Passmed Haematology Mushkies Flashcards
1
Q
Unprovoked DVT investigations?
A
1, Underlying malignancy (CXR, bloods, urine, CT abdo pelvis if >40y/o)
2. Anti-phospholipid Abs
3.
2
Q
Smear/smudge cells?
A
CLL
3
Q
Why recurrent infections in CLL?
A
Due to hypogammaglobulinaemia
4
Q
What AIHA is a complication of CLL?
A
Warm
5
Q
4 complications of CLL?
A
- Anaemia
- Hypogammaglobulinaemia –> recurrent infections
- Warm AIHA
- Richter’s transformation
6
Q
What is Richter’s transformation?
A
When CLL cells enter the lymph node and changer into a high grade, fast-growing NHL (DLBCNHL)
7
Q
Howell-Jolly bodies?
A
Hyposplenism
8
Q
Basophilic stippling?
A
- Lead poisoning
- Thalassaemia
- Sideroblastic anaemia
- Myelodysplasia
9
Q
Cabot rings?
A
Lead poisoning
10
Q
Rouleaux?
A
Chronic inflammation and myeloma
11
Q
Schistocytes?
A
- Intravascular haemolysis
- Mechanical heart valve
- DIC
12
Q
Dohle bodies?
A
Neurtrophil response to infection
13
Q
Hyposplenism on blood film?
A
- Howell-Jolly bodies
2. Siderocytes
14
Q
Causes of hyposplenism?
A
- Splenectomy
- SCD
- Coeliac
- Graves
- SLE
- Amyloid
15
Q
Most common hereditary haemolytic anaemia in people of northern European descent?
A
Hereditary spherocytosis
16
Q
Hereditary spherocytosis inheritance?
A
AD
17
Q
Osmotic fragility test?
A
Hereditary spherocytosis
18
Q
Current main test for hereditary spherocytosis?
A
- Cryohaemolysis testing
2. EMA binding test
19
Q
Mx of hereditary spherocytosis?
A
- Folate replacement
2. Splenectomy
20
Q
What kind of haemolysis is found in hereditary spherocytosis?
A
Extravascular haemolysis
21
Q
G6PDD inheritance?
A
X-linked recessive
22
Q
G6PDD ethinicity?
A
African and Mediterranean
23
Q
Hereditary spherocytosis ethnicity?
A
North European
24
Q
Heinz bodies?
A
- G6PDD
2. Alpha thalassaemia
25
G6PDD test?
Enzyme activity of G6PD
26
Presentation of G6PDD/HS?
1. Neonatal jaundice
2. Gallstones
3. Infection/drugs precipitate haemolysis
27
Thymoma condition association?
1. Myasthenia Gravis (30-40% pts with thymoma)
2. Red cell aplasia
3. Dermatomyositis
4. SLE, SIADH
28
Lambert Eaton syndrome association?
Small cell lung cancer
29
What is a thymoma?
Most common tumour of the anterior mediastinum and is usually detected between the 6th and 7th decades of life
30
How do thymomas cause death?
1. Airway compression
| 2. Cardiac tamponade
31
Transfusion threshold for pts with ACS?
80g/L
32
Transfusion threshold for pts without ACS?
70g/L
33
RBC storage temperature?
4 degrees
34
RBC transfusion time in non-emergency?
90-120 mins
35
Spherocytes causes?
1. Congenital = HS
| 2. Acquired = AIHA
36
AIHA Ix?
Direct Coombs Test
37
Decrease in serum haptoglobins?
Intravascular haemolysis
38
Target cells causes?
1. SCD/Thalassaemia
2. IDA
3. Hyposplenism
4. Liver disease
39
Tear drop poikilocytes?
Myelofibrosis
40
Pencil poikilocytes?
IDA
41
Burr cells aka?
Echinocytes
42
Burr cells?
1. Uraemia
| 2. Pyruvate kinase deficiency
43
Acanthocytes?
Abetalipoproteinaemia
44
Hypersegmented neutrophils?
Megaloblastic anaemia
45
Thrombophilia cause classification?
1. Congenital
| 2. Acquire
46
Acquired thrombophilia causes?
1. Antiphospholipid syndrome
| 2. Drugs, most commonly COCP
47
Congenital thrombophilia causes?
1. Gain of function polymorphisms
| 2. Natural anticoagulant deficiencies
48
Gain of function polymorphisms causing thrombophilia?
1. Factor V Leiden (Activated Protein C resistance)
| 2. Prothrombin gene mutaiton
49
Natural anticoagulant deficiencies causing thrombophilia?
1. Antithrombin III deficiency
2. Protein C deficiency
3. Protein S deficiency
50
Macrocytic anaemia with hypersegmented neutrophils?
Megaloblastic anaemia
51
Macrocytic anaemia causes?
1. Megablastic
| 2. Non-megaloblastic
52
Megaloblastic anaemia causes?
1. Vit B12 deficiency
| 2. Folate deficiency
53
Non-megaloblastic anaemia causes?
1. Alcohol
2. Liver disease
3. Hypothyroidism
4. Pregnancy
5. Reticulocytosis
6. Myelodysplasia
7. Drugs = cytotoxics
54
HL/NHL features?
1. Lymphadenopathy
2. Constitutional B symptoms (fever, weight loss, night sweats, lethargy)
3. Extranodal disease = gastric, BM, lungs, skin, CNS
55
What are the B symptoms?
1. Weight loss >10% in 6m
2. Fever > 38C
3. Night sweats
56
Staging for HL?
Ann Arbor
57
What is the Ann Arbor Staging?
CT CAP for HL
1. Single lymph node region
2. Two or more regions on same side of diaphragm
3. Lymph node involvement on both sides of the diaphragm
4. Extranodal site involvement
58
HL on histology?
Reed Sternberg cells
59
Hodgkins lymphoma classification?
1. Classical (4 types)
| 2. Nodular lymphocyte-predominant HL
60
Classical HL types?
1. Nodular sclerosing HL
2. Mixed cellularity HL
3. Lymphocyte rich HL
4. Lymphocyte deplete HL
61
Tx of HL?
Chemo and radiotherapy
62
Dx of HL?
Histology of lymph node
63
Viral assocation of HL?
EBV
64
Prognosis of HL?
Stage I = 85% survival at 5 years
65
Best HL prognosis subtype?
Lymphocyte rich HL
66
Worst HL prognosis subtype?
Lymphocyte deplete HL
67
Should compression stocking be offered routinely to all pts with DVT?
No
68
What is post-thrombotic syndrome?
Clinical syndrome occurring after thrombosis due to venous outflow obstruction and venous insufficiency, leading to chronic venous hypertension
69
Features of post-thrombotic syndrome?
1. Painful, heavy calves
2. Pruritis
3. Swelling
4. Varicose veins
5. Venous ulceration
70
Mx of post-thrombotic syndrome?
1. Keep leg elevated
| 2. Graduated Compression stockings
71
AML blood film?
Auer rod
72
Pseudo Pelger-Huet cells?
CML
73
What is CLL?
A monoclonal proliferation of well differentiated lymphocytes, usually B cells (99%)
74
Most common leukaemia in adults?
CLL
75
CLL presentation?
1. Usually none
2. Constitutional = anorexia, weight loss
3. Bleeding, infection
76
Ix of CLL?
1. Blood film --> smudge/smear cells
| 2. Immunophenotyping
77
EMA binding test?
Hereditary spherocytosis
78
Platelet transfusion threshold if actively bleeding?
<30
79
Platelet transfusion threshold if severe bleeding at critical site e.g. CNS?
<100
80
Presurgical prophylactic platelet level targets?
1. >50 for most pts
2. 50-75 if high risk of bleeding
3. >100 if at critical site
81
Platelet transfusion threshold if no active bleeding?
>10
82
4 C/Is for platelet transfusions?
1. TTP
2. Heparin induced thrombocytopenia
3. AI thrombocytopenia
4. Chronic BM failure
83
Causes of aplastic anaemia?
1. Idiopathic
2. Congenital = Fanconi's anaemia, dyskeratosis congenita
3. Drugs
4. Toxins e.g. benzene
5. Infections
6. Radiation
84
2 infections that cause aplastic anaemia?
1. Parvovirus
| 2. Hepatitis
85
5 drugs that cause aplastic anaemia?
1. Cytotoxics
2. Phenytoin
3. Chloramphenicol
4. Suphonamides
5. Gold
86
Blood finding of aplastic anaemia?
Pancytopenia
87
Acute haemolytic transfusion reaction management?
1. Stop transfusion
2. Generous fluid resuscitation
3. Inform lab
88
Blood product transfusion complications?
1. Immunological
2. TRALI
3. TACO
4. Infective
5. Other
89
Immunological complications of blood product transfusion?
1. Acute haemolytic
2. Non-haemolytic febrile (<1.5 increase)
3. Allergic/anaphylaxis
90
What is TRALI?
Transfusion associated acute lung injury
91
What is TACO?
Transfusion associated circulatory overload
92
What are 'other' complications of blood transfusion?
1. Hyperkalaemia
2. Iron overload
3. Clotting
93
What causes an acute haemolytic transfusion reaction (AHTR)?
Mismatch of ABO blood group that causes massive intravascular haemolysis
94
Acute haemolytic transfusion reaction features?
1. Abdo and chest pain
2. Agitation
3. Hypotension
95
2 complications of acute haemolytic transfusion reaction?
1. DIC
| 2. Renal failure
96
What causes a non-haemolytic febrile transfusion reaction?
Due to WBC HLA antibodies, often the result of sensitisation by previous pregnancies or transfusions
97
Mx of non-haemolytic febrile transfusion reaction?
Paracetamol
98
Dx of TRALI?
Development of hypoxaemia/ARDS within 6 hours of transfusion (hypoxia, fever, hypotension, pulmonary infiltrates on CXR)
99
Raised ESR and osteoporosis?
Multiple myeloma until proven otherwise
100
MM features?
CRAB + OTHER
1. Hypercalcaemia
2. Renal failure
3. Anaemia
4. Bone disease = pain, osteoporosis, pathological fractures, osteolytic lesions
101
What are the non-CRAB features of myeloma?
1. Lethargy
2. Infection
3. Amyloidosis --> macroglossia, carpal tunnel syndrome, neuropathy, hyperviscosity
102
MM Ix?
1. Monoclonal proteins (IgG or IgA) in the serum and urine (Bence Jones Proteins)
2. Increased plasma cells in bone marrow
3. Whole body MRI/skeletal survey for bone lesions (rain-drop skull)
103
MM Dx?
1 major + 1 minor OR 3 minor --> in a pt with s&s of myeloma
104
Major MM criteria?
1. Plasmacytoma on biopsy
2. 30% plasma cells in a BM sample
3. Elevated M protein levels in blood or urine
105
Minor MM criteria?
1. 10-30% plasma cells in a BM sample
2. Minor elevated blood/urine M protein levels
3. Osteolytic lesions
4. Low levels of Abs
106
Hypercalcaemia in MM mechanism?
1. Main = increased osteoclastic bone resorption caused by local cytokines e.g. IL1, TNF, released by the myeloma cells
2. Impaired renal function
3. Increased renal tubular calcium reabsorption
4. PTHrp release
107
Decreased MCHC?
Microcytic anaemia
108
Increased MCHC?
1. HS
| 2. AIHA
109
Normocytic anaemia with low iron, low TIBC, raised ferritin?
ACD
110
Microcytic anaemia, low ferritin, raised TIBC?
IDA
111
5 causes of normocytic anaemia?
1. ACD
2. CKD
3. Aplastic anaemia
4. Haemolytic anaemia
5. Acute blood loss
112
Epidemiological distribution of Reed-Sternberg cells?
Bimodal, peaks in 3rd and 7th decades
113
Lacunar cells?
Nodular sclerosing HL
114
HL with a large number of Reed Sternberg cells?
Mixed cellularity HL
115
What is myelofibrosis?
A myeloproliferative disorder due to hyperplasia of abnormal megakaryocytes. The resultant release of PDGF stimulates fibroblasts
116
Dry tap?
Myelofibrosis --> trephine biopsy is required
117
vWD bleeding time, plt levels, and APTT?
1. Increased bleeding time
2. Normal plt levels
3. APTT prolonged
118
Most common genetic bleeding disorder?
vWD
119
vWD inheritance?
AD
120
Why does vWD lead to prolonged APTT?
It is a carrier molecule for FVIII which is measured by APTT
121
What is the role of vWF?
A large glycoprotein which forms massive multimers, that promotes platelet adhesion to damaged endothelium. Also acts as a carrier for FVIII
122
3 types of vWD?
1. Type I = partial reduction in vWF (80%)
2. Abnormal form of vWF
3. total lack of vWF (AR)
123
Mx of vWD?
1. Tranexamic acid for mild bleeding
2. DDAVP
3. FVIII concentrate
124
Mechanism of DDAVP tx for vWD?
Raises levels of vWF by inducing release of vWF from Weibel-Palade bodies in endothelial cells
125
How does vWD present?
Mucocutaneous bleeding after mild injury e.g. nose-bleeds, bruising
126
MOA behind myeloma causing a stroke?
Paraproteinaemia --> hyperviscosity --> stroke
127
5 SCA crises?
1. Thrombotis 'painful' crises
2. Sequestration
3. Acute chest syndrome
4. Aplastic
5. Haemolytic
128
SCA thrombotic crises aka?
Painful or vaso-occlusive crises
129
Precipitants of SCA thrombotic crises?
1. Infection
2, Dehydration
3. Deoxygenation
130
Sites of SCA thrombotic crises?
1. AVN of hip
2. Hand-foot syndrome in children
3. Lungs
4. Spleen
5. brain
131
What is SCA sequestration crisis?
Sickling of blood within organs e.g. spleen or lungs causes pooling of blood with worsening of the anaemia
132
Most common cause of SCA death after childhood?
Acute chest syndrome
133
SCA acute chest syndrome features?
1. Dyspnoea
2. Chest pain
3. Pulmonary infiltrates
4. Low pO2
134
Suspicious of myeloma initial investigation?
Urgent protein electrophoresis and Bence Jones Protein urine test (within 48 hours)
135
Mirror image nuclei?
Reed Sternberg cell
136
Lymphadenopathy in HL features?
Painless, non-tender, asymmetrical
137
How long taken for 1 unit RBC transfusion in pt with HF?
3 hours, to reduce chances of pt developing circulatory overload
138
ITP stands for?
Immune/idiopathic thrombocytopenic purpura
139
ITP pathophysiology?
Abs are directed against the glycoprotein IIb/IIIA or Ib-V-IX complex
140
Classification of ITP?
Acute and Chronic
141
Acute ITP features?
1. More common in children, equal sex incidence
2. May follow infection or vaccination
3. Usually self limiting course over 1-2 weeks
142
Chronic ITP features?
1. More common in young/middle-aged women
| 2. Tends to run a relapsing-remitting course
143
What is Evans syndrome?
ITP in association with AIHA
144
How long before an operation should the COCP be stopped?
4 weeks to reduce risk of PE/DVT
145
Drugs that are RFs for VTE?
1. COCP
2. HRT
3. Raloxifene/Tamoxifen
4. Antipsychotics (esp. olanzapine)
146
What percentage of pts with a diagnosed PE have no major RFs?
40%
147
Mx of urticarial blood transfusion reaction without anaphylaxis?
Antihistamine and transfusion temporarily stopped
148
When is FFP needed?
Low levels of coagulation factors with a low INR
149
Most common NHL in UK?
Diffuse Large B cell lymphoma (40%)
150
What is more common, NHL or HL?
NHL
151
RFs for lymphoma?
1. Elderly
2. Caucasian
3. Hx of viral infection, esp EBV
4. FHx
5. Certain chemical agents e.g. pesticides, solvents
6. Hx of chemo or radio
7. Immunodeficiency e.g. transplant, HIV, DM
8. AI disease e.g. SLE, Sjogrens, Coeliac
152
HL characteristic SBA lymphadenopathy feature?
Alcohol-induced pain (only in 10%)
153
B symptoms typically earlier in HL or NHL?
HL
154
Extranodal disease more common in HL or NHL?
NHL
155
Starry sky?
Burkitt's lymphoma
156
Haemophilia inheritance?
X linked recessive
157
What percentage of haemophilia pts have no FHx of the condition?
Up to 30%
158
What is christmas disease?
Haemophilia B
159
Features of haemophilia?
1. Haemarthroses, haemophilia
| 2. Prolonged bleeding after surgery or trauma
160
Coag features of haemophilia?
1. Prolonged APTT
| 2. Bleeding time, thrombin time, prothrombin time all normal
161
What percentage of pts with Haemophilia A develop Abs to FVIII treatment?
10-15%
162
Myelofibrosis, WBC and platelet count early in the disease?
Both high
163
Urate and LDH levels in myelofibrosis?
Both high, reflect increased cell turnover
164
2 characteristic HL FBC features?
Normocytic anaemia, eosinophilia
165
2 major forms of Burkitt's lymphoma?
1. Endemic (African) form = typically involves maxilla or mandible
2. Sporadic form = typically HIV associated, abdominal ileo-caecal tumours most common
166
Burkitt's lymphoma gene translocation?
c-Myc translocation, usually t(8:14)
167
Explain starry sky appearance of Burkitt's lymphoma?
Lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
168
Which Burkitt's lymphoma form is more associated with EBV?
Endemic
169
Management of Burkitt's lymphoma ?
Chemotherapy
170
Common complication of Burkitt's lymphoma tx?
Tumour lysis syndrome
171
What is given before chemo to reduce chance of tumour lysis syndrome occuring?
1. Allopurinol OR
| 2. Rasburicase
172
Rasburicase MOA?
1. A recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin
2. Allantoin is 5-10 times more soluble than uric acid, so renal excretion is more effective
173
5 complications of tumour lysis syndrome?
1. Hyperkalaemia
2. Hyperphosphataemia
3. Hyperuricaemia
4. Hypocalcaemia
5. AKI
174
Classification of generalised lymphadenopathy?
1. Infection
2. Inflammation
3. Malignancy
175
Infective causes of generalised lymphadenopathy?
1. Viral = IM, HIV, Rubella, CMV, PVB19
| 2. TB, toxoplasmosis
176
Neoplastic causes of generalised lymphadenopathy?
1. Leukaemia
| 2. Lymphoma
177
Inflammatory causes of generalised lymphadenopathy?
1. AI = SLE, RhA
2. Sarcoidosis
3. Drugs
178
3 drugs that cause generalised lympahdenopathy?
1. Phenytoin
2. Allopurinol
3. Isoniazid
179
Warfarin duration in pts with a provoked VTE?
3 months, then 'assess the risks and benefits of extending treatment'
180
Warfarin duration in pts with unprovoked VTE?
6 months
181
LMWH duration for pts with active cancer?
6 months
182
Pappenheimer bodies?
Hyposplenism
183
4 cell types with hyposplenism?
1. Target cells
2. Howell Jolly bodies
3. Pappenheimer bodies
4. Acanthocytes
184
What is cryoprecipitate?
1. Blood product made from plasma
| 2. Usually transfused as a 6 unit pool
185
Indications for cryoprecipitate?
1. Massive haemorrhage
| 2. Uncontrolled bleeding due to haemophilia
186
Components of cryoprecipitate?
1. Factor VIII
2. Factor XIII
3. Fibrinogen
4. vWF
187
SCA crisis management?
1. Analgesia e.g. opiates
2. Rehydrate
3. Oxygen
4. Abx if infection
5. Blood transfusion
6. Exchange transfusion e.g. if neuro complications
188
SCA long term management?
1. Hydroxyurea
| 2. Pneumococcal vaccine every 5 yeras
189
Hydroxyurea for SCA MOA?
Increases HbF levels and is used in the prophylactic management of SCA to prevent painful episodes
190
Transmission of which type of infection is most likely to occur following a platelet transfusion and why?
Bacterial, as platelet concentrates are stored at room temperature
191
DIC platelets, PT, APTT, and bleeding time?
Platelets low, all times prolonged
192
4 causes of DIC?
1. Sepsis
2. Trauma
3. Malignancy
4. Obstetric complications e.g. amniotic fluid embolism, HELLP
193
What is the critical mediator of DIC?
Tissue factor
194
Warfarin effect on PT/APTT/bleeding time/plt count?
Prolonged PT
195
Aspirin effect on PT/APTT/bleeding time/plt count?
Prolonged bleeding time
196
Heparin effect on PT/APTT/bleeding time/plt count?
PT may be prolonged, APTT prolonged
197
What does FFP contain?
1. Clotting factors
2. Albumin
3. Immunoglobulin
198
Warfarin reversal strategies?
1. Stop warfarin
2. Vitamin K (IV takes 4-6h to work, oral takes 24hrs to work)
3. Human prothrombin complex (reversal within 1 hour, short half life therefore give with Vit K)
4. FFP (only use if human prothrombin complex is not available)
199
AKI with high phosphate and high uric acid?
Tumour lysis syndrome
200
Grading system for tumour lysis syndrome?
Cairo-Bishop scoring system
201
3 drugs to be avoided whilst pt is on methotrexate?
1. Co-trimoxazole
2. Trimethoprim
3. Chloramphenicol eye drops
202
Causes of a relative polycythaemia (pseudopolycythaemia)?
1. Dehydration
2. Diuretics
3. Gaisbock syndrome
203
Mutation in 95% of pts with Polycythaemia Rubra Vera?
JAK2
204
Features of Polycythaemia Rubra Vera?
1. Hyperviscosity
2. Pruritis
3. Splenomegaly
4. Haemorrhage (secondary to abnormal plt function)
5. Plethoric appearance
6. HTN in 1/3rd of pts
7. Low ESR
205
Waldenstrom's macroglobulinaemia demographic?
Older men
206
Waldenstrom's macroglobulinaemia aka?
Lymphoplasmacytic Lymphoma
207
Waldenstrom's macroglobulinaemia key feature?
Monoclonal IgM paraproteinaemia
208
Presentation of Waldenstrom's macroglobulinaemia?
1. Constitutional symptoms
2. Hyperviscoscity e.g. stroke, visual disturbance
3. Hepatosplenomegaly
4. Lymphadenopathy
5. Cryoglobulinaemia e.g. Raynauds
209
Why does hyperviscosity occur in Waldenstrom's macroglobulinaemia?
Pentameric configuration of IgM increases serum viscosity
210
Haemarthrosis without fracture?
Haemophilia A or B
211
Factors affected by Heparin?
2, 9, 10, 11
212
Factors affected by Warfarin?
2, 7, 9, 10
213
Factors affected by DIC?
1, 2, 5, 8, 11
214
Factors affected by liver disease?
1, 2, 5, 7, 9, 10, 11
215
Bleeding with: increased APTT, normal PT, normal BT?
Haemophilia
216
Bleeding with: increased APTT, normal PT, increased BT?
vWD
217
Bleeding with increased APTT, increased PT, normal BT?
Vit K deficiency
218
Causes of IDA?
1. Inadequate intake
2. Blood loss
3. Poor intestinal absorption
4. Increased requirement
219
Mx of IDA?
Oral ferrous sulfate, pts should continue taking iron for 3m after the iron deficiency has been corrected in order to replenish iron stores
220
2 sources of iron rich food?
1. Dark-green leafy vegetables
| 2. Meat
221
Most common cause of an isolated thrombocytopenia?
ITP
222
Blue line along gum margin?
Lead poisoning (20% pts)
223
Abdo pain and neurological signs ddx?
1. Lead poisoning
| 2. Acute intermittent porphyria
224
Mx of lead poisoning?
1. Dimercaptosuccinic acid (DMSA)
2. D-penicillamine
3. EDTA
4. Dimercaprol
225
Mx of combined Vit B12 and folate deficiency?
IM Vitamin B12 and start folic acid when Vitamin B12 levels are normal
226
Why do you manage B12 before folate?
1. To avoid precipitating SCDSC
| 2. B12 is needed as part of folate metabolism and by increasing folate, you will further deplete B12 levels
227
What is Gaisbock syndrome?
A symptom complex associated with polycythemia that cannot be attributed to a diagnosis of polycythemia rubra vera or to a secondary erythrocytosis that has occurred in response to hypoxemia.In order of decreasing importance, risk factors for the development of Gaisbock's syndrome include male sex, hypertension, smoking, diuretic therapy, obesity, and emotional or physical stress. The polycythemia has been attributed to decreases in plasma volume, and may be associated with increases in blood viscosity and peripheral vascular resistance and a propensity to develop arterial and/or venous thromboses
228
What is a primary cause of polycythaemia?
PRV
229
What are some secondary causes of polycythaemia?
1. COPD
2. Altitude
3. OSA
230
Most common leukaemia in adults in the western world?
B-CLL
231
Which has more marked lymphadenopathy, CLL or CML?
CLL
232
CML translocation?
t(9:22) --> Philadelphia chromosome
233
What happens if CML undergoes blast transformation?
Turns into AML (80%) or ALL (20%)
234
Management of CML?
1. 1st line = imatinib
2. Hydroxyurea
3. IFN-a
4. Allogeneic BM transplant
235
Imatinib MOA?
Tyrosine kinase inhibitor
236
Classification of primary immunodeficiencies?
1. Neutrophil disorders
2. B cell disorders
3. T cell disorders
4. Combined B and T cell disorders
237
Neutrophil disorder immunodeficiencies?
1. Chronic granulomatous disease (CGD)
2. Leukocyte adhesion deficiency (LAD)
3. Chediak-Hidashi syndrome
238
CGD defect?
Lack of NADPH oxidase
239
LAD defect?
Defect of LFA1 integrin (CD18) protein on neutrophils
240
B cell immunodeficiencies?
1. CVID
2. Bruton's X-linked agammaglobulinaemia
3. Selective IgA deficiency
241
Bruton's x-linked agammaglobulinaemia defect?
Bruton's tyrosine kinase (BTK) gene defect
242
Selective IgA defeciency?
Maturation defect in B cells
243
Most common primary Ab deficiency?
Selective IgA deficiency
244
T cell immunodeficiencies?
DiGeorge syndrome
245
DiGeorge syndrome defect?
22q11.2 deletion, failure to develop 3rd and 4th pharyngeal arches
246
Combined T and B cell immunodeficiencies?
1. SCID
2. Ataxia telangiectasia
3. Wiskott-Aldrich syndrome
4. Hyper IgM syndrome
247
SCID inheritance?
Most commonly X-linked recessive
248
SCID defect?
Most commonly IL-2R defect
249
Wiskott-Aldrich syndrome defect?
WASP gener mutation
250
Hyper IgM syndrome defect?
Mutation in CD40 gene
251
Negative Nitroblue-tetrazolium test?
CGD
252
Abnormal dihydrorhodamine test?
CGD
253
Partial albinism, peripheral neuropathy, and recurrent bacterial infections?
Chediak-Higashi syndrome
254
Chediak-Higashi syndrome defect?
Microtubule polymerisation defect
255
Delay in umbilical cord sloughing, absence of pus at sites of infection?
LAD
256
Low IgM, IgA and IgG?
CVID
257
B cell immunodeficiency associated with Coeliac disease?
Selective IgA deficiency
258
Ataxia telangiectasia defect?
DNA repair enzyme defect
259
Ataxia telangiectasia inheritance?
AR
260
Ataxia telangiectasia features?
1. Cerebellar ataxia
2. Telangiectasia
3. Recurrent chest infections
4. 10% chance of malignancy, lymphoma or leukaemia
261
Suspected DVT Ix?
Two-level DVT Wells Score
262
4 causes of severe thrombocytopenia?
1. ITP
2. DIC
3. TTP
4. Haem malignancy
263
Neutropenic sepsis defn?
Neutrophil count <0.5 x0^9 in a pt having anticancer tx and has one of the following:
1. Temp >38
2. Other s&s consistent with clinically significant sepsis
264
If it is anticipated that patients are likely to have a neutrophil count of < 0.5 * 10^9 as a consequence of their chemo treatment, what is the mx?
Offered a fluoroquinolone
265
Abx tx of ?neutropenic epsis?
Tazocin (piperacillin with tazobactam) immediately
266
Pt febrile and unwell after 48 hours of tx of neutropenic sepsis?
Meropenem +/- vancomycin
267
Pt not responding after 4-6 hrs of tx for neutropenic sepsis?
Ix for fungal infections e.g. HR-CT
268
Chronic H.pylori infection leads to what infection?
Gastric MALToma
269
Where does gastric lymphoma typically arise?
Antrum of the stomach
270
HTLV1 malignancy?
Adult T cell leukaemia/lymphoma
271
Malaria associated malignancy?
Burkitt's lymphoma
272
Beta thalassaemia chromosome?
11
273
Features of beta thalassaemia?
1. Presents in st year of life with failure to thrive and hepatosplenomegaly
2. Microcytic anaemia
3. HbA2 and HbF raied
4. HbA absent
274
Management of beta thalassaemia?
Lifelong blood transfusions (maintain Hb and simultaneously uppress enhanced erythropoiesis)
275
MOA of acute haemolytic transfusion rection?
RBC destruction by IgM type Abs
276
All stages of granulocyte maturation seen?
CML
277
When are irradiated blood products used?
If pt is depleted in T lymphocytes, to avoid GvHD caused by engraftment of viable donor T lympchytes
278
G6PDD inheritance?
X linked recessive
279
Pathophysiology of G6PDD?
Reduced G6PD --> reduced glutathione --> increased RBC susceptibility to oxidative stress
280
Bite cells?
G6PDD
281
Drugs that trigger haemolysis in G6PDD?
1. Antimalarials
2. Ciprofloxacin
3. Sulfonamides, sulfasalazine, sulfonylureas
282
Heinz bodies?
G6PDD
283
Pts >60 y/o w/ IDA Ix?
Colonoscopy
284
Philadelphia chromosome is seen in what other cancer in addition to CML?
ALL
285
t(15;17)?
1. Acute promyelocytic leukaemia (APML)
| 2. PML-RARA fusion gene
286
t(8;14)?
1. Burkitt's lymphoma
| 2. c-MYC translocation
287
t(11;14)?
1. Mantle cell lymphoma
| 2. Deregulation of Cyclin D1 (BCL-1) gene
288
t(14;18)?
1. Follicular lymphoma
| 2. Increased BCL2 transcription
289
Preferred NOAC for renal impairment and why?
Apixaban, due to minimal renal drug clearance
290
3 NOACs?
1. Dabigatran
2. Rivaroxaban
3. Apixaban
291
Dabigatran MOA?
Direct thrombin inhibitor
292
Rivaroxaban MOA?
Direct Xa inhibitor
293
Apixaban MOA?
Direct Xa inhibitor
294
Dabigatran excretion?
Majority renal
295
Rivaroxaban excretion?
Majority liver
296
Apixaban excretion?
Majority faecal
297
Indications of NOACs?
1. Prevention of VTE following hip/knee surgery
2. Treatment of DVT and PE
3. Prevention of stroke in non-valvular AF
298
What is a reversal agent for dabigatran?
Idarucizumab
299
Heparin reversal?
Protamine
300
Warfarin reversal?
Vitamin K and FFP
301
Microcytic anaemia causes?
TAILS
1. Thalassaemia
2. ACD
3. IDA
4. Lead poisoning
5. Sideroblastic anaemia
302
IV iron indication?
Pts with IDA prior to surgery where oral iron either cant be tolerated or the time interval is too short
303
Shortest time interval for oral iron to work?
2-4 weeks
304
What can be used to stimulated neutrophil growth in neutropenia secondary to chemo?
Filgrastim, a G-CSF
305
Causes of acquired haemophilia?
1. Elderly
2. Pregnancy
3. Malignancy
4. AI conditions
306
What may myelodysplasia progress to?
AML
307
What proportion of myelodysplasia becomes AML?
A third
308
What is myelodysplasia?
An acquired neoplastic disorder of haematopoietic stem cells
309
FFP universal donor?
AB RhD -ive
310
Why is AB RhD -ive the universal FFP donor?
Produce neither Anti-A or Anti-B and is therefore compatible with all ABO groups
311
MOA of heparin?
Activates antithrombin III
312
A common mode of presentation of SCA in late infancy?
Hand-foot syndrome
313
Microcytosis disproportionate to anaemia?
Beta thalassaemia
314
Most common cause of B12 deficiency?
Pernicious anaemia
315
Causes of B12 deficiency?
1. Pernicious anaemia
2. Post gastrectomy
3. Vegan/poor diet
4. Terminal ileum disease e.g. Crohns
316
Mx of B12 deficiency?
1mg IM hydroxycobalamin 3x week for 2 weeks, then once every 3m
317
Causes of thrombocytosis?
1. Reactive (acute phase reaction)
2. Malignancy
3. Essential
4. Hyposplenism
5. CML/PRV
318
What is essential thrombocytosis?
One of the myeloproliferative disorders which overlaps with CML, PRV and myelofibrosis
319
Mutation found in 50% pts with essential thrombocytosis?
JAK2
320
Burning sensation in the hands and JAK2 mutation?
Essential thrombocytosis
321
Mx of essential thrombocytosis?
1. Hydroxyurea (hydroxycarbamide)
2. IFNa
3. Low dose aspirin to reduce thrombotic risk
322
Haemolytic anaemia classification?
1. Congenital
| 2. Acquired
323
Congenital haemolytic anaemias?
1. Membrane = hereditary spherocytosis/elliptocytosis
2. Metabolism = G6PDD
3. Haemoglobinopathies = SCD, thalassaemia
324
Acquired haemolytic anaemia classification?
1. Immune
| 2. Non-immune
325
Immune acquired haemolytic anaemias?
1. Autoimmune = warm/cold
2. Alloimmune = transfusion reaction, HDN
3. Drugs = methyldopa, penicillin
326
Non-immune acquired haemolytic anaemias?
1. MAHA = TTP/HUS, DIC, malignancy, pre-eclampsia
2. Prosthetic cardiac valves
3. PNH
4. Infection = malaria
5. Drug = dapsone
327
What is SCA?
An AR condition that results from the synthesis of an abnormal Hb called HbS
328
What percentage of UK Afro-carribeans are carriers of HbS?
10%
329
Pathophysiology of SCA?
Polar aa glutamate is replaced by non-polar valine, decreasing water solubility of deoxy-Hb. In the deoxygenated state, the HbS molecules polymerise and cause RBCs to sickle
330
At what pO2 do HbAS pts sickle?
2.5-4 kPa
331
At what pO2 do HBSS pts sickle?
5-6 kPa
332
When should furosemide be given alongside red cell transfusions?
Between every other unit in pts with HF
333
How is tranexamic acid given in a major haemorrhage?
IV bolus followed by an infusion
334
Tranexamic acid MOA?
Antifibrinolytic
335
SCA Hb, MCV and reticulocytes?
Normocytic anaemia with raised reticulocyte count
336
Myeloma Ca, P and ALP?
High Ca, Normal P, Normal ALP
