Haematology Flashcards
(164 cards)
1
Q
Common symptoms for anaemia
A
Weakness, fatigue and lethargy Shortness of breath and reduced exercise tolerance Headaches and syncope Angina and palpitations Conjunctival pallor Tachycardia Tachypnoea Orthostatic hypotension
2
Q
Different types of anaemia and some examples
A
Microcytic (low MCV) e.g. iron deficiency, sideroblastic, thalassemias.
Normocytic (normal MCV) e.g. bone marrow suppression, acute blood loss, chronic diseases.
Macrocytic (high MCV) e.g. B12 and folate deficiency, alcoholism, haemolytic anaemias.
3
Q
What happens in severe anaemia
A
Hyperdynamic circulation - tachycardia, cardiac enlargement, flow murmur.
Also get angina, ankle oedema, dyspnoea at rest.
4
Q
Where is iron absorbed, how is transported and what are some good food sources
A
Absorbed in duodenum and jejunum.
Transported by transferrin
Meat, wholegrain (brown rice), curly kale.
5
Q
Causes of iron deficiency
A
Blood loss in GI bleed (hookworm) or menorrhagia
Inadequate intake in diet
Increased requirement e.g. infants
Poor absorption e.g. GI surgery or coeliac
6
Q
Some signs only really seen in iron deficiency anaemia
A
Koilonychia (spoon nails) Atrophic glossitis (red sore tongue) Angular cheilosis (inflammation at side of mouth) Hair loss Pica (weird food cravings e.g. clay)
7
Q
Patient has iron deficiency anaemia but also complains of sore tongue, sore mouth/lips, difficulty swelling. What is wrong with them and what will you see in endoscopy
A
Plummer-Vinson syndrome
Oesophageal webs
8
Q
Investigations and expected results for iron deficiency anaemia
A
FBC = low Hb, Low MCV, Low MCHC (Hb concentration)
Blood film = microcytic hypochromic anaemia with different sized (anisocytosis) and different shaped (poikilocytosis) cells.
Iron studies = low ferritin, low transferrin saturation, high TIBC (iron binding capacity).
Investigate possible cause = coeliac serology (IgA-tTG), endoscopy for GI malignancy.
9
Q
Management of iron deficiency anaemia
A
Treat cause (Mirena coil for menorrhagia, gluten free diet etc etc).
Oral iron e.g. ferrous sulphate 200mg PO. Should see rise in Hb of 10g/L in a week. Continue for 3 months even if Hb normalises.
If severe - blood transfusion.
10
Q
Side effects of oral iron
A
Nausea
Constipation
Dark stools
Abdo discomfort
11
Q
Differential for low iron but high TIBC (no blood film results yet)
A
Pregnancy. Ferritin may be high in pregnancy.
12
Q
FBC = low iron, low MCV, high MCHC
Film = different sized and shaped, hypochromic.
Iron studies = low ferritin, low transferritin, high TIBC, high reticulocytes.
A
Iron deficiency
13
Q
Sideroblastic anaemia
A
Poor erythropoiesis. Increased iron absorption and iron loading in organs.
X-linked genetic inheritance or acquired from myeloproliferative or myelodysplastic disorders.
Ix = FBC = low Hb. Iron studies = high ferritin, normal TIBC, high serum iron. Blood film = dimorphic population of cells, microcytic, hypochromic. Bone marrow biopsy = ringed sideroblasts.
Rx = pyridoxine and transfusion if severe. Treat cause.
14
Q
Name some chronic diseases which can cause anaemia of chronic disease
A
Renal failure Rheumatoid arthritis SLE Malignancies (lymphoma) Chronic infection (TB)
15
Q
Investigations for anaemia of chronic disease
A
FBC = low Hb.
Blood film = normocytic and normochromic.
Iron studies = low serum iron, normal/raised ferritin, low transferrin, low TIBC.
Raised ESR and other markers of the chronic disease.
16
Q
2 types of macrocytic anaemia
A
Megaloblastic and non-megaloblastic.
Megaloblastic = delayed nuclear maturation e.g. B12 and folate deficiency, cytotoxic drugs.
Non-megaloblastic = alcohol excess, pregnancy, hypothyroidism.
17
Q
Sources of folate and where is it absorbed
A
Green vegetables, nuts, citrus fruits
Proximal jejunum
18
Q
Where is B12 absorbed. What has lots of vitamins B12
A
Ileum.
Fish and dairy and meat.
19
Q
What symptoms do patients with megaloblastic anaemia commonly complain (more than other types of anaemia)
A
Headache
Anorexia
20
Q
Who are at risk of folate deficiency
A
Alcoholics
Pregnancy (take folic acid to prevent rural tube defects)
Over 65yrs
21
Q
Investigations and expected results for folate deficiency anaemia
A
FBC = low Hb, high MCV. Blood film = macrocytic RBC, hypersegmented neutrophils (immature RBC and WBC). Low serum folate. Low reticulocyte count. Bone marrow biopsy shows megaloblasts.
22
Q
Investigating for anaemia
A
FBC
Blood smear
Iron studies
Serum B12 and folate
23
Q
Management of folate deficiency
A
Oral folic acid supplementation.
If severe = packed RBC transfusion.
24
Q
Difference between B12 and folate deficiency anaemia
A
B12 = peripheral neurology, neuropsych signs Folate = no neuro stuff.
25
Who are at risk of B12 deficiency
Vegetarians and vegans π©πΌβπΎπ¨π»βπΎ
| Also Crohn's and gastric by-pass patients.
26
Quick overview of B12 physiology
Binds with intrinsic factor from parietal cells in stomach.
IF-B12 complex travels to terminal ileum.
Complex binds to transcobalamin and realised into blood stream.
27
What colour is the skin of b12 deficiency and why
Lemon tinge - pallor from anaemia + yellow from mild jaundice and haemolysis.
28
What are some neurological s+s in b12 deficiency
```
Psych = dementia, psychosis, depression.
Neuro = SUBACUTE DEGENERATION OF SPINAL CORD = distal sensory loss, absent ankle reflex, brisk knee reflex. LMN and UMN signs. Paraesthesia, numbness, loss of vibration sensation, normal pain and temperature sensation.
```
29
Ix for B12 deficiency anaemia
FBC = low Hb, high MCV
Low serum B12
Blood smear = megalocytic RBC, hypersegmented neutrophils.
Low reticulocyte count.
30
Management of B12 deficiency anaemia
```
Moderate = IM hydroxycobalamin (life long but bi-weekly)
Severe = IM hydroxycobalamin + folic acid and admit to hospital for neuro assessment. Consider blood transfusion.
```
31
Pernicious anaemia
Autoimmune atrophic gastritis - anti parietal cell antibody so no IF to bind to B12.
Similar presentation to B12 deficiency (lemon-tinge, neuro and psych symptoms plus palpitations, fatigue etc etc).
Ix = same as for any anaemia - FBC (low Hb, high MCV), smear (hypersegmented neutrophils).
Special pernicious tests = IF antibody, parietal cell antibody.
Mx = IM hydroxycobalamin.
Associated with other autoimmune conditions e.g. thyroid. Also increased gastric cancer risk.
32
What makes up adult haemoglobin
2 alpha + 2 beta globin chains
33
Ξ±-thalassemia
2 separate alpha globin genes on Chr16 so 4 genres (Ξ±Ξ±/Ξ±Ξ±).
Mutations inherited autosomal recessively.
If all 4 mutated = death in utero (Bart's hydrops).
If 3 mutations = HbH disease. Anaemia, haemolysis (jaundice, hepatosplenomegaly). FBC = low MCV. Blood film shows target/Mexican hat cells. Γ4 tetramers from excess Γ chains on electrophoresis. Mx = folic acid, transfusion, splenectomy.
If 2 mutated = asymptomatic carrier.
If 1 mutated = clinically normal.
Mx with genetic counselling.
34
Good differential for HbH (Ξ± thalassaemia)
Malaria esp as prevalent in same areas.
35
Target cells
Blood film of Ξ± and Γ -thalassaemia disease
36
Γ-thalassaemia pathophysiology
Mutations for Γ-globin on Chr11. Ineffective erythropoiesis.
S+S start as fetal Hb changes to adult Hb (present in first year).
Anaemia
Compensatory erythroid hyperplasia = bony changes in skull, splenomegaly.
37
Γ thalassaemia minor, intermedia or major
- Minor = trait. Asymtpmatic, only β on genetic screening.
- Intermedia = presents later in life e.g toddler or young adult. Less severe symptoms.
- Major = Cooley's anaemia. Absence of HbA, presenting within 1st year of life. Pallor, abdo distention, poor feeding, lack of activity, hepatosplenomegaly, frontal and parietal skull bossing.
38
Ix for Γ thalassaemia
FBC = low Hb, low MCV.
peripheral blood film = tear drop cells, microcytic red cells.
High reticulocyte count
Haemoglobin electrophoresis to determine intermedia/major etc
Raised unconjugated bilirubin.
Skull x-ray = 'hair on end' sign (high marrow activity).
AXR = hepatosplenomegaly.
39
Mx for Γ thalassaemia
Genetic counselling
Dietary advice.
Consider chelation
Regular transfusions (lifelong dependent), splenectomy.
40
Inheritance and pathophysiology of sickle cell anaemia
Autosomal recessive mutation in Γ-chain for Hb creating sickle cell Hb (HbS).
On deoxygenation RBC morph shape to sickle shape. This causes clusters to block blood vessels (esp liver and spleen), intense pain (sickle cell crisis), infections and anaemia.
41
Types of sickle cell disease
- Sickle cell trait = heterozygotes = HbSA, no symptoms, protective against P.falciparum malaria.
- Sickle cell disease = homozygotes = HbSS, symptomatic.
- Can get a patient who inherits heterozygous sickle cell and mild thalassaemia so does present.
42
S+S of sickle cell disease β
```
Present within first year of life.
FHx
Jaundice from haemolysis.
Pallor, tachycardia, fatigue from anaemia.
Splenomegaly
Dactylitis
Leg ulcers
Present in crisis.
```
43
Sickle cell vaso-occuslive crisis.
Vaso-occulsive crisis as lots of Hb is deoxygenated at once.
Subtypes of thrombotic stroke, acute chest syndrome and priapism.
Precipitated by: stress, cold, pregnancy, dehydration, low PaO2 (exercise, anaesthesia, altitude), illness.
S+S: PAIN PAIN PAIN (limb bone), swollen joints, S+S of ischaemic area e.g. acute abdomen.
Mx = A-E resuscitation, IV analgesia (morphine), blood crossmatch, warm IV fluids, consider need for ABx and blood transfusion, inform haematologist.
44
Chronic complications of sickle cell disease
```
Chronic anaemia
Chronic pain
Leg ulcers
Sickle Lungs = breathlessness, chest pain, restrictive lung pathology
Retinopathy
Gallstones
Persistent erection
Pulmonary HTN
lots and lots
```
45
Ix for sickle cell disease
```
Hb electrophoresis = diagnostic.
Peripheral blood smear = sickle cells, target cells and Howell-Jolly bodies from poor splenic function.
FBC = low Hb
Bilirubin = raised
Reticulocyte count = raised
Sickle solubility test
```
46
Acute complications of sickle cell disease
```
Vaso-occlusive crisis of bone.
Mesenteric ischaemia
Acute chest syndrome
Sepsis
Priapism (sustained painful erection)
AKI
Stroke
Aplastic crisis
Sequestrian crisis.
Avascular necrosis of femoral or humeral head.
```
47
Acute chest syndrome
Can lead to ARDS and death.
Chest pain, tachypnoea, cough, SOB.
A-E resuscitation --> oxygen, fluids, analgesia.
Ix = CXR, ABG.
Mx with broad spec ABx (tazosin) and blood transfusion.
48
Describe 2 ways to classify haemolytic anaemia and examples for each.
Intra and extra vascular.
Intravascular = DIC, lack of G6PD.
Extravascular = sickle cell disease, hereditary spherocytosis, immune mediated IgG (lymphoma).
Acquired and Hereditary.
Acquired = immune e.g. IgG and IgM or non-immune e.g. DIC, trauma.
Hereditary = sickle cell disease, G6PD deficiency.
49
Coombs' test
Direct antiglobulin test. Positive in immune mediated haemolytic anaemia.
50
Examples of hereditary causes of haemolytic anaemia
Inherited RBC defect e.g. hereditary spherocytosis.
Abnormal Hb e.g. thalassaemia, sickle cell.
Enzyme defect e.g. G6PD deficiency.
51
Examples of acquired haemolytic anaemia
Immune mediated:
- alloimmune (haemolytic disease of the newborn).
- warm reacting autoimmune (SLE, CLL) IgG
- cold reacting autoimmune (lymphoma) IgM.
- drugs (sulfasalazine, penicillin)
Non-immune mediated:
- infection (Mycoplasma pneumoniae)
- Trauma
- microangiopathic haemolytic anaemia (DIC, HELLP, HUS).
- hypoersplenism
52
Ix for haemolytic anaemia
FBC = low Hb, high reticulocyte, high MCV from reticulocytes.
Peripheral blood film = depended on cause.
Bilirubin = raised in conjugated bilirubin.
Urinalysis = ++RBC.
LDH + haptoglobin = low LDH and high haptoglobin.
Coomb's test = immune or no immune-mediated.
53
Presentation of haemolytic anaemia
Anaemia = pallor, fatigue, SOB, headache,
Haemolysis = jaundice, dark urine, splenomegaly, gallstones.
RFx = FHx, other autoimmune conditions.
S+S of cause.
54
Mx for haemolytic anaemia
All get supportive Mx = folic acid, review meds, transfusion of packed RBC.
Immune mediated Mx = corticosteroids, splenectomy.
55
Drug for sideroblastic anaemia
Pyridoxine
56
Aplastic anaemia pathophysiology and causes
Pancytopenia (thrombocytopenia, neutropenia, anaemia) with hypocellular marrow and the absence of any abnormal cells.
Ax = autoimmune, drugs, viruses (hepatitis, parvovirus), irradiation, inherited (Fanconi anaemia).
57
Where to take bone marrow biopsies
Iliac crest.
| Haemopoises occurs in axial skeleton (e.g. skull, vertebrae, ribs) and long bones.
58
Name 4 types of leukaemia and how they are different from one another
Acute myeloid leukaemia - acute neoplastic proliferation of blasts cells from bone marrow myeloid tissue, blast cells can no mature and differentiate.
Acute lymphocytic leukaemia - acute neoplastic proliferation of immature B and T lymphocytes.
Chronic myeloid leukaemia - slow progression of neoplastic proliferation of blasts cells from bone marrow myeloid.
Chronic lymphocytic leukaemia - slow progression of neoplastic proliferation of dysfunctional B cells.
59
But what actually is leukaemia π€
Excess of abnormal white blood cells in peripheral blood. either myeloid or lymphoid in origin.
60
Which leukaemia can be a product of another cancer and why
Acute myeloid leukaemia from chemotherapy.
61
AML S+S
Acute onset of symptoms.
From marrow failure = anaemia, infections, bleeding and bruising.
From cell infiltration = hepatosplenomegaly, gum hypertrophy, lymphadenopathy
62
Ix for AML
FBC - raised WCC, low Hb. Also do clotting profile, renal function, LFT.
Peripheral blood film - blast cells.
Bone marrow biopsy - β, hypercellulatiry, blast cell infiltration, AUER RODS in myeloblast cells.
63
Mx for acute myeloid leukaemia
Supportive care - exercise regimens, adequate hydration, information on diagnosis, safety net advice or ABx for infections.
Chemotherapy e.g. Azacitidine (pyrimidine analogue) Allopurinol to prevent uraemia from tumour lysis syndrome.
Bone marrow transplant
64
Acute lymphoblastic leukaemia common presentation
CHILDREN. Commonest cancer of childhood πΆ
65
S+S of acute lymphoblastic leukaemia
Marrow failure = anaemia, thrombocytopenia and neutropenia. Esp recurrent chest, mouth, skin or perianal infections.
Infiltration = hepatosplenomegaly, lymphadenopathy, CNS involvement e.g. cranial nerve palsy, meninigms and nuchal stiffness.
66
Ix for acute lymphoblastic leukaemia
FBC =pancytopenia.
U+E = raised calcium and others.
Also do renal function, clotting profile, lactate dehydrogenase and LFTs.
Peripheral blood film = blast cells.
Bone marrow aspiration + trephine biopsy = β, hypercellularity, lymphoblast infiltration.
***LP*** if CNS involvement
CXR = Mediastinal lymphadenopathy causes widened mediastinum, mediastinal mass and pleural effusion.
67
Mx for acute lymphoblastic leukaemia
Supportive = educate, exercise regime, IV fluids, safety net for infections.
Allopurinol (tumour lysis syndrome).
Allogenic bone marrow transplants.
Chemotherapy in 3 stages:
Induction treatment - aim to achieve remission, vincristine, dexamethasone + asparaginase.
Consolidation treatment - exterminate residual disease.
Maintenance treatment - extend remission, methotrexate and mercaptopurine.
68
Chronic myeloid leukaemia causes
Philadelphia chromosome.
69
Describe the Philadelphia chromosome please π
Reciprocal translocation between Chr9 and Chr22 long arms. Creates a BCR-ABL fusion oncogene, tyrosine kinase activity.
If a patient has CML and no Philadelphia chromosome they have a worse prognosis.
Is seen in CML and ALL.
70
Presentation of chronic myeloid leukaemia
```
Gradual onset. 3 phases = Chronic phase, accelerated phase and blast transformation (AML).
Weight loss, fatigue, fever, sweats.
Bleeding and bruising.
Anaemia
Massive splenomegaly
Abdo discomfort and hepatomegaly
Gout (purine breakdown).
```
71
Ix for chronic myeloid leukaemia
FBC - massively raised WCC, low Hb.
Metabolic profile - B12 raised. Urate raised.
Peripheral blood film - high basophils and eosinophils.
Bone marrow biopsy - granulocytic hyperplasia.
Cytogenetics - Philadelphia chromosome.
PCR for BCR-ABL fusion for tyrosine kinase
72
Mx of chronic myeloid leukaemia
Tyrosine kinase inhibitor e.g Imatinib or Dasatinib.
| Allogenic stem cell transplantation.
73
Chronic lymphocytic leukaemia S+S
Commonest leukaemia.
Leukaemic cells infiltrate lymph tissue and haemopoietic tissue (liver, spleen, bone marrow)
Asymptomatic - only found on a routine FBC.
Anaemia, neutropenia. Weight loss, sweats and anorexia.
Big lymphadenopathy (non-tender, rubbery), HepatoSplenomegaly.
74
Ix for chronic lymphoblastic leukaemia
FBC - massive increase in lymphocytes. Low Hb, low neutrophils, low platelets.
Peripheral blood film - smear/smudge cells, spherocytes if haemolysis.
Flow cytometry - +ve dim surface immunoglobulins.
Bone marrow aspiration + trephine biopsy - infiltration with leukaemia cells.
75
Mx of chronic lymphoblastic leukaemia
Supportive care
Chemotherapy e.g. Fludarabine + Rituximab + cyclophosphamide.
radiotherapy
Stem cell transplantation.
76
Richter's syndrome
CLL transformation to aggressive B cell lymphoma.
77
Staging for CLL
Binet
78
3 complications of CLL
Richter's syndrome and B cell lymphoma
Autoimmune haemolytic anaemia
Hypogammaglobulinaemia (increased infection risk)
79
What is a neutropenic regime and when is it used
Prevent neutropenic sepsis. Used in those with a low neutrophil count from chemo or leukaemias!!
Barrier nursing
Avoid Im injections (infected haematoma)
Check FBC, platelets, U+E, LFT, INR, LDH, CRP, blood cultures.
Good hygiene, avoid DRE, oral hygiene and wash perineum after defection.
4 hourly vital signs.
Avoid food with high risk of contamination e.g. raw meat.
80
Complications of leukaemia
Neutropenic sepsis = MASCC assessment tool for risk of serious complications in febrile neutropenia.
Hyperviscosity = leukostasis. Thrombi in brain, lung or heart.
DIC
Tumour lysis syndrome and electrolyte abnormalities.
81
Difference between Hodgkin's and Non-Hodgkin's Lymphoma
Hodgkin's = mirror-image nuclei cells = Reed-Sternberg cells.
Absence of these = Non=Hodgkin's
82
Risk factors for Hodgkin's lymphoma
Epstein-Barr virus
FHx
Young adult from high socio-economic class
83
Difference between leukaemia and lymphoma
```
Leukaemia = neoplasia at bone marrow, circulating.
Lymphoma = neoplasia in lymphatic system, solid.
```
84
S+S of lymphomas
Lymphadenopathy esp cervical, axillary or inguinal. Non-tender, rubbery and superficial.
B symptoms = weight loss, night sweats, pruritus, fever.
SE of obstruction from lymphadenopathy e.g. SVC obstruction (EMERGENCY).
Hepatosplenomegaly.
Cough
85
Ix for lymphomas
```
Laboratory:
FBC - anaemic, low platelets, can have high WCC.
Peripheral blood film
ESR
LFT
Lactate dehydrogenase (prognostic)
Metabolic profile (urate, calcium)
```
Imaging: CT/PET of thorax, abdo, pelvis - mediastinal mass.
DIAGNOSTIC: lymph node excision biopsy. If HODGKIN'S = Reed-Sternberg cells.
86
Staging for lymphoma
Ann Arbor.
Imaging to work out where cancer is (above, below diaphragm)
+ B symptoms present or absent.
87
Mx of lymphoma
```
MDT
Chemotherapy + radiotherapy
R-CHOP regime for NHL
Supportive
Biologics (e.g. CD20 monoclonal antibodies) immunotherapy and cellular therapy.
```
88
Jaw lymphadenopathy cause
Seen in lymph node biopsy
Associative RFx
Burkitt's lymphoma
'Starry sky'
EBV
89
Types of non-hodgkin's lymphoma and presentations
Most are derived from B cell lymphocytes. Most common is diffuse large B-cell lymphoma.
RFx = immunodeficiency as linked to many viruses (EBV, Herpes simplex 8, hep C) and H.pylori.
S+S = lymphadenopathy, B symptoms, shortness of breath, splenomegaly, dependent on type. Gastric symptoms in MALT, orophaynx, skin etc etc.
Rx with chemo (R-CHOP regime) agents include Rituximab, Cyclophosphamide, Pred, Vincristine)
90
S+S more seen in Hodgkin's lymphoma
Alcohol-induced lymph pain.
'Pel Ebstein' swinging high fevers
Pruritus
91
What is myeloma
Plasma cell dyscrasia characterised by malignancy of immunoglobulin producing plasma cells leading to accumulation of abnormal immunoglobulins which can be detected in blood or urine (paraproteinaemia).
92
How to classify multiple myeloma
Based on the immunoglobulin product detected e.g. IgG, IgA in blood or Bence Jones protein in urine.
93
RFx for multiple myeloma
Black ethnicity
Increasing age (over 70yrs)
FHx
94
Complications of multiple myeloma
```
Pathological bone fractures
Spinal cord compression
AKI
Anaemia, thrombocytopenia and bleeding, neutropenia and infections.
Hyperviscosity of blood = stroke, HF.
```
95
S+S of multiple myeloma
Back/bone pain (lower or thoracic area)
Fatigue, weight loss.
Hypercalacemia Sx = bone pain, depression, polyuria, renal colic, constipation.
Sx of spinal cord compression
Sx of hyperviscosity = headache, visual disturbance, cognitive impairment.
O/E:
Hepatomegaly
Brusing and bleeding
96
Ix for multiple myeloma
FBC = normocytic, normochromic anaemia.
ESR = raised.
Metabolic profile = high urea, high calcium.
Peripheral blood film = Rouleaux (aggregation of RBC)
Bone marrow biopsy = increased plasma cells.
Serum and urine electrophoresis = presence of paraprotein/immunoglobulin e.g. IgG, Bence-Jones protein.
Skeletal surgery and whole body MRI/CT.
97
Criteria for multiple myeloma
- Serum or urine electrophoresis confirms paraprotein (serum M protein β₯30 g/L, urine Bence-Jones).
- Monoclonal plasma cells in marrow β₯10%.
- Evidence of end-organ damage e.g. hypercalcaemia, renal impairment, anaemia, lytic lesions in bone.
98
Mx for multiple myeloma
MDT. Information and support for pt and carers.
- Analgesia for back pain e.g pcm or codeine (not NSAID due to renal toxicity).
- Bone disease Rx with bisphosphonates e.g. zolendronic acid.
- Anaemia Rx with blood transfusion or erythropoietin.
- DVT prophylaxis with LMWH
- Prevent AKI with good hydration.
- Chemo e.g. cyclophosphamide, corticosteroids, immunomodulatory drugs.
- Stem cell transplant.
99
1. What are paraproteins?
| 2. What is a Bence Jones proteins
1. Single type of immunoglobulin fragment made by neoplastic cells. Will produce large monoclonal band on electrophoresis whereas normally will be polyclonal.
2. Free Ig light chains of kappa or lambs type. Found in urine as filtered by kidneys.
100
Over 50yrs old and come to GP with back pain.
Do serum protein electrophoresis and ESR to exclude myeloma.
101
Main areas which myeloma affects
```
π¦ π¦ π¦ π¦ π¦ π¦ π¦ π¦
Calcium elevation
Renal insufficiency
Anaemia
Bone disease
```
(CRAB)
102
MCV for alcoholics
Macrocytic due to folate deficiency
103
Severe folate or B12 deficiency
Pancytopenia!!
Slow healing esp of tongue = glossitis.
Homocystineuria
Atherosclerosis from inflammation - increase IHD and stroke.
104
2 neural tube defects due to folate deficiency
Spina bifida
| Anencephaly.
105
Aplastic crisis with sickle cell disease
Parvovirus B19 infection causes massive drop in Hb within 1week.
S+S = splenomegaly, sudden pallor, weakness, circulatory collapse and shock.
Rx = oxygen, fluids, transfusion.
106
Splenic sequestrian crisis in sickle cell disease
Spleen infarction.
Acute, painful splenomegaly. Sudden drop in Hb. Can lead to hypovolvaemic shock.
Rx = oxygen, fluids, transfusion.
107
Management of sickle cell disease
```
Haemotologist referral.
Genetic counselling to family.
Pneumococcal vaccine.
Hydroxycarbamide to prevent crises.
Bone marrow transplant.
Transfusion
Manage complications e.g leg ulcers, analgesia, treat chest infections early.
Prevent obstructive sleep apnoea = hypoxia!
```
108
Inheritance of G6PD deficiency
X linked
109
Differentials for massive splenomegaly
CML
Malaria
Myelofibrosis.
110
Someone's had a splenectomy or their spleen has gone kaput - what is seen on blood film
Howell-Jolly bodies
Increased risk of infection
Life-long ABx e.g. penicillin.
111
How to find out how at risk someone is of febrile neutropenia
MASCC tool.
112
R-CHOP regimine
Non-hodgkin's lymphoma. Big risk of neutropenic sepsis.
```
Rituximab
Cyclophosphamide
Doxorubicin hydrochloride
Vincristine
Prednisolone.
```
113
Causes of leucopenia
```
Drugs = chemotherapy, clozapine.
Haem = myelofibrosis, aplastic anaemia, post bone-marrow transplant.
Systemic = SLE, Hodkin's lymphoma
```
114
Risk factors for venousthrombo emboli
```
DVT or PE
Malignancies
Recently bed-ridden
Previous Hx of DVT or PE
Obesity
Thrombophilia e.g Protein C or S deficiency
Smoking
```
115
How long post DVT or PE are patients on anticoagulants
3 months.
116
Ix for ?DVT
D-dimer = good negative predictive value but not specific.
| Doppler USS
117
Ix for ?PE in PREGNANCY
V/Q scan. Lower radiation to breast than CTPA.
118
2 drugs which you can give IMMEDIATELY IN VTE
Heparin
| Apixaban
119
Where does apixaban and other DOACs work?
Factor Xa
120
Action of warfarin
Vitamin K dependent factors
| 2, 7, 9 and 10.
121
Reversal of warfarin
Immediately with Beriplex = dried prothrombin complex.
| Over 12 hours with vitamin K
122
Reversal of heparin
Protamine sulfate
123
MGUS
Monoclonal Gammopathy of Uncertain Significance
| Asymptomatic premalignant disorder. Presence fo serum M-protein but patient has no symptoms.
124
Causes of thrombocytopenia
Poor production = leukaemia, aplastic anaemia, severe folate deficiency.
Increased destruction = TTP, ITP, HELLP, HUS.
125
ITP
Immune thrombocytopenia purpura.
- Autoimmune, no identifiable cause of thrombocytopenia.
- Kids have viral illness and then develop petechiae on lower limbs. Bruising, mucosal bleeding e.g nose bleeds, menorrahagia, can cause intracranial haemorrhage.
- FBC = low white cells. Peripheral blood film = few white cells.
Rx = Observational monitoring, corticosteroids e.g.prednisolone, IVIG, Rituximab.
126
TTP
Thrombotic thrombocytopenia purpura. Life-threatening!!
- Microangiographic haemolytic anaemia + thrombocytopenic purpura.
- Can also get neurological dysfunction, fever and renal dysfunction.
- S+S = epistaxis, bruising, petechiae, headache, confusion, fever, pallor, jaundice, arthralgia.
- U+E = raised urea and creatinine.
- Blood smear = schistocytes and fragmented erythrocytes.
- Rx = IV plasma exchange, glucocorticoid steroids.
127
Who gets TTP
Post-partum mothers
| ADAMTS13 deficiency
128
Haemophilia
A = loss of factor 8
B = loss of factor 9
X-linked
S+S = bruising and bleeding into joints.
Prolonged aPTT
Rx = recombinant clotting factors.
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Causes of polycythaemia
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Primary = polycythaemia vera.
Secondary = hypoxia e.g. COPD, OSA; excessive EPO e.g. renal artery stenosis; high testosterone in body builders.
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Tear drop cells on blood film
Myelofibrosis, beta thalassaemia
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Target cells on blood film
alpha thalassaemia (Mexican hat cells), sickle-cell disease, hyposplenism, iron deficiency anaemia.
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Macrocytic RBC, hypersegmented neutrophils on blood film
Megaloblastic macrocytic anaemias e.g. B12 and folate deficiency and pernicious anaemia
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Smear/Smudge cellson blood film
CLL
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Rouleaux cells on blood film
Multiple myeloma
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What is Polycythaemia Vera and what are some risk factors ?
Myeloproliferative disorder. Erythrocytosis and often combined with thrombocytosis. Strong associated with JAK2.
RFx:
- FHx
- Budd-Chiari syndrome
- Over age of 40yrs.
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Complications of Polycythaemia Vera and other erythrocytosis/polycythaemia
Increased blood viscosity --> Increased thrombi = VTE, TIA, digital ischaemia.
Polycythaemia Vera = AML progression!!
137
Presentation for polycythaemia
- Asymptomatic but picked up on routine FBC.
- Symptoms of thrombi (PE, DVT, MI).
- Features of haemorrhage (GI, intra-cranial).
- 'Fullness' headache
- Myalgia and muscle weakness.
- Worsen in heat
```
O/E:
Ruddy complexion
Conjunctival plethora
Splenomegaly
Clubbing
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Ix for polycythaemia
FBC: raised Hb (>185g/L), raised haematocrit, raised WCC (PCV),.
Serum erythropoietin (increased in secondary but decreased in PCV).
Raised uric acid in PVC.
JAK2 mutation genetic testing.
LFT, Abdo USS for secondary cause.
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Mx of Polycythaemia Vera
Venesection.
Aspirin.
Hydroxycarbamide to decrease VTE risk.
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Primary myelofibrosis
1. Pathogenesis
2. CFx
3. Ix and expected results
4. Mx
5. Cx
- Bone marrow produces excessive collagen. Limits marrow ability to make blood cells.
- Features = marrow fibrosis and scarring + ***extra medullary haematopoisis***.
- MASSIVE SPLENOMEGALY, bone pain, hepatomegaly, weight loss, pancytopenic symptoms.
- Ix = anaemia. Film has teardrop shaped RBC, blast cells. Dry tap on bone marrow aspiration, bone marrow biopsy shows fibrosis and reticulin deposits.
- Rx = folic acid, JAK2 inhibitor e.g. ruxolitinib, curative haematopoietic stem cell transplant.
- Complications = AML, pulmonary HTN, DVT, PE
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What are Myeloproliferative Disorders
Abnormal, slow growing bone marrow cancers. Produce high amounts of RBC, WBC, platelets.
Includes: CML, Polycythaemia Vera, Myelofibrosis, essential thrombocythaemia.
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What is this describing:
Clonal stem cell disorder causing ineffective haematopoiesis. S+S of anaemia, thrombocytopenia and neutropenia/leukopenia
Myelodysplastic syndrome.
- Most are primary and due to genetic mutations. Can be secondary to chemotherapy and radiotherapy.
- S+S of anaemic, thrombocytopenia and leukopenia.
- Ix = Pancytopenia. β bone marrow aspiration and biopsy = significant dysplasia, clonal cytogenetic abnormalities and changes in at least 1 cell line.
- Rx = Allogenic haematopoietic stem cell transplant.
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Causes of thrombocytosis (high platelets)
Essential thrombocythaemia (a Myeloproliferative disorder)
Inflammation
Infection
Iron deficiency anaemia
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Spherocytes on peripheral blood film
Hereditary sperocytosis
| Autoimmune haemolytic anaemias.
145
Heinz bodies on peripheral blood film
G6PD deficiency
| Alpha thalassaemia
146
Schistocytes (helmet cells) on peripheral blood film
DIC
147
Poikilocytes 'pencils' on peripheral blood film
Iron deficiency anaemia
148
Burr cells/echinocytes on peripheral blood film
Pyruvate kinase deficiency
149
Antiphospholipid syndrome
Coagulation deformities
Livido reticulares
Obstetrics problems
Thrombocytopenia
150
where are ulcers in sickle cell disease found
medial malleolus
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Some immediate blood transfusion complications and brief description of presentation?
- ABO incompatibility haemolytic reaction = as soon as transfusion starter. Chills, fever, flushing, riggers, N+V, chest pain, hypotension, DIC.
- Transfusion-related lung injury = cyanosis, fever, tachycardia, hypotension, dyspnoea.
- Bacterial contamination causing sepsis.
- Air emboli.
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1. Iron chelation agent
| 2. Copper chelation agent
1. desferrioxamine
| 2. penicillamine
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CFx and Mx of aplastic anaemia
S+S of cell deficiencies. Bleeding + bruising, infections, pallor + dyspnoea + fatigue + headache.
Mx = prevent infections, avoid IM injections, if severe allogeneic bone marrow transplant + RBC and Platelet transfusions + ABx.
154
Precipitants for sickle cell crisis/attacks
Cold, hypoxia, infection, dehydration and stress
155
Spleen attack in sickle cell anaemia
Splenic sequestration.
Caused by intrasplenic trapping of red blood cells and the resultant drop in haemoglobin levels.
Splenomegaly, pallor, tachycardia, lethargy, and shock.
Avoid it by reducing precipitants e.g. pneumococcal vaccination.
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Chronic management of sickle cell disease
- Hydroxycarbamide (prevent vaso-occulsive crisis).
- Pneumococcal, Hep B, Meningitis ACWY vaccination.
- Access to specialist psychology support.
- Consider prophylactic penicillin therapy.
- Folic acid supplements.
- Lifestyle advise to avoid triggers e.g. keep warm and hydrated.
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Which acute leukaemia will have CXR signs and what signs are they?
Acute lymphoblastic leukaemia. Mediastinal mass (lymphadenopathy) and pleural effusion
158
Classification for AML
FAB (French American British)
159
Does the Philadelphia chromosome improve or worsen prognosis
Interesting questions.... π΅οΈ
In CML it improves prognosis.
In ALL is worsens prognosis.
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Why does myeloma lead to renal failure?
* free light chains cause tubular damage (cast nephropathy)
* hypercalcaemia
* nephrotoxic drugs
* infection
* dehydration.
161
Name 4 classes of drugs and an example for each plus 1 side effect used in the management of myeloma
1 - corticosteroids e.g. Dexamethasone, fluid retention.
2 - analgesia e.g. co-codamol, constipation.
3 - Alkylating agents e.g. cyclophosphamide, alopecia.
4 - Proteasome inhibitor, Bortezomib, infection risk.
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Myelodysplastic syndrome
1. Causes
2. Complications
3. CFx
4. Ix and expected results
5. Management
1. Genetic/primary, chemotherapy, tobacco, benzene.
2. increases in blast cells further reduce normal cell production, when blasts >20% --> AML.
3. Anaemic, thrombocytopenia, leucopenic.
4. FBC (low Hb, low WCC, low platelets), peripheral blood smear, bone marrow biopsy (dysplasia, blast cells), chromosomal analysis.
5. Erythropoietic growth factors, decitabine (hyypomethylating agent), Lenalidomide, transfusions, ABx.
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Main clinical feature in essential thrombocythaemia
Burning pain and digital iscahemia --> Erythromelalgia.
164
Itching after a hot bath and ruddy complexion
Polycyathmia vera
