Important cancers Flashcards
(190 cards)
1
Q
Inheritance pattern of MEN
A
autosomal dominant
2
Q
What glands are implicated in MEN and what adaptation do they undergo
A
Hyperplasia of several endocrine glands
3
Q
List the 3 variants of MEN
A
Men I
Men II or IIa
Men IIb or III
4
Q
What glands are implicated in MEN I
A
Parathyroid
Pancreatic
Pituitary
5
Q
What glands are implicated in MEN II
A
Pheo/ andreal medullary hyperplasia
Parathyroid hyperplasia
Thyroid - Medullar cancer and C - cell hyperplasia
6
Q
What are the metabolic problems associated with MEN (5)
A
Zollinger-Ellison syndrome, hyperinsulinism, pheochromocytoma, Cushing’s syndrome,
hyperparathyroidism
7
Q
What glands are implicated in MEN IIb or III
A
Pheo
Medullay thyroid hyperplasia
Medullar Ca and C cell hyperplasia of the thyroid
Mucosal neruomas
Marafanoid features
8
Q
What life threatening cancers are associated with MEN
A
Medullary thyroid
Pancreatic
9
Q
2 life threatening sequelae of MEN
A
- Hypertension
- Relentless peptic ulcer syndrome
10
Q
Testing for MEN (what to use and what not to use
A
To use
- Urinary catecholamine estimation
- Plasma calcitonin
Not
- Plasma calcium estimation
- Not serum ionized calcium/phosphate ratio
11
Q
What cells is medullary carcinoma of the thyroid derived from, what do they secrete
A
Parafollicular cells or C cells
Secrete calcitonin
- may also secrete serotonin and prostaglandin
- sometimes carcinoid, rarely ACTH
12
Q
Are MEN genetic
A
arise sporadically 80% of cases, the other 20% from genetic associations (MEN IIa & b)
13
Q
What gene is implicated in medullary thyroid carcinoma (when due to MEN)
A
germ line mutations in the RET proto-oncogenes
14
Q
What is the stroma of medullar carcinoma of the thyroid rich in
A
Amyloid
15
Q
Medullary thyroid carcinoma slow or fast growing
A
slow
16
Q
Biochemically what will people with medullary thyroid carcinoma have or not have
A
Will have high circulating calcitonin
WONT have
- Hypocalcemia
- Hypercalcemia
- Hypothyroidism
17
Q
What is the most common thyroid cancer in adults and children
A
Papillary carcinoma of the thyroid
18
Q
What is papillary thyroid carcinoma strongly assocaited with
A
Radiation - especially in childhood
19
Q
How do people with papillary thyroid cancer often present
A
With nodal metases (50% at time of diagnosis)
20
Q
What is the prognosis of papillary thyroid cancer
A
90% in 20 yrs > the best survival with thyroid neoplasia
21
Q
What dystrophic calcification is papillary thyroid cancer a/w
A
pasmmoma bodies
22
Q
What is the growth pattern of papillary thyroid cancer
A
papillary (often plus follicular) growth pattern > but co-existing with follicular DOES NOT worsen prognosis
23
Q
What hormone is papillary thyroid cancer dependent on and what implications does this have
A
TSH dependent > implications for therapy, relapse during pregnancy, etc…
24
Q
Where do carcinoid tumours arise from (structurally and what type of cell)
A
GIT (including pancreas),
Also bronchi
Thymus
Occasionally ovary
Neuroendocrine cells
25
When are carcinoid tumours often found
Incidentally at surgery or autopsy
26
What type of carcinoid tumours rarely metastaise
appendiceal, rectal carcinoids infrequently metastasize
27
What carcinoid tumours are frequently aggressive
ileal, gastric and colonic carcinoids are frequently aggressive
28
What is the main hormone released in carcinoid syndrome and what cancers is it associated with
Serotonin
- Hepatic mets
29
What are the 4 vasoactive substances that can be released in carcinoid syndrome + other rare hormones
1. serotonin
2. bradykinin & kallikreins
3. prostaglandin
4. histamine
ACTH or gastrin
30
Symptoms of carcinoid syndrome
1. cramping abdominal pains
2. facial flushes
3. tachycardia
4. pulmonary stenosis
5. tricuspid regurgitation
31
When is gastric cancer defined as early
When it is confined to the mucosa and submucosa
32
Post resection survival of gastric cancer
90-95% if confined to mucosa/sub mucosa
33
Risk factors for gastric cancer
diet
smoking
H. pylori infection
autoimmune gastritis
partial gastrectomy
34
Final common pathway for gastric cancer
dysplasia of the gastric mucosa
35
Locations of gastric cancer and their frequencies
50-60% at pylorus & antrum 25% cardia
15-25% body and fundus
36
Name the cancer that is gastric in origin and mets to the ovaries and the cell associated
Krukenberg (signet ring cell)
37
What type of cancer causes rigid thickened stomach wall
Linitis plastica
38
What type of polyps are benign
hyperplastic
juvenile
inflammatory
Peutz-Jeghers polyps
39
What types of polyps are neoplastic
adenomas
(tubular, villous and mixed tubulovillous)
40
Chance of an adenoma <1cm progressing to invasive cancer
1%
41
Chance of an adenoma 1-2cm progressing to invasive cancer
10%
42
Chance of an adenoma >2cm progressing to invasive cancer
45%
43
What 3 factors are implicated in the association between polyp and cancer
- Polyp size
- Relative proportion of villous component
- Degree of cell atypia (dysplasia) in the neoplastic cells
44
Peak incidence of colorecal carcinoma
60-70
45
Proportion of colorectal cancers located in rectosigmoid
<25%
46
Do most colorectal carcinomas occur in people with FAP or UC
No most occur in their absence
47
Is carcinoma insitu considered a malignant lesion and why/why not
- No
- Has not yet acquired attributes that will cause mets
- because lymphatic channels are largely absent from colonic mucosa, intra-mucosal carcinoma is regarded as having little or no metastatic potential
- invasion into submucosa indicates adenocarinoma which has now acquired metastatic potential > real in all case
48
What histological evidence suggests benign colonic epithelial neoplasia
1. adenoma with carcinoma in situ
2. adenoma showing severe epithelial dysplasia with focal intramucosal carcinoma
49
Why is Gardner's syndrome is a clinically more sinister variant of FPC
Aggressive neoplasms, other than colonic carcinoma, may determine mortality in Gardner’s syndrome
50
What is Gardners syndrome (what other things does it exhibit)
- An autosomal dominant familial polyposis variant
- It is an inherited disease that is characterised by gastrointestinal polyps, multiple osteomas (benign bone tumours), and various skin and soft tissue tumours.
- epidermal cysts, fibromatosis, abnormal dentition, higher frequency of duodenal and thyroid cancer
51
What is Gardners syndrome characterised by
lots of adenomatous polyps (minimu 100) in colon & elsewhere in the gut ! risk of becoming adenocarinoma 100%
52
How do you mitigate colon cancer risk in Gardners syndrome
Prophylactic colectomy
53
Average age of onset of Gardners syndrome and when does progression to cancer occur without surgery
- Teens to 20's
- in 10-15 years
54
Tumour marker for Gardeners syndrome
CEA
55
What side of the colon does Gardners cause cancer
Right
56
What is Turcots associated with
FPC and CNS tumours
57
What cancers/ gastric disorders are implicated by dietary factors
FPC
UC
58
What Genes are associated with Gardeners and which isnt
HNPCC
- NOT APC, DNA, K-ras, not DCC, or p53
59
Most common form of renal cancer
RCC
60
Most important risk factor for RCC
Smoking
61
When does RCC present
50-60
62
Is RCC more common in men or women
Men
63
Is RCC genetic
Most sporadic
4% can be rare autosomal dominant forms
64
Does RCC present early or late
Normally presents late with mets, sometimes they are solitary and stay so
65
Clinical manifestations/complications of RCC
1. amyloidosis
2. polycythemia
3. hypercalcemia
4. fever & cachexia
66
Paraneoplastic syndrome associated with RCC (6)
- polycythemia (EPO pure RBC blood volume increase)
- hypercalcemia
- hypertension
- masculinisation or feminization
- Cushing’ s syndrome
- Amyloidosis - AA type
67
Common site of spread for RCC
renal vein extension very common > haematogenous metastases are common
68
Describe the gross appearance of RCC, and what type of cell does it have lots of
- basic yellow-grey color, but variegated with haemorrhage, necrosis, cystic change, etc.
- High lipid content
69
Describe the gross appearance of Wilms tumour
Wilm’s tuour is usually of very uniform cut surface in contrast to the
variegated colours and consistency of RCC
70
Peak age of cervical cancer
40-45
71
Risk factors for cervical cancer
early of first intercourse
multiple sex partners
high risk male sex partners
married women
72
Main HPV types associated with cervical cancer
16, 18, 31, 33
73
Peak incidence of CIN III
~ 30 years
74
% of cervical cancer associated with HPV
85%
75
2 Main groups of testicular cancer
- germ cell tumours (95% of cases)
- nongerminal tumours (stromal or sex cord tumours)
76
Give 2 examples of germ cell testicular tumours
seminoma & teratoma are both germ cell tumours
77
Which is the most commone testicular germ cell cancer
Seminoma
78
Peak incidence of testicular seminoma, and which presents first, seminoma or teratoma
30 (older onset than teratoma)
79
What is the least aggressive of testicular germ cell cancer
seminoma
80
Compare seminoma vs NSGT with respect to radiosensitivity
Seminoma - Highly radiosenitive
NSGT - Radioresistance
81
Is hcg prognostic in testicular seminoma
- No
- Significance remains unknown
82
What are the most clinically important testicular tumours
seminoma and non-seminomatous germ cell tumours (NSGT)
83
Compare the growth and metastasis pattern of NSGT and seminomas
- seminomas remain confined to the testis longer and typically metastasise to LNs with blood spread occurring (when it does) as
a latera phenomenon
- NSGCT metastasize earlier and more frequently by blood stream
84
NSGT management post orchidectomy (if confined to organ)
Intensive surveillance without additional therapy
85
NSGT management for lesions out of the testes
Surgical resection
86
What are testicular teratomas made of and what behaviour do they exhibit
- Often occur as combined growth with seminoma or embryonal carcinoma
- complex mixtures usually have the behaviour of the worse component
- highly malignant
87
Where can extra-testicular teratomas occur
midline region from head to sacrococcygeal area
88
Karyotype associated with ovarian teratomas
46xx
89
Risk factors for prostate cancer
increasing age, race, hormonal influences, genetic factors, ?diet
90
How many patients gave advance cancer on diagnosis with prostate cancer
> 75%
91
Describe the relationship between PSA and prostate cancer
- Correlates well with total tumour volume
- also raised in hyperplasia and in early & localized cancer, so can’t be used alone for reliable early detection of cancer
92
What else causes a high PSA
levels may be elevated in BPH, prostatitis or prostatic infarct
so ~70-80% of men with raised PSA usually have one of these conditions rather than cancer
93
What is PSA
is a glycoprotein secreted by prostate cells into the ejaculate
94
How is prostate cancer best diagnosed
- DRE
- PSA serum level
- Transrectal U/S of prostate and biopsy
95
What biochemical marker is associated with metastatic prostate cancer
prostatic acid phosphatase
96
Where does prostate cancer start and where does hyperplasia occur
Prostate cancer starts in the peripheral zone (hence why cant biopsy)
BPH occurs in peri-urethral zone
97
Where does prostate cancer spread
- when hematogenous spread occurs > mainly to axial skeleton and produces predominantly ‘osteoblastic’ behaviour
- osteoblastic metastases virtually diagnostic of prostatic cancer in males (mets to bone)
98
Describe the relationship between cell differentiation and prostate cancer prognosis
there is good correlation between tumour differentiation and prognosis
99
Is hypercalcemia a feature of prostate cancer
No
100
What structure is commonly involved in local extension of prostate cancer
Seminal vesicles
101
What is prostate cancer commonly associated with (biochemically and common site of met)
- Raised serum acid phosphatase > indicate extra-prostatic invasiona/metastasis
- androgen dependency
- osteoblastic (osteosclerotic) bony metastases
102
Common occurences in advanced prostate cancer (3)
- spinal cord compression
- haematuria
- renal failure from bilateral ureteric obstruction
103
Follow up of localized prostate cancer following definitive therapy
- DRE
- PSA
104
Treatment choices for prostate cancer (5)
1. no initial treatment
2. external beam radiotherapy alone
3. brachytherapy alone
4. external beam radiotherapy + brachytherapy
5. surgery (radical prostatectomy)
105
What factors may influence treatment choice for prostate cancer (4)
1. age of patient >70 yrs with co-morbidity is likely to die from co-morbid diseases, rather than cancer
2. co-morbid illnesses
3. patient preference > as treatment affects continence, rectal irritation, impotence, etc...
4. grade of cancer
- well differentiated > excellent prognosis
- mod-severe differentiated > definite benefits with treatment
106
Is oral anti-andogren therapy alone sufficient for treatment of prostate cancer/ what is its role
No
Only tend to eradicate additional 5-10% of male androgens (which are adrenal derived as opposed to testes)
107
What is the mainstay of prostate cancer treatment
testosterone deprivation therapy
80% are responsive to this
- Surgical (bilateral orchidectomy)
- Medical (LHRH agonist)
Dont need both
108
Side effects of testosterone deprivation therapy (5)
hot flushes
impotence
weight gain
gynaecomastia
mood disturbances
109
What is a neuroblastoma/ where does it occur
Childhood cancer (not a CNS tumour)
- occurs in the adrenal, but arises in primitive cells (neuroblasts) of neural tube origin
- may mature to ganglioneuroma or regress
110
Growth pattern of neuroblastomas
Highly aggressive > have often metastasized at the time of diagnosis
111
What do neuroblastomas secrete
90% of them secrete catecholamines, but HTN is rate
112
Hisotological feature of medulloblastomas and gross appearance
Rosette formation
often well circumscribed, gray, friable, usually extrememly cellular
113
Where does a medulloblastoma form (cell type and location)
- Primitive neurons (ot adrenal medulla)
- Midline in children, more lateral in adults
- Often found near the 4th ventricle
114
Growth pattern of medulloblastomas and where do they spread to
Grow rapidly, highly malignant, poor prognosis if untreated
Near 4th ventricle so spread to CSF and associated with hydrocephalus
115
Proportion of childhood brain tumours that occur exclusively in the cerebellum
20%
116
Treatment and prognosis for medulloblastoma
exclusively radiosensitive
total excision + radiation 75% survival, 5 yrs
117
Inheritance genetics of familial retinoblastoma
######
118
When do wilms tumours present
2-5
119
Are wilms tumours malignant or benign and what is their prognosis
Malignant but good survival >90%
120
Are Wilms tumours genetic or sporadic
Some sporadic but most are genetic and associated with abnormalities in chromosome 11p.
121
3 Malformation syndromes associated with WIlms
- WAGR (Wilms tumour, aniridia, genital anomalies, mental retardation)
> patient with this syndrome have 33% chance developing Wilm’s tumour
> Due to deletions on chromosome 11p band 13 of the Wilms tumour 1 (WT-1) gene
- Denys-Drash syndrome
- Beckwith-Wiedemann syndrome
122
What is hepatoblastoma
- a rare form of primary liver cancer
- a tumour of young childhood, usually fatal within a few years if not resected
123
What are the two anatomic variants of hepatoblastoma
- epithelial (fetal, well differentiated, embryonal, macrotrabecular) > form acini tubules etc vaguely recapitulating liver development
-
- mixed epithelial & mesenchymal type, which contains foci of mesenchymal differentiation > may consist of primitive mesenchyme, osteoid, cartilage, or striated muscle
124
Prognosis of non gestational choriocarcinoma
Terrible prognosis
125
How common is Hydatidform mole
1 in 2000 pregnancies
126
How do you monitor therapy and progress for gestational trophoblastic neoplasia
Plasma HCG
127
How do you treat gestational trophoblastic neoplasia
Highly responsive to chemo
128
Growth pattern of choriocarcinoma of gestational origin
Mets early and widely
129
What happens if you dont treat choriocarcinoma of gestational origin
if untreated, parallels the behaviour of primary ovarian choriocarcinoma
130
What are the clinical manifestations of acute leukaemia (6)
- Anaemia with pallor & fatigue
- Infection with fever
- Thrombocytopenic bleeding > petechii/ecchymoses, epitaxis, gingival
- ALL >> AML for lymphadenopathy, hepatosplenomegaly > leukaemic infiltration
- Bone infiltration (medullary, subperiosteal) > with resorption & pain + hypercalcemia common
- CNS involvement common + mediastinum (ALL); chloromas (AML)
131
Side effect of successful treatment of acute leukemia
successful therapy may precipitate acute gout > because successful therapy > rapid tumour lysis > hyperuricemia > induce symptomatic gout
132
What chromosomal abnormalities are associated with ALL and which have a better prognosis
- hyperdiploidy common - better prognosis
- All translocations are a/w a poor prognosis
133
Cell types associated with ALL and which have a better prognosis
- Most are B cell lineage
- T cell type more commonly has mediastinal mass > poor prognosis
134
Growth profile of ALL, prognosis (comparing kids and adults)
- highly aggressive, but in children responds to therapy
- 80% remission, 60% 5yr ‘cure’ (in kids)
- adult response generally poor
135
What is the most common acute leukemia
ALL (85%)
136
Who gets AML
young adults
137
Important marker for AML
terminal deoxynucleotidyl transferase (TdT) – DNA polymerase is an important marker
138
Prognosis of AML
- remission is common
- relapse is the usual long-term outcome <20% ‘cure’
139
Chronic myeloid leukemia
Myeloproliferative disorder
140
Who gets CLL
Elderly - indolent course
141
Genetic associations of CLL
.Deletions as opposed to translocations like most lymphoid malignancies.
142
What cell type predominates in PRV
- erythroid precursors dominate with absolute increase in RBC mass + WBC + platelets
143
Clinical feature of PRV
increased blood viscosity with vascular stasis & thromboses
144
What occurs with prolonged survival in those with PRV
- may develop ‘spent phase’ > develop a myeloid metaplasia/myelofibrosis picture
- treated patients (alkylating agents) > may develop terminal AML
145
What is myelofibrosis
Clonal proliferation of stem cells (mainly megakaryocytes) that secrete cytokines such as platelet derived growth factor. This results in a reactive fibrosis (not clonal) of the bone marrow. This leads to impaired haematopoesis, cytopenis and extensive extra medullary haematopoesis.
146
Cells seen in peripheral blood of patient with myelofibrosis
1. normoblasts
2. myeloblasts
3. myelocytes
4. NOT megaloblasts
(see tear drop red cells
poikilocytosis and a leukoerythroblastic cell i.e immature cells, like nucleated red blood cells)
147
What is multiple myeloma
− Cancer of plasma cells, should only make up 1% of bone marrow.
− Plasma cells produce different types of antibodies (immunoglobulins) to fight a variety of infections.
− Malignant plasma cells only release one type of immunoglobulin-paraprotein.
(Most common is igG, followed by igA
- Immunoglobulins can be complete or INCOMPLETE (i.e light chains)
148
Clinical features of multiple myeloma (including main causes of death)
Clinical features stem from
1. effects of plasma cell growth in tissues.
2. Production of excessive igG which have abnormal properties.
3. Suppression of normal humoral immunity
- Bone resorption: Fractures, pain hypercalcemia.
- Hyperviscosity, esp. IgA > polymers (from excess Ig production)
- Amyloidosis (Associated with delta light chains in particular)
Main causes of death
- Reduced igG results in increased susceptibility to bacterial/ pyogenic infections. Essentially - T cell functions, well preserved, therefore, viral infection not a problem.
- Renal failure: Multifactorial, but most importantly Bence jones proteins (light chains that are toxic to tubules) and amyloidosis
149
What contributes to renal failure in multiple myeloma (5)
1. amyloidosis (often complicated MM)
2. renal tubular protein casts
3. protein deposition - Bence Jones protein (blocks renal tubules)
4. pyelonephritis (increased susceptibility to pyogenic organisms)
5. hypercalcemia (due to skeletal demineralization)
150
What is Waldenstorms macroglobulinaemia
IgM production by a lymphoma-like malignancy + hyperviscosity & cryoglobulinaemia
151
Pathology of Hodgkins lymphoma (cell type histologically etc)
T cell
- Reed-sternberg cells > malignant large mononuclear cells
- plus large numbers of reacting lymphocytes, plasma cells, and others
-. giving characteristics pleomorphic microscopic appearance
152
5 subtypes of Hodgkins lymphoma
1. Nodular sclerosis
2. Mixed cellularity
3. Lymphocyte rich
4. Lymphocyte depletion
5. Nodular lymphocyte predominance
153
Prognosis and side effects associated with treatment of Hodgkins lymphoma
- excellent outlook; tumour burden correlates inversely with prognosis
- But therapy is followed by risk of ‘second cancer’ – leukaemia, lung cancer, & others
154
Definition of MGUS
Asymptomatic patients with serum M protein <3 g/dL
i.e no CRAB
1% progress to a plasma neoplasm (usually myeloma)
Exhibit the same mutations as multiple myeloma
Progression to myeloma is unpredictable
155
What is smouldering myeloma
Middle ground between MGUS and multiple myeloma
- Serum M protein >3 but patients are asymptomatic
75% of people progress to multiple myeloma
156
What 2 haematological conditions are closely associated with massive splenomegaly
CML
Myelofibrosis
157
What tyrosine kinases are implicated in myeloproliferative disorders
JAK2 (PV, ET, MF)
BCR-ABL (CML)
158
What is ET
- platelets due to megakaryocte proliferation
- Clinically: recurrent haemorrhage and
thrombosis. splenomegaly
- Laboratory: numerous large platelets with
hypercellular marrow.
159
Treatment for PRV and ET
Venesection, aspirin +/- cytoreduction
(usually hydroxycarbamide AKA
hydroxyurea)
160
Compare and contrast Hodgkins lymphoma with NHL with respect to
- Location
- Spread
- Node involvement
- Extra-nodal presentation
Hodgkins
- Localized to a single axial group of nodes (cervical, mediastinal, para-aortic)
- Spread by contiguity
- Mesenteric nodes and Waldeyer ring rarely involved
- Extranodal presentation rare
NHL
- More frequent involvement of multiple peripheral nodes
- Non contiguous spread
- Extra-nodal presentation common
161
Cells in AML (microscopic)
Auer rods
162
What do Reed-sterburg cells do
Release factors that induce accumulation of reactive lymphocytes, macrophages and granulocytes (make up 90% if tumor cellularity)
163
How is Hodgkins lymphoma staged
1. Single lymph node region or single extra-lymphatic origin or site
2. 2 or more regions on same side of diaphragm or localized involvement of an extra-lymphatic organ
3. Both sides of diaphragm with or without involvement fo extra-lymphatic organ or site
4. Diffuse involvement of one or mote extra-lymphatic organs with or without lymphatic involvement
164
What subtypes of HL tend to have what stage
Nodular sclerosis - I or II, tend to be free of systemic manifestations
Mixed cellularity or lymphocyte depletion - III and IV, more likely to have systemic symptoms
165
How does HL tend to spread
Nodal disease
Splenic disease
Hepatic
Marrow
Other tissues
166
What is MDS
Clonal stem cell disorders characterised by maturation defects that result in ineffective haematopoiesis and high risk transformation to AML.
I.e there is a clonal progeny that is able to differentiate but it does it ineffectively
167
What are Myeloproliferative disorders
Mutated tyrosine kinase (or other signalling pathways) that leads to growth factor independent proliferation and survival of marrow progenitors. Differentiation is not impaired so tend to get mature blood elements.
Results in
- Increased proliferative drive of marrow
- Homing of neoplastic stem cells to secondary haemoatopoteic organs
- Variable transformation to spent phase (marrow fibrosis and cytopenias)
- Variable transformation to acute leukemia.
168
What gene/ chromosome is implicated in CML
- BCR gene on chromosome 22 an - - ABL gene on chromosome 9
- Most cases of CML (more than 90%) are caused by the Philadelphia chromosome. Translocation between 22 and 9 (molecular weight of 210)
- majority at least, of the remainder, show the same EFFECTIVE genetic transfer by way of fusion of abl oncogen with bcr
gene on chromosome 22
169
Marrow of CML
very hypercellular with every type of myeloid cel increased with a relative emphasis on the granulocyte lineage rather than the red cell lineage. Platelets and megakaryocytes are also usually
increased.
Sea blue histocytes are characteristic (macrophages)
170
Blood film of CML
greatly increased WBC, often in the 100s, and predominantly
neutrophils and myelocytes.
171
Cell appearance MDS
Dysplastic differentiation!
- Ringed sideroblasts
- Eythroblasts with iron laden mitochondria
- Megaloblasts
- Dhole bodies
- Pseudo-pelger-huet
172
What is CML
The myeloproliferative disorder where there are too many granulocytes and their precursors.
- Unlike acute myeloid leukaemia, we see predominantly mature granulocytes and not an increased number of immature myeloblasts.
173
Is CML pluripotent and if so how do we know
Yes, because you can get lymphoid blast crises or myeloid blast crises.
174
Marrow of PCV
Hypercellular
increase in red cell progentiors is subtle and accompanied by increase in granulocytic and megakaryocytes as well
175
Marrow of ET
Cellularity only mildly increased but you see megakaryocytes
176
What is pathopneumonic for CLL and what other cells are present
Proliferation centres
Smudge cells
lymphocytosis, sometimes as high as several hundred. The malignant lymphocytes are small and rather nondescript, resembling ordinary lymphocytes. The nuclear chromatin pattern is “condensed” suggesting the cell is not dividing very rapidly.
177
Marrow of CLL
Lymphocytes are increased in the marrow, usually massively. In this example we see a hypercellular, diffuse infiltrate. There may be other cytopenias as a result, e.g. anaemia, thrombocytopenia and absolute neutropenia.
178
What is the only difference between CLL and SLL
Degree of peripheral blood lymphocytosis
i.e major overlap of clinical & pathological features with small lymphocytic lymphoma
179
Immunophenotype of CLL/ SLL
- pan B cell markers (CD19, CD20),
- CD5
- surface Ig (usualy igM or igM and igG)
- no TdT or CD10 - i.e. ‘early’ B cell markers are lost
- express kappa or delta, light chains, but NOT both
180
is CLL T or B cell
95% are B cell neoplasms > T cell CLL is rare
181
Clinical presentation of CLL
Normally asymptomatic
When symptoms appears - non specific, fatigue, weight loss
182
Complications of CLL
- Disrupts normal immune function through uncertain mechanisms.
- Hypogammaglobunlinemia is common and contributes to increased susceptibility to infection
- Haemolytic anemia
- Thrombocytopenia
- Transformation into more aggressive cancer - DLBCL (Ritcher syndrome)
- Transformation to blast cell crisis is rare
183
Poor prognostic features associated with CLL
11q and 17p deletions
ZAP-70 protein expression
184
How do you differentiate CML from a leukemoid reaction
Total lack of leukocyte alkaline phosphatase
185
Exposure to what 2 substances increases your risk of bladder cancer and 2 that dont
1. cigarette smoke
2. 2-naphthylamine
3. NOT ! asbestos or lead
186
5 core biopsy results that need excisional biopsy to exclude or diagnose associated cancer
1. a papillary lesion
2. atypical ductal hyperplasia
3. intraduct papilloma
4. radial scar
5. ductal carcinoma in situ
187
2 things that increase your risk of breast cancer and 2 that dont
1. positive family history for breast cancer
2. obesity
NOT ! early first pregnancy, or late menarche
188
4 facts about gallbladder cancers
1. are adenocarcinomas
2. clinical presentation is typically insidious and indistinguishable from the symptoms and signs of benign gallbladder disease !
palpable enlargement of the GB is distinctly unusual
3. have invaded the liver at the time of operation
4. are NOT always a/w presence of gallstones
189
Lymph node biopsy from patient with NHL will show/ not show what 4 things
1. Will NOT show Reed Sternberg cells
2. is unlikely to be composed of malignant T cells than B cells
3. will contain reactive lymphocytes of the same lineage (i.e. T or B) as the neoplastic cells
4. will contain a monoclonal population of tumour cells
190
