Haematology Flashcards
(86 cards)
1
Q
Where does haematopoiesis occur in adults?
A
In bone marrow.
spleen and liver in disease states.
2
Q
What controls erythrocyte production and maturation?
A
EPO (Erythropoietin).
3
Q
What percentage of erythropoietin is produced in the kidney?
A
90%.
4
Q
What is hemoglobin?
A
A protein produced in the mitochondria of erythrocytes that carries O2.
5
Q
List the causes of anaemia.
A
- Increased blood cell loss (acute bleeding, malignancy, GI losses, menorrhagia)
- Decreased production (Iron deficiency, B12 and folate deficiency, reduced EPO, aplastic anaemia)
- Increased breakdown (haemolytic anaemias)
- Increased plasma volume (pregnancy and heart failure)
6
Q
What are the types of anaemia?
A
- Microcytic
- Macrocytic
- Normocytic
- Aplastic
- Haemolytic
7
Q
What characterizes microcytic red cells?
A
Microcytic (<80fL) Red Cells
Hypochromic (MCH <27)
Poikilocytosis (varying shape)
Anisocytosis.(varying size)
8
Q
What are some causes of microcytic anaemia?
A
- Fe Deficiency - Dietary
- Absorption - coeliac/gastrectomy
- Blood Loss - Menorrhagia, GI
- Increase Demand - Growth/pregnancy
- Thalassaemia Trait
- Sideroblastic Anaemia
9
Q
What is the blood film appearance of macrocytic anaemia?
A
Oval Macrocytes > 96fL.
10
Q
What conditions can lead to megaloblastic macrocytic anaemia?
A
- Low B12
- Folate
- Hyper segmented neutrophils >6 lobes per nucleus.
11
Q
What is a megaloblast?
A
An immature erythroblast caused by decreased DNA synthesis.
12
Q
What characterizes normocytic anaemia?
A
Reduced red cell count with normal morphology.
13
Q
List some causes of normocytic anaemia.
A
- Anaemia of chronic disease
- Acute blood loss
- Hypothyroidism
- Uraemia
- Pancytopenia.
14
Q
What is aplastic anaemia?
A
Absence of reticulocytes.
15
Q
What are the primary causes of aplastic anaemia?
A
- Idiopathic (70%)
- Genetic - Fanconi’s anaemia.
16
Q
List some secondary causes of aplastic anaemia.
A
- Chemical - benzene
- Drugs - Chemotherapy, Azathioprine, Carbamazepine
- Infections - EBV, HIV.
17
Q
What is polychromasia?
A
Increased reticulocytes that stain light blue. Seen in haemolytic anaemia
18
Q
What are some inherited causes of haemolytic anaemia?
A
- Membrane - Spherocytosis, Elliptocytosis
- Hb - Thalassaemia and Sickle Cell
- Metabolic - G6PD Deficiency.
19
Q
List some acquired causes of haemolytic anaemia.
A
To have awareness, not know all detail
- Immune - Warm and cold agglutinin, transfusion reaction, organ rejection, haemolysis of newborn
- Non-immune - Valve prosthesis, hypersplenism, burns, malaria, renal and liver failure.
20
Q
What genetic inheritance pattern is associated with Sickle Cell Disease?
A
Autosomal recessive
Sickle Cell Disease is caused by the inheritance of two copies of the HbS gene, one from each parent. (HbSS). If heterozygous then only sickle cell trait (HbSA)
21
Q
What are common complications of Sickle Cell Disease?
A
Vaso-occlusive crises, infection, bleeding
These complications arise due to the sickling of red blood cells which can obstruct blood flow and sickle cells get sequestered and can lead to aplastic anaemia.
22
Q
What laboratory test is used to screen for Sickle Cell Disease in antenatal settings?
A
Hb electrophoresis
This test helps identify the presence of different hemoglobin types, including HbS.
23
Q
What is the typical age group for the diagnosis of Chronic Lymphocytic Leukaemia (CLL)?
A
Leukaemia of old age
24
Q
What is a key symptom of Chronic Myeloid Leukaemia (CML)?
A
Very high WCC
WCC stands for white cell count, which can be significantly elevated in CML.
25
What does the acronym CRAB stand for in the context of Multiple Myeloma?
Calcium raised, Renal failure, Anaemia, Bone pain
## Footnote
These are the common clinical features of Multiple Myeloma.
26
What is the recommended referral for unexplained bruising, fatigue, or abnormal FBC?
Refer to haematology
## Footnote
These symptoms may indicate underlying hematological disorders requiring specialist evaluation.
27
What are the B symptoms associated with lymphoma?
Painless lymphadenopathy, fever, night sweats, weight loss
## Footnote
B symptoms are systemic symptoms that can indicate more aggressive disease.
28
What is the role of Penicillin V in the management of Sickle Cell Disease?
Prevent infections in children
## Footnote
Prophylactic antibiotics like Penicillin V are used to reduce the risk of infections.
29
Fill in the blank: In patients with Sickle Cell Disease, _______ is often used as a treatment to reduce painful episodes.
hydroxycarbamide
## Footnote
Hydroxycarbamide helps to increase fetal hemoglobin levels and reduce sickling.
30
What is a common finding on a blood film for Acute Leukaemia?
Blasts
## Footnote
Blasts are immature white blood cells that are typically increased in acute leukaemia.
31
True or False: Lymphocytosis and smudge cells are associated with Chronic Lymphocytic Leukaemia.
True
## Footnote
Lymphocytosis is an increase in lymphocytes, and smudge cells are fragile lymphocytes seen in CLL.
32
What is a laboratory test used to monitor renal function in patients with Multiple Myeloma?
U&E (Urea and Electrolytes)
## Footnote
Monitoring renal function is crucial as renal impairment can occur due to light chain deposition.
33
What is Philadelphia chromosome found in?
CML
34
What is the most common inherited haemolytic anaemia?
Spherocytosis
35
What is the inheritance pattern of spherocytosis?
AD with 25% spontaneous mutation
36
What virus classically triggers aplastic crisis in spherocytosis
Parvovirus
37
What is commonly seen in spherocytosis?
Jaundice and anaemia with reticulocytes
38
Why does aspirin affect platelet function?
COX inhibitor
39
What is Thalassaemia?
Disordered production of alpha or beta globulin
## Footnote
Thalassaemia is a genetic blood disorder characterized by reduced or absent production of hemoglobin.
40
What are the effects of homozygous alpha thalassaemia?
Death barts hydrops; DEAD AT BIRTH
## Footnote
This condition results from a complete absence of alpha globin chains, leading to severe fetal anemia.
41
What are the effects of heterozygous alpha thalassaemia?
Often asymptomatic, mild anaemia
## Footnote
Individuals may not exhibit significant health issues.
42
What regions are commonly associated with beta thalassaemia?
Mediterranean, Middle East, South Asia
## Footnote
These areas have higher prevalence due to historical genetic factors.
43
What are the effects of homozygous beta thalassaemia?
Bossing of the skull, profound haemolytic anaemia
## Footnote
This condition leads to severe physical deformities and significant health complications.
44
What are the effects of heterozygote beta thalassaemia?
Asymptomatic anaemia
## Footnote
Many individuals may not show symptoms or require treatment.
45
What can be reversed for vitamin K
Warfarin
46
What medication should be avoided in those with warfarin?
NSAIDs
47
If you wanted to measure Heperin levels what would you use? (Don’t normally need to)
Anti Xa
48
What is ITP?
Immune destruction of platelets
## Footnote
Typically affects children (2-6 years) or adults, usually women with conditions like SLE.
49
What are common presentations of ITP?
* Easy Bruising
* Purpura
* Epistaxis
* Menorrhagia
## Footnote
These symptoms occur due to low platelet counts.
50
What is TTP?
Thrombocytopenic Purpura, characterized by platelet consumption due to microvascular thrombosis
## Footnote
Presents with purpura, fever, cerebral dysfunction, anemia, and acute kidney injury (AKI).
51
What is the inheritance pattern of Hemophilia A?
X-linked recessive
## Footnote
Occurs in approximately 1 in 5000 males due to decreased factor VIII.
52
What are the main symptoms of Hemophilia A?
* Spontaneous bleeding
* Joint bleeding
* Easy bleeding after surgery or injury
## Footnote
Patients may experience easy bruising and joint bleeds.
53
What is Christmas Disease?
Hemophilia B
## Footnote
Occurs in approximately 1 in 30,000 males due to factor IX deficiency.
54
What mutations are associated with VWD?
Mutations of VWF on chromosome 12
## Footnote
These mutations result in decreased VWF or morphologic changes.
55
What are the presentations of VWD?
* Easy bruising
* Epistaxis
* Menorrhagia
## Footnote
Very rare joint bleeds compared to Hemophilia.
56
List some management options for ITP.
* Steroids
* Platelets if bleeding or preoperative
* Splenectomy
* Rituximab
* Thrombopoietin Receptor Agonist (Romiplostin)
## Footnote
These treatments aim to increase platelet counts and manage symptoms.
57
What are some treatments for Hemophilia A?
* Factor VIII concentrate
* Desmopressin
## Footnote
Desmopressin can stimulate the release of VWF, aiding in hemostasis.
58
What are some treatments for Hemophilia B?
* Factor IX concentrate
* Desmopressin
## Footnote
Similar to Hemophilia A, but focuses on factor IX.
59
What is the role of Desmopressin in bleeding disorders?
Stimulates the release of VWF and factor VIII
## Footnote
Useful in managing certain bleeding disorders, especially VWD and mild Hemophilia A.
60
What is Glanzmann's thrombasthenia?
A rare platelet membrane deficiency of the Glycoprotein IIb/IIIa protein
## Footnote
Characterized by excess bruising and bleeding in response to trauma and surgery.
61
What is Polycythaemia Rubra Vera (PRV)?
A myeloproliferative disorder characterized by excess red cell production, increased platelets, and white cell count.
## Footnote
95% are JAK2 V617F positive.
62
What are the clinical features of Polycythaemia Rubra Vera?
Headache, pruritus (especially after hot shower), plethoric complexion, night sweats, thrombosis (DVT/PE, stroke), and splenomegaly.
63
What is Factor V Leiden?
An inherited thrombophilia caused by a mutation in Factor V that makes it resistant to activated protein C, leading to a prothrombotic state.
64
What are the clinical features of Factor V Leiden?
Often asymptomatic; may present with DVT/PE. Risk increases with pregnancy, surgery, and use of COCP/HRT.
65
What investigations are used for Polycythaemia Rubra Vera?
T Hb/Hct, L EPO, JAK2 mutation, bone marrow biopsy, thrombophilia screen, and genetic testing for mutation.
66
What is the management for Polycythaemia Rubra Vera?
Venesection to keep Hct < 0.45, low-dose aspirin, hydroxycarbamide if high-risk, consider anticoagulation if thrombotic history, no treatment if asymptomatic, anticoagulation after VTE, and avoid COCP/HRT in known carriers.
67
What is the commonest childhood leukaemia
ALL
68
What is bone pain specific to (in terms of cancer)
ALL
69
In cancer what is skin infiltration specific to
AML
70
What is the commonest leukaemia of adults
AML (median age of presentation 60)
71
When would you need to refer CLL
Usually asymptomatic and managed in GP
Only need to refer if doubling time >6 months and or anaemia or thrombocytopenia develops
72
CML key characteristics
Middle Ages, fatigue, splenomegely
73
What will investigations show with myeloma
High ESR
High calcium
Urinary hence Jones positive
Raised immunoglobulins
74
What is the commonest haematological malignancy?
Lymphoma
## Footnote
Effects males more than females, 5th commonest malignancy in developed world.
75
What type of cells does Hodgkin's lymphoma arise from?
B cells
## Footnote
95% are classical with Reed Sternberg cells, 5% are nodular with popcorn cells.
76
What are the common presentations of Hodgkin's lymphoma?
Cervical lymphadenopathy and cough
## Footnote
Cough is due to mediastinal widening.
77
How is Hodgkin's lymphoma classified for prognosis?
Ann Arbor scale
## Footnote
Staging is classified above diaphragm (better survival) and below diaphragm.
78
What are the management options for Hodgkin's lymphoma?
Radiotherapy, Chemotherapy, Monoclonal Antibodies
## Footnote
Example: Rituximab.
79
How does Non-Hodgkin's lymphoma present?
Painless peripheral lymphadenopathy, mediastinal mass, or B symptoms
## Footnote
There are 7 subtypes of Non-Hodgkin's lymphoma.
80
What is the 10-year survival rate for Non-Hodgkin's lymphoma?
50%
## Footnote
Survival rates are generally worse than Hodgkin's lymphoma.
81
What are the management options for Non-Hodgkin's lymphoma?
Radiotherapy, Chemotherapy, Monoclonal Antibodies, Immune Therapy
## Footnote
Treatment approaches may vary based on subtype.
82
What are B symptoms associated with lymphomas?
Fever, night sweats, and weight loss
## Footnote
These symptoms indicate advanced disease.
83
Fill in the blank: Hodgkin's lymphoma is characterized by _______.
Reed Sternberg cells
## Footnote
These cells are a key diagnostic feature.
84
True or False: Non-Hodgkin's lymphoma has a better prognosis than Hodgkin's lymphoma.
False
## Footnote
Non-Hodgkin's lymphoma generally has worse survival rates.
85
What is the significance of the Ann Arbor staging in lymphomas?
It helps determine prognosis and treatment
## Footnote
Stage 1 and 2 indicate disease above diaphragm, while 3 and 4 indicate below.
86
What are popcorn cells seen in
Nodular Hodgkin lymphoma
