Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

DEFNE Extra Buzzwords > MedGems Haem > Flashcards

MedGems Haem Flashcards

1
Q

A 21 year old woman with transfusion dependent beta thalassaemia normally has a transfusion every two weeks.

She has recently moved into your care and no previous blood results are available.

Pre-Transfusion bloods show a Hb of 76 (low) and a WCC of 23 (Normal Range: <11)

She is clinically well, apyrexial. She denies losing weight or night sweats. Her appetite is good. There are no clinical signs of infection.

What blood test should be ordered to further investigate the raised white cell count given her history?

A

Reticulocyte count

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

A 62 year old man develops palpitations while undergoing chemotherapy for acute myeloid leukaemia.

ECG shows p wave flattening, hyperacute T waves and a broadened QRS compared to an ECG 5 days prior.

What electrolyte abnormality is responsible for the ECG changes?

A

Hyperkalaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Scoring system in Tumour lysis syndrome

A

Cairo Bishop Scoring System

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What chromosomal translocation is associated with Mantle Cell Lymphoma?

A

t(11;14)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

“Significant infiltrates of homogenous lymphocytes. Macrophages are interspersed between sheets of lymphocytes and clearly contain apoptotic lymphocytes”

What lymphoma is associated with this histopathological description?

A

Burkitt’s lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is the inheritance pattern of hereditary elliptocytosis?

A

Autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is the most common cause of iron deficiency anaemia in the developing world?

A

Hookworm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What blood test is a measure of fibrin degredation products and may reflect activity of the body’s anticoagulant system?

A

D-Dimer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

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?

A

Porphyrias

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What blood film finding may be found in multiple myeloma and is reflective of a paraproteinaemia?

A

Rouleaux formation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the reversal agent for rivaroxaban?

A

Andexanet alfa

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the characteristic lymph node biopsy finding associated with Hodgkin lymphoma?

A

REed Sternberg cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is the inheritance pattern of hereditary spherocytosis?

A

Autosomal dominant

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What protein is classically mutated in hereditary spherocytosis?

A

Spectrin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the most common immunoglobulin produced by plasma cells in multiple myeloma?

A

IgG

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Staging system in Hodgkin’s lymphoma

A

Lugano staging classification

used to be Ann Arbor

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

A 34 year old woman suffered a major obstetric haemorrhage (1.5L blood loss) during delivery. She was transfused at the time and the bleeding was successfully controlled.

However, an hour later, she is now bleeding profusely and has lost a further 1L of blood. She has now developed a nosebleed and she is bleeding from her cannula insertion site.

A medical emergency (MET) call is put out along with activation of the major haemorrhage protocol.

30 minutes later, blood tests from the MET call showed the following clotting abnormalities:

Very high INR, very high APTT and a very low platelet count. Her D Dimer is very high.

What is the likely diagnosis?

A

DIC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

A 80 year old man is seen by their GP.

His wife is concerned that his gums have grown. He says when he brushes his teeth, they tend to bleed.

He has had weight loss (10% in the last 6 months) and now bruises easily.

A full blood count shows a low haemoglobin and low platelets. There is an elevated white cell count, with a large number of neutrophils.

A blood film shows the presence of myeloid blast cells.

What is the diagnosis?

A

AML

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

What inherited condition, after cystic fibrosis, is the most common cause of pancreatic exocrine dysfunction in children?

A

Shwachman Diamond syndrome

Shwachman-Diamond syndrome is an inherited, autosomal recessive condition characterised by neutropenia and other classical features.

Generally, there is pancreatic dysfunction - resulting in fatty stools and hepatic impairment.

They also have a very short stature, with most patients being less than the fifth percentile for height and a preponderance of skeletal issues.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What first line small molecule inhibitor is used in the treatment of chronic lymphocytic leukaemia?

A

Ibrutinib

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

A 28 year old woman is 39 weeks pregnant. Her labour is induced.

During the delivery she becomes unwell with rigors, sweating and vomiting.

Her observations are recorded as: pulse rate 147bpm, blood pressure 92/62mmHg.

Bloods reveal an INR of 2.4 (1), APPT 47 seconds (22-42), fibrinogen 0.8 (1.5-4.0g/L), D Dimer 3350 (<250)

What condition has been precipitated by her induction of labour?

A

DIC

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

What description is given to red blood cells which are typically polychromatic and stain heavily for the presence of RNA?

A

Reticulocyte

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

elongated, thin erythrocytes which is highly specific for iron deficiency anaemia

A

Pencil/cigar cell

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

red blood cells with a “bullseye” appearance of a dark central ring surrounded by pallor. Associated with liver disease, hyposplenism and iron deficiency

A

Target cells

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

spherical red blood cells due to membrane damage. Associated with hereditary spherocytosis or haemolytic anaemia

A

Spherocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

fragments of red blood cells due to damage. Associated with haemolytic anaemia

A

Schistocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

red blood cells stacked on top of each other due to inflammation

A

rouleaux

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

the classical finding associated with hyposplenism. Nuclear remnants visible on microscopy

A

Howell-Jolly bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

Denatured haemoglobin within RBCs - classical finding in G6PD

A

heinz bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What drug inhibits the fusion protein BCR-ABL and is used in the treatment of Chronic Myeloid Leukaemia?

A

Imatinib

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

What abnormality is commonly seen on a full blood count of patients with fanconi anaemia?

A

pancytopaenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Which haemoglobin chain is mutated in sickle cell disease?

A

Beta Globin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

What prothrombotic condition is associated with thrombocytopenia and immune complex formation?

A

Heparin induced thrombocytopaenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

A 78 year old woman is suspected to have immune thrombocytopenic purpura.

Her platelet count is 115 (NR >150). She reports no bruising, bleeding or rashes.

What is the most appropriate treatment?

A

No treatment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

What is the classical finding on a full blood count for a patient suspected of dyskeratosis congenita?

A

Pancytopaenia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

What is the most common primary bone marrow failure?
Fanconi anaemia
Diamond-Blackfan anaemia
Shwachman-Diamond syndrome
Dyskeratosis congenita

A

Fanconi Anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

Which primary bone marrow failure will have pancytopaenia?
Fanconi anaemia
Diamond-Blackfan anaemia
Shwachman-Diamond syndrome
Dyskeratosis congenita

A

Fanconi anaemia and Dyskeratosis congenita

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Which primary bone marrow failure will have neutropaenia only?
Fanconi anaemia
Diamond-Blackfan anaemia
Shwachman-Diamond syndrome
Dyskeratosis congenita

A

Shwachman-Diamond syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

Which primary bone marrow failure will have red cells only?
Fanconi anaemia
Diamond-Blackfan anaemia
Shwachman-Diamond syndrome
Dyskeratosis congenita

A

Diamond-Blackfan anaemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

Heparin induced thrombocytopenia is associated with the formation of antibodies directed against what autoantigen?

A

Platelet factor 4

41
Q

What is the most common reaction to transfusion of packed red blood cells?

A

Fever

42
Q

Other than an elevated lymphocyte count, his full blood count is normal.

Immunophenotyping of lymphocytes taken from that blood sample reveal the cells are positive for CD19 and CD5.

A

CLL

43
Q

A 70 year old man is seen in clinic. He describes a history of progressive enlargement of a “lump” in his groin.

USS reveals it to be a lymph node and a biopsy is taken.

What is the likely histopathological type of lymphoma that will be seen on biopsy?

A

Diffuse large B cell

44
Q

Lymphoma - Most common lymphoma in 15-40 year olds

A

Hodgkin’s

45
Q

Lymphoma - Most common lymphoma in >40 year olds

A

Diffuuse large B cell

46
Q

Lymphoma - Most common lymphoma in <15 year olds

A

Burkitt’s Lymphoma

47
Q

Hereditary spherocytosis test

A

Eosin-5-maleimide test

48
Q

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.

What investigation will confirm the diagnosis?

A

Hereditary spherocytosis

49
Q

What clotting factor has the shortest half life?

A

Factor VII

50
Q

A patient with metastatic ovarian cancer is investigated for unilateral lower limb swelling and is confirmed to have a DVT.

What is the most appropriate form of anticoagulation?

A

DOAC - rivaroxaban
if kidney failure -> warfarin

51
Q

A doctor suspects that their patient may be anaemic.

The doctor orders a panel of blood tests and a urine dip.

The urine dip was positive for blood.

A FBC reveals a normocytic anaemia.

The results of Iron Studies are below.

Serum iron Normal
Total Iron Binding Capacity (TIBC) Normal
C Reactive Protein 3 (NR 2-8)
Ferritin Normal
Bilirubin Elevated

A

Haemolytic anaemia

52
Q

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?

A

Calcium

53
Q

What protein is typically defective in hereditary elliptocytosis?

A

Spectrin

54
Q

A 14 year old boy is seen by a haematologist.

He has been referred due to concerns from his GP about a clotting disorder, as he has had recent unexplained petechiae, nosebleeds, tiredness and fatigue.

Bloods from the GP showed a pancytopenia.

The haematologist performs a full physical examination and notes the presence of longitudinal ridging in the nails and areas of whitish grey discolouration in the mouth. There are fine, end inspiratory crackles at both lung bases.

The haematologist suspects a primary bone marrow disorder.

What is the most likely diagnosis?

A

Dyskeratosis congenita

55
Q

What scoring system can be used to calculate the probability of developing heparin induced thrombocytopenia?

A

4T score

56
Q

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?

A

Smouldering multiple myeloma

57
Q

After myelodysplastic syndrome, which haematological malignancy does Fanconi anaemia carry an approximate 10% lifetime risk of developing?

A

AML

58
Q

A blood film is ordered and reveals the presence of immature red blood cells with inclusions of iron deposits in a ring formation.

A

Sideroblastic anaemia

59
Q

What is the most sensitive biomarker for iron deficiency anaemia?

A

Serum Ferritin

60
Q

What molecule binds free haemoglobin in the bloodstream and may be used in the investigation of haemolysis?

A

Haptoglobin

61
Q

What score is used to aid clinical decision making with respect to DVT risk?

A

Well’s score

62
Q

What medication can be given prophylactically to reduce the risk of tumour lysis syndrome in patients undergoing chemotherapy for haematological malignancies?

A

Rasburicase

63
Q

What is the reversal agent for dabigatran?

A

Idarucizumab

64
Q

The M3 subtype of Acute Myeloid Leukaemia can be treated effectively with which first line drug?

A

All TRans Retinoic Acid

65
Q

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?

A

Hodkin’s lymphoma

66
Q

Give an example of a chemotherapy drug which inhibits BCL2 and hence increases lymphocyte apoptosis in CLL.

A

Venetoclax

67
Q

Which cofactor is required for synthesis of the anticoagulant proteins C and S?

A

vitamin k

68
Q

She describes an odd looking cell that she hasn’t seen before in this patient. It has lots of dark blue speckles in the cytoplasm of the cell. It is much larger than the red blood cells.

A

Basophil

69
Q

What chromosomal translocation is associated with Follicular Lymphoma?

A

t(14;18)

70
Q

What drug should be given urgently to a patient on LMWH therapy who has significant, life threatening bleeding?

A

protamine sulfate

71
Q

Serum levels of which electrolyte drop precipitously in tumour lysis syndrome?

A

calcium

72
Q

A 1 month old child is investigated for the cause of a macrocytic anaemia, identified during routine workup for surgery for treatment of her cleft palate. She is noted to be quite underweight. Her brother has suffered from similar issues.

Vitamin B12 and folate levels were normal. Haemoglobin electrophoresis showed elevated fetal haemoglobin, but no other abnormalities. Her reticulocyte count was low.

What is the most likely underlying diagnosis?

A

Diamond-blackfan anaemia

73
Q

She describes a cell that is nearly completely dark purple, except for a thin rim of slightly blue stained cytoplasm. It’s roughly the same size as red blood cells.

A

lymphocyte

74
Q

A 49 year old man is reviewed by his GP after his blood tests suggested a new microcytic anaemia.

His ferritin and serum iron is low. His full blood count, CRP, renal function, LFTs, B12 and folate are otherwise normal.

A blood film reveals the presence of hypochromic erythrocytes with pencil cells.

What is the next most appropriate blood test to request, prior to an OGD and colonoscopy?

A

Anti-tissue transglutaminase antibodies

75
Q

What clotting disorder is associated with a reduced platelet count, elevated PT, elevated APTT and an elevated bleeding time?

A

Disseminated intravascular coagulation

76
Q

What chromosomal translocation is associated with a poor prognosis in Acute Lymphoblastic Leukaemia?

A

t(9;22)

77
Q

What chromosomal translocation is associated with Chronic Myeloid Leukaemia?

A

t(9;22)

78
Q

What molecule, secreted by the liver, inhibits iron absorption from the gut by inhibiting ferroportin?

A

Hepcidin

79
Q

What amino acid is glutamate substituted for in sickle cell disease?

A

Valine

80
Q

B symptoms

?

A

Drenching Night Sweats
Fever
Weight loss

81
Q

A histopathologist looks at a blood film taken from a patient. She notes the presence of smudge cells.

What condition is the patient likely to have?

A

CLL

82
Q

What is the most common cause of B12 deficiency in the UK?

A

Pernicious anaemia

83
Q

A recently immigrated 5 year old girl is seen in secondary care after concerns from her GP about an undiagnosed congenital condition.

She is short (below 5% percentile for age), has malformed fingers and flat, pigmented birthmarks all over her body.

Blood tests show a pancytopenia.

What is the likely diagnosis?

A

Fanconi anaemia

84
Q

A 60 year old woman is seen in the emergency department. She is known to have Immune thrombocytopenic purpura and a previous cholecystectomy.

A contrast enhanced CT Abdomen Pelvis shows the presence of a small amount of free fluid in the abdomen and fat stranding around the ileocecal area in the right iliac fossa.

The surgeons wish to take her to theatre, however, her platelets are 14 (NR >150).

What is an appropriate treatment to raise her platelet count quickly preoperatively?

A

IVIg

85
Q

She says she has seen a number of these cells in this sample, but that they are not too common. This cell is very large and has a bilobed nucleus. She says at least 4 red blood cells could fit inside this cell.

A

Monocyte

86
Q

What blood test should be monitored regularly on a patient treated with unfractionated heparin?

A

APTT

87
Q

A peripheral blood film from a patient with advanced myeloma shows a normocytic, normochromic anaemia.

What other blood film finding may be found in patients with advanced myeloma and reflects a high paraprotein concentration?

A

ROuleaux

88
Q

A 60 year old man is diagnosed with Immune thrombocytopenic purpura.

He reports nosebleeds quite frequently, usually every 2-3 days.

His platelet count is 34.

What is an appropriate treatment to improve his platelet count?

A

prednisolone

89
Q

What coagulation factor decreases after starting warfarin therapy and causes a initial prothrombotic state?

A

Protein C and Protein S

90
Q

Assuming no allergies, what antibiotic should be offered lifelong to patients with a known diagnosis of sickle cell disease to reduce their risk of infection by encapsulated organisms?

A

Pen V

91
Q

What specific type of alpha thalassaemia develops if a patient has three defective alpha globin genes?

A

Haemoglobin H disease

92
Q

What is the predominant form of haemoglobin that is expressed in adults?

A

Hb A1

93
Q

How many alpha globin genes does a normal healthy adult human express?

A

4

Alpha thalassaemia trait - one or two defective alpha globin genes - very little clinical significance. Mild microcytic anaemia.

Haemoglobin H disease - three defective alpha globin genes. Leads to chronic haemolytic anaemia - with associated splenomegaly.

Hydrops fetalis - fatal. Foetuses die in utero from heart failure and anaemia.

94
Q

A 34 year old woman from the South Mediterranean has a routine set of bloods taken at her local GP.

It shows that she has a haemoglobin of 111g/L (normal range: >115g/L). Her MCV is 61 (normal range: 80-100fL). The red cell distribution width is normal.

The GP finds her ferritin and iron levels to be normal. She feels well in herself with no recent infections.

What is the likely cause of her anaemia?

A

Thalassaemia

95
Q

What is the name of the condition that develops if a patient has negligible, but detectable, activity of two beta globin genes?

A

Beta thalassaemia intermedia

96
Q

Give an example of a treatment which increases fetal haemoglobin synthesis and hence can be used to reduce the severity of sickle cell disease or beta thalassaemia?

A

Hydroxycarbamide

97
Q

Rapid reversal of warfarin

A

Prothrombin complex concentrate

98
Q
A

DEFNE Extra Buzzwords (14 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions