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Haematology Diseases Flashcards

1
Q

What is the epidemiology of DVTs?

A

25-50% of surgical patients
65% of below knee DVTs are asymptomatic and rarely embolise to the lung
Commonly occurs after periods of immobilisation

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2
Q

What are the risk factors for DVTs?

A 
Increased age
Pregnancy
Synthetic oestrogen
Trauma or surgery
Past DVT
Cancer 
Obesity 
Immobilisation
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3
Q

What are the causes of DVT?

A

Surgery, immobility, leg fractures, oral contraceptive, malignancy, long haul flights
Genetic: Factor V leiden (5%)m and PT2021OA (3%) = both common in caucasian populations
Acquired: Anti-phospholipid syndrome, lupus anticoagulant, hyperhomocysteinaemia

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4
Q

What are the clinical features of DVT?

A

May be asymptomatic
Pulse is present
Pain in calf- often swelling, redness and engorged superficial veins
With complete occlusion, can result in cyanotic discouration and severe oedema

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5
Q

How is DVT diagnosed?

A

Plasma D dimer= Type of fibronigen degradation product that is released when a clot begins to dissolve. Plasma D dimer is not diagnostic, but a normal result can exclude DVT
Compression ultrasound: if popliteal vein can be squashed shut= No DVT, if not= DVT
Venography
Doppler ultrasound

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6
Q

How is DVT treated?

A

Low molecular weight heparin (e.g. sc enoxaparin) for a minimum of 5 days
Oral warfarin with a target INR of 2.5 by 6 months after
Direct acting oral anticoagulants
Compression stockings
IVC filters

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7
Q

How is DVT prevented?

A

Early mobilisation post op
Compression stockings
Thrombophylaxis

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8
Q

What are the complications of DVT?

A

Pulmonary embolism

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9
Q

What are the causes of heart failure?

A

IHD (Main cause), cardiomyopathy, valvular heart disease, hypertension, cor pulmonale, alcohol excess, pregnancy, obesity etc.

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10
Q

What is the epidemiology of heart failure?

A

25-50% of patients die within 5 years of diagnosis
1-3% of population
10% of elderly

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11
Q

What are the risk factors for heart failure?

A 
65 and over
African descent
Men (due to low oestrogen)
Obesity 
Post MI
Pregnancy
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12
Q

How is heart failure treated?

A

Lifestyle changes= Avoid large meals, lower BMI, smoking cessation, vaccination

  • Diuretics
  • Aldosterone antagonist
  • ACE inhibitors
  • Beta blocker
  • Digoxin
  • Surgical measures
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13
Q

How is heart failure diagnosed?

A
  • Blood tests first line= High BNP
  • Chest X ray= Alveolar oedema, cardiomegaly, effusions
  • ECG= Ischaemia, LV hypertrophy, arrhythmia
  • Echocardiography
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14
Q

What is the clinical presentation of heart failure ?

A

3 cardinal symptoms= Shortness of breath, fatigue, ankle swelling
Dysponea, cold peripheries, raised JVP, Murmurs, cyanosis, hypotension, tachycardia, 3rd and 4th heart sounds, bi-basal crackles, ascites

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15
Q

What are the classes of heart failure?

A

Systolic or diastolic

Acute or chronic

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16
Q

What is systolic heart failure and what causes it?

A

Ventricle cant contract normally, caused by IHD, MI etc

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17
Q

What is diastolic heart failure and what causes it?

A

Ventricle can’t relax fully to properly fill. Caused by hypertrophy (due to hypertension) or aortic stenosis

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18
Q

Briefly explain the pathophysiology of heart failure

A

When the heart begins to fail, there are compensatory changes to maintain cardiac output and peripheral perfusion. However this is overwhelmed, and leads to decompression-

  1. Venous return (preload)= Heart tries to increase force of contraction to make up for low ejection fraction, but this fails
  2. Outflow resistance (afterload)= Increased resistance leads to a lower cardiac output and dilated ventricle
  3. Sympathetics= Baroreceptors become used to new normal
  4. RAAS= Not activated in failure, so less blood supply to overworked myocytes leading to cell death
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19
Q

What is the epidemiology or pernicious anaemia?

A
  • Common in elderly
  • More common in females
  • An association with other autoimmune diseases
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20
Q

What are the risk factors for pernicious anaemia?

A
  • Elderly
  • Female
  • Fair-haired, blue eyes
  • Blood group A
  • Thyroid and Addison’s disease
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21
Q

Briefly explain the pathophysiology of pernicious anaemia

A
  • There are many causes of B12 deficiency including dietary, malabsorbtion and pernicious anaemia
  • An autoimmune disorder in which parietal cells are attacked resulting in atrophic gastritis and the loss of intrinsic factor
  • Therefore B12 can’t be reabsorbed
  • B12 is essential for thymidine and DNA synthesis
  • Delayed nuclear maturation resulting in large RBCs and lower RBC production
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22
Q

What are the general clinical features of anaemia?

A
  • Fatigue
  • Headache
  • Dyspnoea
  • Anorexia
  • Palpitations
  • Pallor
  • Tachycardia
  • Heart failure/ Angina
  • Heart murmur
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23
Q

What are the specific clinical features of pernicious anaemia?

A
  • Generalised anaemia symptoms
  • Lemon yellow skin due to pallor and mild jaundice (excess haemoglobin breakdown)
  • Glossitis and stomatitis/ cheilosis
  • Neurological features if v low B12= Symmetrical paresthesia in fingers and toes, weakness and ataxia, dementia etc.
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24
Q

How is pernicious anaemia diagnosed?

A
  • Blood count and film= RBCs are macrocytic, oval macrocytes, hypersegmented neutrophil polymorphs with 6 nuclei
  • Serum bilirubin is raised due to ineffective erythropoiesis
  • Low serum B12
  • Low Hb
  • Low reticulocytes
  • Intrinsic factor antibodies (insensitive)
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25
Q

How is pernicious anaemia treated?

A
  • If other cause of B12 deficiency, treat cause
  • Injections required if low B12 is due to malabsorption
  • If dietary then give oral B12
  • Replenish B12 stores by IM hydroxocobalamin
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26
Q

What is the epidemiology of iron deficiency anaemia?

A

Most common cause of anaemia worldwide. Seen in 14% of menstruating women

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27
Q

What are the causes of iron deficiency anaemia?

A
  • Blood loss= Menorrhagia, GI bleeding (Hookworm)
  • Poor diet
  • Increased demands= Growth and pregnancy
  • Malabsorption
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28
Q

What are the risk factors for iron deficiency anaemia?

A
  • Undeveloped countries
  • High veg diet
  • Premature infants
  • Introduction of mixed feeding delayed
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29
Q

Briefly explain the pathophysiology of iron deficiency anaemia?

A
  • Iron ions are required for haem synthesis
  • This leads to low haemoglobin
  • Therefore lower RBC formation
  • Microcytic anaemia
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30
Q

What is the average daily intake of iron?

A

15-20 mg

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31
Q

What are the clinical features of iron deficiency anaemia?

A
  • General anaemia symptoms
  • Brittle nails and hair
  • Spoon shaped nails Koilonychia
  • Atrophy of papillae of tounge= atrophic glossitis
  • Ulceration of corner of mouth= angular stomatitis/ cheilosis
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32
Q

How is iron deficiency anaemia diagnosed?

A
  • Blood count and film= RBC are microlytic and hypochromic, poikilocytosis (variation and RBC size), and anisocytosis (Variation in size)
  • Low serum ferritin (But it raises in inflammation so may not be low), and low serum iron
  • Low reticulocytes
  • High serum transferrin receptors
  • Further investigations into possible blood loss
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33
Q

How is iron deficiency anaemia treated?

A
  • Find and treat cause
  • Oral iron e.g. ferrous sulphate (GI Side effects), or if CI, then ferrous gluconate
  • Parenteral iron e.g. IV iron in extreme cases
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34
Q

What is the epidemiology of anaemia of chronic disease?

A
  • 2nd most common anaemia
  • Commonest anaemia in hospital patients
  • Occurs in individuals with chronic infections e.g. TB, Crohn’s, RA, SLE, Malignancy
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35
Q

What is the clinical presentation of anaemia of chronic disease?

A
  • Fatigue, headache and faintness
  • Dyspnoea and breathlessness
  • Anorexia
  • Intermittent claudication
  • Palpitations
  • Angina if pre-existing coronary disease
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36
Q

What are the risk factors of anaemia of chronic disease?

A

Have a chronic disease

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37
Q

What is the pathophysiology of anaemia of chronic disease?

A

There is a decreased release of iron from the bone marrow to developing erythroblasts (early RBC before reticulocytes). An inadequate erythropoietin response to anaemia. Decreased RBC survival

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38
Q

How is anaemia of chronic disease diagnosed?

A
  • Serum iron and total iron-binding capicity (TIBC) low
  • Serum ferritin is normal or raised due to inflammation
  • Serum soluble transferrin receptor level is normal
  • Blood count and film: RBCs are normocytic or microcytic and hypochromic (pale)
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39
Q

How is anaemia of chronic disease treated?

A
  • Treat underlying cause

- Erythropoietin is effective (SE: Flu like symptoms, mild rise in platelet count, hypertension)

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40
Q

What is MCV?

A

Mean corpuscular volume

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41
Q

What is the MCV in normocytic anaemia?

A

Normal

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42
Q

What is the MCV in microcytic anaemia?

A

Low

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43
Q

What is the MCV in macrocytic anaemia?

A

High

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44
Q

What are the main causes of microcytic anaemia?

A
  • Iron deficiency anaemia= most common cause
  • Anaemia of chronic disease
  • Thalassaemia
  • Congenital sideroblastic anaemia
  • Lead poisoning
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45
Q

What are the main causes of macrocytic anaemia?

A
  • Megaloblastic= Vit B12 deficiency (cobalamin), folate deficiency
  • Non megaloblastic= Alcohol, liver disease, hypothyroidism, haemolysis, bone marrow failure, bone marrow infiltration, myeloma
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46
Q

What are the 2 types of macrocytic anaemia?

A
  • Megaloblastic= Presence of erythroblasts with delayed nuclear maturation due to delayed DNA synthesis (These are megaloblasts, large and have no nuclei)
  • Non-megaloblastic= Erythroblasts are normal (normoblastic)
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47
Q

What are the main causes of normocytic anaemia?

A
  • Acute blood loss
  • Anaemia of chronic disease
  • Renal failure
  • Pregnancy
  • Endocrine disorders such as hypopituitarism, hypothyroidism and hypoadrenalism
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48
Q

What is the clinical presentation of normocytic anaemia?

A
  • Fatigue, headache and faintness
  • Dyspnoea and breathlessness
  • Angina if pre-existing coronary disease
  • Anorexia
  • Intermittent claudication
  • Palpitations
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49
Q

How is normocytic anaemia diagnosed?

A
  • Normal B12 and folate
  • Raised reticulocytes
  • Hb down
  • Blood count and film: RBCs are normocytic
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50
Q

How is normocytic anaemia treated?

A
  • Treat underlying cause
  • Improve diet with plenty of vitamins
  • Erythropoietin injections
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51
Q

What are the four types of leukaemia?

A
  • Acute myeloid leukaemia
  • Acute lymphoblastic leukaemia
  • Chronic myeloid leukaemia
  • Chronic lymphocytic leukaemia
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52
Q

What are the clinical features of acute myeloid leukaemia?

A
  • Anaemia= Low Hb so breathlessness, fatigue, angina, and claudication. There is pallor and cardiac flow murmur
  • Infection due to low WCC- fever, mouth ulcers
  • Bleeding due to low platelets- Bleeding, bruising, hepatomegaly, splenomegaly, gum hypertrophy, DIC
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53
Q

How is acute myeloid leukaemia treated?

A
  • Blood and platelet transfusion
  • Neutropenia may lead to deadly infections- treat with prophylactic antivirals, antifungals and antibacterials
  • Allopurinol (prevents tumour lysis syndrome)
  • IV fluids via Hickman line
  • Chemotherapy
  • Marrow transplant
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54
Q

How is acute myeloid leukaemia diagnosed?

A
  • WCC is often raised, but can be normal or even low
  • Bone marrow biopsy
  • Differentiation from ALL is based on microscopy, immunophenotyping and molecular methods
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55
Q

What are the complications of acute myeloid leukaemia?

A
  • Infection= Common organisms present oddly with few antibodies being made
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56
Q

What are the clinical features of chronic lymphocytic leukaemia?

A
  • Often no symptoms= presenting as a surprise on a routine FBC
  • May be anaemic (due to haemolysis or marrow infiltration) or infection prone
  • If severe; weight loss, sweats and anorexia
  • Enlarged, rubbery, non-tender nodes
  • Hepatosplenomegaly
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57
Q

How is chronic lymphocytic leukaemia diagnosed?

A
  • Blood count= normal or low Hb
  • Raised WCC with very high lymphocytes
  • Smudge cells may be seen in vitro
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58
Q

What are the complications of chronic lymphocytic leukaemia?

A
  • Autoimmune haemolysis
  • Increased infection risk - hypogammaglobulinaemia
  • Marrow failure
  • Progression
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59
Q

How is chronic lymphocytic leukaemia treated?

A
  • Blood transfusion
  • Human IV immunoglobulins
  • Chemo or radiotherapy
  • Try stem cell transplant
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60
Q

What is the prognosis of chronic lymphocytic leukaemia?

A
  • 1/3 will never progress
  • 1/3 progress slowly
  • 1/3 will progress actively
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61
Q

What is the epidemiology of chronic myeloid leukaemia?

A
  • Most exclusively a disease of adults
  • Occurs between 40-60 yrs
  • Slight male predominancy
  • More than 80% have Philadelphia chromosome
62
Q

What is the epidemiology of chronic lymphocytic leukaemia?

A
  • Most common leukaemia

- Occurs predominantly later in life

63
Q

What are the clinical features of chronic myeloid leukaemia?

A
  • Symptomatic anaemia e.g. breathlessness
  • Abdominal discomfort= splenomegaly
  • Weight loss, pallor, fatigue
  • Fever and sweats
  • Features of gout due to purine breakdown
  • Bleeding due to platelet dysfunction
64
Q

How is chronic myeloid leukaemia diagnosed?

A
  • Blood count= v high wcc, low Hb (normochromic and normocytic)
  • Bone marrow aspirate= hypercellular
65
Q

How is chronic myeloid leukaemia treated?

A
  • Oral imatinib

- Stem cell transplant

66
Q

What is the epidemiology of acute lymphoblastic leukaemia?

A
  • Most common between 2-4 (commonest cancer in childhood)
67
Q

What is chronic lymphocytic leukaemia?

A
  • Accumulation of mature B cells that have escaped apoptosis and undergone cell-cycle arrest
68
Q

What is chronic myeloid leukaemia?

A
  • Uncontrolled clonal proliferation of myeloid cells
69
Q

What is acute myeloid leukaemia?

A
  • Neoplastic proliferation of blast cells derived from marrow myeloid (giving rise to basophils, neutrophils, eosinophils etc)
70
Q

What is acute lymphoblastic leukaemia?

A
  • Malignancy of immature lymphoid cells (give rise to T and B cells). Arrests the maturation and promotes uncontrolled proliferation of immature blast cells. Majority of cases derive from B cell precursors
71
Q

What are the clinical features of acute lymphoblastic leukaemia?

A
  • Anaemia= low Hb. Resulting in breathlessness, fatigue, angina, claudication
  • Infections
  • Bleeding= Bruising, bleeding, bone pain, hepatosplenomegaly, lymphadenopathy, Headache, nerve palsies
72
Q

How is acute lymphoblastic leukaemia diagnosed?

A
  • FBC and blood film= WCC is usually high, blast cells on film and in bone marrow
  • CXR and CT to look for mediastinal and abdominal lymphadenopathy
  • Lumbar puncture to look for CNS involvement
73
Q

How is acute lymphoblastic leukaemia treated?

A
  • Blood and platelet transfusion
  • Neutropenia- treat prophylactically with antibacterials, antifungals and antivirals
  • Allopurinol to prevent tumour lysis syndrome
  • IV fluids via Hickman line
  • Chemotherapy
  • Marrow transplantation
74
Q

What are the risk factors for Hodgkin’s lymphoma?

A
  • Affected siblings
  • EBV
  • SLE
  • Obesity
  • Post transplantation
75
Q

What is the epidemiology of Hodgkin’s lymphoma?

A
  • Male predominance
  • Majority occurs in teenagers and elderly
  • EBV
76
Q

What are the two types of Hodgkin’s lymphoma?

A
  • Classical Hodgkin’s lymphoma

- Nodular lymphocyte predominant Hodgkin’s lymphoma

77
Q

What is the emergency presentation of Hodgkin’s lymphoma?

A
  • Infection
  • Superior vena cava obstruction, with increased jugular venous pressure
  • Sensations of fullness in the head
  • Dyspnoea
  • Blackouts
  • Facial oedema
78
Q

What are the clinical features of Hodgkin’s lymphoma?

A
  • Often only a painless “rubbery” cervical lymphadenopathy
  • A smaller proportion of patients (often young women) present with disease localised to the mediastinum with a cough
  • Others present with generalised disease including hepatosplenomegaly, weight loss, fever and night sweats
79
Q

How is Hodgkin’s lymphoma diagnosed?

A
  • CT/MRI of chest, abdomen and pelvis for staging
  • Lymph node excision or bone marrow biopsy- will see “popcorn cells”
  • Bloods- High ESR/ Low Hb= Worse prognosis, High serum lactate dehydrogenase
  • Immunophenotyping
  • Cytogenetics
  • PET scan
80
Q

How is Hodgkin’s lymphoma treated?

A
  • Combination chemotherapy
  • If less advanced, short course of chemotherapy, followed by radiotherapy
  • If advanced, longer course of chemotherapy
81
Q

What are the stages of Hodgkin’s lymphoma?

A 
1= Single lymph node
2= 2+ Lymph nodes on same side of diaphragm
3= Nodes on both sides of diaphragm
4= Spread beyond nodes
Also A (no systemic symptoms) or B (Symptomatic)
82
Q

What is the epidemiology of Non-Hodgkin’s lymphoma?

A
  • Around 80% is of B cell origin

- Around 20% is of T cell origin

83
Q

What are the risk factors of Non-Hodgkin’s lymphoma?

A
  • Family history is linked with a minor increase in risk
84
Q

What are the clinical features of Non-Hodgkin’s lymphoma?

A
  • Nodal disease (75%) e.g. superficial lymphadenopathy
  • Extranodal disease (25%) = skin, oropharynx, gut, bone, small bowel etc
  • Systemic B symptoms= Fever, weight loss, night sweats
  • Pancytopenia- anaemia, infection and bleeding
85
Q

What are the classifications of Non-Hodgkin’s lymphoma?

A
  • Low/indolent grade= slow growing, usually advanced at presentation, incurable. Median survival of 9-11 yrs
  • High grade= Usually has nodal presentation, 1/3 of cases have extranodal involvement
86
Q

How is Non-Hodgkin’s lymphoma diagnosed?

A
  • Raised lactose dehydrogenase reflects worse prognosis
  • Lymph node excision/ bone marrow biopsy
  • Marrow and node biopsy for classification
  • CT/MRI of chest, abdomen and pelvis for staging
  • Immunophenotyping
  • Cytogenetics
87
Q

How is Non-Hodgkin’s lymphoma treated?

A
  • R CHOP= Rituximab, cyclophosphamide, hydroxy-duanorubioin, Oncovin (brand name for vincristine), prednisolone
  • In low grade, radiotherapy may be curative
88
Q

Briefly explain the pathophysiology of Burkitts lymphoma?

A
  • The accumulation of malignant plasma cells in the bone marrow
  • In myeloma, the malignant plasma cells produce an excess of one type of immunoglobulin (usually IgG) and other immunoglobulins are low
89
Q

What is the epidemiology of Burkitts lymphoma?

A
  • Disease of old age
  • Peak age is 70 yrs
  • More common in Afro-Carribbean populations
90
Q

What are the clinical features of Burkitts lymphoma?

A
  • Renal failure (as raised immunoglobulins are proteins which precipitate and deposit in organs especially kidneys)
  • Anaemia= neutropenia or thrombocytopenia resulting in infection, bleeding, fatigue and pallor
  • Bone lytic lesions= bone pain and fractures
91
Q

How is Burkitts lymphoma diagnosed?

A
  • Blood: normocytic normochromic anaemia, raised ESR, rouleaux formation on blood films
  • U&Es: Raised calcium and alk phosphatase
  • Bence-jones protein in urine
  • X ray shows lytic lesions
  • Monoclonal protein band in serum or urine
  • Raised plasma cells on bone marrow biopsy
92
Q

How is Burkitts lymphoma treated?

A
  • Bone pain supported with analgesia and bisphosphonates for fracture
  • Anaemia treated with RBC transfusion and EPO
  • Rehydrate
  • Renal dialysis
  • Treat infections
  • Chemotherapy
  • Stem cell transplant
93
Q

What are the clinical features of myeloma?

A

OLD CRAB

  • Old age
  • Calcium elevated
  • Renal failure
  • Anaemia
  • Bone lytic lesions
94
Q

How is myeloma diagnosed?

A
  • Blood: normocytic normochromic anaemia, raised ESR, rouleaux formation on blood films
  • U&Es: Raised calcium and alk phosphatase
  • Bence-jones protein in urine
  • X ray shows lytic lesions
  • Monoclonal protein band in serum or urine
  • Raised plasma cells on bone marrow biopsy
95
Q

How is myeloma treated?

A
  • Bone pain supported with analgesia and bisphosphonates for fracture
  • Anaemia treated with RBC transfusion and EPO
  • Rehydrate
  • Renal dialysis
  • Treat infections
  • Chemotherapy = VAD
  • Stem cell transplant
96
Q

What is polycythaemia?

A

An increase in haemoglobin, haemocrit and red cell count by concentration.

97
Q

What are the types of polycythaemia?

A
  • Absolute (increase in RBC mass) = Polycythemia vera, hypoxia etc
  • Relative (Decreased plasma vol and normal RBC mass) = dehydration, apparent polycythaemia
98
Q

What is over anticoagulation?

A
  • Excess use of anticoagulation therapy
99
Q

What are the clinical features of over anticoagulation?

100
Q

What are the risk factors for over anticoagulation?

A
  • Iatrogenic (inappropriate prescription of warfarin/ heparin)
101
Q

What are the treatments for over anticoagulation?

A
  • Warfarin= Phytomenadione

- Heparin= Protamine sulfate

102
Q

Briefly explain the pathophysiology of polycythaemia vera

A
  • A clonal stem cell disorder resulting in a malignant proliferation of a clone derived from one pluripotent marrow stem cell
  • The erythroid progenitor offspring are unusual in not needing EPO to avoid apoptosis
  • This results in the excess proliferation of RBCs, WBCs, and platelets which causes raised haemocrit
103
Q

What are the possible complications of polycythaemia vera?

A

Thrombosis and haemorrhage

104
Q

What are the clinical features of polycythaemia vera?

A
  • May be asymptomatic
  • May present with vague symptoms due to hyperviscosity: headaches, tiredness, dizziness, tinnitus, visual disturbances
  • Severe itching
  • Erythromelalgia- burning sensation in fingers and toes
  • Gout
  • Hypertension, angina, intermittent claudication
  • Plethoric complexion
  • Hepatosplenomegaly
105
Q

How is polycythaemia vera diagnosed?

A
  • Blood count= raised WCC and platelets, raised Hb
  • Presence of JAK2 mutation on genetic screen
  • Bone marrow biopsy showing prominent erythrothyroid, granulocytic and megakarocytic proliferation
  • Serum EPO low
106
Q

How is polycythaemia vera treated?

A
  • No cure
  • Venesection= aims to lower PCV and platelet count
  • Chemotherapy= hydroxycarbamide and low dose bulsulfan for those who dont tolerate venesection
  • Low dose aspirin
  • Radioactive phosphorus
  • Allopurinol to reduce gout
107
Q

What causes immune thrombocytopenia purpura?

A

Immune destruction of platelets

108
Q

How is immune thrombocytopenia purpura diagnosed?

A
  • Bone marrow examinaiton= shows thrombocytopenia with increased/normal megakaryocytes
  • Platelet autoantibodies in 60-70%
109
Q

How is immune thrombocytopenia purpura treated?

A
  • First line= Corticosteroids e.g. prednisolone, and IV immunoglobulins
  • Second line= Splenectomy, or if this fails, immunosuppression
110
Q

What are the clinical features of immune thrombocytopenia purpura?

A
  • Easy bruising
  • Epistaxis
  • Menorrhagia
  • Purpura
  • Gum bleeding
111
Q

What is the aetiology of thrombotic thrombocytopenia purpura?

A
  • Idiopathic
  • Autoimmune e.g. SLE
  • Cancer
  • Pregnancy
  • Drug associated e.g. quinine
112
Q

What are the clinical features of thrombotic thrombocytopenia purpura?

A
  • Floroid purpura
  • Fever
  • Fluctuating cerebral dysfunction
  • Haemolytic anaemia with red cell fragmentation, often accompanied by AKI
113
Q

How is thrombotic thrombocytopenia purpura diagnosed?

A
  • Lactate dehydrogenase is raised due to haemolysis
114
Q

How is thrombotic thrombocytopenia purpura treated?

A
  • Plasma exchange to remove antibody to ADAMTS-13 as well as provide a source of ADAMTS-13
  • IV methylprednisolone
  • IV Rituximab
115
Q

What are the clinical features of sickle cell disease?

A
  • Acute pain in hands and feet. Worse in cold.
  • Anaemia
  • Pulmonary hypertension
  • Growth delay
  • Recurrent infection
  • Hepatomegaly and liver dysfunction
  • Retinopathy, vitreous haemorrhage
116
Q

Briefly explain the pathophysiology of sickle cell disease?

A

Changes in the alpha-alpha sequence of a Hb subunit causes a faulty Hb complex. This distorts the shape of rbc into sickles when deoxygenated, which are easily destroyed and occlude vessels easily. This process worsens with repeated oxygenation/deoxygenation.

117
Q

What is the epidemiology of sickle cell disease?

A
  • More common in African populations
118
Q

What are the risk factors for sickle cell disease?

A
  • African

- Family history

119
Q

How is sickle cell disease diagnosed?

A
  • Identified in neonatal screening
  • Blood film= sickled cells
  • Sickle solubility test will be positive
  • Hb in range of 60-80g/L, raised reticulocyte count
120
Q

How is sickle cell disease treated?

A
  • Folic acid
  • Pain relief
  • BMT in severe disease
  • Oral hydroxycarbamide
121
Q

What are the causes of DIC?

A
  • Massive activation of the coagulation cascade
  • Sepsis
  • Major trauma
  • Advanced cancer
  • Obstetric complications
122
Q

What is the epidemiology of DIC?

A

Never occurs in isolation

123
Q

What are the clinical features of DIC?

A
  • Patient is acutely ill and shocked
  • Bleeding may occur from mouth, nose and venepuncture sites
  • Widespread ecchymoses
  • Confusion
  • Bruising
124
Q

How is DIC diagnosed?

A
  • Severe thrombocytopenia
  • Decreased fibrinogen
  • Increased FDPs e.g. D dimer
  • Blood film= Fragmented RBC
  • Prolonged PTT, Activated partial thromboplasmin time and Thrombin time
125
Q

How is DIC treated?

A
  • Treat underlying condition
  • Replace platelets if very low via transfusion
  • Fresh frozen plasma to replace coagulation factors
  • Cytoprecipitate to replace fibrinogen and coagulation factors
  • RBC transfusion in patients who are bleeding
126
Q

What are the clinical features of thalassemia?

A
  • Variable
  • Alpha presents in utero, whilst beta in infancy
  • Can be asymptomatic if heterozygotes or severe anaemia in homozygotes, with failure to thrive and bone deformities
127
Q

Briefly explain the pathophysiology of thalassemia?

A

Defective versions of either alpha or beta subunits of Hb= inbalance of subunits available= Precipitation of globin chains within RBC (or precursor) = Cell damage, death of precursor and haemolysis

128
Q

What is the epidemiology of thalassemia?

A
  • 1% Carriers of beta

- 5% Carriers of alpha

129
Q

How is thalassemia diagnosed?

A
  • Either genetic testing or haemoglobin electrophoresis
130
Q

How is thalassemia treated?

A
  • Homozygotes= blood transfusions to avoid complications. Iron chelating agents for overload (deferoxamine). Ascorbic acid increases iron excretions in urine.
  • More severe= BMT
131
Q

What are possible complications of thalassemia?

A
  • Iron overload, endocrine dysfunction
132
Q

What are the clinical features of glucose-6-phosphate dehydrogenase deficiency?

A
  • Most are asymptomatic
  • Neonatal jaundice
  • Haemolytic anaemia
  • Acute haemolysis
133
Q

What is the aetiology of glucose-6-phosphate dehydrogenase deficiency?

A
  • X linked recessive

- Linked to Fava beans

134
Q

What is the epidemiology of glucose-6-phosphate dehydrogenase deficiency?

A
  • Most common metabolic RBC disorder
  • Affects predominantly in Africa, around the Mediterranean and in the Middle East
  • Heterogenous X linked = More common in males
135
Q

How is glucose-6-phosphate dehydrogenase deficiency diagnosed?

A
  • Direct measurements of enzymes in RBC
  • Blood film during attack= Irregularly contracted cells, bite cells, reticulocytosis
  • G6PD enzyme levels low
136
Q

How is glucose-6-phosphate dehydrogenase deficiency treated?

A
  • Avoid fava beans

- Transfusion if necessary

137
Q

What are the clinical features of malaria?

A
  • Most with falciparum present in first month
  • No specific symptoms
  • Fever, chills, rigor, cough, myalgia, hepatosplenomegaly
  • Severe: Impaired consciousness, shortness of breath, bleeding, fits, hypovolaemia
138
Q

What are the risk factors for malaria?

A
  • Poor
  • Young
  • Pregnant
  • Elderly
  • Recently travelled abroad
139
Q

How is malaria diagnosed?

A
  • Blood smear with giemsa stain
140
Q

How is malaria treated?

A
  • Non falciparum: chloroquine
  • Falciparum: Quinine sulfate, atovaquone-proguaril and artemether with lumefantrine
  • Severe: IV quinine dihydrochloride
141
Q

Briefly explain the pathophysiology of haemophilia A

A
  • Factor VIII Deficiency

- X linked

142
Q

Which is more common; haemophilia A or haemophilia B?

143
Q

How is haemophilia diagnosed?

A
  • FBC= Low heamocrit, low Hb
  • Normal prothrombin time
  • Prolonged partial thromboplastin time
  • Low factor VIII/ IX
144
Q

What are the clinical features of Haemophilia A?

A
  • Mild disease bleed after trauma
  • Bleeding following venepuncture
  • Neonatal bleeding
  • GI bleeds
  • Haematuria
145
Q

How is haemophilia A treated?

A
  • Prophylactic factor VIII
  • Desmopressin for factor VIII
  • Fresh frozen plasma containing factor VIII for acute bleeds
146
Q

How is haemophilia B treated?

A
  • Recombinant factor IX
  • Vaccinated against Hep A and B
  • Medical emergency bracelet
147
Q

Briefly explain the pathophysiology of haemophilia B?

A
  • Factor IX deficiency

- Male predominance (X linked)

148
Q

What are the clinical features of haemophilia B?

A
  • Bruising
  • Epistaxis
  • Pallor
  • Haemoptysis
  • Heavy bleeding following trauma
  • Joint pain and stiffness
  • Headache, irritability, vomitting
149
Q

How is arterial thrombosis treated?

A

Anti-platelets e.g. aspirin, clopidogerel

150
Q

How is venous thrombosis treated?

A

Anti-Coagulants e.g. warfarin, heparin, NOACs

151
Q

What are the clinical features of arterial thrombosis?

A
  • Diminished or absent pulse
  • Pain= intermittent claudication
  • Cold
  • Rubor

Phase 2a (31 decks)

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