / results help to differ it from secondary thrombocytosis? [2]
Essential:
- Dysregulated megakaryocyte proliferation
- Platelet count consistently high
Secondary:
- Triggered by infection, trauma, bleeding, hyposplenism
- Transiently raised platelet count
What are specific signs associated with anaemia of iron deficiency? [4]
- Koilonychia (spoon shaped nails)
- Angular stomatitis (inflammation of corners of mouth)
- Restless legs syndrome
- Hair loss
- Post-cricoid webs
Describe how Fe tests would help to diagnose ACD [3]
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
What are specific signs associated with anaemia of vit. B12 deficiency? [7]
- 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
Describe the treatment regime for pernicious anaemia
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
Which investigations are used to diagnose folate deficiency? [1]
Red cell folate is a better measure of levels than serum folate, since levels are affected even with a short period of deficiency.
How do you treat folate deficiency? [1]
Folic acid is usually given as a once daily oral dose of 5 mg for up to four months.
Describe how you would treat a patient with hereditary spherocytosis in:
- neonates [2]
- infants (>28 days old), children, and adults [5]
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
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
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
Hyposplenism
Hyposplenism
Hyposplenism
Autoimmune haemolytic anaemia is which type of hypersensitivity reaction?
Type I
Type II
Type III
Type IV
Type II
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
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
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
Describe how you manage essential thrombocytosis (depending if they are low or high risk)
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
How do you differentiate between an aplastic and sequestration crisis in SCA? [1]
Aplastic: reduced reticulocytes
Seq. crisis: increased reticulocytes
What is the inheritance patten of thalassaemias? [1]
Both alpha and beta conditions are autosomal recessive
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)
Mutation leading to absent production (0)
Mutation leading to reduced production (+)
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.
Compared to alpha thal: just genetic testing
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.
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
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
subtertian malaria (fever < every 48hrs) is caused by
Plasmodium falciparum
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae
Plasmodium knowlesi
Plasmodium falciparum
quartan malaria (fever spikes every 72 hours is caused by
Plasmodium falciparum
Plasmodium vivax
Plasmodium ovale
Plasmodium malariae
Plasmodium knowlesi
Plasmodium malariae
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.
What determines if malaria is uncomplicated or complicated? [3]
Criteria for uncomplicated malaria includes:
* Parasitaemia < 2%
* No schizonts on film
* No clinical complications
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
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.
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
Which autoimmune conditions increase the liklihood of HL? [3]
- rheumatoid arthritis
- systemic lupus erythematosus
- sarcoidosis
What are the two types of HL? [2]
Classical Hodgkin’s lymphoma (95%)
Nodular lymphocyte-predominant Hodgkin’s lymphoma (5%)
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
Which of the following types of classical HL is the most common?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Nodular sclerosis
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
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
Which of the following types of classical HL is most associated with HIV patients?
Lymphocyte-depleted
Nodular sclerosis
Lymphocyte-rich
Mixed cellularity
Lymphocyte-depleted
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
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
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
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)
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.
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
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.
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
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.
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
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
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
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.
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.
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
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
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
Which of the following is most likley to have caused bladder atony?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Vincristine
Which of the following is most likley to have caused Hep B reactivation
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Rituximab
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
Which of the following is most likley to cause an infusion reaction?
Rituximab
Cyclophosphamide
Doxorubicin
Vincristine
Prednisolone
Rituximab
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
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
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
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
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
Which lymph nodes does Burkitt’s lymphoma typically present in? [1]
Abdomen and mesenteric lymph nodes
