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Haematology Flashcards

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1
Q

What does Hb have to be below in anaemia?

A

<135 g/L for men and <115 g/L for women

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2
Q

Name 3 causes of microcytic anaemia

A

Iron deficiency, thalassaemia and sideroblastic anaemia

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3
Q

Name 4 causes of normocytic anaemia

A

Acute blood loss, anaemia of chronic disease, bone marrow failure, renal failure

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4
Q

Name 4 causes of macrocytic anaemia

A

B12/folate deficiency, alcohol excess/liver disease, haemolytic, hypothyroidism

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5
Q

Name 4 causes of iron deficiency anaemia

A

GI bleeding/chronic blood loss, menorrhagia, poor diet, malabsorption

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6
Q

Give 3 investigations for iron deficiency anaemia

A

FBC (low hb, mcv)
Ferritin (low)
Blood film

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7
Q

How do you treat iron deficiency anaemia?

A

Oral iron - ferrous sulphate or IV iron infusion

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8
Q

Give 3 side effects of ferrous sulfate

A

Nausea, abdominal discomfort, constipation

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9
Q

Give 3 causes of anaemia of chronic disease

A

Chronic infection, malignancy and renal failure

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10
Q

Give 4 side effects of erythopoietin treatment

A

Flu like symptoms, hypertension, high platelets and VTE

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11
Q

What is seen on the blood film in B12/folate deficiency?

A

Hypersegmented neutrophils

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12
Q

Give 3 clinical features of B12 deficiency

A

Irritability, peripheral neuropathy, depression

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13
Q

How do you treat B12/folate deficiency?

A 
IM hydroxycobalamin (B12)
Folic acid
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14
Q

Give 5 causes of haemolytic anaemia

A 
Autoimmune (would see spherocytosis)
Sickle cell anaemia
Hereditary spherocytosis
G6PDH deficiency (Heinz bodies)
Drug induced
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15
Q

Give 5 causes of neutrophilia

A 
Bacterial infection
Inflammation eg MI
Drugs (steroids)
Disseminated malignancy
Trauma/surgery/haemorrhage
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16
Q

Give 5 causes of neutropenia

A 
Post-chemotherapy
Severe sepsis
Hypersplenism eg Felty's syndrome
Bone marrow failure (leukaemia etc)
Viral infections
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17
Q

Give 4 causes of raised lymphocytes

A

Viral infections
Chronic infections eg TB and hepatitis
Leukaemias (CLL)
Lymphomas

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18
Q

Give 5 causes of decreased lymphocytes

A 
Steroid therapy
HIV infection
Post chemotherapy
Post radiotherapy
Marrow infiltration
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19
Q

Give 5 causes of raised eosinophils

A 
Allergy
Parasitic infection
Drug reactions eg erythema multiform
Eczema, psoriasis
Eosinophilic leukaemia
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20
Q

Give 7 causes of pancytopenia

A 
Aplastic anaemia
Acute leukaemias
Myelodysplasia
Myeloma
Myelofibrosis
Lymphoma (marrow infiltration)
Hypersplenism - haemolytic anaemias, cirrhosis
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21
Q

Give 2 drugs that can cause agranulocytosis

A

Clozapine and carbimazole

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22
Q

Give 6 causes of thrombocytopenia due to decreased production of platelets

A 
Leukaemias
Lymphoma (marrow infiltrations)
Chemotherapy
Alcohol
Myelofibrosis
Viral infections
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23
Q

Give 6 causes of thrombocytopenia due to decreased platelet survival

A 
ITP
TTP
DIC
Post transfusion
Heparin induced
HELLP syndrome
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24
Q

What can be used to treat ITP?

A 
Prednisolone
IV IG
Rituximab
Splenectomy
TPO receptor agonists
Thrombopoietin analogues
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25
Give 5 symptoms of hyperviscosity syndrome
``` Lethargy Confusion Reduced cognition Abdominal pain Visual disturbance ```
26
Give 4 causes of hyperviscosity syndrome
Polycythaemia vera Leucocytosis Myeloma Waldenstroms macroglobulinaemia
27
What is the treatment of hyperviscosity syndrome?
Underlying cause Polycythaemia - venesection Leukapheresis in leukaemia (raised WCC) Plasmapheresis in myeloma and waldenstroms
28
Give 6 causes of splenomegaly
``` CML Myelofibrosis Malaria Portal hypertension Lymphoma Haemolytic anaemia ```
29
What does the blood film show after splenectomy?
Howell-Jolly bodies, pannenheimer bodies, target cells
30
What organisms are dangerous for people who have had their spleen out?
Strep pneumonia, haemophilia influenza and neisseria meningitidis (encapsulated)
31
What measures are taken to reduce risks for people post splenectomy?
Immunisations (pneumococcal, flu and meningococcal) Lifelong prophylactic antibiotics - penicillin V or erythromycin Pendants, bracelets or cards for awareness Caution travelling abroad
32
Why shouldn't long term steroids be stopped suddenly?
Risk of addisonian crisis due to adrenal insufficiency
33
What conditions could be made worse by steroids?
Diabetes, osteoporosis, hypertension, TB, chickenpox
34
What are the main side effects of steroids?
Weight gain, myopathy, osteoporosis, cushings, depression, psychosis, increased susceptibility to infections
35
What should you prescribe alongside steroids?
Calcium and vitamin D supplements | Bisphosphonates
36
Which patients require CMV negative blood products?
Pregnant women and neonates up to 28 days (CMV can cause sensorineural deafness and cerebral palsy)
37
Which patients require irradiated blood products?
Hodgkin's lymphoma Stem cell transplant recipients Some chemo patients
38
When are packed red cells indicated?
Acute blood loss, chronic anaemia where Hb < 70 or symptomatic anaemia
39
How long are packed red cells administered over?
2-4 hours
40
When is a platelet transfusion indicated?
Haemorrhagic shock, profound thrombocytopenia <20 or preoperatively where platelets <50
41
How long are platelets administered over?
30 mins
42
What does fresh frozen plasma consist of?
Clotting factors
43
When is fresh frozen plasma indicated?
DIC, haemorrhage secondary to liver disease, all massive haemorrhages
44
How long is fresh frozen plasma administered over?
30 mins
45
What does cryoprecipitate consist of?
Fibrinogen, von willebrands factor, factor VIII and fibronectin
46
When is cryoprecipitate indicated?
DIC where fibrinogen is low, von willebrands disease or massive haemorrhage
47
How long is cryoprecipitate administered over?
Stat
48
What are the main complications of transfusions?
``` Acute haemolytic reaction Anaphylaxis Transfusion Associated Circulatory Overload Transfusion Related Acute Lung Injury Bacterial contamination ```
49
What are the clinical features of acute haemolytic reaction from transfusions?
``` Fever Hypotension Urticaria Abdomen/chest pain Agitation ```
50
What will be seen in investigations for acute haemolytic reaction from transfusions?
FBC - low Hb High LDH High bilirubin Positive direct antiglobulin test
51
What is the management of acute haemolytic reaction from a transfusion?
STOP THE TRANSFUSION Tell haematologist/blood bank Fluid resuscitation Give O2
52
What are the clinical features of transfusion associated circulatory overload (TACO)?
Dyspnoea Raised JVP Tachycardia Basal crackles
53
What is the management of transfusion associated circulatory overload (TACO)?
STOP THE TRANSFUSION Chest X Ray Oxygen IV Furosemide 40mg
54
What are the clinical features of transfusion related acute lung injury (TRALI)?
``` Dyspnoea Cough Hypoxia Frothy sputum Fever ```
55
What is seen on the chest x ray of TRALI?
White out
56
What is the management of transfusion related acute lung injury (TRALI)?
STOP THE TRANSFUSION 100% oxygen Treat as ARDS Remove donor from the donor panel
57
What is the pathophysiology of TRALI?
Antileucocyte antibodies in donor plasma causes ARDS
58
What are 3 delayed complications of transfusions?
Infection (eg hepatitis, HIV) Graft vs Host disease (due to HLA mismatch) Iron overload (especially in repeated transfusions)
59
Which is the most common childhood cancer?
ALL
60
What is the peak age of onset of ALL?
2-4 years of age
61
What is ALL associated with?
Down's syndrome, Philadelphia chromosome
62
How does ALL present?
Pancytopenia - anaemia, infection, bleeding Bone pain Lymphadenopathy Hepatosplenomegaly
63
What is seen on the blood film in ALL?
Blast cells
64
What tests need to be done on the bone marrow biopsy?
Flow cytometry/immunophenotyping - differentiates between myeloid and lymphoid Cytogenetics - to look for translocations etc
65
What is the most common translocation in childhood ALL?
t(12;21) - TEL-AML fusion gene
66
What chemotherapy induces remission in ALL?
Vincristine, prednisolone, L-asparaginase and daunorubicin
67
What is imatinib?
Tyrosine kinase inhibitor
68
What is the median age of onset for AML?
67 years
69
What is AML associated with?
- Myelodisplastic syndromes - Progression from CML - Complication of chemotherapy eg for lymphoma - Down's syndrome
70
How does AML present?
Fatigue, fever, anaemia, bleeding, gum hypertrophy, hepatosplenomegaly, leukostasis
71
How can Leukostasis present?
Respiratory distress and altered mental status
72
What investigations are required in AML?
FBC - low Hb, low platelets LDH - raised Bone marrow biopsy + flow cytometry and cytogenetics
73
What does the bone marrow biopsy show in AML?
Presence of Auer rods found in AML myeloblast cells
74
What is the chemotherapy for AML?
Daunorubicin and cytarabine
75
What is the median age at diagnosis for CML?
60-65 years
76
Which leukaemia is most associated with the Philadelphia chromosome?
CML
77
What is the main association with CML?
Philadelphia chromosome - t (9;22) translocation
78
What fusion gene does the Philadelphia chromosome form?
BCR/ABL fusion gene (on chromosome 22) which has tyrosine kinase activity
79
Which drug is good at targeting conditions associated with the Philadelphia chromosome like CML?
Imatinib (tyrosine kinase inhibitor)
80
What are the 3 stages of CML?
1) Chronic phase - asymptomatic for 4-5 years 2) Accelerated phase - getting increasingly anaemic and thrombocytopenia, more blasts taking up blood and bone marrow 3) Blastic phase - blastic transformation (AML)
81
What are some clinical features of CML?
Fatigue, night sweats, weight loss, hepatosplenomegaly, fever, symptoms of anaemia
82
What is seen on the blood film of CML?
All stages of maturation
83
What is seen on the bone marrow biopsy of CML?
Hypercellular, excess myeloid cells
84
How do you manage CML?
Imatinib, 2nd gen BCR-ABL inhibitors like dasatinib, hydroxycarbimide, bone marrow/stem cell transplant
85
What is the median survival for CML?
5-6 years
86
What is CLL?
Progressive accumulation of a malignant clone of functionally incompetent B cells
87
What is the median age at diagnosis for CLL?
72 years
88
How does CLL present?
``` Often asymptomatic Infection prone Fatigue B symptoms Lymphadenopathy Splenomegaly ```
89
What is seen on the blood film for CLL?
Smudge cells, lymphocytosis
90
What investigations are needed in CLL?
FBC, blood film, bone marrow biopsy, flow cytometry (CD5, CD19, CD20, CD23)
91
What is the natural history of CLL?
1/3rd never progress 1/3rd progress slowly 1/3rd progress actively
92
What is Richter's syndrome?
Transformation from CLL to high grade lymphoma
93
How do you treat CLL if indicated?
FLudarabine + rituximab + cyclophosphamide Radiotherapy for lymphadenopathy and splenomegaly Steroids reduce autoimmune haemolysis Transfusions when needed Human IV IG for recurrent infections
94
What are the 3 main myeloproliferative disorders?
Polycythaemia vera, essential thrombocythaemia and myelofibrosis
95
What mutations are myeloproliferative disorders associated with?
JAK2 (main one) MPL CALR
96
Give an example of a JAK2 inhibitor
Ruxolitinib
97
What is polycythaemia vera?
Malignant proliferation of pluripotent stem cell - excess red blood cells, white blood cells and platelets Leads to hyperviscosity and thrombosis
98
In what percentage of polycythaemia vera cases is the JAK2 mutation present?
>95%
99
How does polycythaemia vera present?
``` May be asymptomatic Headaches, dizziness, visual disturbance (hyperviscosity) Pruritis - especially after a hot bath Erythromelalgia Splenomegaly Facial plethora ```
100
What does the bone marrow biopsy show in polycythaemia vera?
Hypercellularit with erythroid hyperplasia
101
What investigations would you do in polycythaemia vera?
FBC - shows increased all cells Bone marrow biopsy Serum erythopoietin would be low
102
How do you mange polycythaemia vera?
Venesection in younger people Hydroxycarbamide Aspirin 75mg
103
What can polycythaemia transform into?
Myelofibrosis in 30% | Acute leukaemia in about 5%
104
What is essential thrombocythaemia?
Clonal proliferation of megakaryocytic leading to persistently high platelets
105
How does essential thrombocythaemia present?
``` Asymptomatic in 50% Thrombosis Haemorrhage (if >1500) Splenomegaly Headache Syncope ```
106
How do you manage essential thrombocythaemia?
Plateletphoresis, aspirin 75mg, hydroxycarbimide
107
What can essential thrombocythaemia transform into?
Myelofibrosis or polycythaemia vera
108
What are some secondary causes of high platelets?
Bleeding, surgery, iron deficiency, inflammation, infection, trauma
109
What is myelofibrosis?
Hyperplasia of megakaryocytes - produces platelet-derived growth factor - intense marrow fibrosis and haematopoiesis in the spleen (massive hepatosplenomegaly)
110
How does myelofibrosis present?
``` Weight loss Night sweats Fever SPLENOMEGALY Bone marrow failure (anaemia, infections, bleeding) Bone pain ```
111
What is seen on the blood film in myelofibrosis?
Characteristic teardrop RBCs
112
How do you manage myelofibrosis?
``` Folic acid and vitamin B6 Cytoreductive drugs Allopurinol JAK-2 inhibitors Splenectomy Stem cell transpolant ```
113
What are the two peaks of Hodgkins lymphoma?
Young adults in 3rd decade and over 70s
114
What are some risk factors for Hodgkins lymphoma?
EBV infection, mononucleosis, HIV
115
What are the characteristic cells in Hodgkins lymphoma?
Reed-Sternberg cells (multinucleate giant cells)
116
How does Hodgkins lymphoma present?
``` Enlarged painless lymph node B symptoms Pruritis Alcohol induced lymph node pain Hepatosplenomegaly ```
117
How do you investigate Hodgkins?
``` FBC Blood film LDH, urate, ESR Lymph node biopsy (excision node biopsy) Chest X ray CT/PET for staging ```
118
What is the Ann Arbor staging?
I - Confined to single lymph node region II - 2 or more areas on the same side of diaphragm III - nodes on both sides of the diaphragm IV - spread beyond nodes eg liver, bone marrow A - no symptoms B - fever, night sweats, weight loss >10% in 6 months
119
What is the chemotherapy for Hodgkins lymphoma?
ABVD - adriamycin (doxorubicin), bleomycin, vincristine and dacarbazine
120
What are some complications of the treatment Hodgkins?
Infertility, secondary cancers, AML, hypothyroidism from radiotherapy
121
What percentage of Non Hodgkins lymphomas are B cell vs T cell?
70% B cell, 30% T cell
122
What are the main high grade non Hodgkins lymphomas?
Diffuse large B cell lymphoma, Burkitt's lymphoma, mantle cell lymphoma
123
What are the main low grade non Hodgkins lymphomas?
Follicular lymphoma, MALT lymphoma, waldenstroms
124
What are some risk factors for non Hodgkins lymphoma?
HIV, immunodeficiency, EBV, HTLV-1, H pylori
125
How can low grade lymphomas present?
Painless, slowly progressive lymphadenopathy B symptoms Hepatosplenomegaly Pancytopenia
126
How can higher grade lymphomas present?
Rapidly growing and rapid lymphadenopathy B symptoms Hepatosplenomegaly Obstructive hydronephrosis secondary to retroperitoneal lymphadenopathy
127
How may burkitts lymphoma present?
Large abdominal mass and symptoms of bowel obstruction
128
How is high grade non Hodgkins lymphoma managed?
Rituximab, Cyclophosphamide, Hydroxydaunorubicin, Vincristine and Prednisolone
129
What is rituximab?
Monoclonal antibody against B cells - anti CD20
130
What is myeloma?
Malignant proliferation of the plasma cells of the bone marrow - overproduction of a monoclonal antibody - organ dysfunction
131
Which is the commonest myeloma>
IgG (2/3rds) followed by IgA (1/3)
132
What is the peak age of onset of myeloma?
70 years
133
Which ethnicity is myeloma more common in?
Black
134
What are the clinical features of myeloma?
- Hypercalcaemia - Anaemia, neutropenia or thrombocytopenia - Renal impairment - Osteolytic bone lesions - Recurrent bacterial infections
135
Why is there renal impairment in myeloma?
Due to deposition of light chains in the renal tubules, Hypercalcaemia and hyperuricaemia
136
How do you investigate myeloma?
- FBC, ESR, Plasma viscosity - Calcium, U+Es, creatinine - Serum protein electrophoresis - Urine electrophoresis - bence jones protein - Bone marrow biopsy - increased plasma cells - Serum free light chain assay - Whole body MRI scan or low dose CT - X-ray - pepper pot skull, fractures, vertebral collapse
137
How is myeloma managed?
Supportive - analgesia for bone pain, transfusion, orthopaedic procedures, rehydration Chemotherapy - lenolidomide, bortezomib and dexamethasone Autologous stem cell transplant
138
What are the complications of myeloma?
Hypercalcaemia Spinal cord compression Hyperviscosity AKI
139
What are the vacancies of the immunoglobulins?
IgM - pentimer - increased risk of hyperviscosity IgA - dimer IgG - single
140
What immunoglobulin is associated with waldenstroms macroglobulinaemia?
IgM - risk of hyperviscosity

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