Haematology Flashcards

1
Q

Define anaemia

A

A low level of haemoglobin in the blood.

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2
Q

Examples of microcytic anaemia

A

TAILS
Thalassaemia
Anaemia of chronic disease
Iron deficiency anaemia
Lead poisoning
Sideroblastic anaemia

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3
Q

Examples of normocytic anaemia

A

3 As and 2 Hs
Acute blood loss
Anaemia of chronic disease
Aplastic anaemia
Haemolytic anaemia
Hypothyroidism

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4
Q

Examples of macrocytic anaemia

A

Megaloblastic - B12, folate deficiency
Normoblastic - alcohol, reticulocytosis, hypothyroidism, liver disease, azathioprine.

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5
Q

History of anaemia

A

Tiredness
Shortness of breath
Headaches
Dizziness
Palpitations
Worsening of other conditions

Iron deficient
Pica - crave abnormal things like dirt
Hair loss

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6
Q

Clinical signs of anaemia

A

Pallor
Tachycardia
Raised respiratory rate
Koilonychia - iron
Angular cheilitis - iron
Atrophic glossitis - iron
Brittle hair - iron
Jaundice - haemolytic
Bone deformities - thalassaemia
Oedema HTN and excoriations - CKD

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7
Q

Investigations in anaemai

A

Bloods - MCV, B12, folate, ferritin, film
OGD + colonoscopy - cancer?
Bone marrow biopsy if cause unclear

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8
Q

Causes of iron deficiency anaemia

A

Insufficient dietary iron
Increased requirements - pregnancy
Iron being lost - slow bleed, colon cancer
Inadequate iron absorption
Most common cause - menorrhagia

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9
Q

Management of iron deficiency anaemia

A

Investigate cause - ?cancer
Blood transfusion
Iron infusion
Oral iron supplement

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10
Q

Define pernicious anaemia

A

An autoimmune condition attacking the parietal cells or intrinsic factor. This prevents the absorption of vitamin B12 and they become deficient.

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11
Q

History of B12 deficiency

A

Peripheral neuropathy
Loss of vibration sense or proprioception
Visual changes
Mood or cognitive change

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12
Q

Management of pernicious anaemia

A

If dietary deficient - cyanocobalamin
IM hydroxocobalamin - B12 replacement
Treat B12 before folate -spinal cord degeneration

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13
Q

Define haemolytic anaemia

A

Anaemia caused by the destruction of red blood cells. Caused often by inherited condition .

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14
Q

Inherited haemolytic anaemias

A

Hereditary spherocytosis
Hereditary elliptocytosis
Thalassaemia
Sickle cell anaemia
G6PD dificiencty

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15
Q

Acquired haemolytic anaemia

A

Auto immune
Alloimmune - transfusion reactions, haemolytic disease of newborn
Paroxysmal nocturnal haemoglobinuria
Microangiopathic haemolytic anaemia
Prosthetic valve related

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16
Q

Clinical signs of haemolytic anaemia

A

Splenomegaly
Jaundice
Normocytic anaemia

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17
Q

Investigations in haemolytic anaemia

A

FBC - normocytic anaemia
Film - schistocytes
Direct coombs test - positive in autoimmune haemolytic anaemia

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18
Q

Define hereditary spherocytosis

A

Most common inherited haemolytic anaemia - causing sphere shaped red blood cells that are fragile and easily broken down when passing through the spleen

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19
Q

History of hereditary spherocytosis

A

Jaundice
Gall stones
Splenomegaly
Aplastic crisis - parvovirus

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20
Q

Management of hereditary spherocytosis

A

Folate supplementation
Splenectomy
Cholecystectomy - stones

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21
Q

Define G6PD deficiency

A

Defect in the red blood cell enzyme G6PD
X linked recessive condition causing crises that are triggered by infection medications or fava beans.

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22
Q

Classic presentation of G6PD deficieny

A

Mediterranean or African patient turns jaundiced and anaemic after eating broad beans, an infection or treatment with antimalarials.

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23
Q

What is seen on blood fil in G6PD deficiency

A

Heinz bodies - individual blobs seen inside rbcs - denatured globin.

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24
Q

Define target cells

A

Central pigmented area surrounded by a pale arrea, surrounded by a ring of thicker cytoplasm on the outside - bull’ eye

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25
What conditions would you see target cells in
Iron deficiency anaemia Post splenectomy
26
Define Howell-Jolly bodies
Individual blobs of DNA material seen inside RBCs,
27
What conditions would you see Howell-Jolly bodies in
Post splenectomy Severe anaemia
28
Define reticulocytes
Immature RBCs - still have RNA in, appears in a mesh like (reticular) pattern
29
What conditions would you see reticulocytes in
Normal to have 1% or RBCs as reticulocytes Goes up if increased turnover Haemolytic anaemia
30
Define schistocytes
Fragments of red blood cells - indicate physical damage by trauma
31
What conditions would you see schistocytes in
Networks of small clots Haemolytic uremic syndromes DIC TTP Metallic heart valves Haemolytic anaemia
32
Define sideroblasts
Immature RBCs that contain blobs of iron
33
What conditions would you see sideroblasts in
Myelodysplasic syndrome
34
Define smudge cells
Ruptured white blood cells occur during the process of preparing the blood film due to aged or fragile cells
35
What conditions would you see smudge cells in
Chronic lymphotic leukaemia
36
Define spherocytes
Spherical red blood cells
37
What conditions would you see spherocytes in
Autoimmune haemolytic anaemia Hereditary spherocytosis
38
Define thalassaemia
A autosomal recessive genetic defect that changes the protein chains that make up haemoglobin.
38
Define thalassaemia
A autosomal recessive genetic defect that changes the protein chains that make up haemoglobin.
38
Define thalassaemia
A autosomal recessive genetic defect that changes the protein chains that make up haemoglobin.
39
Clinical signs of thalasaemia
Microcytic anaemia Fatigue Pallor Jaundice Gallstones Splenomegaly Poor growth and development Pronounced forehead and malar eminences
40
Investigations in thalassaemia
FBC Haemoglobin electrophoresis DNA testing Screening in pregnancy
41
Features of iron overload
Fatigue Liver cirrhosis Infertility Heart failure Arthritis Diabetes Osteoporosis and joint pain
42
Define alpha-thalassaemia
Thalassaemia caused by defects in the alpha globin chain - issues with chromosome 16
43
Management of alpha thalassaemia
Monitoring FBC Monitoring complication Blood transfusions Splenectomy Bone marrow transplant - can be currative
44
Define beta thalassaemia
Defects in the beta globin chains - defect on chromosome 11
45
Define beta thalassaemia minor
Carriers of abnormal gene - one abnormal and one normal. Causes mild microcytic anaemia - often no active treatment
46
Define beta thalassaemia intermedia
Two abnormal copies of the beta globin gene - either two defective or one defective and one deletion
47
Define beta thalassaemia major
Patients who are homozygous for the deletion genes - no functioning beta-globin gene Severe microcytic anaemia, splenomegaly, bone deformities
48
Define sickle Cell anaemia
A autosomal recessive condition causing production of sickle shaped RBCs. Defect on chromosome 11 on the beta globin gene. Makes RBCs more fragile leading to haemolytic anaemia.
49
What affect does sickle cell disease have on malaria
Having one copy of the sickle-cell trait reduces the severity of malaria
50
Investigations in sickle cell anaemia
Newborn screening heel prick Film
51
Complications of sickle cell anaemia
Anaemia Increased risk of infection Stroke Avascular necrosis Pulmonary hypertension Priapism CKD Sickle cell crises Acute chest syndrome
52
Management of sickle cell anaemia
Avoid dehydration Ensure vaccines up to date Antibiotic prophylaxis Hydroxycarbamide - stimulate HbF production Blood transfusion - in severe anaemia Bone marrow transplant - can be curative
53
Define sickle cell crises
Ubrella term for a spectrum of acute crises related to the condition Range from mild to life threatening, can be spontaneous or triggered by infection, dehydration, cold or significant events
54
Examples of sickle cell cises
Vaso-occlusive crisis - painful crisis Splenic sequestration crisis Aplastic crisis Acute chest syndrome
55
Define vaso-occlusive crisis
Caused by sickle shaped blood cells clogging capillaries causing distal ischaemia - associated with dehydration and raised haemocrit.
56
Presentation of vaso-occlusive crisis
Pain Fever Triggering infection Priapism - urological emergency
57
Define splenic sequestration crisis
Caused by red blood cells blocking blood flow within the spleen - acutely enlarged and painful spleen Leads to pooling of blood in the spleen, severe anaemia and circulatory collapse
58
Management of splenic sequestration
Supportive Blood transfusion Fluid resuscitation Splenectomy
59
Define aplastic crisis
Describes a situation where there is temporary loss of the creation of new blood cells Causes significant anaemia requiring blood transfusion - is often transient
60
Cause of aplastic crisis
Parvovirus B19
61
Define acute chest syndrome
Fever or respiratory symptoms with new infiltrates seen on CXR
62
Management of acute chest syndrome
Abx or antivirals Blood transfusion Incentive spirometry - encourages deep breathing Artificial ventilation - NIV
63
Define leukaemia
Cancer of a particular line of the stem cells in bone marrow causing unregulated production of certain blood cells.
64
Main types of leukaemia
Acute myeloid leukaemia Acute lymphoblastic leukaemia Chronic myeloid leukaemia Chronic lymphocytic leukaemia
65
What ages do the leukaemia occur
ALL CeLLmates have CoMmon AMbition Under 5 and >45 - ALL >55 - CLL >65 - CML >75 - AML
66
History of leukaemia
Fatigue Fever Failure to thrive Pallor Petechiae and abdominal bruising due to thrombocytopaenia Abnormal bleeding Lymphadenopathy Hepatosplenomegaly
67
Differentials for petechiae
Leukaemia Meningococcal septicaemia Vasculitis Henoch-Schonlein purpura Idiopathic thrombocytopenia purpura NAI
68
Investigations in leukaemia
FBC!! Blood film Lactate dehydrogenase - raised but not specific Bone marrow biopsy CXR Lymph node biopsy Lumbar puncture - CNS involvement CT, MRI, PET
69
Define acute lymphoblastic leukaemia
Malignant change to one of the lymphocyte precursor cells - causes acute prolifferation of a single type of lymphocyte (usually B-lymphocytes)
70
What is seen on blood film in ALL
Blast cells
71
What genetics are associated with ALL
Philadelphia chromosome
72
Define Chronic lymphocytic leukaemia
Chronic proliferation of a single type of well differentiated lymphocyte (usually B). Occurs in older ages. Easily transforms into high grade lymphoma - Richter's transformation
73
What is seen on blood film in CLL
Smear or smudge cells
74
Define chronic myeloid leukaemia
Malignant production of myoblasts
75
What are the phases of CML
Chronic Accelerated Blast
76
What is the chronic phase of CML
Lasts around 5 years and is often asymptomatic and is diagnosed incidentally
77
What is the accelerated phase of CML
Occurs where the abnormal blast cells take up high proportion of cells in the bone marrow and blood - patients become more symptomatic, anaemia and thrombocytopaenia and immunocompromised
78
What is the blast phase of CML
Follow acceleratsed phase and involves even high proportions of blast cells - severe symptoms and is often fatal
79
What genetics are associated with CML
Philadelphia chromosome
80
Define AML
Most common type of leukaemia with a few differnt types.
81
What is seen on AML blood film
High proportion of blast cells These blasts have rods in the Auer rods
82
Management of leukaemia
Chemo Steroids Radio Bone marrow transplant Surgery
83
Complications of leukaemia
Failure Stunted growth Infections Neurotoxicity Infertility Secondary malignancy Cardiotoxicity Tumour lysis syndrome
84
Define tumour lysis syndrome
Caused by the release of uric acid from cells that are being destroyed by chemotherapy
85
Management of tumour lysis syndrome
Allopurinol Rasburicase Reduce uric acid levels Monitor potassium and phosphate
86
Define lymphoma
A group of cancers that affect the lymphocytes inside the lymphatic system. These cancerous cells proliferate within the lymph nodes and cause them to become abnormally large
87
Main types of lymphoma
Hodgkin's Non-Hodgkin's
88
Define Hodgkin's lymphoma
Lymphoma with the presence of Reed-Sternberg cells on biopsy, 1 in 5 are Hodgkin's. Peak ages are 20 and 75
89
Risk factors for Hodgkin's lymphoma
HIV Epstein-Barr virus Autoimmune conditions - rheumatoid, sarcoid FH
90
History of Hdogkin's lymphoma
Enlarged non tender rubbery lymph node - often neck, axilla or inguinal. Fever Weight loss Night sweats Fatigue Itching Cough Shortness of breath Abdo pain Recurrent infections
91
Investigations in Hodgkin's lymphoma
Lactate dehydrogenase - not specific Lymph biopsy - reed sternberg CT, MRI, PET
92
What is seen on biopsy of Hodgkin's lymphoma
Reed-Sternberg cell
93
What staging system is used for lymphoma
Ann Arbor staging - Stage 1 - confined to one region of lymph nodes Stage 2 - mor than one region but same side of diaphragm Stage 3 - lymph nodes above and below diaphragm Stage 4 - widespread involvement including non-lymphatic organs
94
Management of Hodgkin's lymphoma
Chemo - risk of leukaemia and infertility Radio - risk of other cancers, damage to tissue, hypothyroidism
95
Define non-hodgkin's lymphoma
Any lymphoma with out the presence of Reed-Sternberg cells
96
Examples of non-hodgkin's lymphoma
Burkitt lymphoma MALT lymphoma Diffuce large B cell lymphoma
97
Risk factors for non-hodgkin's lymphoma
HIV EBV H. pylori - MALT Hep B or C Pesticides - trichloroethylene FH
98
Management of non-hodgkin's lymphoma
Watchful waiting Chemo Monoclonal antibodies Radiotherapy Stem cell transplant
99
Define myeloma
Cancer of the plasma cells - b lymphocytes that produce antibodies
100
Define multiple myeloma
Where myelomas affect multiple areas of the body.
101
Define myeloma bone disease
Results from increased osteoclast activity and suppressed osteoblast resulting in more bone being reabsorbed
102
Complications of myeloma
Pathological fractures Hypercalcaemia Myeloma renal disease Infection Pain Renal failure Anaemia Hypercalcaemia Peripheral neuropathy Spinal cord compression Hyperviscocity
103
Define myeloma renal disease
Development of renal impairment as a result of myeloma by causing - High levels of Ig block the tubules Hypercalcaemia Dehydration Medication
104
Features of myeloma
CRAB Calcium - elevated Renal failure Anamia - normocytic, replacement of bone marrow Bone lesions/pain
105
Risk factors for myeloma
Age Male Black african FH Obesity
106
Investigations in myeloma
FBC, Calcium, ESR, plasma viscosity, serum protein electrophoresis BLIP Bence-Jones protein - urine electrophoresis Light chain assay Immunoglobulins Protein electrophoresis Bone marrow biopsy XR, MRI, CT
107
Signs of myeloma on xray
Punched out lesions Lytic lesions Raindrop skull
108
Management of myeloma
Chemo Stem cell transplant VTE prophylaxis Bisphosphonates Radio - bones Orthopaedic surgery Cement augmentation Manage complications of myeloma
109
What genetics are myeloproliferative disorders associated with
JAK2 MPL CALR
110
Define meyloproliferative disorders
Uncontrolled proliferation of a single type of stem cell - have potential to progress to AML
111
Examples of myeloproliferative disorders
Primary myelofibrosis - haemoatopoietic stam cells Polycythaemia Vera - reythroid cells Essential thrombocythaemia - megakaryocytes
112
Define myelodysplastic syndrome
Caused by bone marrow cells not maturing properly and not producing healthy blood cells/
113
Define thrombocytopenia
Low platelet count
114
Causes of poor platelet production
Sepsis B12 or folate deficiency Liver failure Leukaemia Myelodysplastic syndrome
115
Causes of platelet destruction
Medication - sodium valproate, methotrexate, isotretinoin, antihistamines, PPIs, Alcohol Immune thrombocytopenic purpura TTP Heparin induced thrombocytopenia Haemolytic-uraemic syndrome
116
History of thrombocytopenia
Incidental Spontaneous bruising Prolonged bleeding - nosebleeds, gums, heavy periods, blood in urine/stool
117
Define immune thrombocytopenic purpura
Also called autoimmune, idiopathic and primary Condition where antibodies are created against platelets
118
Management of immune thrombocytopenic purpura
Prednisolone IV Ig Rituximab Splenectomy
119
Define thrombotic thrombocytopenic purpura
Conition where tiny blood clots develop throughout small vessels using up platelets causing thrombocytopenia, bleeding under the skin and other systemic issues. affectrs small vessesl so is a microangiopathy.
120
Management of TTP
Plasma exchange Steroids Rituximab
121
Define von Willebrand disease
Deficiency, absence or malfunctioning of von Willebrand factor often caused by an autosomal dominant gene
122
History of von Willebrand disease
Bleeding! Bleeding gums Nose bleeds Heavy menstrual During surgery or trauma FH!
123
Management of von Willebrand disease
Managed only in response to major bleeding or trauma, or in preperation for surgery Desmopressin - stimulates release of VWF VWF infusion Factor VII Heavy periods Tranexamic acid Mefanamic acid Norethisterone COCP Mirena Hysterectomy
124
Define haemophillia
X linke recessive severe bleeding disorders.
125
Which factor is affected in each haemophilia A and B
A - Factor VIII B - Factor IX
126
History of haemophilia
Bleeding! Intracranial, haematoma, cord - neonates Joints and muscles
127
Management of haemophilia
Replace specific clotting factor Acute Infusion Desmopressin Antifibrinolytics - tranexamic acid
128
Define venous thromboembolism
Common condition where clots develop in the circulation and become dislodged and occlude small blood vessels
129
Risk factors for DVT or embolism
Immobility Recent surgery Long haul travel Pregnancy Oestrogen! - HRT, contraceptive Malignancy Polycythaemia Systemic lupus erythematosus Thrombophilia
130
Examples of thrombophilias
Antiphospholipid syndrome Factor V Leiden Antithrombin deficiency Hyperhomocysteineaemia Prothrombin gene variant Activated protein C resistance
131
DVT presentation
Unilateral painful swollen leg Calf swelling - >3cm Dilated superficial veins Tenderness Oedema Redness
132
What score is used to assess risk of DVT or PE
Wells score
133
Investigations in PE
CTPA
134
Management of DVT
Anticoagulation
135
Define Budd-Chiari syndrome
Blood clot developlable statess in the hepatic vein blocking outflow of blood. Associated with hyper-coagu
136
Triad in Budd-Chiari syndrome
Abdo pain Hepatomegaly Ascites