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Haematology Flashcards

1
Q

What are the protein chains that make up HbA, HbA2 and HbF?

A

HbA: 2 alpha, 2 beta
HbA2: 2 alpha, 2 delta
HbF: 2 alpha, 2 gamma

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2
Q

What might you ask in the history of a patient presenting with anaemia?

A

Fatigue
SOB
Dizziness/syncope
Exercise tolerance
Chest pain
Any signs of bleeding: epistaxis, menorrhagia, haematuria, haempytysis, malaena
Red flags: weight loss, night sweats, lymphadenopathy, change in bowel habit
PMH: any other chronic diseases- eg. coeliac, IBD
Diet
Smoking/alcohol
Drug history

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3
Q

How can you classify anaemias and what are the causes of each?

A

Microcytic: MCV <80: Iron deficiency anaemia, anaemia of chronic disease, thalassaemia. sideroblastic anaemia.
Normocytic: MCV 80-100: haemolytic anaemias, anaemia of chronic disease, acute haemorrhage, bone marrow failure (aplastic anaemia), renal failure
Macrocytic: MCV 100: B12/folate deficiency, alcohol excess, hypothyroidism, liver disease, antifolaxe drugs

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4
Q

A 25 year old comes to GP practice as she says she is feeling tired all the time. She says she has started feeling more out of breath recently as well just when walking short amounts and has felt a bit dizzy occasionally. On examination she looks a bit pale and you notice her nails look flat, almost ‘spoon shaped’. You review for any blood loss and there is none acute but she says she gets very heavy periods every month. Given the likely diagnosis, what investigations are you going to do?

A

Iron deficiency anaemia secondary to menorrhagia
Ix:
Bloods: FBC and blood film: microcytic hypochromic anaemia, B12 and folate, iron studies- low Fe, low ferritin and raised TIBC, U&Es, LFTs, clotting profile
COELIAC SCREEN- anti-TTG and IgA ab

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5
Q

What are the causes of iron deficiency anaemia?

A

Blood loss- menorrhagia, IBD, gastric ulcer, varies
Malabsorption eg. coeliac, IBD
Poor dietary intake
Colon cancer in older men

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6
Q

What additional investigation would you do if the patient was over 60, male and had a history of weight loss and night sweats in a patient with iron deficiency anaemia?

A

Endoscopy and colonoscopy- check for colon cancer

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7
Q

How do you treat iron deficiency anaemia?

A

Iron replacement oral - sachets/tablets for 3m

If malabsorption/can’t take: IV

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8
Q

What side effects do you need to warn patients of when starting iron replacement?

A

Constipation, black stools, abdo pain, N&V

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9
Q

A 30 year old woman presents to clinic as she is feeling tired all the time. She says she has started feeling more out of breath recently as well just when walking short amounts and has felt a bit dizzy occasionally. On examination she looks a bit pale and you notice her tongue looks a bit swollen and red. She also says she has been struggling to swallow her food recently and feels like it gets stuck in her throat. What is the likely diagnosis and cause?

A

Plummer Vinson syndrome due to oesophageal webs: iron def anaemia + dysphagia + glossitis

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10
Q

How do you treat Plummer Vinson syndrome?

A

Iron supplementation to tx the iron def anaemia

Surgery to widen the oesophagus

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11
Q

A 7 month old boy is brought in by his dad because the child is failing to grow appropriately to his growth charts. He has a short stature given his age and looks a bit pale on examination. The dad says he’s noticed him more lethargic lately. On examination, you see a very prominent forehead and on palpation of the abdomen you can feel some splenomegaly. What is the diagnosis?

A

Beta thalassaemia

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12
Q

What is the pathophysiology behind beta thalassaemia?

A

Defect in beta globin gene causing no beta chains to be produced

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13
Q

What changes would you see to HbA, HbA2 and HbF in beta thalassaemia?

A

Absent beta chains will cause low HbA (2 alpha, 2 beta) but raised HbA2 (2 alpha, 2 gamma) and HbF (2 alpha, 2 delta)

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14
Q

Why do you get skull bossing and hepatosplenomegaly in beta thalassaemia?

A

Extramedullary haemotopoiesis- trying to produce more Hb so get production in liver and spleen
Bone marrow expands in skull to compensate and increase more Hb- skull bossing

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15
Q

How do you treat beta thalassaemia?

A

Lifelong blood transfusions + iron chelation to stop overload
May need splenectomy if massive splenomegaly

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16
Q

What changes would you see to HbA, HbA2 and HbF in alpha thalassaemia?

A

HbA= 2 alpha, 2 beta. Decreased
HbA2= 2 alpha, 2 gamma. Decreased
HbF= 2 alpha, 2 delta . Decreased
Tx with blood transfusions if severe

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17
Q

A 30 year old woman comes to GP as she is complaining of increasing fatigue all the time. She also gets intermittently out of breath more frequently and has had some headaches. On examination, she has a large red sore tongue and some ulcers at the corners of her mouth. She says in the last few days she’s noticed some numbness in her toes too. PMH: hypothyroidism and Addison’s disease. Given the likely diagnosis, what investigations will you do?

A

B12 deficiency caused by pernicious anaemia
Ix:
FBC and blood film: MCV >100 and megaloblasts, U&Es, LFTs, clotting
Parietal cell antibodies and intrinsic factor antibodies
B12 and folate measurements

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18
Q

What are some causes of B12 deficiency?

A

Poor diet- b12 is in meat, fish, diary
Malabsorption: Crohn’s, coeliac disease
Pernicious anaemia- autoimmune disease to parietal cells - decreased intrinsic factor production
Gastrectomy

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19
Q

A 30 year old woman comes to GP as she is complaining of increasing fatigue all the time. She also gets intermittently out of breath more frequently. On examination, she has a large red sore tongue and some ulcers at the corners of her mouth. She says in the last few days she’s noticed some numbness in her toes too. PMH: hypothyroidism and Addison’s disease. Given the likely diagnosis, what is the main worry in this patient i.e what complication might she have developed?

A

B12 deficiency caused by pernicious anaemia

Subacute degeneration of the spinal cord- paraesthesia, hyporeflexia , increased tone (mixed UMN and LMN signs)

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20
Q

How do you treat B12 deficiency?

A

B12 injections/oral: hydroxycobalamin

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21
Q

A 25 year old pregnant woman comes to GP as she is complaining of increasing fatigue all the time. She also gets intermittently out of breath more frequently and has had some headaches. On examination she has a sore red tongue. You do a FBC and a blood film which shows a MCV of 110 and blood film shows megaloblasts. What is the likely diagnosis and cause? What medication should she be on given this diagnosis and why?

A

Folate deficiency due to increased requirements of folate in pregnancy
Folic acid to prevent neural tube defects

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22
Q

What are the causes of folate deficiency?

A

Dietary: found in greens, nuts, fruit
increased requirements in pregnancy, malignancy, diabetes
Malabsorption : Coeliac, Crohn’s
Drugs; methotrexate, trimethoprim

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23
Q

A lady has been diagnosed with B12 and folate deficiency secondary to her Crohn’ disease. You decide to prescribe supplements. What will you be giving and which one first?

A

B12 - hydoxycobalamin before folate supplements- can worsen neurological symptoms otherwise.

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24
Q

A child presents to clinic with her mum who says child has been more fatigued and lethargic in the last few months. She looks pale on examination and has a bright erythematous rash on her cheeks. Her mum says she has had quite a few coughs and colds in the last few months as well. You take bloods and it shows a low Hb (MCV normal), low WCC and low platelets. What is the likely diagnosis?

A

Aplastic anaemia secondary to parvovirus b19 (slapped cheek)

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25
What are the causes of aplastic anaemia?
Bone marrow failure - Idiopathic - Congenital: Fanconi's - radial ray defect , Diamond-Blackfan anaemia - Secondary: parovirus B19 infection, radiation, drugs
26
What investigations will you do in someone with aplastic anaemia?
Bloods: FBC low Hb and MCV normocytic, low WCC and low platelets, LFTs Blood film Bone marrow biopsy
27
What signs might you see with a patient who has a haemolytic anaemia?
Jaundice Anaemia - pallor, Hepatosplenomegaly
28
What are some causes of haemolytic anaemias?
``` G6PD deficiency Sickle cell Hereditary spherocytosis Autoimmune - warm/cold, ABO incompatibility, Rhesus disease DIC TTP HUS Drugs- penicillin ```
29
What investigations would you do in a patient with a haemolytic anaemia?
Bloods: FBC- raised reticulocytes, MCV normocytic, U&E, LFTs, bilirubin- raised unconjugated Blood film Coombs/direct antiglobulin test- autoimmune causes
30
A 10 year old child presents with his mother. He has been feeling a lot more tired in the last few months than usual and has been getting more SOB. His mum says he has been a lot paler too and she thinks he looks a bit yellow now too. On examination, you can feel some splenomegaly as well. They moved here from Norway about 1 year ago. What is the likely diagnosis?
Hereditary spherocytosis: jaundice + pallor + splenomegaly
31
What is a common gastro condition associated with hereditary spherocytosis?
Gallstones
32
What investigations will you do in a child you suspect has hereditary spherocytosis?
Bloods: FBC- low Hb, MCV normal, raised reticulocytes , LFTs- normal Blood film: spherocytes Bilirubin: raised unconjugated
33
How do you treat hereditary spherocytosis?
If Hb <70/symptomatic anaemia: blood transfusion Splenectomy Folic acid supplementation- needed to synthesise RBCs
34
A 13 year old boy has come to A&E as he is complaining of very severe pain in his arms and hands. His fingers look very swollen on examination. He says he has had a cold recently and has been feeling unwell with that. What condition do you need to rule out when a child presents with swollen painful fingers?
Sickle cell anaemia! he is having a vast-occlusive crises causing dactylitis triggered by infection
35
What is the pathophysiology behind sickle cell anaemia?
Defect in the beta globin causing glutamine to change to valine and producing HbS.
36
What are the symptoms of sickle cell anaemia?
``` Anaemia- pallor, SOB, lethargy Jaundice Splenomegaly Stroke Priapism ```
37
What investigations are you going to do in a child with sickle cell anaemia?
Bloods: FBC- low Hb,MCV normal, raised reticulocytes, raised bilirubin, LFTs Blood film: sickle shaped RBCs Hb electrophoresis: HbA reduced, HbA2 normal, HbF raised, HbS raised.
38
A 13 year old boy has come to A&E as he is complaining of very severe pain in his arms and hands. His fingers look very swollen on examination. He says he has had a cold recently and has been feeling unwell with that. His observations show he has a low BP, and is cold. He has a past medical history of sickle cell disease. What is this diagnosis and how are you going to treat it?
``` Sickle cell vasocclusive crises - limbs Tx: ABCDE IV fluids IV Anaglesia- likely needs morphine IV Antibiotics - broad spectrum Oxygen if needed Blood/exchange transfusion ```
39
What are some triggers for vast-occlusive crises in sickle cell anaemia?
Cold Infection Dehydration Hypoxia
40
A 13 year old boy has come to A&E as he is complaining of severe chest pain and shortness of breath. On examination he is breathing very fast, and has a RR of 25. He has a bit of a fever and says he's been feeling a bit run down in the last few days. He has a past medical history of sickle cell disease. What is this diagnosis and how are you going to treat it?
``` Acute chest syndrome - sickle cell crises Tx: ABCDE Oxygen IV analgesia IV antibiotics borad spec Salbutamol if wheeze present ```
41
How do you treat sickle cell anaemia?
Immunisations Prophylactic antibiotics - penicillin Hydroxycarbamide- reduce crises
42
What inheritance pattern is sickle cell anaemia?
Autosomal recessive
43
You are bleeped to see a patient on the haematology ward who has acutely become jaundiced. He is also describing feeling quite fatigued and thought he looked quite pale earlier. He has a catheter present which is passing very dark urine and he looks yellow on examination. He has a PMH of rheumatoid arthritis and said today he was started on a new drug for it called hydroxychloroquine. What is the likely diagnosis and what you would see on blood film?
G6PD deficiency- crises triggered by anti-malarial drug eg. chloroquine, hydroxychloroquine. Blood film: Heinz bodies and bite cells.
44
What inheritance pattern is G6PD deficiency?
X linked recessive
45
What are triggers for crises in patients with G6PD deficiency?
``` Fava beans Anti-malarials e.g chloroquine, hydroxychloroquine. Ciprofloxacin Sulfonlyureas Infection ```
46
What investigations will you do in a patient with G6PD deficiency?
Bloods: FBC- low Hb, raised reticulocytes. Raised bilirubin. LFTs (if jaundiced)- will be normal. Blood film: Heinz bodies and bite cells
47
How do you treat G6PD deficiency?
Blood transfusion if needed Avoid triggers Splenectomy Folic acid
48
What are the two types of autoimmune haemolytic anaemia and what test can confirm the diagnosis?
Warm and cold | Coombs test or direct antiglobulin test (DAT)
49
What other investigations (than Coombs/DAT) will you do in autoimmune haemolytic anaemia?
FBC: low Hb, raised reticulocytes, LFTs, raised bilirubin, U&Es
50
What are some causes of warm autoimmune haemolytic anaemia?
Idiopathic | Secondary: CLL, other AI diseases- SLE, IBD, penicillin
51
What are some causes of cold autoimmune haemolytic anaemia?
Infection- mycoplasma, EBV | Primary
52
A child comes into A&E with his mother. She is incredibly worried as he has had an of bloody diarrhoea. You take routine blood tests and these show a low Hb , low platelets and a raised creatinine along with a raised urea. What is the likely diagnosis? What would you see on blood film?
Haemolytic uraemic syndrome | Shistocytes - fragmented RBCs from MAHA
53
What is the triad of features in Haemolytic uraemic syndrome ?
MAHA- haemolytic anaemia + thrombocytopenia + AKI
54
What is the most common cause of Haemolytic uraemic syndrome?
E.coli infection causing bloody diarrhoea
55
How do you treat Haemolytic uraemic syndrome ?
Fluids- maintain hydration. If can't drink: IV Blood transfusions if severe anaemia May need dialysis if AKI- refractory hyperkalaemia, fluid overload, signs of uraemia
56
A young woman presents to A&E with new onset confusion and has had a seizure this morning. Observations show her to be feverish and her blood tests show low platelets, low haemoglobin and very high creatinine and urea. What is the likely diagnosis?
TTP | Thrombotic thrombocytopenic purpura
57
What are the symptoms of TTP?
Low platelets + MAHA + AKI + neuro symptoms and fever
58
How do you treat TTP?
Acutely: plasma exchange and steroids | Longer term: aspirin to reduce platelet aggregation and clot formation
59
What are the common clinical features of leukaemia?
``` Anaemia - lethargy Neutropenia- increased infections Thrombocytopenia- bleeding, bruising/petechiae Weight loss and night sweats Bone pain Hepatosplenomegaly ```
60
What is the difference between acute and chronic leukaemia?
Acute: malignancy of the immature blasts eg. myeloblasts/lymphoblasts Chronic: malignancy of of mature cells eg. granulocytes or lymphocytes
61
A 6 year old boy with Down's syndrome presents with his mum to GP. His mum says he has been feeling more tired lately and she has noticed he has more bruises covering his body that have been appearing randomly lately. On examination you can feel some lymphadenopathy and hepatosplenomegaly. Given the likely diagnosis, what investigations do you want to do?
Acute lymphoblastic leukaemia Bloods: FBC- low Hb, low platelets, raised WCC, U&E, LFTs Blood film- immature blasts Bone marrow biopsy and aspirate: excess blasts >20%
62
A man comes in to the GP with a 6 month history of excessive tiredness. He has also noticed bruises appearing on his body and some weight loss. He says his gums have been bleeding every time he's brushing his teeth and they feel sore. What type of acute myeloid leukaemia is this?
M5- acute monocytic leukaemia = gum infiltration and hypertrophy
63
What are the two types of AML? What do they present with?
M3- acute promyeloytic leukaemia- DIC | M5- acute monocutic leukaemia- gum infiltration
64
What investigations would you do in a patient with AML?
Bloods- FBC- low Hb, low platelets, low neutrophils Blood film: Auer rods Bone marrow aspirate + biopsy= excess blasts
65
You have diagnosed your patient who has had tiredness, easy bruising and abdo pain with massive splenomegaly with chronic myeloid leukaemia. What finding will you see on genetic study, blood film and bone marrow aspirate and biopsy?
Genetic study: Philadelphia chromosome Blood film: mature granulocytes Bone marrow aspirate and biopsy: mature granulocytes
66
You have diagnosed your patient who has had tiredness, easy bruising and abdo pain with massive splenomegaly with chronic myeloid leukaemia. What treatment can you offer him?
Tyrosine kinase inhibitor- imatinib
67
A 75 year old man presents to GP fatigue, weight loss, increased bruising. On examination you feel hepatosplenomegaly. You request blood tests, the FBC shows raised WCC (lymphocytes) and the blood film shows smear cells. What is the likely diagnosis and prognosis?
CLL - good prognosis and relatively benign
68
What are two complications that can develop in a person with CLL?
Warm autoimmune haemolytic anaemia | Transformation into lymphoma
69
A 25 year old male patient presents to the general practitioner with a 4 week history of an enlarging left neck mass. He reports the mass is generally painless, but he notices it is sometimes painful after he drinks alcohol. He denies any other symptoms and has no past medical history of note. Physical examination reveals non-tender cervical lymphadenopathy. Excisional lymph node biopsy reveals giant multi-nucleated cells. What is the most likely diagnosis and what are the main risk factors?
Hodgkin's lymphoma | RF: EBV, family history, young adult of high SES
70
What implies a poor prognosis in lymphoma?
Presence of B symptoms - weight loss, night sweats, fatigue, fever
71
A 20-year-old male presents to his GP with persistent cervical lymphadenopathy and fever weight loss and night sweats. He is referred for a biopsy which show Reed-Sternberg cells. On further investigation it is noted that he has inguinal lymphadenopathy that also show Reed-Sternberg cells on biopsy. There is no evidence of liver, spleen or marrow involvement. Given his likely diagnosis what stage is his disease?
Stage 3: cervical lymphadenopathy + inguinal (2 or more places either side of diaphragm)
72
What is the staging system used in Hodgkin's lymphoma?
Ann Arbour Stage 1: one region of lymph nodes Stage 2: two or more lymph node regions one side of diaphragm Stage 3: Two or more lymph nodes above and below diaphragm (eg. cervical + inguinal) Stage 4: Widespread disease
73
What are the two types of Non-Hodgkin's lymphoma?
Low grade eg. follicular - slow to progress, asymptomatic, incurable High grade eg. diffuse B cell- fast progression, sx, treatable.
74
A 65-year-old male presents to his GP with ongoing lethargy. He reports that he has been “slower than usual mentally” and has not been feeling himself recently. He has a background of well-controlled hypertension on amlodipine, and he has a 20 pack-year smoking history. The GP is unsure of the diagnosis and conducts a routine set of blood tests: Hb 101 (low), raised creatinine and urea, raised calcium What is the likely diagnosis?
Multiple myeloma
75
What are the features of multiple myeloma?
``` CRAB Hypercalcaemia Renal failure Anaemia Bone pain ```
76
What is multiple myeloma?
Malignant proliferation of plasma cells
77
How would you investigate for multiple myeloma?
Bloods: FBC- anaemia? , U&Es- renal failure?, CRP, LFTs, Ca- raised? 1st line: serum and urine electrophoresis- serum: paraprotein bands, urine: Bence-Jones protein Blood film: Rouleaux formation Bone marrow biopsy: plasma cell inflation >10% Imaging: CT/MRI full body
78
You are reviewing a patient's blood results. His bloods have showed a pancytopenia, his blood film shows tear drop shaped cells and his bone marrow aspirate has been dry and difficult to drain. What is the likely diagnosis
Myelofibrosis Bloods: pancytopenia Blood film: tear drop cells 'poikilocytes' Bone marrow aspirate: dry- fibrosed bone marrow
79
What happens in myelofibrosis?
Hyperplasia of megokaryoctes (precursor to platelets) causing fibrosis of bone marrow
80
A 71-year old male presents to the emergency department with a transient loss of vision in his left eye for 10 minutes, which has since resolved. He mentions that he has recently noticed a strange burning sensation in his hands and distal arms, in addition to an itching sensation across his chest and arms after he takes a hot bath. He had a previous stroke three years ago, but is otherwise fit and well. What is the likely diagnosis?
Polycythemia rubra vera | Amaurosis fugax- hypervisocity
81
What happens in polycythemia rubra vera?
Malignant proliferation of RBCs--> high Hb and viscous blood
82
What are some symptoms and signs in polycythemia rubra vera?
``` May have strokes, DVT, PE (increased risk of thrombosis) Headache Blurred vision Palmar erythema Flushed Itching after bath- release of histamine ```
83
What will you see with Hb and EPO in polycythemia rubra vera?- primary v secondary
Primary: Hb will be raised, EPO will be low - negative feedback from Hb Secondary: Hb raised, EPO raised eg. in hypoxia
84
How do you treat polycythemia rubra vera?
Antiplatelets - 75mg aspirin for VTE prophylaxis Venessction if younger/mild Hydroxycarbamide if >60, prev VTE
85
What happens in essential thromboytosis?
Uncontrolled proliferation of megakaryocytes and so platelets
86
What is the main worry in essential thromboytosis?
Platelets are too high and dysfunctional- increased risk of thrombosis
87
How do you treat essential thromboytosis?
Antiplatlet- aspirin | Hydroxycarbamide if >60, prev VTE
88
What is primary haemostasis?
Adhesion of platelets to damaged vessel wall and formation of the platelet plug
89
What is secondary haemostasis?
Activation of the coagulation cascade leading to fibrin formation and reinforcing platelet plug
90
What are the three pathways in coagulation cascade and which clotting factors are involved? Which pathway does PT and APTT act?
Extrinsic - Tissue factor, factor 7 . PT Intrinsic - Factors 8, 9, 11, 12. APTT Common- Factors 2 (prothrombin), 5, 10. APTT + PT
91
How does warfarin affect coagulation cascade? i.e which factors are affected and is PT and APTT raised or decreased?
``` Warfarin inhibits factors 2, 7, 9, 10 2= common pathway - APTT +PT 7= extrinsic pathway- PT 9,10= intrinsic pathway- APTT PT and APTT prolonged (APTTT more) ```
92
An 8 year old girl has come to clinic with his mother. She describes these sudden new onset small dots that look like bruises on his skin that have just appeared in the last few days. He doesnt have any other medical conditions but was unwell with a viral chest bug 2 weeks ago that has settled now. On examination, you notice widespread petechiae and purpura. Observations are stable and he looks well on examination. What could be the diagnosis? What condition do you need to rule out?
Idiopathic thrombocytic purpura 2-3 weeks after viral infection - URTI/gastroenteritis CLL!
93
What investigations do you want to do in a patient with suspected ITP?
Bloods: FBC- low platelets, U&E, LFTs, CRP | Blood film and bone marrow biopsy- to rule out CLL and will also show megakarycytes on biopsy
94
What happens in ITP?
IgG antibodies attack and destroy platelets
95
How do you treat ITP?
``` 80% - self limiting 6-8w If doesnt/major bleeds: - Oral prednisolone -IvIg Platelet transfusions if acute ```
96
The 8 year old girl with diagnosed ITP went into remission with a course of oral prednisolone. However she has come back 8 months later for a check and her platelet count is still very very low. What has she got now and how can you treat?
Chronic ITP Lifestyle: avoid contact sports - risk of splenic rupture If prolonged bleeding, affecting ADLs: -Rituximab -Splenectomy + lifelong Abx prophylaxis - very last line
97
A 20 year old woman comes to clinic as she is reporting new onset bruising on her body. She says it started last week and she is noticing bruises appearing randomly on her body. She's also had about 5 nose bleeds in the lat week which she has never had before and has noticed her gums have been bleeding when brushing her teeth, again something that has never happened before. On questioning, her joints and muscles feel fine and not painful or swollen. What is the likely diagnosis? What is the inheritance pattern of this diagnosis?
Von Willebrand disease | Autosomal dominant
98
What is the difference in how Von Willebrand presents compared to haemophilia ?
VWd: Mucosal bleeding- gums, menorrhagia, epistaxis Haemophilia: intra-articular joint bleeds and muscle bleeds.
99
What is von willebrand factor?
Carrier for factor 8
100
How would you investigate for von-willebrand disease?
Plasma levels of von willebrand factor Prolonged (raised) APTT - factor 8 deficiency Platelet number will be normal
101
How do you treat Von Willebrand disease? Active bleeding, maintenance and symptomatic
if actively bleeding: von willebnrad factor concentrate Desmopressin - careful as can cause dilutional hyponatraemia Symptomatic: transexamic acid mouthwash, contraceptives for menorrhagia
102
What is the inheritance pattern of Haemophilia A and B?
X linked recessive
103
What factors are deficiency in Haemophilia A and B?
A: factor 8 B: factor 9
104
What are the symptoms/signs in Haemophilia A and B?
IA/joint bleeds, muscle bleeds- severe Prolonged bleeding after surgery -mild Prolonged bleeding after venepuncture - moderate May have joint deformity from repeated joint bleeds
105
What investigations will you do in haemophilia a and b?
Bloods: FBC- Hb only low if recent bleeding Factor 8 and 9 Clotting: platelet number normal, APTT prolonged (normalises on mixing study)
106
How can you treat Haemophilia A and B? active bleeding, maintenance, advice to give
Active bleeding: recombinant factor infusions Maintenance: mild HA: desmopressin. Prophylactic factor 8 and 9 infusions. Avoid IM injections, NSAIDs, aspirin
107
What are some early (<24h) complications that can occur with blood transfusions?
Anaphylaxis Fever- febrile transfusion reaction. Slow infusion and give paracetamol Acute haemolytic reaction: ABO incompatibility, rhesus Transfusion associated cardiac overload- cardiogenic oedema: SOB, oedema, wheeze, cough. Tx: diuretics TRALI: transfusion associated lung injury (ARDS- SOB, cough). Tx: oxygen
108
What are some delayed (>24h) complications that can occur with blood transfusions?
Infection Iron overload Post-transfusion purpura

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