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Year 2: Clinical Pathology > Haematology > Flashcards

Haematology Flashcards

1
Q

Haemopoiesis

A

Production of blood cells and plasma that occurs in the bone marrow

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2
Q

Multipotent haemopoietic stem cell - does what?

A

Can divide/ differentiated into any cell linage and produce mature blood cells

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3
Q

two Cell linages - and the mature cells they produce?

NOTE: ‘White (immune) blood cells’ come from 2 different linages

A

Myeloid - erythrocytes, platelets, monocytes, neutrophil, basophil and eosinophil

Lymphoid - Lymphocytes - T cells, B cells and natural killer cells

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4
Q

What are granulocytes? 4 Examples (what they do)

A

Myeloid linage cells with granules in the cytoplasm eg

Neutrophils - phagocytosis
Eosinophils - parasitic infection
Basophils - allergic/ innate reactions
Mast cells - release histamine in allergic reaction

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5
Q

What do monocytes do?

A

Myeloid linage.
Monocytes in the blood BUT macrophages in tissue
Antigen presenting Cells, Phagocytosis

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6
Q

Specialized monocytes in specific tissues?

A

Kupffer cells in the liver

Langerhan cells in the skin

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7
Q

Stem cells division methods (4) - what they produce + affect on stem cells pool?

Symmetrical
Asymmetrical
Lack of Self renewal
Lack of self renewal 2

A

Symmetric = self renewal of 2 stem cells = increase pool
Asymmetric = 1 Progenitor 1 stem cells = maintain pool
Lack of self renewal
= produce two Progentior cells = reduce pool
= don’t divide = maintain pool

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8
Q

Progenitor cell?

A

A pluripotent stem cells that are either common meyloid or common lymphoid progentior cell
Remain in blood for lifetime

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9
Q

Meylopoesis

A

Production of meyloid linage cells

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10
Q

Lymphopoesis

A

Production of lymphoid linage cells

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11
Q

Mature cells - lifespan in the blood?

A

Limited
Erythrocytes = 120 days
Neutrophils = 6 - 10 hours

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12
Q

Haemopoesis in the feotus? –> child?

A

Aortic gonad mesonephros –> Liver –> spleen

–> Bone marrow when born (production in liver and spleen stop)

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13
Q

Aneamia = ?

A

Reduced Erythrocytes

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14
Q

Raised erythrocytes?

A

polycythaemia

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15
Q

Reduced neutrophils ? (often due to drugs)

A

Neutropenia

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16
Q

Neutrophillia (usually due to inflammation and what type of infection?)

A

Raised neutrophils

Due to BACTERIAL infection

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17
Q

Increased eosinophils? - due to parasitic infection and allergies

A

Eosinophilia

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18
Q

Basophillia - is due to?

A

Increased Basophils

Due to chronic myeloid leukeamia

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19
Q

Raised Monocytes is called and occurs when?

A

Monocytosis - during TB infection

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20
Q

Reduced lymphocytes

Increased lymphocytes

A 
Reduced = Lymphopenia 
Increased = Lymphocytosis
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21
Q

Causes of increased lymphocytes?

A

Viral infection eg. glandular fever (EBV)

Lymphocytic leukaemia

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22
Q

Increased plasma cells - what called and why?

A

Plasmacytosis

Due to infection or myeloma

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23
Q

Thrombocytopenia?

A

Reduced platelets

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24
Q

What measurements does a full blood count include?

A 
Hb concentration
Mean cell volume
Mean cell Hb
White blood cell count
Platelet count
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25
Testing blood cells includes? (4)
Full blood count Blood film/ cultures - for pathogens in blood (sepsis Coagulation screen - time taken to clot in certain agents Bone marrow - aspirate and Trephine (core biopsy)
26
What is EDTA?
The preservative used to collect blood into
27
When are blood results outside the 'normal ranges' considered normal?
If they are normal for that patient in that clinical situation eg. low WBCC due to recent bone marrow transplant
28
Microcytic hypochromic aneamia? MCV ? MCH Causes (3)?
Small RBC low RBCC Low MCV and low MCH iron deficient, Thalasaemia - other blood disorders
29
Normocytic normochromic aneamia? MCV ? MCH Causes (4)?
Normal sized BUT low RBCC Low MCV and normal MCH Blood loss, kidney disease (other chronic disease), bone marrow failure
30
Macrotcytic aneamia? MCV ? Causes (3)?
Large RBC low RBCC High MCV Vit B12 or folate deficiency, Haemolytic anaemia, myelodysplasic syndrome
31
Myelodysplasia syndrome what is it?
Strain of cancers Mutation in stem cells Immature blood cells can't mature or leave the bone marrow Abnormal cells that do reach the blood = cytopenias
32
Characteristics of Myelodysplasia?
``` Hypogranular neutrophils Aneamia Thrombocytopenia Neutropenia Oval macrocytes Increased cellular activity in bone marrow ```
33
Symptoms of myelodysplasia syndrome (4)
Fatigue dysppnoea Infections bruising and bleeding
34
Refractory aneamia - blood test measurement?
High haemoglobin (MHC) Normal WBCC and platelets Low RBCC
35
Refractory aneamia (type of myelodysplasia) - what is it? results in what appearance of RBC? (4)
``` Increased erythoid precursors in the bone marrow RBCs are Different shaped (dimorphic) Different sizes (anisocytic) Loss biconcave appearance Stomatocytes - slit in them ```
36
Treatment for refractory aneamia
Iron chelation or frequent blood donation to reduce iron overload Erythropoietin injections = normal RBC produced
37
Leucodepletion?
Removal of WBC from donated blood
38
Blood plasma from donations is separated and used in? (3)
Fresh frozen plasma cryopercipitate Fractionation - Forming factor concentrates (clotting cascade factors), immunogloblins and albumin
39
Packed Red blood cell donation - storage
4 degrees for 35 days | electrolyte solution
40
'Group and screening' of recipient before blood transfusion involves?
Grouping by ABO group and Rh | Screening = For antibodies against any specific antigens
41
'Crossmatching' of recipients and donors blood before transfusion involves?
Patients plasma + sample of donor blood --> agglutination? or haemolysis occur? (counter indication)
42
Transfusion threshold? | Values?
Level to which Hb most drops before a Packed RBC transfusion is required <70 g/L = acute anaemia <80 g/L for those with cardiovascular disease
43
Indicators for packed RBC trasnfusion for hemorrhage?
30-40% blood loss = transfusion considered | >40% blood loss = transfusion required
44
Objectives for transfusion in chronic aneamia? | Targets?
Due myeloid failure syndromes Symptoms relief - fatigue Prevent long term ischemic injury to organs Hb 80-100 g/L - above which is iron overload
45
Objectives and risk of transfusion in Thalasameia? Targets?
Supression of endogenous erythropoetin BUT Risk of iron overload Hb 100-120 g/L
46
Counter indications for Pack RBC donations? (3)
Patients wishes and believes Adverse effect and risk of cross reaction Alternatives: - correcting aneamia (iron or B12) - Correcting anticoagulation - stopping medications
47
Platelet donations - storage
Room temp for 5 days
48
Adult Theraputic dose (for platelet donation?)
4 pooled donations (separated from full blood)
49
Indication for Platelet transfusion (3) plus examples
Thrombocytopenia, platelet dysfunction OR bleeding prevention Eg. Haemorrhage Bone marrow dysfunction + complication (sepsis) Prophylaxis for surgery
50
Counter indications for platelet transfusion (2)
Herparin induced thrombocytopenia | rare clotting disorders
51
Fresh Frozen plasma (FFP) donations - storage?
-30 degrees stored for 24 months - thawed before use
52
Indications for FFP?
Where clotting factors are required | Surgery, Haemorrhage AND rare clotting disorders
53
Counter indications for FFP?
Warfarin reversal | Single factor deficiencies
54
PCC - prothrombin complex concentrate - what is it? what is it used for?
Plasma derived clotting factors plus vit K | reversal of life threatening over anti-coagulation (bleeding)
55
Acute transfusion reactions - immunological cause? (4) + timings after transfusion
Heamolytic transfusion reactions (ABO incompatibility) - 15 mins Tranfusion related acute lung injury (TRALI) - 72-90 hours Allergies - urticarial rash Anaphylaxis
56
Acute non immunological transfusion reactions? (2)
Transfusion associated circulatory overload (TACO) | Febrile non-haemolytic transfusion reaction (FNHTR)
57
Long term immunological transfusion reactions *3 - 14 days
Delayed haemolytic reactions
58
Long term non immunological transfusion reactions | examples
Transfusions transmissible infections (TTI) | eg. Hep B and C, HIV, prion disease
59
Symptoms of acute ABO incompatibility? | Cause?
``` Acute renal failure, microvascular thrombosis and vasodilation Fever Chills Back pain Shock - hypotension ``` Human error
60
TRAIL - cause and effect? | Main donations
Transfusion related acute lung injury Activated WBC against HLAs of donors blood --> lung infiltrates (seen on Xray) FFP and platelets
61
Risk factors for overload (TACO)
Elderly, children and those Left ventricle dilations
62
Delayed haemolytic reactions - what its it? how presents?
IgG against ABO blood groups | Presents as jaundice, fatigue and fever with low Hb
63
Heritable bleeding disorders?
Often one defect
64
Von Willeband factor?
Stimulates the production of a fibrin clot | Interacts with vessel wall and platelets
65
Clinical presentation of Von Willeband disease
Petechiae - non blanching, non palpable rash Superfical bruise Bleeds are prolonged but not recurrent
66
Von willebane disease is?
Hereditary bleeding disorder with varying penetrance
67
3 types of Von willeband disease
Type 1 = mild. (dominant) Reduced amount (not made or quickly destroyed) Type 2 = normal amount BUT defective Type 3 = completely absent (recessive)
68
Treatment for Von willeband disease (4)
Antifibrolytics eg. tranexamic acid Desmopressin - stimulates vWF production Plasma derived vWF from donors OCP - for menorrhagia
69
Co-agulation defects ?
Deficiency in one of the co-agulation factors
70
Clinical presentation of co-agulation defects? (5)
``` Deep muscular bleeds - haematomae Joint bleeds - Haemarthrosis Retroperintoneal bleeds Prolong and recurrent bleeds After trauma Spontaneous bleeds ```
71
Haemophillia - two types? inheritance?
Type A = VIII deficient Type B = IX deficient or christmas disease Both X linked recessive Abnormal activated partial prothrombin time
72
Treatment of haemophillias
Replace clotting factors with FFP Desmopressin antifibrolytic Icing and rest
73
Acquired bleeding disorders
Often defects in many factors
74
Vit K deficiency - what factors affected?
``` Vitamin K dependent factors II VII IX X ```
75
Causes of vitamin K deficiency ? (4)
Antibiotics Neonates Obstructive jaundice Prolong nutritional deficiency
76
Massive Transfusion syndrome - what cause it (2)? what it results in?
Transfusion of blood volume equivalent to total volume in less then 24 hours loss of 50% of blood volume in 3 hours Dilation depletion of clotting factors
77
DIC - what does it stand for?
Disseminate intravascular coagulation
78
What is disseminate intravascular coagulation?
General disruption to the physiological balance of procoagulative and anticoagulant mechanism eg. excessive use of platelets and clotting factors
79
Acute causes of DIC (4)
Sepsis Trauma Acute heamolysis (ABO mismatch) Tissue necrosis
80
Chronic causes of DIC (3)
Malignancy Liver disease Retained dead feotus
81
Treatment for DIC
Treat underlying cause (Eg. chemo, antibiotics) | Supportive treatment = maintain profusion, folic acid and vit K supplements
82
Indicators for emergency management of DIC (3)
Platelet <50 g/L = platelet transfusion PT or APTT ratio >1.5 = FFP given Fibrinogen <1g/L = antifibrolytics
83
APTT - Activated partial thromboplastic times - what does it test?
Time for blood clot via the intrinsic pathway (when reacted with silica)
84
PT/INR - prothrombin time/ internalised normal ratio
Assess the extrinsic pathway (Tissue factor is added)
85
TCT - Thrombin clotting time
Time till clot forms when thrombin added
86
Warfarin - what kind of anticoagulant. How monitored?
Vit K antagonist | Monitored by PT/ INR --> aka verse the average time
87
Warfarin drug interactions - what increase bleeds (6)(potentiate)
``` Ampicillin Erythromycin NSAIDs Anti -acids Corticosteroids Amidodarone - AF treatment ```
88
Warfarin drug interactions - that increase clotting (antagonist) (5)
``` Rifampicin Carbamazepine (epilepsy) Cholesterol lowering Kidney failure (K+ sparing) Vit K ```
89
Reversing Warfarin Life threatening? Non life threatening?
WITH HOLD warafin and give vit K | Life threatening = also give Factor IV and Prothrobin complex concentrate (PCC)
90
Heparin - how does it work?
Stimulates exogenous fribolytic mechanisms
91
Heparin - over anti-coagulation reversal treatment?
Stop heparin | Administer Protamine
92
LMWH - low molecular weight heparin --> advantages?
More predictable = monitor less Longer half life = administer less better bioavaiability
93
Examples of LMWH (3)
Deltaparin tinzaparin enoxaparin
94
Myeloproliferative disorder that results in 'thick blood'?
Polycytheamia vera - increased RBC, (platelets and neurtophils)
95
HCT? - in a blood test and EPO in a blood test?
``` HCT = the % of RCB out of total blood cells EPO = amout of erythropoetin in the blood ```
96
What are secondary polycytheamias? | What are relative polycytheamias?
Secondary = to another disease eg. kidney failure, COPD, chronic hypoxia Relative = due to normal RBC but low plasma levels due to dehydration
97
Signs and symptoms of polycythemai vera (10)
``` Plethora - excessive bodily fluid Itching - when in hot water Splenomegaly Engorged retinal vessels Headache Malaise Tinnitus gout Gangrene peptic ulcer ```
98
Cause of Polycytheamia - with elevate EPO? | with normal EPO
``` Elevate = kidney disorders Normal = JAK2 gene mutation and Polycytheamia vera ```
99
Essential thrombocytosis
Myeliodproliferative disease --> increased platelets | Increased thrombotic risk
100
Essential thromocytosis - differential diagnosis for raised platelets? (7)
``` Surgery Infections Inflammation Malignancy iron deficient hyposplenism Drugs ```
101
Diagnosis of Essential Thrombocytosis? | Genes that cause?
Exclude other causes Persistent platelets over >450x10^9/L ``` Genes JAK2 (50% of cases) CALR (45% of cases - JAK2 negative) ```
102
Treatment of thrombocytosis?
Assess clotting risk | Aspirin 75 mg daily
103
Idiopathic myelofibrosis - presents as?
``` Many cytopenias Large spleen (splenomegaly) ```
104
Genetic involved in myelofibrosis
JAK2 and CALR
105
Treatment and prognosis for myelofibrosis?
JAK2 inhibitors Bone marrow transplant 5 years - not good
106
What is chronic myeloid leukaemia ? - myleoproliferative disorder Mutation?
Pluripotent stem cell disorder T(9:22) or Philadelphian translocation on BCR-ABL fusion gene Too many Mature WBC in the blood
107
Presentation and symptoms of myeloid leukaemia (5)
``` Leucocytosis Aneamia Splenomegaly - due to infarction Venous occlusion (renal and DVT) Gout ```
108
Treatment of Myeloid leukaemia?
Oral cytotoxic drugs eg. busulphan Interferons Allogenic bone marrow transplant (50% curative) Imatinib = Tyrosine kinase inhibitors for BCR-ABL genes
109
IgM - what is it released?
When a B cells is exposed to an antigen in the blood | initial immune response
110
Class switching of IgM --> another Ig. When/ where does it happen? What cells can express all the Igs
Class switching occurs when naivee B cells are exposed to antigen in primary lymphoid tissue and they mature into PLASMA or MEMORY cells Therefor Plasma cells are monoclonal and express Ig G, E, D, A, and M
111
Plasma cell differentiation pathway (4) - in the lymph tissue
Proliferation - only those with highly specific receptors = monoclonal Immunoglobin somatic hypermutation and class switching - heavy chains change Selection - only those with high affinity for the antigen Differentiation --> mature cell
112
Immunogloblins Light chains = heavy chains = - held together by disulphide bonds
``` Light = kappa or lambda Heavy = A, G, E, M or D ```
113
Plasma Dyscrasias - caused by?
Disorders of plasma cells Abnormal proliferation of monoclonal plasma cells
114
Detecting plasma cells and Igs
Serum Protein electrophoresis (ablumin, Ig alpha 1 and 2, Ig Beta and Ig gamma) Immunofixation - to find specific monoclonal Igs
115
Detection of the a Plasma Dyscrasia
'M spike' Protein electrophorsis shows that M protein is being released by the defective monoclonal plasma cells
116
Most common Plasma Dyscrasias
MGUS - monoclonal gammopathy of uncertain significance
117
Diagnosis myeloma? - MRE criteria = test - CRAB criteria = symptoms
``` Bone marrow biopsy + blood test + MRI = MRE myeloid defining events >60% clonal plasma cells in biopsy >1 bone lesion on MRI over 5mm >100 mg/L of serum light chains ``` C- hypercalceamia R - renal insufficiency A - anaemia B - bone lesions
118
``` Symptoms of CRAB Hypercalceamia Kidney insufficiency Anaemia Bone lesions ```
C = constipation, confusion, anorexia and polyuria R = confusion, oliuria A = fatigue B = bone pain back an ribs
119
MGUS -monoclonal gammopath of uncertain significance diagnosis? % progression to Myeloma?
Serum M protein <30 g/L 30% progress to myeloma (others can progress lymphoproliferary disorders)
120
Amyloid Light chain Amyloidosis - what is it?
Defective genes = missfolding of Ig light chains --> aggregate = beta plated fibrils
121
Affect of AL Amyloidosis?
Proteinuria 3g/day Liver and cardiac symptoms Fibrils seen in urine by urine PCR
122
Non hodgkins lymphoma - what is it? | Causes?
Enlargement of LN in certain patterns + splenomegaly Malignant or non malignant (infection, inflammation) - most rule out
123
2 types of non-hodgkins lymphoma?
High grade + acute onset - systemic symptoms (weight loss) eg. Burkitts Lymphoma Low grade + chronic onset - clinically well, clear bloods, relapsing patterns eg. Follicular lymphoma
124
Follicular lymphoma? - causes
Type of non hodgkins - gradually increasing size of LN Genetic component - T(14:18) BCL2 protooncogene = neoplastic cells aren't apoptosed
125
Prognosis of Folliucular lymphoma? - what is it called (5) aspects
FLIPI - follicular lymphoma internation prognostic index ``` Age >65 Ann Arbor staging 4 + nodules low Hb LDH levels ```
126
Hodgkin's Lymphoma - what is it?
Neoplastic lymphocytes called Hodgkin Reed-sternberg (HRS) cells - specific histology
127
Hodgkins lymphoma - affects? (3) 85% 5 year survival
LN sclerosis Reduction in number of lymphocytes Mixed cellular presentation
128
Chronic Lymphocytic leukamia - what is it? | Presents with?
Disorder of mature B cells Lymphadenopathy and splenomegaly Lymphocytosis in blood tests Bone marrow failure --> systemic symptoms
129
Grading system and Complications (5) of Chronic Lymphocytic leukamia?
Grading = Binet's System ``` Complications = Progression to higher grade leukemias Recurrent infections Haemolytic aneamia Neutropenia Thrombocytopenia ```
130
Haemoglobin switches at birth? Chromosomes 16 and 18 involved
Feotus = HB-F At birth = HB-F (50%): HB-A (50%) Neonatial = HB-A Adult = HB-A2
131
Why HB-F haemoglobin in utrio?
Higher affinity for Oxygen so can compete with mothers HB-A2
132
Difference in RBC structure in children VS adults?
``` Larger RBC higher Haemocrit (% RBC/ total cells) ```
133
Lymphocytes in the feotus - Passive immunity - Own immune system?
IgG - crosses the placenta IgA, D, E, G, M - in breast milk Own antibodies not made til 2 - 3 months Satisfactory immune response at 6 months
134
Platelets in the fetus - when do they reach adult level | any differences VS adults?
18 weeks gestation | Less responsive to some agents BUT more responsive to vWF
135
Coagulation factors in feotus? | Vit K in the feotus?
Do not cross placenta - prevents clotting in the embillicus Reach adult levels by 6 months Vit K = only 10% of mothers --> require supplements
136
Causes of congential anemia (7) + examples
Haemoglobin sysnthesis issues eg. thalassaemia and sickle cell Bone marrow failure Peripheral destruction - auto antibodies (RHs incompatibility) Membrane defects eg. Hereditary spherocytosis Enzyme defects eg. G6DP Twin - twin transfusion (shared placenta) = blood loss in one Fetomaternal haemorrhage on deliver = blood loss
137
Causes of Acquired anemia in neonates? (4)
iron B12 and folate deficient (malnutrosed) Bone marrow failure Haemolytic anemia Blood loss + bleeding disorders (rare)
138
Changes in RBC during pregnancy? (3) | at 32 weeks gestation
Plasma volume and MCV increases by 50% Haemodilation (red cell mass) increase by 25% = macrocytic Anaemia is common
139
Leucrocytic changes in pregnacy? | 2nd trimester
Neutrophillia (increase)
140
Gestational platelet count?
Raises till 20 weeks Falls again (thrombocytopenia) - should always check this is not for a pathological reason Recovers rapidly after birth
141
Coagulation in pregnancy?
Prothrombotic or hypercoagulative state Decreases in Anticoagulants and fibrolysis Increase in coagulation factors
142
Testing for blood disorders in pregnancy? (3)
Genetic screening - Paternal testing Antenatal screening - amnio and villus for those high risk patients Post natal - heal prick test for Haematopathologies (SC and thalaseamia)
143
Testing for haemoglobinopathologies (3)
Hb electrophoresis Heat instability + O2 dissociation curve DNA analysis
144
Sickle Cell - what is it/ what goes?
Single point mutation in beta globin = HBS. | Polymerises in low O2 = tactoids --> distort the shape of cell
145
Affect of Sickle cell
HbS = charged --> seen on electrophoresis Reduced life span of cells = haemolytic aneamia Reduced O2 delivery Haemostatic activation = microvascular thrombosis and inflammatory style disorders
146
Compliactions of sickle cell
Vasculooclusion - Dactylitis, chest and ab pain Septiceamia Aplastic crisis - sudden stop in production of RBC = aneamia Hyposplenism Renal disease Avascular necrosis (femoral head) Leg ulcers, gall stones, rentinopathy
147
Treatment/ management (3)
Hydroxycarbomide - revert some HbS --> HbF prevents some aggregations Transfusions, hydration and analgesics - for acute crisis Penicillin prenatally for 6 months
148
Thalasaemia - what is it?
Genetic mutation - heterogeneous | Reduced synthesis of certain Hb chains = imbalance of chains --> pathology
149
Types of Thalaseamia Based on what chain is not produced - And severity
Alpha Beta - Minor = Carriers looks like iron deficient - small pale RBC - Intermediate = clinical symptoms BUT variable genotype - Major = life threatening
150
Affects of Thalaseamia B
Excessive alpha chains - ineffective erythropoiesis + reduced RBC life span --> aneamia Increases bone marrow activity - skeletal deformities, increased iron absorption, organ damage Enlarged/ overactive spleen - increased transfusion requirements
151
Other symptoms of thalaseamia B Intermedia (5) Major (presentation)
intermediate = Anaemia, pulmonary hypotension, bone changes, leg ulcers, hypothyroidism Major = presents as aneamia 1st/ 2nd year of life
152
Treatment of Thalasemia (5)
``` Transfusion Iron chelation (Desferrioxamine) Hormone replacements Monitoring Genetic counselling and screening ```
153
Arterial VS Venous thrombotic event
Arteriole = plaque rupture --> endothelial injury and platelet aggregation Venous = Virchows triangle (hypercoagubility, statis and endothelial injury) --> Coagulation response
154
Arterial VS Venous thrombotic conditions?
Arteriole = MI or ischemic stroke - Treat with anti platelets Venous = VTE including DVT and PE. Treat with anti-coagulants
155
Pro -coagulant produces (2) anticoagulant reagents (5)
Pro = Clotting factors and platelets Anti = Protein C Protein S Fibrinogenolytic system Anti-thrombin
156
VTE - PE and DVT - percentage of each condition that also has a silent version of the other?
VTE = venous thromboembolism 50% DVT have an asymptomatic PE 80% of PE have a silent DVT
157
Characteristics of VTE
Recurrent Post thrombotic syndrome - damage valves and swelling Mainly hospital acquired - due to hypercoagulative state
158
Risk factors for VTE (11) Past medical Current conditions Drugs
``` Active/ past cancer Family history Major trauma/ surgery recent pregnancy Dehydration Obesity Varicose veins OCP - oestrogen Periods of immobility Hormone replacement therapy Thrombophilias ```
159
Prophylaxis prevention of VTE (all patients assessed for clotting risk when admitted to hospital) - Non-pharmacological - Pharmacological
Non pharma = TED stockings + encourage early mobility after surgery Pharma = LMWH - low molecular weight heprin Eg. Fondaparinux Rivaroxaban and Apixaban - inhibitor factor Xa Dabigatran - inhibit thrombin
160
3 Step diagnosis
1) Wells score = at high risk? Risk factors, signs and symptoms 2) D-dimer = if a fibrin clot has been formed 3) Ultrasound DVT = duplex scan + compression PE = chest CT + dye to color vessels
161
Treatment of VTE (3)
Start on stronger dose LMWH for 5 days eg. Trinzaparin Overlap with Warfarin - monitor Prothrombin time Continue Warfarin for 3 - 6 months till IMR 2.5
162
Thrombophillia - what is it? | Signs: get a VTE when ... (4)?
Famillia or acquired haemostatic mechansim --> predisposes someone to a thrombotic event ``` Patient will get a VTE Spontaneously At a young age Of disproportionate severity Recurrent ```
163
3 hereditary Thrombophillias
Activate C reactive protein OR FV Ledien - polymorphims reacts with envrio factors --> Protein C isn't broken down Prothrombin - point mutation = deficient in antithrombin Dysfibrinogenaemia - misfolded fibrinogen
164
Autoimmune Thrombophillia? Seen in which conditions? Affects
Antiphospholipid syndrome (APS) or Hugh's syndrome- antibody against negatively charged phosolipids Lupus, Vasculitis, Syphilis, connective tissue disorders Venous thrombotic events and miscarriages
165
Pernicious aneamia - What causes - What it results in
Autoimmune disease that destroys gastric parietal cells and intrinsic factor RESULTS IN no uptake of B12 --> megablastic aneamia
166
Reasons for Iron deficiency ?
Bleeding - GI, malignant, menstruation Increased demand - pregnancy and puberty Decreases intake/ absorption - Coeliac disease
167
Test for iron deficiency? | Treatment for iron deficiency (3)?
Blood film for ferritin levels (GOLD STANDARD) Low Ferritin = low iron % hypochromic cells Treat = oral iron (GI issues), IV iron, IM iron (painful)
168
Haemolysis? what is it?
Shortened red cell life span
169
Cause of haemolysis - in side cell - in cell membrane - outside cell
Inside cell = haemoglobinpathology (sickle cell) or enzyme defect (G6PD) Cell mem = Spherycytosis (hereditary) Outside = Antibodies (autoimmune), drugs, toxins, heart valves
170
Thrombocytopenias ?? Disorders that cause them = ITP and TTP
Low platelet count ``` ITP = immune thrombocytopenia Purpura TTP = Thrombotic Thrombocytopenia Purpura ```
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ITP - immune thrombocytopenia | - what is it associated with?
Autoimmune disorder against the bodies platelets Associated with autoimmune diseases, lymphoma and HIV NOTE: Chronic = seen in adults Acute = seen in children (following a viral infection)
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Presentation of ITP | Treatment of ITP (4)
Bruising and petechiae Steriods, immunosupressive drugs, splenectomy, Thrombomimic drugs
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TTP - thrombotic thrombocytopenia - what is it?
Autoantibody immune inhibition of ADAMTS 13 due to gene mutation = vWF deficiency
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TTP - signs and symptoms (5)
``` Fever neurological symptoms Haemolysis Microvascular occlusions Kidney problems ```
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TTP - treatments (3)
Plasma exchange FFP Steriods Monitor ADAMTS-13 for relapse and control
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ACUTE myeloid leukaemia - what is it?
Mutation in a clone of the myeloid progenitor cells in the bone marrow --> Blast (immature) cells in the blood (holes in their nucleus)
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Signs and symptoms of acute myeloid leukeamia
Aneamia Recurrent overwhelming infections - gram negative Easy bruising Gum hypertrophy and gingivitis
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Classification of Myeloid leukamias by? | Subtypes?
Morphology | Subtypes = M1 M3 M5 and M6
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Treatment of Acute Myeloid leukamia
Induction treatment - using chemo to send disease into remission Bone marrow transplant - in the young
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Acute Lymphoblastic leukemia - what is it?
mutation in the lymphoid progenitor cells in the bone marrow | --> lymphoblastic cells in the blood (large cytoplasms)
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Presentation of acute lymphoblastic leukemia?
Aneamia easy bleeding/ bruising Organ infiltration of organs - eg. spleen, liver, meninges, skin Severe Infections - oral candida
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Management of Acute lymphoblastic leukamia? | Specific for meningeal effects?
Induction therapy - chemo Bone marrow transplant - in poor prognosis patients For Meningeal effects = intrathecal methotrexate and cranial irradiation
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Diagnosis and monitoring of AML and ALL (4) | Genetic analysis techniques
Morphology - from blood films Cytochemistry - staining proteins to determine linage Immunological markers - Fluorescence activating short cell (FASC) Cytogenetic - FISH: abnormalities that correlate to prognosis + response to treatment Molecular techniques (future) - PCR to track the changes in genes post treatment (check effectiveness)
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Complications of AML and ALL treatments ? How managed? (2)
Post chemo neutropenia --> infection risk = Neutropenic Sepsis (very low neutrophils) Broad spectrum antiB - Tazocin and Gentamicin Prophylaxis Anti B (levofloxacin) and isolation
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C1 protein deficiency (compliment system)?
Hereditary angioedema
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Gene that is mutation in hereditary angioedema?
SERPING1 gene - auto dom
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Sings and symptoms of hereditrary angioedema? | Attacks triggered by ? (3)
Episodic attacks of oedema in the face, genitals, extremities, GI tract and airways Swelling in intestinal mucous membranes = coelic-like pain and vomitting Triggered by trauma, viral infections, surgery

Year 2: Clinical Pathology (10 decks)

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