Haem hard to recall Flashcards
(191 cards)
1
Q
What is the most important cell in the initiation of normal haemostasis?
A
Endothelial cell
2
Q
What is the main component involved in stabilising the primary haemostatic plug?
A
fibrin
3
Q
Which protease assists in the break down of blood clots by binding to the clot and localising agents which break it down?
A
Tissue plasminogen-activator (t-PA)
4
Q
Name a potent inhibitor of plasmin in the blood
A
a2 macroglobulin
5
Q
Which product of the cyclic endoperoxides induces platelet aggregation?
A
Thromboxane A2
6
Q
What does an increase in both APTT and PT suggest?
A
Vit K deficiency
7
Q
Which inherited disease has a raised APTT and bleeding time but a normal PT?
A
Von Willebrand deficiency
8
Q
What would you expect to find on blood film of ITP?
A
Megakaryocytes
9
Q
Which protein, important in haemostasis, is vitamin K dependent but is not a serine protease?
A
Protein S
10
Q
What synthesises tissue factor, vWF, prostacyclin, plasminogen activator, antithrombin III and thrombomodulin?
A
Vascular endothelium
11
Q
Which key clotting factor activates both factors V and VIII, and also activates protein C?
A
Thrombin
12
Q
Symptoms of osler-webu-rendu syndrome? AKA hereditary haemorrhagic telangiectasia
A
Recurrent nose bleeds
Anaemia symptoms
Menorrhagia
13
Q
What would a purple rash on bottom and lower legs be pathognomonic of?
What are some other symptoms associated with it
A
Henoch Schonlein Purpura
Abdo pain
Joint pain
14
Q
What drug potentiates antithrombin III. Usually given subcutaneously. Can cause osteoporosis and hyperkalaemia?
A
Dalteparin (LMWH)
15
Q
What type of heparin is reversible?
A
Unfractionated
16
Q
What is used to monitor patients undergoing warfarin therapy?
A
PT
17
Q
What is used to monitor patients undergoing unfractionated heparin therapy?
A
APTT
18
Q
What Reflects the amount and activity of fibrinogen?
A
Thrombin time
19
Q
In patients with cancer and acute venous thromboembolism, the most effective drug at reducing the risk of recurrent VTE is?
A
LMWH dalteparin
20
Q
Which anticoagulant drug most likely to cause thrombocytopaenia with paradoxical thrombosis?
A
Unfractionated heparin
21
Q
What drug is given as thrombotic prophylaxis in DIC?
A
LMWH dalteparin
22
Q
BM features of Chronic Myelomonocytic Anaemia?
A
Ring sideroblasts
High monocyte count
Auer rods
23
Q
What does Refractory Anaemia with excess Blasts in Transformation predispose you to?
A
AML
24
Q
Bone marrow findings of 5q syndrome?
A
Hyperplasia of hypolobulated micromegakaryocytes
25
What treatment does 5q syndrome respond well to?
Lenalidomide
26
Blood film features of Refractory Anaemia with excess Blasts?
Pelger-Huet neutrophils
| Blast cells
27
What would you expect to see on blood film of someone with myelofibrosis?
Tear drop poikilocytes
28
What is imatinib used to treat?
BCR-ABL positive CML
29
What do auer rods indicate?
AML
30
Describe Richter's transformation
CLL converts to diffuse large B cell lymphoma
31
What cancer is bcr-abl involved in?
CML
32
Patients with which inherited disorder have an increased risk of developing acute leukaemia?
Down's
33
What is the commonest adult leukaemia?
CLL
34
Why might CML present with a painful toe?
Increased mass of turning-over cells generates urate
35
Dykeratosis congenita triad?
Nail dystrophy
Leukoplakia
Cutaneous manifestations e.g. areas of hyperpigmentation
36
What test is used for paroxysmal nocturnal haemoglobinuria?
Ham's test (positive)
37
A 19 year old Jewish male presenting with multiple pathological fractures and hypersplenism. What condition do they have?
Gaucher's disease
38
In which condition might a Donath-Landsteiner be positive?
Paroxysmal cold haemoglobinuria
39
Dapsone is used to treat dermatitis herpetiformis. Dapsone is known to cause haemolysis in which patients?
G6PD deficient patients
40
What characterises delayed haemolytic transfusion reaction (DHTR)?
Manifests days to weeks after transfusion
Falling Hb
Jaundice
Haemoglobinuria
41
Infection with which virus may result in a false positive Paul Bunnell test?
parvovirus B19
42
What infection are patients with sickle cell disease at risk of causing aplastic crisis in?
Bone marrow findings?
parvovirus B19
| Red cell aplasia
43
What is a cause of anaphylaxis to blood transfusion?
IgA deficiency
44
What is Found in patients with Myeloma with low serum albumin and oedema?
Amyloid
45
Which monoclonal immunoglobulin appears as a dense narrow band (M band) on electrophoresis?
Paraprotein
46
What is stimulated by IL-6 secreted by cells in the vicinity of the myeloma cells. Produces radiological changes resembling those of generalized osteoporosis?
Osteoclasts
47
Misfolded protein deposited in myeloma-associated amyloidosis?
AL amyloid
48
A condition involving a low level of paraprotein in the blood in usually asymptomatic elderly patients?
MGUS
49
Induction chemotherapy regimen with the best evidence pre autologous stem cell transplant in multiple myeloma
Lenalidomide + low dose dexamethasone
50
Monoclonal immunoglobulin raised in macroglobinaemia?
IgM
51
Factor(s) associated with a very poor prognosis in multiple myeloma?
Anaemia & renal failure
52
Proteasome inhibitor drug which is active in myeloma?
Bortezomib
53
Tx regimen with the best evidence in multiple myeloma if not suitable for autologous SCT
Melphalan + Pred + Thalidomide = first line
54
3 diagnostic criteria for MM?
1. Presence of a serum or urine monoclonal protein. No minimum value.
2. Presence of clonal plasma cells in bone marrow >10%
3. Presence of end organ damage due to the plasma cell dyscrasia: CRAB
Note - second it's symptomatic it's myeloma
55
2 diagnostic criteria for smouldering myeloma?
1. Serum monoclonal protein >=30g/L and/or marrow plasma cells >=10%
2. AND No CRAB
56
3 diagnostic criteria for MGUS?
1. Serum monoclonal protein present but <30g/L
2. Marrow plasma cells <10%
3. AND No CRAB
57
Which paraprotein is most commonly raised in patients with multiple myeloma?
IgG
58
Which cytokine is an important growth factor in the development of myeloma?
IL-6
59
Elevated levels of what imply a poor prognosis in myeloma patients?
Beta2 microglobulin
60
What is Waldenstrom's macroglobulinaemia?
What do the cells secrete and what is their appearance?
Who is it seen in?
Median survival?
Low grade non-Hodgkin's lymphoma
Lymphoplasmacytoid in appearance and secrete IgM
Elderly men
3-5 years
61
A 43 year old man presents to his GP with concerns over a tender lump on the left side of his abdomen. Investigation showed a normal haemoglobin but a mildly raised white-cell count (13.2 x 109/l). On the blood film these cells were mainly small mononuclear cells resembling lymphocytes. These cells stained positively for tartrate-resistant acid phosphatase.
Hairy Cell Leukaemia
62
Best Tx for corticosteroid refractory haemolytic anaemia?
Splenectomy
63
Best Tx for CLL?
Cyclophosphamide, fludarabine and rituximab
64
Best Tx for CML?
Imatinib
65
What operation are DiGeorge children ineligible for?
Thymectomy
66
What drug is used in solid organ transplantation, multiple sclerosis, NHL. Depletes lymphocytes? Side effects include ITP and Graves disease.
Campath (anti CD52, alemtuzumab)
67
Anti-proliferative used in polycythaemia rubra vera and essential thrombocythaemia?
Hydroxyurea
68
What drug Reduces platelet count in thrombocythaemia but causes palpitations, tachycardia, fluid retention?
Anagrelide
69
Which Rescue drug after high dose methotrexate is used for gestational trophoblastic disease or intrathecal ALL?
Leucovorin (Folinic acid, Formyl tetrahydrofolate)
70
Advanced Hodgkins disease should be treated with?
Combination chemo
71
Pathological stage IA or IIA Hodgkins disease may be treated with?
ABVD combination chemotherapy + radiotherapy if required
72
A classical but rare constitutional symptom of Hodgkin lymphoma?
Pel-Ebstein fever
73
An 81 year old man with known prostatic carcinoma presents to his GP with severe bone pain. Blood tests reveal a mild anaemia and peripheral blood film shows nucleated red blood cells and immature myeloid cells. What are these haematological features collectively known as?
Leucoerythroblastic anaemia
74
A 21 year old student recently returning from India complains to his GP of cough, headache and diarrhoea. He is febrile and rose spots are present on his chest. Blood culture reveals salmonella typhi. Which immune cells out of the list are most likely to be raised?
Monocytes
75
Anaemia and neutropenia are more common in which of the above autoimmune disease?
SLE
76
What infection could cause a Monocytosis but with a normal neutrophil count?
Brucella infection
77
What could cause a neutrophilia with visible toxic granulation and vacuoles on the blood film? The monocyte count is normal.
Acute fungal infection
78
A 22 year old female who has recently undergone surgery presents with difficulties breathing and swelling of the face, hands and feet. She also complains of severe abdominal pains. She has experienced similar problems in the past. Diagnosis?
C1 inhibitor deficiency
79
What is C1 inhibitor deficiency associated with?
malignant B-cell lymphoma
80
Difference between VWD and Haemophilia in terms of APTT, PT and bleeding time?
Haemophilia: prolonged APTT, NORMAL PT and bleeding time
VWD: prolonged APTT and bleeding time, normal PT
81
A 3 month old boy, of Greek origin is brought to A&E by his mother straight from his christening. On examination, he is crying and appears jaundiced. Mild splenomegaly is felt in his abdomen. His mother says he has a fever and she notes that his urine was very dark when she changed his nappy. Investigation of his blood film reveals polychromatic macrocytes and irregularly shaped red blood cells. You also note his “special christening clothes” that his mother proudly tells you have been in the family for generations, they smell strongly of moth balls. Assay for G6PD reveals a normal level. Dx?
G6PD deficiency
82
A 6 year old boy from Greece presents to paediatric outpatients with severe anaemia. On examination he is anaemic and has enlarged maxilla and prominent frontal and parietal bones and hepatosplenomegaly. A skull X-ray reveals expansion of the medullary cavity giving rise to a ‘hair on end’ appearance. What is the likely diagnosis?
Beta thalassaemia
83
You are asked to see a neonate who while being treated for a urinary tract infection with Nitrofurantoin has developed jaundice. The blood film shows Heinz bodies, “bite cells” and polychromasia. What is the likely diagnosis?
G6PD deficiency
84
Who does nitrofurantoin Tx typically cause a jaundice in?
Patients with G6PD deficiency
85
What proteins are mutated in hereditary spherocytosis?
Spectrin
86
What blood test is used clinically to measure the activity of the extrinsic and common clotting pathways?
PT
87
What first line small molecule inhibitor is used in the treatment of chronic lymphocytic leukaemia?
Ibrutinib
88
An 18 year old presents to their GP with a neck lump.
It is approximately 3.5cm, mobile and untethered in the posterior triangle of the neck. On further questioning, he reports weight loss and drenching night sweats.
A blood film showed the presence of multinucleated and reactive lymphocytes.
What is the most likely diagnosis?
Hodgkin's lymphoma
89
What abnormality is commonly seen on a full blood count of patients with fanconi anaemia?
Pancytopenia
90
What blood test is a measure of fibrin degredation products and may reflect activity of the body's anticoagulant system?
D dimer
91
What are inclusions of clusters of nuclear DNA within erythrocytes commonly called when seen in a peripheral blood film?
Howell-Jolly bodies
92
What stereotypical description relating to erythrocytes can be seen on a blood film with a patient with myelofibrosis?
Dacrocyte (tear drop)
93
A bone marrow biopsy for a patient suspected of having multiple myeloma shows 15% clonal plasma cells in the bone marrow. She does not have any symptoms of end organ damage.
What is the diagnosis?
Smouldering myeloma
94
A GP suspects a clotting disorder and orders a panel of bloods, including inflammatory markers and a clotting screen. Given the most likely diagnosis, what blood test would you expect to be elevated?
APTT (most likely a haemophilia)
95
What description is given to red blood cells which are typically polychromatic and stain heavily for the presence of RNA?
Reticulocyte
96
What is the most common immunoglobulin produced by plasma cells in multiple myeloma?
IgG
97
What molecule binds free haemoglobin in the bloodstream and may be used in the investigation of haemolysis?
Haptoglobin
98
What is the M3 subtype of Acute Myeloid Leukaemia and what drug can it be treated effectively with?
Acute promyelocytic leukaemia
| All Trans Retinoic Acid
99
A patient has a normocytic anaemia, an elevated unconjugated bilirubin, elevated LDH and low haptoglobin. A peripheral blood film reveals spherocytes. Direct Coomb's test is negative. Dx and what investigation will confirm the diagnosis?
Hereditary spherocytosis
| Eosin-5-maleimide test
100
What is the classical finding on a full blood count for a patient suspected of dyskeratosis congenita?
Pancytopenia
101
Which chemical ion is required to convert Factor IX to Factor X in the intrinsic pathway of the clotting cascade and Factor VII to Factor X in the extrinsic pathway of the clotting disorder?
Calcium
102
A 63 year old man is admitted to hospital with severe pain in his right flank which comes and goes in waves. He cannot remain still while the pain is at its worst.
He has had similar episodes in the past which resolved without him seeing a doctor. He has no other past medical history of note.
Blood tests reveal an elevated calcium (3.1, NR 2.2-2.6); a normocytic anaemia (Hb 90, NR >120); and an elevated urea and creatinine.
After further questioning, he reports pain in his back and his legs which has progressively been worsening.
What is the likely underlying diagnosis?
Multiple myeloma
103
What is the inheritance pattern of hereditary spherocytosis?
Autosomal dominant
104
What is the inheritance pattern of hereditary elliptocytosis?
Autosomal dominant
105
Which haemoglobin chain is mutated in sickle cell disease?
Beta globin
106
Symptoms of Diamond-Blackfan anaemia?
Present in infancy
Bloods - Anaemia, low reticulocytes and elevated fetal haemoglobin
Bone marrow biopsy - decreased erythroid precursors
Craniofacial malformations e.g. cleft palate
Cardiac defects
107
First line Tx in polycythaemia?
Phlebotomy
108
What blood film finding may be found in multiple myeloma and is reflective of a paraproteinaemia? What else does it cause
Rouleaux
| Causes raised ESR
109
What are inclusions of denatured haemoglobin within erythrocytes commonly called when seen in a peripheral blood film?
Heinz body
110
What protein is typically defective in hereditary elliptocytosis?
Spectrin
111
The myeloproliferative syndromes, which include essential thrombocytosis, all carry a risk of transformation to what malignancy?
AML
112
Which clotting factor is prevented from being degraded through binding with Von Willebrand Factor in circulation?
Factor VIII
113
What is the most common cause of B12 deficiency in the UK?
Pernicious Anaemia
114
What is the term given to a group of genetic condition which affect the synthesis of the haem molecule, which is vital for the function of haemoglobin, peroxidase and P450 enzymes?
Porphyria
115
What enzyme is inhibited by warfarin and leads to a reduction in clotting factors II, VII, IX and X?
Vitamin K epoxide
116
Which cofactor is required for synthesis of the anticoagulant proteins C and S?
Vitamin K
117
What 2 disorders does fanconi anaemia increase your risk of?
AML
| Myelodysplastic syndrome
118
Signs in Fanconi anaemia?
Skeletal abnormalities (abnormal thumbs)
Short stature
Cafe au lait spots
Pancytopenia
119
What molecule, secreted by the liver, inhibits iron absorption from the gut by inhibiting transferrin?
Hepcidin
120
Symptoms of Shwachman Diamond syndrome?
Neutropenia
Pancreatic dysfunction
Short stature
121
Classic triad of symptoms in Dyskeratosis Congenita?
```
Nail dystrophy
Oral leukoplakia
Skin pigmentation
+ associated bone marrow failure
+ pulmonary fibrosis
```
122
What are hypersegmented neutrophils pathognomic of?
Megaloblastic anaemia
123
Primary causes of aplastic anaemia?
Fanconi anaemia
| Dyskeratosis congenita
124
What cytokine mediates anaemia of chronic disease and what is the mechanism?
IL6
| Induces hepcidin production - reduces iron levels
125
What is the serum iron, ferritin and TIBC in anaemic of chronic disease?
```
Iron = low
Ferritin = high
TIBC = low
```
126
Causes of basophilic stippling?
Lead poisoning
127
Which antibody mediates warm AIHA?
IgG
128
Which antibody mediates cold AIHA?
IgM
129
Causes of warm AIHA?
SLE
Penicillin
CLL
130
Causes of cold AIHA?
```
Mycoplasma
EBV
CLL
Lymphoma
Idiopathic
```
131
Blood film of G6PD?
Bite cells
| Heinz bodies
132
G6PD precipitants of oxidative crises?
```
Primaquine
Sulphonamides
Aspirin
Fava beans
Henna
```
133
Features of paroxysmal nocturnal haemoglobinuria? Remember PNH
pancytopenia with aplastic anaemiaintravascular
New thrombus - thrombophilia
haemolytic anaemia - haematuria (esp at night)
134
Causes of anisocytosis?
Iron deficiency anaemia (most common)
Thalassaemia
Megaloblastic anaemia
Sideroblastic anaemia
135
Causes of howell-jolly bodies?
Splenectomy
| Sickle cell disease
136
What are Cabot rings and what causes them?
Looped structures within RBCs
| Caused by megaloblastic anaemia (B12 deficiency, pernicious anaemia, lead poisoning)
137
What causes target cells?
Thalassaemia
Asplenia
Liver disease
138
What are pappenheimer bodies and what causes them?
Granules of iron within RBCs
Lead poisoning
Sideroblastic anaemia
Haaemolytic anaemia
139
Presenting features of TTP?
```
MAHA
Renal failure
Thrombocytopenia
Fever
Neurological signs - stroke/headache
```
140
A mutation in which gene allows formation of VWF multimers to cause platelet thrombi and organ damage in TTP?
ADAMST13
141
2 roles of vWF?
Platelet adhesion
| Factor 8 production
142
Which cofactors has low activity in vWD?
Low ristocetin cofactor activity
| Poor ristocetin aggregration
143
What mutation occurs in Glanzmann's thrombasthenia?
GlpIIb-IIIa
144
What mutation occurs in bernard-soulier syndrome
GlpIb
145
Which thrombophilia is caused by a substitution mutation?
prothrombin g20210a
146
Which thrombophilia is associated with impaired degradation of factor Va and VIIIa?
Protein S deficiency
147
Which factors does antithrombin inhibit?
Factor 10a
| Thrombin
148
Symptoms of GVHD following blood transfusion?
Diarrhoea
Maculopapular rash
Skin necrosis
149
What reaction can occur following a blood transfusion in someone with IgA deficiency?
Anaphylaxis
150
Which tumour cells express tartrate-resistant acid phosphatase?
Hairy cell leukaemia
151
What cell markers do hairy cell leukaemias express
CD25
| CD11c
152
Cell markers on AML immunophenotyping?
CD13
CD33
CD34
153
The nuclei of which cancer cells has a characteristic cloverleaf appearance?
Adult T cell leukaemia
154
Mutation in acute promyelocytic leukaemia?
PML-RARA fusion gene
155
Mutation in Burkitt's and what disease is associated?
EBV
| Translocation of c-myc on chromosome 8
156
What is Pel-Ebstein fever and what is it associated with?
Intermittent fever every 2 weeks
| Hodgkin's
157
MAntle cell leukaemia mutation and overproduction of which protein?
Chromosome 11 and 14 translocation
| Cyclin D1
158
Follicular lymphoma mutation and overproduction of which protein?
Chromosome 14 and 18 translocation
| BCL-2
159
Blood findings in sarcoidosis?
Monocytosis
160
Main feature of TRALI?
Non-cardiogenic pulmonary oedema (no fluid overload)
161
Histology of trali?
massive pulmonary oedema
| granulocyte aggregation within pulmonary microvasculature
162
Most severe ABO incompatibility?
A and O
163
Features of febrile haemolytic transfusion reactions?
| Mx?
Less than 24 hours after transfusion
Fever
Slow transfusion +
164
What condition are pigment gallstones associated with?
hereditary spherocytosis
| any haemolytic anaemia tbh
165
What conditions do you see pencil cells in?
Iron deficiency anaemia
Thalassaemia
Pyruvate kinase
166
What conditions do you see target cells in? 3Hs
Hepatic failure
Hyposplenism
Haemoglobinopathies
167
What conditions do you see acanthocytes in (spiky RBCs)
Hyposplenism
alphabetalipoproteinaemia
Chronic liver disease
alpha-thal trait
168
Conditions where splenectomy may be beneficial? The PIIES
```
Thalassaemia
Pyruvate Kinase deficiency
Immune haemolytic anaemia
ITP
Elliptocytosis
Spherocytosis
```
169
Prophylaxis post splenectomy?
Penicillin V
Pneumococcal vaccine
Meningococcal vaccine
Hib vaccine
170
3Cs of aplastic anaemia?
Cytotoxics
Carbamazepine
Chloramphenicol
171
Cause of chondrocalcinosis?
Haemochromatosis
172
What conditions can cause an ESR >100?
Myeloma
Leukaemia
Aortic aneurysm
Malignant prostate cancer
173
What is combined polycythaemia?
Increased RBC mass
| Decreased plasma volume
174
What happens to red cell mass when someone is dehydrated?
It stays the same
175
Another name for non-classical Hodgkin's lymphoma?
| What is its unique feature?
Nodular lymphocytic HL
| Popcorn cells - weird Reed Sternberg cells - lymphocytic and histiocytic cell
176
Clinical features of G6PD?
Jaundice
Tiredness
Haematuria
177
CML translocation important to remember?
t(9,22)
178
How can mild vWD be treated?
Desmopressin
179
What drugs precipitates renal failure in MM?
NSAIDs
180
How to differentiate between TACO and TRALI?
TRALI is differentiated from TACO by the presence of hypotension in TRALI vs hypertension in TACO
181
What are pappenheimer bodies associated with?
Hyposplenism
182
Gold standard test for paroxysmal nocturnal haemoglobinuria?
Flow cytometry for CD59 and CD55
183
What are Aschoff bodies?
granulomatous nodules found in rheumatic heart fever
184
Venetoclax is a BCL2 inhibitor used for B-cell CLL. Which cellular process does this act on?
Apoptosis
185
```
A 55 year old man attends his GP with severe fatigue and back pain. Investigations:
Creatinine 635 (high)
IgG 3.5 (low)
IgM 0.3 (low)
Serum protein electrophoresis – no paraprotein
Kappa light chains 0.9 (low)
Lambda light chain 1970 (very high)
K/L ratio 0.0005 (low)
What is the cause of the renal failure?
```
B cell leukaemia
186
CAR-T cells against CD19 treat haematological malignancy of what cell type?
B cell
187
Cause of left shift and toxic granulation on blood film?
reactive neutrophilia
188
A bleeding patient has a prolonged aPTT and PT, but normal platelets and fibrinogen. Which blood component should they be given?
FFP
189
A 75 y/o woman develops neutropenic sepsis secondary to myelodysplasia. Her FBC is significantly worse than 6 weeks prior, with marked pancytopenia. Blood film shows numerous large cells of primitive appearance. What is the most likely diagnosis?
Progression to acute myeloid leukaemia
190
```
55 y/o male smoker on furosemide has the following test results:
Hb 178 (high)
Hct 51% (high
Red cell mass 25 (low end of normal)
Plasma volume 26 (low)
What is the most likely diagnosis?
```
Relative polycythaemia
191
A 61 year old lady complains to her GP of morning stiffness and aching in her hips. She also suffers from anorexia, fatigue and occasional night sweats. She is found to have a raised ESR. Of note she has had giant cell arterititis in the past. What is the most likely diagnosis from the list above
Polymyalgia rheumatica
