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2a - SBCS > Haematology > Flashcards

Haematology Flashcards

1
Q

What is anaemia

A

Defined as low level of Hb in the blood

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2
Q

State the 3 main categories of anaemia and give their MCV

A

MCV= Mean cell/corpuscular volume (femtolitres)
* Microcytic - MCV<80 = small RBCs
* Normocytic - MCV 80-100 = normal RBCs
* Macrocytic - MCV>100 = large RBCs

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3
Q

What Hb levels indicates anaemia

A

Women: <120 g/L
Men: <130 g/L

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4
Q

Give 4 generic symptoms of anaemia

A
  • tiredness
  • shortness of breath
  • headaches
  • palpitations
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5
Q

Give 3 generic signs of anaemia

A
  • Tachycardia
  • Conjunctival pallor
  • Pale skin
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6
Q

5 causes of microcyctic anaemia

A

TAILS:
* Thalassaemia
* Anaemia of Chronic Disease
* Iron Deficiency
* Lead Poisoning
* Sideroblastic Anaemia

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7
Q

What is iron deficiency anaemia

A

Non inherited Fe deficiency that impairs Hb synthesis

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8
Q

4 causes of Fe deficiency anaemia

A
  • Hookworm - GI blood loss
  • Malnutrition
  • Malabsorption conditions (coeliac)
  • Increased requirements during pregnancy
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9
Q

Signs specific to Fe anaemia

A
  • Koilonychia - spoon shaped dent in nails
  • Angular cheilitis - red sores around mouth
  • Brittle hair and nails
  • Atrophic glossitis - smooth and thin tongue
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10
Q

Describe the investigation of Fe deficiency anaemia

A
  • Full blood count (FBC) = microcytic
  • Fe studies = low FE, Low ferritin, low transferrin saturation
  • Blood film - hypochromic RBC
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11
Q

What is the main drug used to treat Fe deficiency anaemia
* Inc SE & alt

A
  • Ferrous sulphate
  • SE: diarrhoea/constipation, black stool
  • If poorly tolerated consider ferrous gluconate
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12
Q

What is total iron binding capacity

A

Describes the affinity for Fe to bind to protein

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13
Q

Function of transferrin and ferritin

A

Ferritin - Fe storage
Transferrin - protein that facilitates Fe absorption

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14
Q

What is thalassaemia

A
  • Inherited genetic defect in the protein chains that make up Hb
  • autosomal recessive
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15
Q

Explain the pathophysiology of thalassaemia

A

Normal Hb consists of 2 alpha and 2 beta globin chains
* Defects in alpha-globin chain = alpha thalassaemia
* Defects in beta-globin chains = beta thalassaemia (mc)

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16
Q

Signs and symptoms of thalassaemia (4)

A
  • hepatosplenomegaly
  • Pronounced forehead and cheekbones due to extra RBC production
  • Failure to thrive
  • pallor and lethargy
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17
Q

What chromosome is the gene that codes for alpha-globin found on

A

chromosome 16

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18
Q

What chromosome is the gene that codes for beta-globin found on

A

Chromosome 11

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19
Q

Describe the 2 different gene defects in beta-thalassaemia

A
  • Defective copies (Df) that retain some function
  • Deletion (Dl) genes where there’s no function in beta-globin
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20
Q

Briefly describe the 3 types of beta-thalassaemia

A
  • Minor (carrier) - one abnormal and one normal beta-globin gene = mild microcytic anaemia
  • Intermedia - 2 abnormal copies: 2 df/ 1df + 1 dl = marked anaemia
  • Major - homozygous deletion genes = severe anaemia
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21
Q

Diagnosis of thalassaemia

A
  • FBC + blood film = microcytic and hypochromic RBCs, target cells, Heinz bodies (alpha)
  • Hb electrophoresis - diagnose globin abnormalities
  • DNA testing
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22
Q

Treatment for thalassaemia

A
  • Blood transfusion
  • Splenectomy reduce transfusion comp
  • Fe chelation (deferasirox) - prevent Fe overload
  • Bone marrow transplant
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23
Q

Complication of thalassaemia

A

Organ failure

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24
Q

What is sideroblastic anaemia

A

Disorder where there the body has iron available but can’t incorporate it into Hb
* Often inherited and linked to the mitochondria

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25
Q

Identifying features of sideroblastic anaemia (investigation results)

A
  • Hypochromic microcytic anaemia
  • High ferritin iron and transferrin saturation
  • Basophilic stripling of RBCs
  • Iron overload = iron deposition in BM = increased staining with prussian blue
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26
Q

Give 4 causes of normocytic anaemia

A
  • Haemolysis
  • Chronic disease
  • Aplastic anaemia
  • Acute bleeding
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27
Q

How are haemolytic and non-haemolytic normocytic anaemias distinguished from each other

A
  • Low reticulocytes = Non haemolytic
  • High reticulocytes = Haemolytic
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28
Q

Name 4 haemolytic anaemias

A
  • Sickle cell
  • G6PDH deficiency
  • Autoimmune haemolytic
  • Hereditary spherocytosis
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29
Q

Give 2 causes of non haemolytic anaemia

A
  • Aplastic
  • CKD
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30
Q

What is aplastic anaemia

A

Condition that occurs when your bone marrow can’t make enough new RBCs for normal body function

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31
Q

What is sickle cell anaemia

A

Autosomal recessive condition that causes crescent shaped RBCs

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32
Q

Explain the pathophysiology of sickle cell anaemia

A
  • Abnormal gene for beta-globin that produces the abnormal variant HbS
  • This makes RBCs more fragile and more easily destroyed
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33
Q

Signs and symptoms of sickle cell anaemia

A
  • general anaemia Sx - pallor, lethargy, tachycardia
  • Pre-hepatic jaundice
  • Failure to thrive
  • Persistent pain in skeleton/chest/abdo
  • Dactylitis - swollen dorsa of hands and feet
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34
Q

Diagnosis of sickle cell anaemia

A
  • New born screening - heel prick test
  • DNA based assays
  • Hb electrophoresis - GS
  • FBC + blood film - normocytic + normochromic with high reticulocytes and sickled RBCs
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35
Q

Treatment of sickle cell anaemia

A
  • Acute attacks: IV fluid, paracetamol + Ab if infection
  • Hydroxycarbamide (hydroxyurea) - stimulate production of HbF
  • Blood transfusion
  • Bone marrow transplant
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36
Q

What is sickle cell crisis
Give examples

A

Spectrum of acute crisis relating to condition
* Vaso-occlusive crisis - sickled cells clog BVs
* Splenic sequestration - RBCs blood flow within spleen
* Acute chest crisis - pul vessel vaso-occlusion = resp distress

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37
Q

Give 3 factors that can trigger sickle cell crisis

A
  • Infection
  • Cold
  • Hypoxia
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38
Q

How can complications of sickle cell disease be prevented

A
  • keeping warm
  • up to date with vaccines
  • regular blood transfusion
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39
Q

What is glucose-6-phosphate dehydrogenase deficiency

A
  • X linked recessive enzyme deficiency causing decreased lifespan of RBC
  • intravascular haemolysis
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40
Q

Epidemiology of G6PDH deficiency

A
  • MC in Africa/Middle Eastern
  • affects more males as X linked
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41
Q

Signs and symptoms of G6PDH deficiency

A
  • Mostly asymptomatic
  • neonatal Jaundice
  • Exacerbated by eating broad beans
  • Pallor
  • Nausea
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42
Q

Diagnosis of G6PDH deficiency

A
  • Heinz bodies on blood film
  • Bite cells (membrane indentation)
  • FBC - normocytic
  • Raised reticulocytes
  • Raised unconjugated bilirubin and lactate dehydrogenase
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43
Q

Tx for G6PDH deficiency

A

Blood transfusions
Avoid triggers - oxidative drugs, broad beans, infection
Folic acid
Splenectomy

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44
Q

What is autoimmune haemolytic anaemia (AIHA)

A

Ab are created against the patient’s RBCs
* warm and cold types (warm mc)

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45
Q

Investigation for AIHA

A
  • +ve Direct coombs test - agglutination of RBCs with coombs agent
  • Blood film - spherocytes and high reticulocytes
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46
Q

What is hereditary spherocytosis

A
  • Auto dom condition that causes sphere shaped RBC that are fragile and easily break down
  • mc inherited haemolytic anaemia in N. europe
  • Extravascular haemolysis
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47
Q

Presentation of hereditary spherocytosis (3)

A
  • Jaundice
  • Gallstones
  • Splenomegaly
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48
Q

How is hereditary spherocytosis treated

A
  • Splenectomy
  • transfusion if needed
  • Folate supplements
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49
Q

Describe the 2 categories of macrocytic anaemia

A
  • Megaloblastic - anaemia is the result of impaired DNA synthesis preventing the cell from dividing normally
  • Non-megaloblastic
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50
Q

Causes of megaloblastic anaemia

A

B12 deficiency
Folate deficiency

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51
Q

Give 4 causes of non-megaloblastic anaemia

A
  • Alcohol
  • Liver disease
  • Hypothyroidism
  • pregnancy
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52
Q

What is pernicious anaemia

A

Autoimmune destruction of parietal cells leading to vitamin B12 deficiency

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53
Q

Causes of B12 deficiency

A
  • Pernicious anaemia
  • malnutrition
  • Gastric/ intestinal surgery
  • Malabsorption disorders - Crohn’s, coeliac disease
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54
Q

Explain the pathophysiology of B12 deficiency

A
  • B12 must bind to intrinsic factor to be absorbed into the terminal ileum
  • IF is produced by gastric parietal cells
  • Ab against IF = decreased absorption of B12
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55
Q

Neurological symptoms of B12 deficiency (3)

A
  • loss of vibration sense and proprioception
  • mood/cognitive changes
  • Pins and needles
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56
Q

Sx of B12 deficiency ( exc neuro)

A
  • Jaundice (late sign)
  • Glossitis
  • Angular cheilitis
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57
Q

4 foods that Vit B12 are found in

A

Exclusively animal-derived products
* meat
* Salmon
* eggs
* Milk

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58
Q

Investigation of B12 deficiency

A
  • FBC - macrocytic anaemia
  • Peripheral blood smear - megaolcytes
  • Low serum B12
  • Anti-IF Ab - specific to pernicious anaemia
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59
Q

Treatment for B12 deficiency

A
  • Dietary advise - multivitamin supplements
  • pernicious anaemia - IM hydroxycobalamin
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60
Q

If a patient has a combination and B12 and folate deficiency which is treated first and why

A

B12 deficiency treated first as treating with folic acid can deplete B12 more and mask symptoms of B12 deficiency

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61
Q

Function of folate

A

Folate is absorbed in the proximal jejunum and is needed for DNA replication

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62
Q

Causes of folate deficiency

A
  • Poor nutrition - found in broccoli, legumes (chickpeas) and fruits
  • Malabsorption - coeliac and tropical sprue
  • Increased demand in pregnancy
  • Drugs
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63
Q

Give 3 drugs that can cause folate deficiency

A
  • Sulfasalazine
  • Trimethoprim
  • methotrexate
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64
Q

Presentation of folate deficiency

A
  • Severe: glossitis and angular cheilitis
  • General anaemia Sx - SOB, fatigue, pallor, tachycardia
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65
Q

Investigation of folate deficiency

A
  • FBC and blood smear - macrocytic anaemia and megaloblasts
  • Low serum folate
66
Q

Treatment of folate deficiency

A
  • Oral folic acid
  • Treat underlying disorder
  • Multivitamin supplements
67
Q

What is leukaemia

A

Broad name for cancer of the blood cells
Type of leukaemia depends on type of blood cell (myeloid/lymphoid) and how rapidly they progress

68
Q

What are the 4 main types of leukaemia

A
  • Acute myeloid leukaemia (AML)
  • Acute lymphoblastic leukaemia (ALL)
  • Chronic myeloid leukaemia (CML)
  • Chronic lymphocytic leukaemia (CLL)
69
Q

What is acute myeloid leukaemia

A

Myeloblast (immature WBCs) proliferation

70
Q

Give 3 RFs of AML

A
  • Down’s syndrome
  • Radiation
  • Age >65
71
Q

Signs and symptoms of AML

A
  • infections
  • Pallor
  • Petechiae - small non-blanching spots due to thrombocytopenia
  • Swollen gums
  • Lymphadenopathy
  • Splenomegaly
72
Q

Investigations of AML

A

FBC + blood film:
* Pancytopenia
* Auer rods - rods in cytoplasm of blast cells
BM biopsy: >20% myeloblasts
acute promyelocytic leukaemia t(15,17)

73
Q

Treatment for AML

A
  • Anaemia - red cell transfusion
  • Neutropenia - Ab
  • Stem cell transplant
  • Thrombocytopenia - platelet transfusion, tranexamic acid
  • Chemo: cytarabine+ ananthracycline (e.g. daunorubicin)
  • All-trans retinoic acid (ATRA/tretinoin) used in aggressive subtype = acute promyelocytic leukaemia (APML)
74
Q

What is chronic myeloid leukaemia

A
  • Overproduction of myeloid progenitor
  • Has 3 typical phases: chronic, accelerated and blast (worst)
75
Q

Describe the genetic change that is characteristic of chronic myeloid leukaemia

A

Philadelphia chromosome:
*translocation of genes between chromosome 9 and 22 = t(9:22)
* BCR-ABL gene formed

76
Q

How does chronic myeloid leukaemia present(4)

A
  • splenomegaly
  • Left upper quadrant pain or fullness
  • Excessive sweating
  • Weight loss
77
Q

Investigations of CML

A
  • Cytogenetic studies - Philadelphia chromosome t(9,22)
  • BM biopsy - granulocytic hyperplasia
  • FBC - leukocytosis, anaemia, thrombocytosis (chronic) or thrombocytopenia (blast crisis)
  • decreased leukocyte alkaline phosphatase
78
Q

Treatment of chronic myeloid leukaemia

A
  • Tyrosine kinase inhibitor (imatinib)
  • Induction chemo + allogeneic stem cell transplant
79
Q

What is acute lymphoblastic leukaemia (ALL)

A

Malignant lymphoblast proliferation

80
Q

Risk factors of ALL

A

*MC childhood (<6) malignancy
* Downs syndrome
* Radiation exposure

81
Q

Presentation of acute lymphoblastic leukaemia

A

*Fever, weight loss, fatigue
* hepatosplenomegaly
* Bruising, nose bleeds
* Bone pain

82
Q

Diagnosis of A.L.L

A
  • BM aspiration and trephine biopsy: >20% lymphoblasts = diagnostic
  • FBC - leukocytosis, thrombocytopenia and normocytic anaemia with low reticulocytes
  • Blood film - leukemic lymphoblasts (small, coarse chromatin, clumped together)
  • Cytogenetic and molecular studies - detect abnormalities e.g t(12;21) or t(9;22)
83
Q

Treatment of A.L.L

A
  • Induction therapy: prednisolone + Cyclophosphamide + Vincristine + doxorubicin
  • +ve Ph chromosome - TKI (imatinib)
  • Consolidation: cyclophosphamide + crisantaspase
  • Stem cell transplant
  • CNS prophylaxis - methotrexate
84
Q

What is chronic lymphocytic leukaemia

A
  • Chronic proliferation of a single type of well differentiated lymphocyte (usually B)
  • mc leukaemia overall
85
Q

Who does CLL usually affect

A

Males, over age of 55

86
Q

How does CLL present

A
  • Often asymptomatic but can present with infections, anaemia, bleeding and weight loss
  • lymphadenopathy (non-tender swollen lymph nodes)
87
Q

Chromosomal changes seen in chronic lymphocytic leukaemia

A
  • Deletion of long arm of chrom 13 - mc, good prognosis
  • Deletion of part of short arm of chrom 17 - poor prognosis
88
Q

Diagnosis of CLL

A
  • Blood film: smudge/ smear cells - aged/fragile WBCs rupture and leave a smudge on film
  • FBC - lymphocytosis, low platelets, anaemia
89
Q

Treatment of CLL

A
  • Early stage, Asymptomatic - observation
  • FCR - fludarabine, cyclophosphamide, rituximab
  • del (17p) mutation: targeted therapies - acalabrutinib
  • Allogeneic stem cell transplant
90
Q

Complications of CLL

A
  • Richter transformation - CLL transforms into high-grade NH lymphoma
  • warm autoimmune haemolytic anaemia
  • hypogammaglobulinaemia
91
Q

What are lymphomas

A
  • Group of cancers that affect lymphocytes in the lymphatic system
  • Cancerous cells proliferate within the lymph nodes causing lymphadenopathy
92
Q

What are the 2 main categories of lymphoma

A
  • Hodgkin’s (HL) - specific disease
  • Non-hodgkin’s (NHL) - all other lymphomas
93
Q

Signs and symptoms of lymphoma

A
  • Lymphadenopathy - usually painless and non-tender but will experience pain after drinking alcohol in Hodgkin’s, mc in neck
  • B symptoms - fever (>38 ), weight loss (>10% in 6m), night sweats
  • Pruritus
94
Q

Causes and RFs of HL

A
  • caused by proliferation of lymphocytes
    RF:
  • Age - bimodal age distribution with peak around age 20 and 75
  • HIV
  • Epstein-barr virus
95
Q

Describe the investigation of HL

A
  • Lymph node biopsy (diagnostic) : +ve Reed-Sternberg
  • Raised lactate dehydrogenase
  • CT/MRI for staging
  • Eosinophilia
  • normocytic anaemia
96
Q

Describe reed Sternberg cells and which lymphoma has them

A

abnormally large B cells that are multinucleated or have a bilobed nucleus with prominent eosinophilia inclusion-like nuclei (owl eye)
Hodgkin’s lymphoma

97
Q

What is Ann Arbor staging

A
  • System used for both hodgkins and non-hodgkins lymphoma
  • puts importance on whether affected nodes are above or below diaphragm
  • 4 stages
  • A/B = without/with symptoms
98
Q

Treatment for HL

A

ABVD chemotherapy:
- Doxorubicin (Adriamycin)
- Bleomycin
- Vinblastine
- Dacarbazine

99
Q

3 side effects of chemotherapy

A
  • Alopecia
  • Nausea and vomiting
  • bone marrow failure
100
Q

Give 2 types of NHL

A
  • Burkitt lymphoma associated with EBV, malaria and HIV
  • Diffuse large B cell lymphoma (mc)
101
Q

Investigations of NHL

A
  • Lymph node biopsy - no reed sternberg cells, confirm type of NHL
    • Starry sky appearance on biopsy = burkitts
  • CT/MRI chest, abdo, pelvis for staging
102
Q

Treatment for NHL

A
  • R-CHOP-21 Chemo:
  • Rituximab (mAb)
  • Cyclophosphamide
  • doxorubicin
  • vincristine (oncovin)
  • prednisolone
103
Q

Describe tumour lysis syndrome

A
  • Complication of chemo
  • when a large number of cancer cells die within a short period, releasing their contents (uric acid) in to the blood
  • This accumulates in the kidneys and can cause AKI
104
Q

What can be given prior to chemo to avoid tumour lysis syndrome occurring

A

Rasburicase
* a recombinant version of urate oxidase, an enzyme which catalyses the conversion of uric acid to allantoin
* Allantoin is more soluble than uric acid = more effective renal excretion

105
Q

What is multiple myeloma

A
  • Neoplastic proliferation of a plasma cell
  • Results in large quantities of a single Ab; typically IgG (55%) or IgA (20%)
106
Q

Who is most affected by MM (2)

A
  • Over 70
  • Afro-Caribbean
107
Q

Signs and symptoms of MM

A

CRAB:
* Ca elevated
* Renal failure
* Anaemia
* Bone lesion/pain

108
Q

Describe the investigation and diagnosis of MM (5)

A
  • Urine dipstick: Bence Jones proteins (free lights chains in urine)
  • Skeletal survey - osteopenia, osteolytic lesions
  • FBC + blood film: normocytic normochromic anaemia, raised ESR and rouleaux formation (RBC aggregation)
  • Serum electrophoresis - high levels of Ig (separate proteins based on weight
  • XRAY - raindrop skull
  • BM biopsy (diagnostic) - >10% plasma cells
109
Q

Define ESR and what would a raised ESR signify

A
  • Erythrocyte sedimentation rate
  • Measures how quickly RBC settle at bottom of a test tube
  • High ESR = high levels of inflammation
110
Q

Treatment for MM (5)

A
  • Chemotherapy - dexamethasone AND thalidomide
  • stem cell transplant
  • bone disease - Bisphosphonates (zoledronic acid)
  • Analgesia - avoid NSAIDs
  • DVT prophylaxis - aspirin
111
Q

What is PT/INR

A
  • Prothrombin time test (11-13.5s)/ international normalized ratio
  • Measures time taken for blood to clot via the extrinsic pathway sample
  • Norm INR = 0.8-1.1; Warfarin INR = 2-3
112
Q

Give 4 things that can cause a higher INR value

A
  • Anticoagulants
  • Liver disease
  • Vit K deficiency
  • Disseminated intravascular coagulopathy
113
Q

Describe the activated partial thromboplastin time (aPTT)

A
  • Measures time taken for blood to clot via intrinsic pathway
  • Norm = around 35-45s
114
Q

What conditions affect APTT

A
  • Haemophilia A
  • Haemophilia B
  • Von Willebrand’s disease
115
Q

What is thrombin time

A
  • Tests how fast fibrinogen is converted to fibrin (clot if formed) by thrombin
  • 12-15s
116
Q

What is polycythaemia

A

Erythrocytosis (high [RBC])

117
Q

What are the causes of polycythaemia

A

*Absolute - mutation (JAK2) and polycythaemia vera (cancer)
* Secondary - Altitude, dehydration, COPD (hypoxia)

118
Q

Symptoms of polycythaemia vera (5)

A
  • Itchy after a bath
  • Burning sensation in fingers and toes (erythromelalgia)
  • splenomegaly
  • Headaches
  • ruddy cyanosis
119
Q

Causes of polycythaemia vera

A

Janus kinase 2 (JAK2) mutation causing BM cells to produce too many RBCs

120
Q

Investigations for polycythaemia vera

A
  • FBC: raised WBC & platelets
  • Raised Hb
  • Genetic test: JAK2
121
Q

Tx for polycythaemia vera

A
  • No cure, aim is to maintain normal blood count
  • Phlebotomy
  • hydroxycarbamide - reduce platelet count
  • low dose aspirin
122
Q

What is immune thrombocytopenia purpura (ITP)

A
  • Autoimmune destruction of platelets
  • Primary - mc in children aged 2-6
  • Secondary- mc in adult women >65
123
Q

Causes of primary and secondary ITP

A
  • 1 - post viral infection
  • 2 - malignancy, HIV
124
Q

Signs and symptoms of ITP

A
  • Purpuric rash - purple/red rash
  • Heavy periods
  • Can be asymptomatic
  • bruising
  • no systemic symptoms
125
Q

Investigation and diagnosis of ITP

A
  • FBC - thrombocytopenia
  • Platelet autoantibodies
  • BM biopsy - increased megakaryocytes
126
Q

Tx for ITP

A
  • Prednisolone (reduce inflammation)
  • IV IgG
  • Splenectomy
  • no treatment required for children with no significant bleeding
127
Q

What is thrombotic thrombocytopenic purpura (TTP)

A

Platelet deficiency caused by a deficiency in vWF cleaving protein

128
Q

RFs of TTP

A
  • Adult females
  • Associated with: HIV and cancer
129
Q

Signs and symptoms of TTP

A
  • Purpuric rash
  • Menorrhagia (heavy periods)
  • Fever
  • Abdominal pain
  • may have neuro changes
130
Q

Investigation and diagnosis of TTP

A
  • Thrombocytopenia
  • Blood smear - fragmented RBC
  • Deficiency in ADAMTS13 protein
131
Q

Treatment for TTP

A
  • Prednisolone (steroid)
  • rituximab (monoclonal Ab)
  • IV plasma exchange
132
Q

What is myelofibrosis

A
  • Where proliferation of the cell line leads to fibrosis of the bone marrow
  • can be caused by myeloproliferative disorders
133
Q

Name 3 common myeloproliferative disorders

A
  • Primary myelofibrosis
  • Polycythaemia Vera
  • Essential thrombocythemia
134
Q

What is the proliferating cell line in essential thrombocythemia

A

megakaryocyte

135
Q

What is the proliferating cell line in polycythaemia Vera

A

Erythroid cells

136
Q

What is the proliferating cell line in primary myelofibrosis

A

haematopoietic stem cell

137
Q

What is haemophilia

A
  • X-linked recessive bleeding disorder
  • Haemophilia A is caused by factor VIII deficiency
  • B is caused by factor IX deficiency
138
Q

What is the most common type of haemophilia

A

A

139
Q

Signs and symptoms of haemophilia

A
  • excessive bruising/ haematomas
  • fatigue
  • Menorrhagia
  • Spontaneous bleeding into muscles and joints (haemoathrosis)
140
Q

Investigations and diagnosis of haemophilia

A
  • Normal prothrombin time (extrinsic pathway)
  • prolonged activated partial thromboplastin time (intrinsic pathway )
  • Coagulation assay - reduced/absent factor 8/9
141
Q

Treatment for haemophilia

A
  • IV F8/9
  • Desmopressin - release stored f8
    • Antifibrinolytic to inhibit plasmin - e.g. tranexamic acid
142
Q

What is von Willebrand disease (vWD)

A
  • Autosomal dominant mutation causing vWF deficiency
143
Q

Explain the pathophysiology of VWD

A
  • vWF assists platelet plug formation
  • Decreased vWF results in more spontaneous bleeds and bruising
144
Q

How does VWD present

A
  • Nose bleeds
  • Menorrhagia
  • Spontaneous bleeds
145
Q

Describe the investigation and diagnosis of VWD

A
  • Normal PT and raised APTT
  • Reduced plasma vWF
  • normal platelets
146
Q

Treatment for VWD

A
  • Desmopressin - raises levels of vWF
  • Tranexamic acid - useful for Menorrhagia or nosebleeds
  • Combined oral contraceptive pill - menorrhagia
147
Q

What is Disseminated intravascular coagulopathy (DIC)

A
  • Systemic activation of coagulation cascade
  • Results in thrombosis of small vessels
148
Q

Causes of DIC

A
  • Sepsis
  • Malignancy
  • Transplant rejection
149
Q

Describe the investigations and diagnosis of DIC

A
  • Low fibrinogen levels
  • Prolonged PT and APTT
  • Thrombocytopenia
150
Q

Treatment of DIC

A
  • Fresh frozen plasma - replace clotting factors
  • Platelet transfusion
  • RBC transfusion
151
Q

What is G6PDH deficiency

A
  • X linked recessive condition causing decreased lifespan of RBC
152
Q

How is hereditary spherocytosis treated

A
  • Splenectomy
  • Folate supplements
153
Q

What is malaria

A

A parasitic infection caused by the plasmodium family

154
Q

How is malaria spread

A

Spread through bites from the female anopheles mosquitos that carry the disease

155
Q

What are the 4 species of plasmodium

A

P. falciparum
* P. vivax
* P. ovale
* P. malariae

156
Q

Which species of plasmodium is most severe and common

A

P. falciparum

157
Q

Which plasmodium can relapse and why

A

P. vivax and P. ovale as they can lay dominant in the liver for years

158
Q

RF for malaria

A
  • African countries
  • Infants
  • Pregnancy
  • Old age
159
Q

Signs and symptoms of malaria

A
  • Fever, sweats
  • Malaise
  • Hepatosplenomegaly
  • shortness of breath
160
Q

Describe the investigation and diagnosis of malaria

A
  • Travel history
  • hick and thin blood film over 3 consecutive days
161
Q

How is malaria treated

A
  • Quinine and doxycycline
  • IV artesunate in severe/ complicated malaria

2a - SBCS (9 decks)

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