The White Cell Flashcards
1
Q
What cell types are included in a WC differential?
A
Neutrophils Lymphocytes Eosinophils Monocytes Basophil
2
Q
What is the function of Neutrophils?
A
Ingest/kill bacteria, fungi, cellular debris
3
Q
What is the function of Lymphocytes?
A
Produce antibodies for cell-mediated immunity
4
Q
What is the function of Eosinophils?
A
Role in allergic reactions, defence of parasitic infection
5
Q
What is the function of Monocytes?
A
Precursor to tissue macrophages
6
Q
What is the function of Basophils?
A
Release histamine in inflammatory reactions
7
Q
What is Leukocytosis?
A
Increase in WCC
8
Q
What are the causes of a neutropenia?
A
Viral infection Severe sepsis Neutrophil antibodies (SLE) Bone marrow failure Hypersplenism Cytotoxic drugs
9
Q
What are the causes of a neutrophilia?
A
Bacterial infection Inflammatory reactions Disseminated malignancy Stress Myeloproliferative conditions Corticosteroid therapies
10
Q
What is agranulocytosis, and what drug causes it as a s/e?
A
Complete absence of circulating neutrophils
Carbimazole
11
Q
What are the causes of a lymphopenia?
A
Bone marrow failure Corticosteroid therapies SLE Uraemia HIV infection Cytotoxic drugs
12
Q
What are the causes of a lymphocytosis?
A
Viral infections
Chronic infections
Myeloproliferative conditions
13
Q
What are leukaemias?
A
Malignant proliferations of blood forming cells
- acute/chronic
- myeloid/lymphoid
14
Q
Describe the difference b/w myeloid/lymphoid cell lineages?
A
Haemopoietic stem cell –> common myeloid/lymphoid progenitor
- common myeloid –> erythrocytes, mast cells, megakaryocytes (form platelets), myeloblasts
- common lymphoid –> NK cells, T/B lymphocytes
15
Q
What cell lines do myeloblasts go on to form?
A
Monocytes
Basophils
Neutrophils
Eosinophils
16
Q
What are the myeloproliferative conditions?
A
Myelofibrosis
Myelodysplasia
Polycythaemia vera
PRE-LEUKAEMIC
17
Q
What is Acute Lymhoblastic Luekaemia (ALL)?
A
Malignancy of lymphoid cells (T/B cells) leading to uncontrolled proliferation of immature blast cells
-eventual bone marrow failure & tissue infiltration
18
Q
What is the aetiology of ALL?
A
Most common childhood malignancy
- 3-7yrs average age of dx
- 80% of childhood leukaemias
- rare in adults
- more common in certain syndromes (e.g. Downs)
19
Q
What is the prognosis of ALL?
A
Children (<10yrs) = good, cure rates >80%
20
Q
What are poor prognostic factors in ALL?
A
Older age of presentation Male sex B-cell disease Presence of Philadelphia chromosome -9:22 translocation
21
Q
What is Acute Myeloid Leukaemia (AML)?
A
Malignancy of blast cells from marrow myeloid elements
22
Q
What are the risk factors for AML?
A
Can be de novo OR Myeloproliferative conditions Previous chemo Ionising radiation Genetic syndromes
23
Q
What is the aetiology of AML?
A
Median age of presentation at 65 yrs
- can occur at any age
- incidence increases w/ age
24
Q
What is the prognosis of AML?
A
Rapidly progressive
20% 3yr survival after chemo
25
How do acute leukaemias present?
B sx
Bone pain
Sx of marrow failure
Hepatomegaly/splenomegaly
26
What are B sx?
```
Fatigue
Wt loss
Night sweats
Fevers
Pruritis
```
27
What are the sx of marrow failure?
Anaemia (SOB on exertion, weakness)
Leukopenia (recurrent infections)
Thrombocytopenia (bleeding & brusing)
28
What is important to remember about the leukopenia in acute leukaemias?
Pts have leukocytosis BUT immature cells so present as leukopenia
29
What investigations are appropriate in suspected acute leukaemia?
```
FBC
Blood film
CXR (T-cell ALL, ?mediastinal widening)
Bone marrow aspiration
PET scanning (?mets)
U&Es, LFTs, cardiac function testing
```
30
What are the general principles of managing acute leukaemias?
Supportive care
| Use of antibiotics
31
What supportive care should be given in acute leukaemia?
```
Nurse w/ full barrier nursing
Hickman line inserted
High-calorie diet
Frequent blood/platelet transfusions
Allopurinol (prevent tumour lysis syndrome)
Frequent bloods/obs
```
32
When should antibiotics be given in acute leukaemia?
Temp >38o on 2 occassions >1hr apart
- assume sepsis
- start broad spectrum a/b until afebrile for 72 hrs
- often cephalosporin + gentamycin
33
What specific management steps are taken in ALL?
```
High dose chemo to induce remission
-consolidation w/ high/med dose blocks over months
-2 yrs maintenance therapy
Consider marrow transplant
-necessary if Philadelphia chromosome
```
34
What specific management steps are taken in AML?
```
Intensive chemo
If poor prognosis
-allogenic marrow transplant from HLA-matched siblings
-allows further high dose chemo
If intermediate prognosis
-autologous marrow transplants
-further, lower dose chemo
```
35
What is the aetiology of Chronic Myeloid Leukaemia (CML)?
Rare in childhood
Most common 40-60yrs
Philadelphia Chr present in 95%
-better prognosis
36
What are the sx of CML?
```
Insidious B sx, detected by chance (30%)
Gout (purine breakdown)
Abdo discomfort (splenomegaly)
```
37
What are the signs of CML?
Splenomegaly
Hepatomegaly
Anaemia/thrombocytopenia
38
What investigations are appropriate in suspected CML?
```
FBC (WCC inc)
Blood film (myeloid cells inc)
Bone marrow biopsy
CT/PET
Cytogenic analysis of blood/marrow for Philadelphia Chr
```
39
What are the management options for CML?
```
Imatinib chemo
-response rate >90%
Stem cell transplantation
-only treatment to achieve remission
-significant mortality/morbidity
```
40
What is the prognosis of CML?
Median survival 6yrs
41
What are the three stages of CML?
```
Chronic phase
-few sx, lasts for yrs
Accelerated phase
-inc sx, difficulty controlling counts
Blast transformation
-features of AML, death
```
42
What is the aetiology of Chronic Lymphocytic Leukaemia (CLL)?
Most common leukaemia
2x as common in males
Median age of presentation 7yrs
43
What is the underlying pathophysiology of CLL?
Accumulation of mature B-cells (escaped apoptosis)
| Inc mass of immune-incompetent cells --> Bone marrow failure
44
What are the sx of CLL?
Asymptomatic, incidental finding on FBC
Anaemic/infection prone
B sx (if severe)
45
What are the signs of CLL?
Enlarged, non-tender lymph nodes
| Hepatosplenomegaly
46
What investigations are appropriate in suspected CLL?
```
FBC (inc WCC, AI haemolysis later)
Blood film (smudge cells)
```
47
What is the rule of 1/3s for CLL?
W/o treatment
- 1/3 will never progress
- 1/3 will eventually progress
- 1/3 will progress from diagnosis
48
What are the management options for CLL?
Treatment only indicated if symptomatic/markers of poor prognosis
Chemo/radio
49
What is the prognosis of CLL?
Depends on Rai stage, anywhere from 1yr to 13yrs mean survival
-death usually due to infection/Richter's syndrome
50
What is Richter's syndrome?
Transformation of CLL to aggressive lymphoma
| -usually diffuse large B cell lymphoma
51
What is myeloma?
Malignant clonal proliferation of plasma cells (derived from B lymphocytes)
52
What is the monoclonal band/paraprotein of myeloma?
Single clone of plasma cells produce single immunoglobulin
| -seen on serum/urine electrophoresis as monoclonal band
53
What is the aetiology of myeloma?
Average age presentation 70yrs
| More common in Afro-Carribean populations
54
What are the core clinical features of myeloma?
CRAB
- calcium (hypercalcaemia)
- renal (impairment)
- anaemia (bone marrow failure)
- bone (osteolytic lesions due to osteoclast activation)
55
What are the presenting sx of myeloma?
```
Backache
Pathological fractures
Hypercalcaemia (bones, stones, moans, groans)
Infection
Anaemia
Bleeding
Renal impairment (20% at dx)
```
56
What investigations are appropriate in suspected myeloma?
```
FBC (normochromic, normocytic anaemia, WCC dec)
Blood film (rouleaeux)
ESR (raised)
U&Es (deranged)
Ca (raised)
Alk Phos (norm)
Serum/urine electrophoresis
Urine Bence-Jones protein (+ve)
Skeletal XR (lytic lesions)
Bone marrow biopsy
```
57
What are the management options for myeloma?
Supportive therapy
Chemotherapy
Radiotherapy
Bone marrow stem cell transplants if <70
58
What are the complications of myeloma?
Hypercalcaemia
Spinal cord compression
Hyperviscosity
Acute renal failure
59
What is the prognosis of myeloma?
Original cell v. resistant, often returns
Med survial 3-4yrs
Death from renal failure/infection
60
What are lypmhomas?
Malignant proliferations of lymphocytes, usually B-cells
- commonly accumulate in peripheral lymph nodes
- can accumulate in peripheral blood/infiltrate organs
61
What are the two main types of lymphoma?
Hodgkin's (15%)
| Non-Hodgkin's (85%)
62
What is the defining characteristic of Hodgkin's lymphoma?
Presence of Reed-Sternberg cells
| -binucleate 'mirror cells' on biopsy
63
What is the aetiology of Hodgkin's lymphoma?
Peak incidence at 20-35yrs & 50-70yrs
| More common in males (2:1)
64
What are the risk factors for Hodgkin's lymphoma?
Affected sibling
EBV
SLE
Obesity
65
What are the sx of Hodgkin's lymphoma?
B sx, w/ profuse night sweats (25%)
Lymph node pain induced by alcohol
SVC/bronchial obstruction
Pleural effusion
66
What are the signs of Hodgkin's lymphoma?
Enlarged, non-tender, rubbery lymph nodes (cervical)
Lymphaenopathy
Hepato-splenomegaly (50%)
Cachexia/anaemia
67
What is the prognosis of Hodgkin's lymphoma?
Slow growing, localised, rarely fatal
| Better in lymphocyte predominant disease
68
What is the aetiology of Non-Hodgkin's lymphoma?
All lymphomas w/o Reed-Sternburg cells
| Peak incidence at 70yrs
69
What are the two main classifications of Non-Hodgkin's lympoma?
High grade
| Low grade
70
Describe high grade Non-Hodgkin's lymphoma
Divide rapidly
Present w/ rapid onset lymphadenopathy
Aggressive
Better prognosis if identified/treated
71
Describe low grade Non-Hodgkin's lymphoma
Divide slowly
Present insidiously
Widely disseminated at dx
Often incurable
72
What are the sx of Non-Hodgkin's lymphoma?
```
Nodal disease (lymphadenopathy in 75%)
Extranodal disease (25%)
-oropharynx, skin, CNS, gut or lung
B sx (wt loss if disseminated)
Bone marrow failure
```
73
What are the appropriate investigations in Non-Hodgkin's lymphoma?
Bloods - FBC, ESR, LFT, U&Es, LDH, Ca
Lymph node excision biopsy
Image guided biopsy
Staging CT
74
What are the management options for Non-Hodgkin's lymphoma?
Chemotherapy
Radiotherapy
Chemo-radiotherapy
75
What is the prognosis for Non-Hodgkin's lymphoma?
```
Survival variable
Poor prognostic signs are
->60yrs
-disseminated disease
-raised LDH
```
76
How are Non-Hodgkin's lymphomas stages?
Ann-Arbor system
77
What are myeloproliferative disorders?
Disease where clones of haemopoetic stem cells proliferate in the marrow, but retain the ability to differentiate
78
What are the common myeloproliferative disorders?
Essential thrombocytosis
Myelofibrosis
Polycythaemia rubra vera
79
What is essential thrombocytosis?
Clonal proliferation of megakaryocytes --> persistently raised platelets
-often asymptomatic
80
What are the sx of essential thrombocytosis?
Often asymptomatic
Microvascular occlusion
Bleeding sx
Arterial/venous thrombosis
81
What is Polycythaemia rubra vera (PCV)?
Malignant proliferation of a clone derived from one pluripotent marrow cell
-not requiring EPO to avoid apoptosis
82
What are the common sx of PCV?
Asymptomatic
| Arterial/venous thrombosis (>60yrs)
83
What are the main complications of PCV?
```
Serum hyperviscosity (excess RBCs & WCs)
Thrombotic complications (excess platelets)
```
84
How is PCV diagnosed?
Increased red cell mass
| Investigation for JAK 2 mutation
85
What are the rarer presentations of PCV?
Vague hyperviscosity sx (headache, dizziness, tinnitus, facial plethora, erythromelagia)
Splenomegaly
Gout
86
How is PCV managed?
Repeated venesection
| Low dose aspirin
87
What is erythrromelagia?
Burning sensation in fingers/toes
88
What is Primary Myelofibrosis?
Hyperplasia of megakaryocytes
- produces excess platelet derived growth factor
- marrow fibrosis & metaplasia
- 2o haematopoiesis in spleen
89
What are the sx of Primary Myelofibrosis?
B sx
Abdo discomfort (massive splenomegaly)
Bone marrow failure
90
What is the main risk of essential thrombocytopenia & PCV?
May progress to myelofibrosis OR AML
| -risk relatively rare
91
What is aplastic anaemia?
Stem cell disorder leading to pancytopenia/hypoplastic marrow
92
What are the causes of aplastic anaemia?
Autoimmune (drugs, viruses, irradiation as triggers)
| Inherited (Fanconi)
93
What are the sx of aplastic anaemia?
Bone marrow failure
| Pancytopenia
94
How is aplastic anaemia diagnosed?
Bone marrow biopsy
95
How is aplastic anaemia managed?
```
Blood product transfusion
Immunosuppression in AI
Allogenic bone marrow transplant
-curative
-only in younger pts
```
