Haem Qs Flashcards
1
Q
The most important cell in the initiation of normal heaemostasis
A
Endothelial cell
2
Q
A serine protease which assists in the break down of blood clots by binding to the clot and localising agents which break it down.
A
tPA
3
Q
A potent inhibitor of plasmin in the blood
A
a2 macroglobulin
4
Q
A single chain glycoprotein, synthesised by the liver and endothelium, which has strongly anticoagulant action and is important in the mode of action of heparin.
A
Antithrombin 3
5
Q
This product of the cyclic endoperoxides induces platelet aggregation
A
Thromboxane A2
6
Q
A 6 foot 7 inch rower presents to his GP complaining of easy skin bruising. On further examination he is found to have pectus excavatum, lax joints and a high-arched palate
A
Ehers-Danlos
7
Q
Abnormal pathway with prolonged APTT
A
Common or intrinsic. A deficiency of any factor except VII and XIII can raise the APTT
8
Q
Common pathway factors
A
1, 2 5 10
9
Q
Extrinsic pathway factors
A
7
10
Q
Intrinsic pathway factors
A
8, 9 11 12
11
Q
A 25 year old man presents to the Emergency Department a day after attending his dentist for a routine check-up. After treatment at the dentists the previous day, his gums had not stopped bleeding. On investigation, his APTT and bleeding time are prolonged but a normal PT
A
vWF deficiency
12
Q
A 16 year old girl presents to the Haematology Outpatients clinic describing a fluctuating history of easy bruising, epistaxis and menorrhagia. On investigation there is a thrombocytopaenia with increased megakaryocytes on BM examination
A
Autoimmune thrombocytopenic purpura
13
Q
Which protein, important in haemostasis, is vitamin K dependent but is not a serine protease?
A
Protein S
14
Q
Which option is required as a cofactor for protein C activity?
A
Protein S
15
Q
Which option synthesises tissue factor, vWF, prostacyclin, plasminogen activator, antithrombin III and thrombomodulin?
A
Vascular endothelium
16
Q
Which key clotting factor activates both factors V and VIII, and also activates protein C?
A
Thrombin
17
Q
A 37 year old mother of 4 children, presents to her GP because of recurrent nose bleeds and feeling tired all the time and heavy periods.
A
Osler-Weber Rendu (hereditary telangectasia). There is a structural abnormality of the blood vessels, resulting in telangiectases, which are thin walled so are likely to bleed. This leads to haemorrhage and anaemia. It is more common in females, and may not present until later in life. Epistaxis is the commonest presenting symptom. This patient is feeling tired, not just because of her 4 children, but because she also has iron deficiency anaemia.
18
Q
22 year old Saharawi refugee presents with anaemia, weight loss, loose stools and blood tests reveal an increased PT and slightly increased APTT, with normal thrombin time and platelet count.
A
Vitamin K deficiency. Prevalence of coeliac disease is highest in Saharawi refugees. This patient has coeliac disease, and as a result of malabsorption is losing weight and has loose stools (steatorrhoea), and vitamin K deficiency. The blood results related to vitamin K deficiency.
19
Q
5 year old boy has the following blood results: normal PT, increased APTT, normal platelet count, decreased VIII:C and decreased vWF.
A
vWF. The most common hereditary bleeding disorder, affect 1% of the population. vWF is a carrier protein for factor VIII and stabilises it. Mutation is in chromosome 12.
20
Q
A 56-year-old woman returns to the Vascular Clinic with recurrence of her L leg ulcer after the area has been knocked by a shopping trolley. On examination the ulcer is situated above the medial malleolus, its dimensions being 6cm x 5cm. The base is filled with yellowish slough and the surrounding area is erythematous, with prominent oedema.
A
Postphlebitic syndrome. Complication of DVT. Signs
Pain, heaviness, itching, swelling, varicose veins, ulcer, haemosiderin.
21
Q
A drug that is administered intravenously and has a rapid effect by potentiating the action of antithrombin. Action can be reversed quickly which is of relevance in myocardial infarction patients who may require early invasive treatment (ie PTCA).
A
Unfractionated heparin
22
Q
Potentiates antithrombin III. Usually given subcutaneously. Can cause osteoporosis and hyperkalaemia.
A
LMWH
23
Q
Used to monitor patients undergoing warfarin therapy.
A
PT
24
Q
Used to monitor patients undergoing unfractionated heparin therapy.
A
APTT
25
Reflects the amount and activity of fibrinogen.
Thrombin Time
26
Antiplatelet action. Licensed for secondary prophylaxis of stroke. More effective than aspirin alone. Cheap.
Dipyridamole modified release and aspirin
27
Antiplatelet action. Licensed for primary prevention of stroke in aspirin allergic patients, secondary prevention of stroke (but expensive) and in acute myocardial infarction in addition to aspirin.
Clopi
28
Dangerous antiplatelet combination with no added efficacy and increased GI bleed.
Aspirin and Clopi
29
10mg, 10mg, 5mg, measure on 4th day then every 2 days
5mg, 5mg, 5mg, 5mg, measure on 5th day, 8th day and then every 4 days
Which is current model of starting warfarin?
5mg, 5mg, 5mg, 5mg, measure on 5th day, 8th day and then every 4 days
30
In patients with metallic heart valves, this drug is the most effective anticoagulant
Warfarin
31
Side effects of this anticoagulant include cutaneous necrosis
Warfarin
32
The anticoagulant drug most likely to cause thrombocytopaenia with paradoxical thrombosis
Heparin
33
Indicated as thrombotic prophylaxis in DIC
LMWH
34
Contra-indicated if recent sore throat, if ever used before, or in the presence of proliferative retinopathy.
Streptokinase
35
A 65 year old patient presents with hepatosplenomegaly. He is mildly anaemic and thrombocytompenic. A blood monocyte count of 1.2 x 109/l is observed. Bone marrow aspirate reveals ring sideroblasts at 15% of total blasts. Auer rods are observed.
CML
36
An alcoholic presents to your clinic with anaemia. Sideroblasts are observed on morphological examination
Secondary Sideroblastic Anaemia
37
A 58 year old lady complains of lethargy and “easy bruising”. She presents with purpura. Her FBC reveals Hb 10.5g/dl; WBCs 2.3x109/l and platelets 8x109/l. Blood film reveals
Refractory Cytopaenia with Multilineage Dysplasia
38
A 78 year old male patient with recurring infections of the face and maxillary sinuses associated with neutropenia. His bloods are: Hb 9.8 g/dl; WBC 1.3x109/l; Neutrophils 0.3x109/l; platelets 38x109/l.The lab informs you that there are Blasts approximately compromise 17% of bone marrow aspirate.
Refractory Anaemia with excess of Blasts II
39
65y man, fever, SOB, lost 5kg weight in last few months/ Previously diagnosed wiht refractory anaemia with excess of blasts in transformation zone. . Blast cell count is approx 30% nucleated cells
AML
40
A 34 year old man with peripheral cytopenia suffers from bleeding gums. Peripheral blood shows 5% blast cells and bone marrow 42% blast cells.
AML
41
A 74 year old woman with high-normal platelet count. Bone marrow aspirate shows hyperplasia of hypolobulated micromegakaryocytes. Responds well to lenalidomide.
5q syndrom
42
A 20 year old man with hepatitis C complains of fatigue and breathlessness and bruises very easily.
Secondary aplastic anaemia
43
This patients blood film shows classic Pelger-Huet neutrophils and bone marrow blasts make up 15% of cells.
Refractory anaemia with an excess of blasts
44
In this case haemoglobin is normal but there is a reduction in platelets and neutrophils
Refractory cytopenia with multilineage dysplasia
45
A 64 year old man complains of headaches, fatigue and itchy skin, particularly evident after a hot bath. He has a long-standing history of alcohol abuse and drug history reveals that he taking thiazide diuretics. On examination, you note that he is thin with sunken eyes.
Pseudopolycythaemia
46
A 55 year old female has a past medical history of deep vein thrombosis. She also complains of easy bruising. Her platelet count is 770 x109/L, CRP is 4mg/L. You prescribe aspirin.
Essential thrombocythaemia. While there is an excess of platelets these platelets dont function properly so there is bleeding.
47
A 53 year old man goes to see his doctor about an embarrassing problem. It seems that his friends have nicknamed him ‘Rudolph’. You seem quite confused, but all becomes apparent when he points to his nose, which appears red. After further questioning, he describes a ‘burning’ sensation on his nose, hands and feet and visual disturbances. You send him for ‘blood tests’. What do you expect to be raised?
Haematocrit
48
A consultant grills you on a ward round: there is a patient with a WBC of 140 x109/L, Hb 12 g/dL, Platelet count 320 x109/L. She complains of tiredness, night sweats, fever and abdominal pain. Her spleen is markedly enlarged. Blood film shows blasts, neutrophils, basophils. How would you treat her?
Imatinib
49
You are asked to see a 76 year old man on the wards, who presented with fatigue, dyspnoea, bleeding gums and nightsweats. His abdomen is massively enlarged. You read his notes and find ‘bone marrow aspirate: ‘dry tap’. What would you expect to see on the blood film?
Tear drop poilikocytes
50
A 64-year old woman receiving long-term chemotherapy for lymphoma presents with worsening bone pain, recurrent fever and night sweats. Blood film shows blast cells with Auer rods
AML
51
A 61-year-old man with CLL presents with recurrent pneumonia and haemoptysis. On fibreoptic bronchoscopy, the patient is found to have an endobronchial mass. The biopsy shows anaplastic, large cell lymphoma.
Richter's syndrome. CLL develops into a rapid growing diffuse b cell lymphoma. 5-10% of CLL
52
A 5-year-old girl presents with failure to thrive, recurrent fever and bruising. Immunotyping reveals the presence of CD10.
ALL
53
A patient has acute lymphoblastic leukaemia. A bone marrow biopsy will show infiltration by which cells?
Blast cells
54
Patients with this inherited disorder have an increased risk of developing acute leukaemia.
Down's
55
The commonest adult leukaemia.
CLL
56
A 50yr old man presents to his GP complaining of weight loss, tiredness, easy bruising and a painful big toe. On examination his spleen is massively enlarged. Investigation shows a raised serum urate. The peripheral blood film is abnormal, showing proliferation of which type of cell?
Neutrophils. CML has a high turnover of urate producing cells.
57
A 65yr old lady is seen in the haematology clinic where she has been treated for 7 years with Imantinib for chronic myeloid leukaemia. Having been previously well, she is now complaining of shortness of breath and general weakness. Examination reveals splenomegally. Her peripheral blood film has changed from previous appointments and reflects the progression of her disease. Which type of cell is now proliferating?
Blast cells. this is the blast phase of CML
58
A 70yr old man complains of a year’s history of fatigue, weight loss and recurrent sinusitis. His white cell count is raised with a lymphocytosis of 283x109 /L. Blood film shows features of haemolysis and Coomb’s test is positive. Further investigation show the bone marrow, blood and lymph nodes are infiltrated with which cell population?
Clonal B lymphocytes. This is CLL which can be associated with AIHA and ITP. This is Evans syndrome.
59
A 10 year old boy presenting with tan-to-grey hyperpigmented patches on his upper trunk, neck and face, nail ridges and leukoplakia.
Dyskeratosis congentia.
Abnormal skin pigment, nail dystrophy and leukoplakia. has a varying phenotype. Disorder of telomeres. Results in progressive bone marrow failure.
M>F
60
An 83 year old woman presents to the AandE Departments with severe weakness and shortness of breath on minimal exertion. On examination, masses are felt in both left and right upper quadrants of the abdomen. Blood film show leukoerythroblastic cells and teardrop poikilocytes.
Myelofibrosis.
| Could be AML but usually have less splenomegaly.
61
A 35 year old women complains of abdominal pain. There is associated pancytopenia, and a Ham’s test is positive.
PNH. Can get abdo pain in PNH.
62
Complications of splenectomy
Increased susceptibility to infection with encapsulated organisms. As Pneumnococcus and meningococcus are oposined and then removed by the spleen. Will nee prophylactic Penicillinf for life. Will need vaccinations.
Splenectomy also causes and initial rise in platelet and potential thrombotic risk.
63
A 19 year old Jewish male presenting with multiple pathological fractures and hypersplenism.
Gaucher's disease (glucosylceramide lipidosis)
Lysosome storage disorder where sphingolipids accumulated in cells.
Signs:
heptosplenomegaly. Hypersplenism and pancytopenia.
Joint and bone pain, osteoporosis. Skin pigmentation.
64
A woman aged 69 with no past medical history except osteoarthritis suffered acute haemolysis after her right hip was replaced. There was no evidence of splenomegaly or lymphadenopathy and both warm and cold antibody tests were negative. When she had the left hip replaced 2 years later she again developed haemolysis soon after the anaesthetic. She was tested for IgG and IgE antibodies to the anaesthetic. Seven months later she had a revision arthroplasty and haemolysed again. Which condition might she have?
Cephalosporin-induced haemolytic anaemia
65
In which condition might the sucrose and Ham acid haemolysis tests be positive?
PNH
66
Mr RX came to Fulham from Thailand last year. He has inflammatory bowel disease and is taking dapsone for his dermatitis herpetiformis. Which of the above conditions would be likely and clinically relevant?
G6PD. Dapsone can cause drug induced HA especially in people with G6PD
67
A 29 year old man noticed he had yellow eyes and dark urine. He was always tired, and could not take part in sports as he easily became short of breath. There was no itching, fever or bleeding, and he was not taking any drugs. On examination he was anaemic, jaundiced, afebrile and had no palpable lymphadenopathy, hepatosplenomegaly or rash. His blood tests showed Hb 5.4g/Dl and raised WCC (40 x 109/L), bilirubin (47 µmol/l), aspartate transaminase (90iu/L) and lactate dehydrogenase (5721iu/l). The blood film showed polychromic nucleated red cells and spherocytes and the reticulocyte count was 9%. Direct Coombs' test revealed IgG and C3 on the red cell surfaces. The serum contained a warm non-specific autoantibody (i.e. it reacted with all the red cells in the test panel). Antinuclear antibodies and rheumatoid factor tests were negative and immunoglobulin levels were normal; there were no paraprotein bands in his serum. He failed to respond to high-dose corticosteroids and had a splenectomy three weeks later.
Primary AIHA
68
In which condition might a Donath-Landsteiner be positive?
Paroxysmal cold haemoglobinuria.
69
A 49 year old female complains of abdominal pain and vomiting a few minutes after the start of transfusion. O.E she is tachycardic and has facial flushing. Urinalysis reveals haemoglobinuria.
Immediate haemolytic transfusion reaction.
70
A 28 year old female presents with fever
Febrile non haemolytic transfusion reaction
71
44 year old male develops a pyrexia of >40 C and marked hypotension very shortly after receiving a blood transfusion. He has never had a transfusion before and there is no evidence of ABO blood group / rhesus incompatibility.
bacterial contamination
72
A 78 year old man is found collapsed in his bathroom at home. Tests reveal that he is anaemic and has raised urea and creatinine levels. Over the next 24 hours he receives 3 litres of fluid and a blood transfusion. He then becomes dyspnoeic. Examination reveals a raised JVP, basal crepitations and ankle oedema.
Fluid overload
73
An 18 year old African girl known to suffer from sickle cell anaemia develops a fever, sudden lethargy, pallor and arthralgia two months post-transfusion. Investigations reveal bone marrow red cell aplasia and a positive Paul-Bunnell test.
B19 parovirus infection
74
A 32 year old Irish man was noted to have a temperature of 36.9ºC and blood electrolyte results of: urea = 5.2 mmol/L, sodium = 144 mmol/L, potassium = 5.5 mmol/L, calcium = 2.5 mmol/L after receiving 10 units of blood over a 24 hour period.
Transfusion related fluid overload
75
A 55 year old British female developed an itchy, diffuse erythematous rash on both arms fifteen minutes after receiving a blood transfusion. This rash responded well to acrivastine.
Allergic reaction to transfusion
76
A 36 year old Italian gentleman presented with severe bruising across the dorsal aspect of his left arm one week post-transfusion. FBC results included: Hb = 15g/dL, platelets = 8x109/L and WBC = 5.5x109/L
Post-transfusion purpura
77
Patient develops increasing restlessness and chest pain during blood transfusion. Haemoglobinuria is also present.
ABO incompatible
78
Patient develops shortness of breath, dry cough and chills. Donors blood was found to contain anti-leucocyte antibodies with similar specificity to the patient’s white blood cell antigens.
baterial infection
79
Patient has a severe anaphylactic reaction soon after transfusion. Signs and symptoms include wheeze, raised pulse, low blood pressure and laryngeal oedema.
Anaphylaxsis due to IgA deficiency.
80
A 33 year old male was brought in from a serious RTA. He was given a donor transfusion on the ward. He later developed a fever and complained of suffereing from a 'dry cough'. The patient became increasingly breathless.
TRALI
81
A 33 year old patient has just given birth to her second child. The baby is jaundiced and placed under a UV lamp. What investigation would you perform to test for foetal haemoglobin-containing red cells?
Keihauer test. Test for fetal haemoglobin in maternal circulation.
82
A 25 year old man vomits and becomes restless on receiving a blood transfusion, he also complains of chest and abdominal pain.
Immediate haemolytic transfusion reaction.
83
Found in patients with Myeloma with low serum albumin and oedema
Amyloid
84
A reactive increase in number of plasma cells in the marrow and polyclonal increase in Igs.
Inflammation
85
Stimulated by IL-6 secreted by cells in the vicinity of the myeloma cells. Produces radiological changes resembling those of generalized osteoporosis.
Osteoclasts
86
Misfolded protein deposited in myeloma-associated amyloidosis
AL
87
Induction chemotherapy regimen with the best evidence pre autologous stem cell transplant in multiple myeloma
Lenalidomide and low dose dexamethasone
88
Factor(s) associated with a very poor prognosis in multiple myeloma
Anaemia and renal failure
89
Proteasome inhibitor active in myeloma
Bortezomib
90
Asymptomatic, serum monoclonal protein 25g/L, marrow plasma cells 5%, serum Ca 2.3 mmol/L, estimated GFR 100mL/min, Hb 12.5 g/dL, skeletal survey normal
mgus
91
Asymptomatic, serum monoclonal protein 30g/L, marrow plasma cells 10%, serum Ca 2.3 mmol/L, estimated GFR 100mL/min, Hb 12.5 g/dL, skeletal survey normal
Smoldering multiple myeloma
Serum moloclonal protein at least 30g/l or marrow plasma cells >10%
NO CRAB
92
Asymptomatic, serum monoclonal protein 10g/L, marrow plasma cells 15%, serum Ca 2.3 mmol/L, estimated GFR 30 mL/min, Hb 8 g/dL, lytic vertebral lesions
Multiple myeloma as has osetolytic lesions
93
Previously fit 35yo Afro Carribean female, sudden onset back pain, no history of trauma, 1 month history polyuria, urine specific gravity raised but dipstick negative for protein (and normal in all other respects), estimated GFR 70mL/min, vertebral fracture and bilateral femoral neck fractures on radiograph.
Myeloma. Light chains dont show up on urine dipstick but raise the specific gravity. This plus crab = myeloma
94
Which cytokine is an important growth factor in the development of myeloma?
IL-6
95
Elevated levels imply a poor prognosis in myeloma patients.
beta2 microglobulin
96
A 65 yr old man presents to his GP with general malaise, weight loss and visual disturbances that he likens to looking through a watery car windscreen. On examination he has peripheral lymph node enlargement. Protein electrophoresis shows an IgM paraprotein.
Waldenstrom's macroglobulinaemina
97
A 60 yr old woman presents to her GP with shortness of breath and ankle oedema. When examined the patient has bilateral weakness and paraesthesia of her hands. Investigations revealed a monoclonal proliferation of plasma cells and the presence of Bence Jones proteins.
Primary Amyloidosis.
Produce light chains (AL amyloid), these in urine are bence jones proteins. This deposit in the kidney causing oedema, SOB. AL in preipheral nervous system is causing paraesthesia.
98
A 52 yr old woman who has a 5 yr history of rheumatoid arthritis and has recently been found to have proteinuria. Further examination revealed hepatosplenomegaly. A rectal biopsy stained with Congo Red showed apple green birefringence in polarised light.
Secondary Amyloidosis.
| 2nd to chronic inflammatory disease. Is caused by AA amyloid.
99
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.
| Lymphocytes plus positive Tartrate resistant acid phosphotase.
100
Treatment for steroid resistant haemolytic anaemia
Splenectomy
101
CLL treatment
cyclophosphamide, fludarabine and rituximab
102
Tx for multiple myeloma (usually)
Lenalidomide (Revlimid) and low dose dexamethasone followed by autologous stem cell transplant (SCT)
103
Tx for smoldering myeloma and MGUS
Regular surveillance but no active treatment
104
DiGeorge children are ineligible for this operation.
Thymectomy
105
Used in solid organ transplantation, multiple sclerosis, NHL. Depletes lymphocytes. Side effects include ITP and Graves disease.
Campath (anti CD52, alemtuzumab)
106
Add-on anti-proteasome drug for myeloma
Bortezomib
107
Anti-proliferative used in polycythaemia rubra vera and essential thrombocythaemia
Hydroxyurea
108
Reduces platelet count in thrombocythaemia but causes palpitations, tachycardia, fluid retention
Anagrelide
109
Standard prophylaxis accompanying a once weekly myelosuppressant when used in rheumatoid arthritis
Folate
110
Rescue drug after high dose methotrexate is used for gestational trophoblastic disease or intrathecal ALL
Leucovorin (Folinic acid, Formyl tetrahydrofolate)
111
Used in solid organ transplantation, multiple sclerosis, NHL. Depletes B lymphocytes more than T lymphocytes.
Rituximab (anti CD20)
112
Myeloma grade
Variable B cell
113
Burkitts lymphoma grade
Aggressive B cell
114
ATLL grade
aggressive T cell
115
Grade of most easily curable B cell neoplasm
Aggressive B cell
| As chemo attacks rapidly dividing cells the more aggressive the more succeptible to chemo
116
Grade of follicular lymphoma
Indolent B cell
117
Stage 2A or 2B hodgkins are treated with
ABVD combination chemotherapy + radiotherapy if required
118
A subtype of Non-Hodgkin lymphoma associated with very aggressive disease.
Burkitts
119
A classical but rare constitutional symptom of Hodgkin lymphoma.
Pel-Ebstein Fever
120
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
121
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
122
A 51 year old man is having work up for palliative surgery due to gastric adenocarcinoma. He is found to be anaemic, with high a reticulocyte count and fragmented red blood cells on blood smear. What is this anaemia known as?
Microangiopathic haemolytic anaemia
123
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
124
Anaemia and neutropenia are more common in which autoimmune disease?
SLE
125
A peripheral blood smear of a thrombocytopenic patient raised Lactate dehydrogenase and unconjugated bilirubin shows fragmented Red blood cells
Microangiopathic haemolytic anaemia
126
A markedly raised haemoglobin in a patient with renal cell carcinoma.
Secondary true polycythaemia
127
Neutrophilia with visible toxic granulation and vacuoles on the blood film. The monocyte count is normal.
Acute fungal infection
128
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.
C1 inhibitor deficiency
129
A female infant presents to A&E with symptoms of hypoxia. It is noted that she has dactylitis.
Sickle cell disease
130
A 4 year old male presents to A&E with pallor, bruising and bone pain. O/E: hepatosplenomegaly is noted. Blood analysis reveals a reduced Hb and a raised WCC. Blast cells are noted on the blood film
Acute Lymphoblastic Leukaemia
131
A routine blood sample is taken from an asymptomatic neonate. Results reveal a raised Hb, a raised WCC and a raised MCV. Both Hb A and F are present.
Normal neonate
132
A 3 month old boy, of Greek origin is brought to AandE 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.
G6PD. In a haemolytic crisis there is a reticulocyte response with increased young red cells entering the blood. These cells have higher levels of G6PD which can elevate the measured level during a crisis. Need to measure G6PD after the crisis.
Reticulocytes give polychromic macrocytosis.
133
A 6 month old girl is brought to her GP by her Greek Cypriot parents. They complain that she looks small compared to their neighbour’s baby of the same age. They also think her face looks funny. On examination, you note pallor and jaundice, the baby’s skull appears bossed and there is maxillary prominence. There is also marked hepatosplenomegaly. Her blood film shows a microcytosis and haemoglobin analysis shows high levels of HbF and HbA2
Thal major (beta)
134
A young boy is referred to you because of prolonged bleeding following circumcision. You also note some bleeding of the gums. Coagulation tests reveal a normal PT but a raised APTT and an increased Bleeding Time. Analysis of clotting factors reveals a low Factor VIII.
vWF.
vWF = normal PT, raised APTT and raised bleeding time. With decreased F8 as vWF is a carrier for factor 8.
Haemophillia A = normal PT, raised APTT and NORMAL bleeding time, with low F8
Platelets levels can be normal in vWF (type 2) but they are dysfunctional.
135
A baby with Down’s syndrome is noted, on routine blood testing, to have large numbers of circulating megakaryocyte blast cells and nucleated red blood cells. A repeat blood film 2 months later is normal.
Transient Abnormal Myelopoiesis. Associated with Down's syndrome.
136
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
137
A 9 month old boy presents to AandE with excessive bleeding following circumcision. Laboratory investigations reveal a prolonged activated partial thromblastin time (APTT), normal prothrombin time (PT) and a factor VIII level which is 10% of normal. Bleeding time is normal. What is the likely diagnosis?
Haemophillia A
138
A peripheral blood film from an anaemic patient shows abnormally shaped, nucleated red blood cells and immature myeloid cells
Leukoerythroblastic anaemia
139
Imatinib treats?
CML
140
Chlorambucil treats?
CLL
141
Lenalidomide + low dose dexamethasone followed by autologous stem cell transplantation treatment?
Multiple myeloma
142
Treatment for High grade non-hodgkins lympphoma?
Rituximab +/- CHOP
143
ABVD 2-4 cycles + involved field (IF) radiotherapy
Hodgkins stage 1 or 2
144
Venesection + aspirin treatment?
Polycythamia RV
145
ABVD 6-8 cycles then PET to look for residual disease + IF radiotherapy. Treatment?
Hodgkins stage 3-4
146
(Hydroxyurea or Anagrelide) + aspirin. Treatement?
Essential Thrombocythamia
