Haematology Flashcards
1
Q
What is anisocytosis
A
Variation in size of RBCW
2
Q
Causes of anisocytosis
A
- Myelodysplastic syndrome
- Many types of anaemia, e.g. iron deficiency, pernicious, autoimmune haemolytic anaemia
3
Q
What are target cells
A
RBC with central pigmented area surrounded by pale area, surrounded by ring of thicker cytoplasm on the outside
4
Q
Causes of target cells
A
- Iron deficiency anaemia
- Post splenectomy
- Sickle cell/thalassaemia
- Liver disease
5
Q
What are Heinz bodies
A
Individual blobs (inclusions) seen inside RBC - denatured haemoglobin
6
Q
Causes of Heinz bodies
A
- G6PD deficiency
- Alpha thalassaemia
7
Q
What are Howell-Jolly bodies
A
Individual blobs of DNA material seen inside RBC
8
Q
Causes of Howell-Jolly bodies
A
- Splenectomy or non-functioning spleen
- Severe anaemia
9
Q
What are reticulocytes
A
Immature RBC - larger than normal RBC, and still have RNA material in them
10
Q
Cause of high retic %
A
Rapid turnover of RBC, e.g. haemolytic anaemia
11
Q
What are schistocytes
A
Fragments of RBC
12
Q
Causes of schistocytes
A
- Microangiopathic haemolytic anaemia (small blood clots obstruct small blood vessels)
- Metallic heart valve replacement
13
Q
Causes of microangiopathic haemolytic anaemia
A
- Haemolytic uraemic syndrome
- Disseminated intravascular coagulation
- Thrombotic thrombocytopenic purpura
14
Q
What are sideroblasts
A
Immature red blood cells with a nucleus surrounded by iron blobs, seen in sideroblastic anaemia
15
Q
What is sideroblastic anaemia
A
When bone marrow cannot incorporate iron into the haemoglobin molecules
16
Q
Causes of sideroblastic anaemia
A
- Genetic defect
- Myelodysplastic syndrome
17
Q
What are smudge cells?
A
Ruptured WBC, occur when preparing blood film when cells are aged or fragile
18
Q
Causes of smudge cells
A
Chronic lymphocytic leukaemiaW
19
Q
What are spherocytes
A
Sphere shaped red blood cells without bi-concave disk shape
20
Q
Causes of spherocytes
A
Autoimmune haemolytic anaemia
Hereditary spherocytosis
21
Q
Causes of tear drop poikilocytes
A
Myelofibrosis
22
Q
Causes of basophilic stippling
A
- Lead poisoning
- Thalassaemia
- Sideroblastic anaemia
- Myelodysplasia
23
Q
Causes of pencil poikilocytes
A
Iron deficiency anaemia
24
Q
Causes of Burr cells (echinocytes)
A
Uraemia
Pyruvate kinase deficiency
25
Causes of acanthocytes
Abetalipoproteinaemia
26
Causes of hypersegmented neutrophils
Megaloblastic anaemia
27
Causes of microcytic anaemia
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemiaC
28
Causes of normocytic anaemia
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism
29
Causes of megaloblastic macrocytic anaemia
B12 deficiency
Folate deficiency
30
Most common acute leukaemia in adults
AML
31
Features antiphospholipid syndrome
- Predisposition to arterial and venous thromboses
- Recurrent fetal loss
- Thrombocytopenia
32
Most common condition antiphospholipid syndrome occurs secondary to
SLE
33
Complications antiphospholipid syndrome in pregnancy
Recurrent miscarriage
IUGR
Pre-eclampsia
Placental abruption
Pre-term delivery
VTE
34
Management antiphospholipid syndrome in pregnancy
Low dose aspirin once pregnancy confirmed on urine testing
LMWH once fetal heart seen on USS, discontined at 34 weeks
35
Investigation findings haemolytic anaemia
Anaemia
Reticulocytosis
Low haptoglobin
Raised LDH and indirect bili
Spherocytes and reticulocytes on blood film
36
Investigation findings specific to autoimmune haemolyic anaemia
Positive direct antiglobulin test (Coombs' test)
37
Where does haemolysis occur warm AIHA
Extravascular sites, e.g. spleen
38
Causes warm AIHA
Idioapthic
Autoimmune disease, e.g. SLE
Neoplasia - lymphoma, CLL
Drugs, e.g. metyldopa
39
Management warm AIHA
Treatment of underlying disease
Steroids +/- rituximab
40
What temp does warm AIHA occur best at
Body temp
41
What temp does cold AIHA occur best at
4 c
42
What antibody usually involved in warm AIHA
IgG
43
What antibody usually involved in cold AIHA
IgM
44
Where does haemolysis occur in cold AIHA
Intravascular
45
Features cold AIHA
Symptoms of Raynaud's and acrocyanosis
46
Causes cold AIHA
Neoplasia, e.g. lymphoma
Infections, e.g. mycoplasma, EBV
47
Presentation beta thalassaemia major
Presents in first year of life
Failure to thrive
Hepatosplenomegaly
48
Blood results beta thalassaemia major
Microcytic anaemia
HbA2 and HbF raised
HbB absent
49
Management beta thalassaemia major
Repeat transfusion
50
Prevention of iron overload caused by repeated transfusion in beta thalassaemia major
Iron chelation, e.g. desferrioxamine
51
Typical blood film hyposplenism
Howell-Jolly bodies
Pappenheimer bodies
Siderotic granules
Acanthocytes
52
Typical blood film iron deficiency anaemia
Target cells
Pencil poikilocytes
53
Blood film iron deficiency anaemia combined with B12/folate deficiency
Dimorphic film - mixed microcytic and macrocytic cells
54
Typical blood film myelofibrosis
Tear drop poikilocytes
55
Typical blood film intravascular haemolysis
Schistocytes
56
Typical blood film megaloblastic anaemia
Hypersegmented neutrophils
57
Features non-haemolytic febrile reaction to blood transfusion
Fever
Chills
58
Management non-haemolytic febrile reaction to transfusion
Slow or stop transfusion
Paracetamol
Monitor
59
Features minor allergic reaction to blood transfusion
Pruritis
Urticaria
60
Management minor allergic reaction to blood transfusion
Temporarily stop transfusion
Antihistamine
Monitor
61
Features anaphylaxis to blood transfusion
Hypotension
Dyspnoea
Wheezing
Angioedema
62
Management anaphylaxis blood transfusion
Stop transfusion
IM adrenaline
ABC
63
Features acute haemolytic reaction to blood transfusion
Fever
Chest and abdominal pain
Hypotension
Agitation
64
Management acute haemolytic reaction to transfusion
Stop transfusion
Confirm diagnosis - check matching details, send blood for direct Coombs, repeat typing and x match
Supportive care - fluid resus
65
Complications acute haemolytic transfusion reaction
Disseminated intravascular coagulation
Renal failure
66
Features transfusion associated circulatory overload
Pulmonary oedema
Hyertension
67
Treatment transfusion associated circulatory overload
Slow or stop transfusion
Consider IV loop diuretic and oxygen
68
What is transfusion related acute lung injury
Non-cardiogenic pulmonary oedema thought to be secondary to increased vascular permeability caused by host neutrophils that become activated by substances in donated blood
69
Features transfusion related acute lung injury
Hypoxia
Pulmonary infiltrates on CXR
Fever
Hypotension
Symptoms develop within 6 hours of transfusion
70
Management transfusion related acute lung injury
Stop transfusion
Oxygen and supportive care
71
Antibody involved in acute haemolytic transfusion reaction
IgM
72
What kind of pathogens are RBC most at risk of transmitting
Viral - HIV, HBV, HCV
73
What kind of pathogens platelets most at risk of transmitted
Bacteria (stored at room temp, increasing risk of bacterial proliferation)
74
Most common bacterial contaminants platelets
Staphylococcus epidermidis
Bacillus cereus
75
Measures taken to reduce risk of vCJD transmission from blood transfusion
All donations undergone removal of white cells to reduce any vCJD infectivity
Plasma derivatives have been fractionated from imported plasma rather than being sourced from UK donors
Recipients of blood components excluded from donating blood
76
Use of packed red cells
- Chronic anaemia
- Cases where infusion of large volumes of fluid may result in cardiovascular compromise
77
Use of platelet rich pplasma
Thrombocytopaenia and bleeding or requiring surgery
78
Use of platelet concentrate
Thrombocytopenia
79
What does FFP contain
Clotting factors
Albumin
Immunoglobulin
80
How much is unit of FFP
200-250ml
81
Dose FFP
12-15ml/kg
82
Use FFP
- Clinically significant but not major haemorrhage in patients with prothrombin time ratio or APTT ratio of >1.5
- Prophylaxis in patients undergoing major surgery with risk of significant bleeding
83
Universal donor FFP
AB (lacks any anti-A or anti-B antibodies)
84
What does cryoprecipitate contain
Concentrated factor VIII, von Willebrand factor, fibrinogen, factor XIII, and fibronectin
85
Use cryoprecipitate
-Patients with clinically significant but not major haemorrhage and fibrinogen concentration <1.5g/L
E.g.;
DIC
Liver failure
Hypofibrinogenaemia secondary to massive transfusion
- Prophylaxis in patients undergoing invasive surgery where risk of significant bleeding and fibrinogen <1.0
- von Willebrand disease
- Emergency treatment haemophiliacs when specific factors not available
86
What is SAG-mannitol blood
Blood with all plasma removed and replaced with a solution of;
- Sodium chloride
- Adenine
- Anhydrous glucose
- Mannitol
87
How much SAG-M blood can be given
4 units
88
Which blood components must be cross matched
Packed red cells
FFP
Cryoprecipitate
Whole blood
89
Which blood componenets can be ABO incompatible in adults
Platelets
90
Use of cell saver devices
Avoid the use of infusion of blood from donors into patients, e.g. Jehovah's witnesses
May reduce risk of blood borne infection
91
Contraindication cell saver devices
Malignant disease (may facilitate dissemination)
92
How long does IV vitamin K take to reverse warfarin
4-6 hours
93
How long does oral vitamin K take to reverse warfarin
24 hours
94
Dose of FFP in warfarin reversal
30ml/kg
95
Limitations of FFP
Need to give at least 1L fluid in 70kg person - not appropriate in fluid overload
Need blood group
Only use if human prothrombin complex not available
96
How long does human prothrombin complex take to reverse warfarin
1 hour
97
Limitation human prothrombin complex
Short half life - give with vitamin K
98
Purpose of irradiated blood
Depleted of T lymphocytes - avoid transfusion-associated graft versus host disease caused by engraftment of viable donor T lymphocytes
99
Requirements CMV negative blood
Granulocyte transfusions
Intra-uterine transfusions
Neonates up to 28 days post EDD
Elective transfusions during pregnancy (not during labour or delivery)
100
Requirements irradiated blood
Granulocyte transfusions
Intra-uterine transfusions
Neonates up to 28 days EDD
Bone marrow/stem cell transplants
Immunocompromised, e.g. chemo, congenital
Current or previous Hodgkin's lymphoma
101
Hb transfusion threshold
70g/L
80g/L if ACS
102
Hb target after transfusion
70-90g/L
80-100g/L in ACS
103
What is Burkitt's lymphoma
High grade B cell neoplasm
104
Major forms Burkitts' lymphoma
Endemic form
Sporadic form
105
Where does endemic form Burkitt's lymphoma typically involve
Maxilla or mandibleW
106
Where does sporadic form Burkitt's lymphoma affect
Abdominal (ileo-caecal)
107
Risk factors sporadic Burkitt's lymphoma
HIV
108
What is associated with the development of endemic form Burkitt's lymphoma
EBV
109
Microscopy findings Burkitt's lymphoma
'Starry sky' appearance - lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
110
Management Burkitt's lymphoma
Chemotherapy
111
Complication chemo in Burkitt's lymphoma
Tumour lysis syndrome
112
Prevention of tumour lysis syndrome in Burkitt's lymphoma
Rasburicase given before chemo
113
Complications tumour lysis syndrome
- Hyperkalaemia
- Hyperphosphataemia
- Hypocalcaemia
- Hyperuricaemia
- Acute renal failure
114
Most common leukaemia in adults
CLL
115
Complications chronic lymphocytic leukaemia
Anaemia
Hypogammaglobulinaemia → recurrent infections
Warm autoimmune haemolytic anaemia
Richter's transformation
116
What is Richter's transformation
When leukaemia cells enter the lymph node and change into high grade fast growing non-Hodgkins lymphoma
117
Symptoms Richter's transformation
Lymph node swelling
Fever without infection
Weight loss
Night sweats
Nausea
Abdominal pain
118
Pathophysiology CLL
Monoclonal proliferation of well-differentiated lymphocytes, almost always B-cells
119
Presentation CLL
Often asymptomatic, picked up with incidental finding of lymphocytosis
Constitutional - anorexia, weight loss
Bleeding
Infections
Lymphadenopathy
120
FBC CLL
Lymphocytosis
Anaemia (due to marrow replacement or AIHA)
Thrombocytopenia (due to marrow replacement or ITP)
121
Blood film CLL
Smudge cells
122
Key investigation CLL
Immunophenotyping
123
Genetics CML
Philadelphia chromosome present in more than 95% of patients
124
What is Philadelphia chromosome
Translocation between long arm of chromosome 9 and 22
125
Presentation CML
Anaemia - lethargy
Weight loss
Swelling
Splenomegaly → abdo discomfort
126
Blood film CML
Increase in granulocytes at different stages of maturation +/- thrombocytosis
May undergo blast transformation
127
First line treatment CML
Imatinib
128
Other treatments CML
Hydroxyurea
Interferon alpha
Allogenic BMT
129
What is cryoglobulinaemia
Presence of immunoglobulins which undergo reversible precipitation at 4 degrees C and dissolve when warmed to 37
130
Antibodies type I cryoglobulinaemia
Monoclonal - IgG, IgM
131
Associations type I cryoglobulinaemia
Multiple myeloma
Waldenstrom macroglobulinaemia
132
Antibodies type II cryoglobulinaemia
Mixed monoclonal and polyclonal, usually with rheumatoid factor
133
Associations type II cryoglobulinaemia
Hepatitis C
Rheumatoid arthritis
Sjorgen's
Lymphoma
134
Antibodies type III cryoglobulinaemia
Polyclonal, usually with rheumatoid factor
135
Associations type III cryoglobulinaemia
Rheumatoid arthritis
Sjorgen's
136
Presentation cryoglobulinaemia
Vascular purpura
Distal ulceration
Arthralgia
Diffuse glomerulonephritis
Raynaud's in type I
137
Investigation findings cryoglobulinaemia
Low complement, esp C4
High ESR
138
Management cryoglobulinaemia
Treatment of underlying condition
Immunosuppression
Plasmapheresis
139
Use DOACs
Prevention of stroke in non-valvular AF
Prevention of VTE following hip/knee surgery
Treatment of DVT and PE
140
Reversal agent dabigatran
Idarucizumab
141
Reversal agent rivaroxiban
Andexanet alfa
142
Reversal agent apixaban
Andexanet alfa
143
Reversal agent edoxaban
No authorised reversal agent
144
Coag results DIC
Low platelets
Low fibrinogen
High PT and APTT
High fibrinogen degradation products
145
Coag results warfarin
Prothrombin time increased
APTT normal
Bleeding time normal
Platelet count normal
146
Coag results aspirin
Prothrombin time normal
APTT normal
Bleeding time prolonged
Platelet count normal
147
Coag results heparin
Prothrombin time often normal, may be prolonged
APTT prolonged
Bleeding time normal
Platelet count normal
148
What is factor V leiden
Inherited thrombophilia - gain of function mutation of factor V leiden protein → activated factor V is inactivated 10x more slowly by activated protein C than normal
149
Inheritance G6PD deficiency
X-linked recessive
150
Factors precipitating crisis in G6PD deficiency
Drugs
Fava beans
151
Drugs precipitating crisis in G6PD deficiency
Anti-malarials - primaquine
Ciprofloxacin
Sulph- group drugs - sulphonamides, sulphasalazine, sulfonylureas
152
Features G6PD deficiency
Neonatal jaundice
Intravascular haemolysis
Gallstones
Splenomegaly
153
Blood film G6PD deficiency
Heinz bodies
Bite and blister cells
154
Diagnosis G6PD deficiency
G6PD enzyme assay
155
When should G6PD deficiency assay be performed for the diagnosis of G6PD deficiency
Around 3 months after acute episode of haemolysis, otherwise may get false negative results
156
Ethnicity G6PD deficiency
African
Mediterranean
157
Inheritance haemophilia
X-linked recessive
158
Cause haemophilia A
Deficiency factor VIII
159
Cause haemophilia B
Deficiency factor IX
160
Features haemophilia
Haemoarthroses
Haematomas
Prolonged bleeding after surgery or trauma
161
Blood results haemophilia
Prolonged APTT
Bleeding time, thrombin time, prothrombin time normal
162
Inheritance hereditary angioedema
Autosomal dominant
163
What is hereditary angioedema
Low plasma levels of C1 inhibitor protein → uncontrolled release of bradykinin → oedema of tissues
164
Presentation hereditary angioedema
Attacks may be preceded by painful macular rash
Painless, non-pruritic swelling of subcutaneous/submucosal tissues
May affect upper airways, skin, or abdominal organs (can present as abdo pain)
165
Acute management hereditary angioedema
IV C1-inhibitor concentrate
FFP if this is not available
166
Prophylaxis hereditary angioedema
Danazol (anabolic steroid)
167
Investigation findings hereditary angioedema
C1-INH level low during attach
Low C2 and C4 levels seen, even between attacks
168
Inheritance hereditary spherocytosis
Autosomal dominant
169
Presentation hereditary spherocytosis
- Failure to thrive
- Jaundice, gallstones
- Splenomegaly
- Aplastic crisis precipitated by parvovirus infection
170
Bloods hereditary spherocytosis
MCHC elevated
Spherocytes
Increase in reticulocytes
171
Diagnosis hereditary spherocytosis
If family history of HS, typical clinical features, and lab investigations, do not need any other tests
If diagnosis equivocal, EMA binding test and cryohaemolysis test
For atypical presentations, electrophoresis analysis of erythrocyte membranes
172
Management acute haemolytic crisis in hereditary spherocytosis
Treatment usually supportive
Transfusion if necessary
173
Longer term treatment hereditary spherocytosis
- Folate replacement
- Splenectomy
174
Ethnicity hereditary spherocytosis
Northern European
175
What is Hodgkin's lymphoma
Malignant proliferation of lymphocytes characterised by presence of Reed-Sternberg cells
176
Best prognosis Hodgkins lymphoma subtype
Lymphocyte predominant
177
Worst prognosis Hodgkin's lymphoma subtype
Lymphocyte depletedM
178
Most common Hodgkin's lymphoma subtype
Nodular sclerosing
179
Cells associated with nodular sclerosing Hodgkin's lymphoma
Lacunar cells
180
Cells associated with mixed cellularity Hodgkin's lymphoma
Large number of Reed-Sternberg cells
181
B symptoms in Hodgkin's lymphoma
Weight loss >10% in last 6 months
Fever >38
Night sweats
182
Factors associated with poor prognosis Hodgkin's lymphoma
B symptoms
Age >45
Stage IV
Haemoglobin <10.5
Lymphocyte count <600
Male
Albumin <40
WCC >15
183
Staging system Hodgkin's lymphoma
Lugano classification
184
Management Hodgkin's lymphoma
Chemo (mainstay) +/- radiotherapy
Haematopoietic cell transplantation - may be used for relapsed or refractory classic Hodgkin lymphoma
185
ITP in children vs adults
In children, acute thrombocytopenia may follow infection or vaccination
Adults tend to have more chronic condition
186
Presentation ITP
May be incidental following routine bloods
- Petechiae, purpura
- Bleeding
Catastrophic bleeding e.g. intracranial is uncommon
187
First line treatment ITP
Oral prednisolone
188
Treatment ITP in active bleeding/needs urgent procedure
Pooled normal human immunoglobulin
189
What is Evan's syndrome
ITP in association with autoimmune haemolytic anaemia
190
Main causes iron deficiency anaemia
Excessive blood loss
Inadequate dietary intake
Poor intestinal absorption
Increased iron requirements
191
Features iron deficiency anaemia
Fatigue
SOBOE
Palpitations
Pallor
Nail changes - koilonychia
Hair loss
Atrophic glossitis
Post-cricoid webs
Angular stomatitis
192
FBC IDA
Hypochromic microcytic anaemia
193
Ferritin IDA
Low
Can be falsely normal/high if co-existing inflammation as is acute phase reactant
194
TIBC/transferrin IDA
High
Transferrin saturation will be low
195
Blood film IDA
Anisopoikilocytosis (RBC different sizes and shapes)
Target cells
Pencil poikilocytes
196
Hb cut offs for endoscopy investigation within 2 weeks
Post-menopausal women ≤10
Men ≤11
197
Management IDA
Oral ferrous sulphate - continue for 3 months after iron deficiency corrected to replenish stores
198
SEs iron supps
Nausea
Abdominal pain
Constipation/diarrhoea
199
Features lead poisoning
Abdominal pain
Peripheral neuropathy, mainly motor
Neuropsychiatric features
Fatigue
Constipation
Blue lines on gum margin
200
Lead levels in blood considered significant
>10mcg/dl
201
FBC lead poisioning
Microcytic anaemia
202
Blood film lead poisioning
Red cell abnormalities, inc basophilic stippling and clover-leaf morphology
203
Management lead poisoning
Various chelating agents;
- DMSA
- D-penicillamine
- EDTA
- Dimercaprol
204
Megaloblastic causes of macrocytic anaemia
- Vitamin B12 deficiency
- Folate deficiency
205
Normoblastic causes of macrocytic anaemia
- Alcohol
- Liver disease
- Hypothyroidism
- Pregnancy
- Reticulocytosis
- Myelodysplasia
- Cytotoxics
206
Causes microcytic anaemia
- Iron deficiency anaemia
- Thalassaemia
- Congenital sideroblastic anaemia
- Anaemia of chronic disease (but more common normocytic)
- Lead poisoning
207
Causes normal haemoglobin with microcytosis
- Thalassaemia
- Polycythaemia rubra vera
208
Types of monoclonal gammopathy of undetermined significance
Non-IgM MGUS
IgM MGUS
Light chain MGUS
209
Non-IgM MGUS may progress too...
Multiple myeloma
AL amyloidosis
210
IgM MGUS may progress too...
Waldenstrom macroglobulinaemia
Lymphoma
211
Light chain MGUS may progress too...
Renal disease
AL amyloidosis
212
Features MGUS
Usually asymptomatic
10-30% have demyelinating neuropathy
213
MGUS vs myeloma
MGUS has:
- Normal immune function
- Normal beta-2 microglobulin levels
- Lower level of paraproteinaemia than myeloma (<30g/L IgG or <20g/L IgA)
- Stable level of paraproteinaemia
- No clinical features of myeloma, e.g. lytic lesions, renal disease
214
What is myelofibrosis
A myeloproliferative disorder caused by hyperplasia of abnormal megakaryocytes → stimulation of fibroblasts → fibrosis of bone marrow
215
Where does haematopoiesis occur in myelofibrosis
Liver and spleen
216
Presentation myelofibrosis
- Anaemia
- Massive splenomegaly
- Hypermetabolic symptoms - weight loss, night sweats
217
FBC myelofibrosis
Anaemia
High WCC and platelets early in disease
218
Film myelofibrosis
Tear drop poikilocytes
219
Bone marrow biopsy myelofibrosis
Unobtainable - dry tap - trephine biopsy needed
220
Other blood tests myelofibrosis
High urate
High LDH
221
What is multiple myeloma
Haematological malignancy characterised by plasma cell proliferation
222
Features multiple myeloma
- Hypercalcaemia
- Renal damage - dehydration and increased thirst
- Anaemia
- Bleeding (thrombocytopenia)
- Bone pain and pathological fractures
- Increased risk of infection
- Amyloidosis
- Carpal tunnel syndrome
- Neuropathy
- Hyperviscosity
223
Causes renal impairment in myeloma
- Monoclonal production of immunoglobulins → light chain depositions within renal tubules
- Amyloidosis
- Nephrocalcinosis
- Nephrolithiasis
224
Where is bone pain most common in myeloma
Back
225
Blood film myeloma
Rouleaux formation
226
Protein electrophoresis findings myeloma
Raised concentrations of monoclonal IgA/IgG proteins
227
X-ray findings myeloma
Rain drop skull
228
Diagnostic criteria multiple myeloma
One major + one minor, or 3 minor with signs/symptoms
Major:
- Plasmacytoma
- 30% plasma cells in bone marrow sample
- Elevated levels of M protein in blood or urine
Minor:
- 10-30% plasma cells in bone marrow sample
- Minor elevations in level of M protein in blood or urine
- Osteolytic lesions
- Low levels of antibodies in the blood
229
Viral causes neutropenia
HIV
EBV
Hepatitis
230
Drug causes neutropenia
Cytotoxics
Carbimazole
Clozapine
231
Other causes neutropenia
Benign ethnic neutropenia
Haematological malignancy - myelodysplastic malignancies, aplastic anaemia
Rheumatological conditions
SLE
Rheumatoid arthritis
Severe sepsis
Haemodialysis
232
Timeline neutropenic sepsis
Most commonly occurs 7-14 days after chemo
233
Diagnostic criteria neutropenic sepsis
Neuts <0.5 in patient having anti-cancer therapy and:
- Temp >38 or
- Other signs/symptoms consistent with clinically significant sepsis
234
Most common bacteria causing neutropenic sepsis
Coag negative, gram +ve bacteria, particularly staph epidermidis
235
Prophylaxis neutropenic sepsis
If anticipated that patients likely to have neutrophil count <0.5, should be offered a flouroquinolone
236
First line antibiotics neutropenic sepsis
Tazocin.
237
Management neutropenic sepsis if still febrile and unwell after 48h IV Abx
Meropenum +/- vanc
238
Management neutropenic sepsis ongoing after 4-6 days IV Abx
Fungal infection investigations, e.g. HRCT
239
Causes normocytic anaemia
Anaemia of chronic disease
Chronic kidney disease
Aplastic anaemia
Haemolytic anaemia
Acute blood loss
240
What is paraproteinaemia
A haematological condition characterised by abnormal overproduction of a single type of immunoglobulin or immunoglobulin fragment
241
Causes of benign paraproteinaemia
Monoclonal gammopathy of undetermined significance
Transient paraproteinaemia, e.g. following infection
242
Causes of malignant paraproteinaemias
- Multiple myeloma
- Waldenstrom macroglobulinaemia
- Primary amyloidosis
- B-cell lymphoproliferative disorders, e.g. CLL, non-Hodgkin lymphoma
243
Clinical features paraproteinaemia
- Hyperviscosity syndrome
- Neuropathy, e.g. sensory, motor, or autonomic dysfunction
- Renal dysfunction
- Haematologic abnormalities, e.g. anaemia, thrombocytopenia, leukopenia
244
What is paroxysmal noctural haemoglobinuria
An acquired disorder leading to haemolysis (mainly intravascular) of haematological cells
245
Features paroxysmal noctural haemoglobinuria
- Haemolytic anaemia
- RBC, WBC, platelets, or stem cells may be affected - can be pancytopenia
- Haemoglobinuria
- Thrombosis
- Aplastic anaemia in some patients
246
Diagnostic test paroxysmal noctural haemoglobinuria
Flow cytometry of blood to detect low levels of CD59 and CD55
247
Management paroxysmal noctural haemoglobinuria
Blood product replacement
Anticoagulation
Eculizumab
Stem cell transplant
248
Platelet threshold for transfusion pre-surgery/invasive procedure
>50 for most patients
50-75 if high risk of bleeding
>100 if at critical site, e.g. CNS
249
Platelet threshold for transfusion if no active bleeding
10
250
Conditions contraindicating platelet transfusion
- Chronic bone marrow failure
- Autoimmune thrombocytopenia
- Heparin induced thrombocytopenia
- Thrombotic thrombocytopenic purpura
251
Platelet threshold for transfusion clinically significant bleeding
<30
Lower threshold if severe bleeding or bleeding at critical sites, e.g. CNS
252
Causes relative polycythaemia
Dehydration
Stress - Gaisbock syndrome
253
Causes primary polycythaemia
Polycythaemia rubra vera
254
Secondary causes polycythaemia
COPD
Altitude
OSA
Excessive erythropoietin
255
Causes of excessive erythropoietin → polycythaemia
Cerebellar haemangioma
Hypernephroma
Hepatoma
Uterine fibroids
256
How to differentiate between true (primary/secondary) polycythaemia and relative polycythaemia
Red cell mass studies - in true, total red cell mass >35 in males and >32 in women
257
What is polycythaemia vera
Myeloproliferative disorder caused by clonal proliferation of marrow stem cells leading to increase in red cell volume, often accompanied by overproduction of neutrophils and platelets
258
Features polycythaemia vera
- Pruritis, typically after a hot bath
- Splenomegaly
- Hypertension
- Hyperviscosity - arterial thrombosis, venous thrombosis
- Haemorrhage
259
ESR in polycythaemia vera
Low
260
First line investigations in polycythaemia vera
FBC/film
JAK2 mutations
Serum ferritin
Renal and liver function tests
261
Findings FBC in polycythaemia vera
Raised haematocrit
Neutrophils, basophils and platelets raised in half of patients
262
Management polycythaemia vera
- Aspirin
- Venesection
- Chemotherapy
263
Complications polycythaemia vera
Thrombotic events
Myelofibrosis
Acute leukaemia
264
Features post-thrombotic syndrome
- Painful, heavy calves
- Swelling
- Varicose veins
- Venous ulceration
265
Treatment post-thrombotic syndrome
Compression stockings
Keep legs elevated
266
Why increased risk of DVT/PE in pregnancy
- Increase in factors VII, VIII, X, fibrinogen
- Decrease in protein S
- Uterine presses on IVC causing venous stasis in legs
267
Management DVT/PE in pregnancy
SC LMWH
268
Age of onset homozygous sickle cell
4-6 months (abnormal HbSS molecules take over from fetal haemoglobin)
269
Diagnostic investigation sickle cell anaemia
Haemoglobin electrophoresis
270
Prophylactic management of sickle cell
Hydroxyurea - increases HbF levels
271
Vaccination sickle cell
Pneumococcal polysaccharide vaccine every 5 years
272
Precipitants thrombotic (painful) crises
- Infection
- Dehydration
- Deoxygenation, e.g. high altitude
273
Complications thrombotic crises sickle cell
Infarcts in various organs;
- Avascular necrosis of hip
- Hand-foot syndrome in children
- Lung infarct
- Splenic infact
- Brain infarct
274
What is acute chest syndrome sickle cell
Vaso-occlusion in pulmonary microvasculature → infarction in lung parenchyma
275
Presentation acute chest syndrome in sickle cell
- Dyspnoea
- Chest pain
- Pulmonary infiltrates on CXR
- Low pO2
276
Management acute chest syndrome in sickle cell
- Pain relief
- Resp support, e.g. oxygen
- Antibiotics
- Transfusion
277
Purpose antibiotics in acute chest syndrome
Infection may precipitate acute chest syndrome
Clinical findings can be difficult to distinguish from pneumonia
278
Purpose transfusion in acute chest syndrome
Improves oxygenation
279
Cause aplastic crises in sickle cell
Infection with parvovirus
280
Investigation findings aplastic crises in sickle cell
- Sudden fall in Hb
- Reduced retic count (due to BM suppression)
281
What is sequestration crises sickle cell
Sickling within organs, e.g. spleen and lungs, causing pooling of bloods with worsening of anaemia
282
Retic count in sequesteration crises
High
283
Indications for transfusion in sickle cell
- Severe or symptomatic anaemia
- Pregnancy
- Pre-op
284
Indications for exchange transfusion in sickle cell
Acute vaso-occlusive crisis (stroke, acute chest syndrome, multiorgan failure, splenic sequesteration)
285
Causes of massive splenomegaly
Myelofibrosis
CML
Visceral leishmaniasis
Malaria
Gaucher's syndrome
286
Causes of non-massive splenomegaly
- Portal hypertension
- Lymphoproliferative disease, e..g CLL, Hodgkin's
- Haemolytic anaemia
- Infection - hepatitis, glandular fever
- Infective endocarditis
- Sickle cell, thalassaemia
- RA
287
Causes of thrombocytosis
- Reactive
- Malignancy
- Essential thrombocytosis
- Hyposplenism
288
What is essential thrombocytosis
Myeloproliferative disorder - megakaryocyte proliferation results in overproduction of platelets
289
Features essential thrombocytosis
- Platelet count >600
- Thrombosis (venous or arterial) or haemorrhage can be seen
- Burning sensation in hands
290
Genetics essential thrombocytosis
JAK2 mutation found in 50% of patients
291
Management essential thrombocytosis
- Hydroxyurea
- Interferon-alpha in younger patients
- Low dose aspirin to reduce thrombotic risk
292
Use tranexamic acid
- Menorrhagia
- Trauma
293
Role of tranexamic acid in trauma
Benefit when administered in the first 3 hours
Given as IV bolus → infusion
294
Prevention tumour lysis syndrome
- IV fluids
- Rasburicase if high risk, allopurinol if lower risk
295
Underlying conditions increasing risk of VTE
Malignancy
Thrombophilia
Heart failure
Antiphospholipid syndrome
Behcet's
Polycythaemia
Nephrotic syndrome
Sickle cell
Paroxysmal noctural haemoglobinuria
Hyperviscosity syndrome
Homocystinuria
296
Thrombophilias eg
Activated protein C resistance
Protein C deficiency
Protein S deficiency
297
Subclavian vs femoral CVC for VTE risk
Femoral higher risk than subclavian
298
Medications increasing VTE risk
COCP
HRT (combined>oestrogen only)
Raloxifene, tamoxifen
Antipsychotics, esp olanzapine
299
What is required for absorption of vitamin B12
Binding to intrinsic factor
300
Where is intrinsic factor secreted from
Parietal cells in stomach
301
Where is intrinsic factor absorbed
Terminal ileum
302
Causes B12 deficiency
- Pernicious anaemia (most common)
- Post gastrectomy
- Vegan/poor diet
- Disorders/surgery of terminal ileum, e.g. Crohn's (disease activity or post-resection)
- Metformin (rare)
303
Features of B12 deficiency
- Macrocytic anaemia
- Sore tongue and mouth
- Neurological symptoms
- Neuropsychiatric symptoms, e.g. mood disturbance
304
Features neurological symptoms of B12 deficiency
Dorsal column usually affected first (joint position, vibration) prior to distal parasthesia
305
Treatment B12 deficiency with no neuro involvement
1mg IM hydroxycobalmin 3/week for 2 weeks, then once every 3 months
306
Treatment B12 deficiency if also deficient in folic acid
Important to treat B12 def first, to avoid precipitating subacute combined degeneration of cord
307
Inheritance von Willebrands
Autosomal dominant
308
Common types of bleeding in vWD
Epistaxis and menorrhaga
309
Type 1 vWD = ?
Partial reduction in wVF
310
Type 2 vWD = ?
Abnormal form of vWF
311
Type 3 vWD = ?
Total lack of vWF
Autosomal recessive
312
Coag results vWD
Prolonged bleeding time
APTT may be prolonged
Factor VIII may be mod reduced
Defective platelet aggregation with ristocetin
313
