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haematology to work on Flashcards

1
Q

what are the investigations for CLL?

A

● Normal or low Hb
● Raised WCC with very high lymphocytes
● Blood film – smudge cells may be seen in vitro

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2
Q

what is the treatment for CLL?

A
  • Watch and wait
  • Chemotherapy
  • Monoclonal antibodies, e.g. rituximab
  • Targeted therapy, e.g. bruton kinase inhibitors (ibrutinib)
  • human IV immunoglobulins
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3
Q

what is the epidemiology of ALL?

A

Mainly a childhood disease
highest prevalence between 2-4yrs
Associated with Down’s syndrome
Associated with ionising radiation

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4
Q

what are the investigations for ALL?

A

● FBC and blood film
o WCC = high
o Blast cells on film and in bone marrow

● CXR and CT scan looking for mediastinal and abdominal lymphadenopathy

● Lumbar puncture to look for CNS involvement

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5
Q

What are signs and symptoms of Hodgkins lymphoma?

A
  • Fever and sweating
  • Enlarged rubbery non-tender nodes
  • Systemic ‘B’ symptoms, e.g. fever
  • Painful nodes on drinking alcohol
  • some patients (commonly young women) have disease localised to the mediastinum
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6
Q

What blood results may you seen in someone with Hodgkins lymphoma?

A 
High ESR 
FBC - anaemia (normochromic normocytic)
Reed Sternberg cells
Low Hb 
High serum lactase dehydrogenase
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7
Q

Describe the staging for Hodgkins lymphoma

A

Stage 1 = confined to a single lymph node region

Stage 2 = Involvement of two or more nodal areas on the same side of the diaphragm

Stage 3 = Involvement of nodes on both sides of the diaphragm.

Stage 4 = Spread beyond the lymph nodes e.g. liver.

Each stage is either ‘A’ - absence of ‘B’ symptoms or ‘B’ - presence of ‘B’ symptoms

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8
Q

What the treatment for Hodgkins lymphoma?

A

ABVD chemotherapy regime

marrow transplant

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9
Q

What are the possible complications of treatment for Hodgkins lymphoma?

A
  1. Secondary malignancies
  2. IHD
  3. Infertility
  4. Nausea
  5. Alopecia
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10
Q

what are the signs and symptoms of non-hodgkins lymphoma?

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms, e.g. fever
GI and skin involvement

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11
Q

What is the treatment for non-hodgkins lymphoma?

A

Steroids
Monoclonal antibodies to CD20 -> Rituximab
CHOP regimen

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12
Q

what are the causes of microcytic anaemia?

A
  1. Iron deficiency
  2. Chronic disease - cancer, HF, CKD
  3. Thalassaemia
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13
Q

what are the causes of normocytic anaemia?

A
  1. Acute blood loss
  2. Chronic disease
  3. Combined haematinic deficiency (iron and B12 deficiency)
  4. renal failure
  5. pregnancy
  6. hypopituitarism, hypothyroidism, hypoadrenalism
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14
Q

Give 3 causes of macrocytic anaemia

A
  1. B12/folate deficiency
  2. Alcohol excess –> liver disease
  3. Haemolytic anaemia
  4. Hypothyroidism
  5. bone marrow infiltration/failure
  6. myeloma
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15
Q

What pathological changes occur due to anaemia?

A
  1. Heart/liver fat change
  2. Ischaemia
  3. Skin/nail atrophy
  4. CNS death
  5. Aggravate angina and claudication
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16
Q

Give 3 signs of anaemia

A
  1. Conjunctival pallor
  2. Hyper-dynamic circulation
  3. Tachycardia
  4. Systolic flow murmur
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17
Q

What investigations might you do in someone with anaemia?

A
  1. FBC and Blood film
  2. Reticulocyte count
  3. B12, folate and ferritin levels
  4. U+E’s, LFT’s, TSH levels
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18
Q

Give 3 causes of iron deficiency anaemia

A
  1. Blood loss
  2. Poor absorption
  3. Decreased intake in diet
  4. Increased demand - growth/pregnancy
  5. Hookworm
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19
Q

What would the results of investigations be for someone with iron deficiency anaemia?

A

MICROCYTIC HYPOCHROMIC ANAEMIA

Low ferritin, low reticulocyte

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20
Q

what are the causes of B12 deficiency/pernicious anaemia?

A
  1. Autoimmune parietal cell destruction (pernicious)
  2. Poor diet
  3. Ileectomy
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21
Q

What are the specific signs of B12 deficiency/pernicious anaemia?

A

Inflamed tongue (glossitis), angular stomatitis, mild jaundice
Neurological - paraesthesia, psychological problems, ataxia
- dementia and visual disturbances

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22
Q

What would the results of the investigations be for someone with B12 deficiency/pernicious anaemia?

A

MACROCYTIC NORMOCHROMIC ANAEMIA with polysegmented neutrophils
Autoantibodies = intrinsic factor and coeliac antibodies(intrinsic factor antibodies are diagnostic)

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23
Q

Give 3 inherited causes of haemolytic anaemia

A
  1. RBC membrane defect
  2. Abnormal Hb - thalassaemia’s, sickle cell
  3. Enzyme defects - Glucose-6-phosphate dehydrogenase deficiency
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24
Q

Give 3 acquired causes of haemolytic anaemia

A
  1. Autoimmune
  2. Drug induced
  3. Secondary to systemic disease
  4. Malaria
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25
What is the management for haemolytic anaemia
Dietary = folate and iron supplementation AI cause = immunosuppression Surgical = splenectomy
26
Give 3 acute complications of sickle cell disease
1. Painful crisis 2. Sickle cell syndrome - blockage of blood vessels within bone leading to pain 3. Strokes in children 4. Infections
27
Give 3 chronic complications of sickle cell disease
1. Renal impairment 2. Pulmonary hypertension 3. Joint damage 4. growth and development problems 5. cardiomegaly, MI 6. hepatomegaly 7. retinopathy 8. impaired placental blood flow
28
Describe the treatment for sickle cell disease
- Precipitating factors should be avoided - e.g. infection, dehydration, cold - Folic acid - Acute attacks: - IV fluids - Analgesia - Oxygen - Possible blood transfusions - Oral hydroxycarbamide to reduce frequency of crises - Stem cell transplant long term management - hydroxyurea - increases HbF levels so is used as prophylaxis - pneumococcal vaccine
29
what are the clinical features of G6PD deficiency?
``` Most are asymptomatic Pallor Fatigue Palpitations Shortness of breath Jaundice Exacerbated by ingesting fava beans ```
30
Name 3 things that can precipitate G6PD deficiency
1. Broad beans / fava beans 2. Infection 3. Drugs - Primaquine, sulphonamide, quinolones
31
What is polycythaemia?
- increased RBC count - increased haematocrit - Due to a JAK2 mutation
32
What stimulates EPO?
Tissue hypoxia
33
what are the secondary causes of absolute polycythaemia?
hypoxia - Smoking - Lung disease - Cyanotic heart disease - High altitude - obstructive sleep apnoea Excess EPO - renal disease alcohol
34
what are the clinical features of polycythaemia?
``` Headaches and dizziness Itching Fatigue Tinnitus Erythromelalgia – burning sensation in fingers and toes Hypertension Angina Hepatosplenomegaly (distinguishes 1o from 2o) ```
35
What is the treatment for polycythaemia?
``` No cure Treatment aim is to maintain normal blood count Venesection - relieves symptoms Low does aspirin Radioactive phosphorus in those over 70 ```
36
What can cause decreased platelet production?
1. Congenital causes (e.g. malfunctioning megakaryocytes) 2. Infiltration of bone marrow (e.g. leukaemia) 3. Alcohol 4. Infection (e.g. HIV/TB) 5. Reduced TPO 6. Aplastic anaemia
37
What can cause increased platelet destruction?
1. Autoimmune (e.g. ITP) 2. Hypersplenism 3. Drug related (e.g. heparin induced) 4. DIC and TTP --> increased consumption
38
What can cause reduced platelet function?
1. Congenital abnormality 2. Medications - aspirin 3. VWF disease 4. Uraemia
39
Give 4 causes of bleeding
1. Trauma 2. Platelet deficiency - thrombocytopenia 3. Platelet dysfunction - aspirin induced 4. Vascular disorders
40
Give 3 things that can cause coagulation disorders
1. Vitamin K deficiency 2. Liver disease 3. Congenital - haemophilia
41
What is thrombotic thrombocytopenia (TTP)?
- Widespread adhesion and aggregation of platelets leads to microvascular thrombosis and consumption of platelets - Occurs due to a reduction in ADAMTS-13 – a protease that is normally responsible for the degradation of vWF
42
what are the clinical features of thrombotic thrombocytopenia (TTP)
- Flu-like symptoms - fever, fatigue, aches - Purpura - Epistaxis (nosebleeds) - Easy bruising - Menorrhagia (heavy periods) - Haemoptysis (coughing up blood) - Headache - Abdominal pain - GI bleeding - Chest pain (rare)
43
What is the treatment for thrombotic thrombocytopenia (TTP)?
- Plasma exchange (removed ADAMTS13 autoantibodies) - Splenectomy - IV steroids - IV methylprednisolone - IV rituximab - folic acid - platelet transfusion = contraindicated
44
What is immune thrombocytopenia (ITP)?
Antibodies formed against platelet --> autoimmune platelet destruction via reticuloendothelial system
45
Give 3 causes of immune thrombocytopenia (ITP)
``` Primary = viral infection (children) Secondary (chronic, adults) - Autoimmune conditions - Malignancies - CLL - Infections - HIV/Hep C ```
46
what is the clinical presentation of immune thrombocytopenia (ITP)?
Can by asymptomatic - Purpura - bruising and purple/red rash - Epistaxis (nosebleeds) - Menorrhagia (heavy periods) - Prolonged bleeding from the gums - Severe headache - Vomiting - Fatigue
47
What is the management for immune thrombocytopenia (ITP)?
- first line = Corticosteroid (prednisolone) or IV immunoglobulin - IV IgG - second line = Splenectomy or immunosuppression (rituximab)
48
Give 4 causes of folate deficiency
1. Dietary 2. Malabsorption 3. Increased requirement - pregnancy 4. Folate antagonists - methotrexate
49
Give 3 signs of haemolytic anaemia
1. Pallor 2. Jaundice 3. Splenomegaly
50
Give 2 specific symptoms of iron deficiency anaemia
1. Koilonychia - spoon nails 2. Brittle hair and nails 3. Atrophic glossitis
51
Give 3 causes of neutrophilia
1. Infection 2. Inflammation 3. Cancer 4. CML = primary cause
52
Give 3 causes of lymphocytosis
1. Viral infection 2. Inflammation 3. Cancer 4. CLL = primary cause
53
Give 3 causes of thrombocytosis
1. Infection 2. Inflammation 3. Cancer 4. Essential thrombocythaemia = primary cause
54
Give 3 causes of neutropenia
1. Underproduction = marrow failure, marrow infiltration, marrow toxicity (e.g. drugs) 2. Increased removal = autoimmune, Felty's syndrome, cyclical
55
Give 2 causes of thrombocytopenia
1. Production failure - marrow suppression, marrow failure | 2. Increased removal - immune response (ITP), consumption (DIC), splenomegaly
56
Give 4 risk factors for febrile neutropenia
1. If the patient had chemotherapy <6 weeks ago 2. Any patient who has had a stem cell transplant <1 year ago 3. Any haematological condition causing neutropenia 4. Bone marrow infiltration 5. those on methotrexate, carbimazole and clozapine
57
Give a risk for spinal cord compression
Any malignancy that can cause compression - bone metastasis | commonly seen in myeloma and lymphoma
58
What is hyper viscosity syndrome?
Increase in blood viscosity usually due to high levels of immunoglobulins
59
How does heparin work?
Indirect factor II (thrombin) inhibitor It binds to and activates antithrombin which then inhibits thrombin and factor Xa
60
What 3 blood test values would be increased in someone with polycythaemia?
1. Hb 2. RCC 3. PCV
61
what are the clinical features of chronic lymphoblastic leukaemia (CLL)?
- often no symptoms - may be anaemic - weight loss, sweats, anorexia - hepatosplenomegaly - enlarged, rubbery, non-tender nodes
62
what are the complications of CLL?
- autoimmune haemolysis - increased infection risk - marrow failure - richter's syndrome - transformation to aggressive lymphoma
63
what are the 2 different groups of macrocytic anaemia?
- megaloblastic = erythroblasts with delayed nuclear nmaturation - non-megaloblastic = normal erythroblasts
64
how is vitamin B12 absorbed in the body?
binds to intrinsic factor (made by parietal cells) and absorbed in terminal ileum
65
what is the role of vitamin B12?
essential for thymidine and DNA synthesis
66
what are the risk factors for folate deficiency?
- elderly - poverty - alcoholic - pregnant - crohn's/coeliac
67
what are the risk factors for pernicious anaemia?
- female - elderly - fair hair and blue eyes - blood group A - thyroid and addison's disease
68
what is the clinical presentation of folate deficiency?
- may be asymptomatic - may present with anaemia symptoms - glossitis - no neuropathy
69
what investigations should be undertaken in folate deficiency?
- blood count and film = megaloblastic, RBCs macrocytic - low serum and red cell folate low - serum bilirubin may be raised
70
what is the management for acute attacks in sickle cell anaemia?
IV fluids analgesia oxygen antibiotics
71
what investigations should be undertaken for G6PD?
blood film - irregularly contracted cells, bite cells, reticulocytosis G6PD enzyme levels low
72
what is the treatment for G6PD?
- blood transfusion | - stop exacerbating drugs
73
what is the epidemiology of non-hodgkins lymphoma?
rare before 40 | strong link between EBV and Burkitt's lymphoma
74
which chemotherapy regimen is used for treating non-hodgkin's lymphoma?
R-CHOP
75
what investigations should be undertaken for polycythaemia?
- blood count = raised WBC and platelets - raised Hb - JAK2 mutation on genetic screen - bone marrow biopsy - proliferation of granulocytes and megakaryocytes - low serum EPO
76
which investigations should be undertaken for immune thrombocytopenia purpura (ITP)?
- bone marrow examination = thrombocytopenia with normal/increased megakaryocytes - platelet autoantibodies
77
what are the causes of thrombotic thrombocytopenic purpura (TTP)?
``` idiopathic autoimmune e.g. SLE cancer pregnancy drug related ```
78
what is the clinical presentation of haemophilia A?
- mild = bleed after trauma - moderate = bleeding after venepuncture and trauma - severe = neonatal bleeding, spontaneous bleed, GI bleeds, haematuria
79
what is the clinical presentation of haemophilia B?
- bruising - nosebleeds - pallor - heavy bleeding from minor injury - bleeding from tooth loss in childhood - joint pain and stiffness - headache, vomiting - GI bleed
80
what is the clinical presentation of von Willebrnad's disease?
- Bleeding from mucosa - Epistaxis (nose bleeds) - Menorrhagia (heavy periods) - Spontaneous bleeding - Blood clots during childbirth (rare) - Spontaneous death (rare)
81
what is the management for von Willebrand's disease?
- Education on bleeding risks - Stop any antiplatelet drugs and NSAIDs - Tranexamic acid - used for minor bleeds - Combined oral contraceptive to control menorrhagia in women - Desmopressin -> increases vWF levels - Platelet transfusions can be helpful in some - Family screening should be offered
82
what are the causes of aplastic anaemia?
- inherited - idiopathic - benzene - chemotherapeutic drugs - antibiotics - infections - EBV, HIV and TB
83
what investigations are undertaken for aplastic anaemia?
- blood cell count - pancytopenia and low reticulocyte count bone marrow examination - hypocellular with increased fat spaces
84
what is the treatment for aplastic anaemia?
- treat cause - antibiotics for neutropenia - RBC transfusion and platelets - bone marrow transplant - immunosuppressive therapy - ATG and ciclosporin if under 40
85
what are the causes of haemolytic anaemia?
- RBC membrane defects - spherocytosis - enzyme defects - G6PD - alpha and beta thalassaemia - sickle cell anaemia - autoimmune haemolytic anaemia
86
what are the different types of non-hodgkins lymphoma?
low grade = follicular lymphoma high grade = diffused large B cell lymphoma very high grade = burkitt's lymphoma
87
what are the different types of von Willebrand's disease?
- 3 types: Type 1, 2, 3, platelet type - Type 1 is the most common (60-80%) - Platelet type is the most rare (70 cases)
88
what are the investigations for von Willebrand's disease?
- FBC - Fibrinogen level - Platelet count - normal - Clotting screen- Plasma vWF decreased - Factor VIII levels - can be decreased as vWF is not present to protect it
89
what are the investigations for haemophilia B?
- Normal prothrombin time - Prolonged activated partial thromboplastin time - Low factor IX levels - Endoscopy if GI bleed suspected
90
what are the clinical features of homozygous sickle cell anaemia?
Acute pain in hands and feet Long bone pain, e.g. femur, spine, ribs Cognitive defects in children Pulmonary hypertension and chronic lung disease (most common cause of death) Splenic sequestration (cells get trapped)
91
what is the epidemiology of G6PD deficiency?
More common in Africa, the Middle East and SE Asia | More common in males
92
what is the clinical presentation of microcytic anaemia?
in addition to anaemia symptoms: ● Brittle hair and nails ● Spoon shaped nails – koilonychia ● Atrophy of papillae of tongue – atrophic glossitis ● Angular stomatitis – ulceration of corners of the mouth
93
what are the clinical features of malaria?
Non-specific symptoms - Fever, chills - Headache - Cough - Splenomegaly Severe disease in P. falciparum - Shortness of breath - Fits and hypovolaemia - AKI and nephrotic syndrome
94
when do clinical features of malaria present?
Symptoms occur from 6 days post infection P. vivax and P. ovale commonly present 6 months post infection - can relapse as it can lay dormant in the liver for years.
95
what is the treatment for malaria?
- Chloroquine for non-falciparum malaria - Oral quinine sulphate for falciparum - Add IV quinine dihydrochloride for severe disease Do not treat those with G6PD deficiency Need specialist help
96
what are the risk factors for iron deficiency anaemia?
● Undeveloped countries ● High vegetable diet ● Premature infants ● Introduction of mixed feeding delayed
97
what is the pathophysiology of anaemia of chronic disease?
● Decreased release of iron from bone marrow to developing erythroblasts ● Inadequate erythropoietin response to anaemia – decreased RBC survival
98
what do the investigations for anaemia of chronic disease show?
● Low serum iron and iron-binding capacity ● Increased or normal ferritin due to inflammatory response ● RBCs normo/micro-cytic and hypochromic (pale) ● Serum soluble transferrin normal
99
what is the management for anaemia of chronic disease?
● Treat underlying chronic condition | ● Erythropoietin is effective in raising the Hb level and is used in cases of renal disease and inflammatory disease
100
what are the side effects of oral iron treatment?
nausea, abdominal discomfort, diarrhoea/constipation, black stool
101
what are the side effects of erythropoietin treatment?
flu-like symptoms, hypertension, raised platelets, thromboembolism
102
how long do folate stores last for?
4 months
103
what are the main causes of aplastic anaemia?
- inherited - Fanconi's anaemia - idiopathic- benzene, toulene, glue sniffing - chemotherapeutic drugs - antibiotics - infections - EBV, HIV, TB
104
what is the treatment of aplastic anaemia?
- treat cause - RBC transfusion - bone marrow transplant - immunosuppressive therapy - ATG and ciclosporin
105
what is the long term management for sickle cell anaemia?
- hydroxyurea - increases the HbF levels and is used in the prophylactic management of sickle cell anaemia to prevent painful episodes - pneumococcal vaccine
106
how is heparin treatment reversed?
- proteamine
107
what is the mechanism of action for ondansetron?
5HT3 antagonist
108
What investigations might you do in someone who you suspect has myeloma?
``` FBC - anaemia, raised ESR Blood film - rouleaux Serum and urine electrophoresis - Bence Jones protein in urine X-rays - pepper pot skull, fractures CT - bone lesions, cord compression ```
109
What does a diagnosis of myeloma require?
1. Monoclonal protein band in serum or urine 2. Increased plasma cells on bone marrow biopsy 3. Hypercalcaemia/renal failure/anaemia 4. Bone lesion on skeletal survey
110
Describe the treatment for symptomatic myeloma
``` Bone pain = analgesia Bisphosphonates (zolendronate) Blood transfusion - for anaemia Fluids and dialysis - renal failure Chemotherapy Stem cell transplant ```
111
What are the risk factors for AML?
1. Preceding haematological disorders 2. Prior chemotherapy 3. Exposure to ionising radiation 4. Down's syndrome
112
what are the clinical features of AML?
- Anaemia -> breathlessness, fatigue, pallor - low WCC = Infection - low platelets = bleeding/bruising - Hepatosplenomegaly - Peripheral lymphadenopathy - Gum hypertrophy - Bone marrow failure and bone pain
113
What investigations do you do on someone who you suspect has AML?
- FBC - anaemia, thrombocytopaenia - WCC often raised (can be normal/low) - Blood film - leukaemic blast cells - Bone marrow biopsy - Auer rods
114
What would you expect to see on an FBC and bone marrow biopsy in someone you suspect to have AML?
FBC = anaemia and thrombocytopenia and neutropenia | BM biopsy = leukaemic blast cells (with Auer rods)
115
what are the clinical features of CML?
1. Symptomatic anaemia 2. Abdominal pain = splenomegaly 3. Weight loss, tiredness, palor 4. Gout - due to purine breakdown 5. Bleeding - due to platelet dysfunction 6. fever and sweats in absence of infection
116
what are the investigations for CML?
- FBC - anaemia, raised myeloid cells, high WCC (eosinophilia, basophilia, neutrophilia) - Increased B12 - Blood film - left shirt, basophilia - Bone marrow biopsy - increased cellularity (hypercellular) - Philadelphia chromosome seen in 80+% of cases  t(9;2) - Stimulates cell division
117
What is the treatment for CML?
- Chemotherapy - Tyrosine kinase inhibitors, e.g. Imatinib - Given orally - Stem cell transplant
118
what are the clinical features of ALL?
- Anaemia = breathlessness, fatigue, pallor - Low WCC = Infection - low platelets = bleeding and bruising - Hepatosplenomegaly - Peripheral lymphadenopathy - CNS involvement - headache, cranial nerve palsies - SVC obstruction, dilated superficial chest veins - Bone marrow failure and bone pain
119
What is the treatment for ALL?
- Blood and platelet transfusion - Chemotherapy - Steroids - Allopurinol to prevent tumour lysis syndrome - Intrathecal drugs, e.g. methotrexate - Acute control of infections with IV antibiotics - Neutropenia makes this high risk - Stem cell transplant
120
Describe the treatment for AML
- Blood and platelet transfusions - IV fluids - Allopurinol to prevent tumour lysis - Infection control with IV antibiotics - Chemotherapy - Steroids - Sibling matched allogenic bone marrow transplant

Phase 2A revision (38 decks)

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