cancers of the blood Flashcards

1
Q

what is multiple myeloma?

A

malignant disorder of plasma cells - b cells

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2
Q

what occurs within MM?

A

excessive secretion of monoclonal AB

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3
Q

what is the path of MM and the monoclonal AB?

A

excessive AB secretion
1. development of monoclonal gammopathy of undetermined significance MGUS
2. progression to MM

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4
Q

what is MGUS?

A

where most cases of MM arise from?
1. inciting event: initial cytogenic event abnormality - abnormal plasma cells clone secreting AB
2. progression to MGUS to MM: more cytogenic changes including change to bone marrow - bone cell infiltration

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5
Q

what are the clinical features of MM?

A

C: calcium - high
R: renal impairment
A: anaemia
B: bone disease

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6
Q

at what point is hypercalcaemia a medical emergency?

A

greater than or higher to 2.9mmol/L

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7
Q

why is anaemia seen in MM?

A

EPO deficiency

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8
Q

how is bone disease seen on imaging?

A

lytic lesions

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9
Q

what are the red flag symptoms seen in MM?

A

weight loss
anorexia
generalised weakness and excessive fatigue

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10
Q

apart from CRAB, what other features are seen in MM?

A

paraesthesia, splenomegaly, hepatomegaly, lymphadenopathy, neuro conditions: hyperviscosity conditions, spinal cord compression

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11
Q

what is hyperviscoisty syndrome?

A

headache, blurred vision, SoB, mucosal bleeding

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12
Q

how do you screen for MM?

A

investigate for monoclonal AB- protein electrophoresis and immunofixation

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13
Q

what immunoglobulins are assessed for?

A

igM - electrophoresis
immunofixation - higher ratio of heavier chains

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14
Q

how do you manage bony pain in MM?

A

bisphosphonates

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15
Q

what is ALL classically associated with?

A

most common in paeds
down syndrome

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16
Q

what is CLL most associated with?

A

warm haemolytic anaemia, Richter’s transformation and smudge cells

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17
Q

what is ritcher transformation?

A

CLL can transform into into aggressive lymphoma

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18
Q

what is CML linked to?

A

three phases - long chronic phase
philadelphia chromosome

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19
Q

what is AML linked to?

A

transformation from myeloproliferative
auer rods

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20
Q

what is burkitts lymphoma?

A

high grade B cell neoplasm

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21
Q

what are the two major forms of burkitts lymphoma?

A

endemic - african - maxilla/ mandible
sporadic- ileo caecal - usually HIV pts

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22
Q

what is burkitts associated with?

A

c-myc gene translocation t(8:14)
EBV linked to african form

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23
Q

what would microscopy show in burkitts?

A

starry sky
lymphocyte sheet interspaced with macrophages containing dead apoptic tumour cells

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24
Q

how is burkitts managed?

A

chemo - which can cause tumour lysis syndrome

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25
what would indicate tumour lysis syndrome?
high k+ high phosphate low ca2+ acute renal failure hyperuricaema
26
what can help prevent tumour lysis syndrome in chemo for burkitts?
rasburicase
27
what is hodgkins lymphoma?
malignant proliferation of lymphocytes
28
what is seen on microscopy for hodgkins?
reed-sternberg cells
29
what age is hodgkins lymphoma most common
20s and 60s
30
what are RF for hodgkins lymphoma?
EBV HIV
31
how does hodgkins lymphoma present?
lymphadenopathy - cervical/ supraclavicular - then axilla then inguinal painless, non tender B symptoms mediastinal mass - cough, weird CXR
32
what is seen with alcohol and hodgkins lymphoma?
alcohol induced pain
33
what are B symptoms ?
weight loss fever night sweats pruritic
34
what investigations are done for hodgkins lymphoma and what is seen?
FBC - normocytic anaemia hypersplenism coombs +ve eosinophilia high LDH lymph node biopsy - reed sternberg cells
35
why is there eosinphillia in hodgkins?
more cytokines eg IL- 5
36
which hodgkins prognosis is worse?
lymphocyte depleted B symptoms - usually poor prognosis
37
which hodgkins prognosis is best?
lymphocyte predominant
38
what are the different chemo combos in hodgkins lymphoma?
ABVD: standard BEACOPP: more toxic but more likely to go into remission
39
what are the management options of hodgkin lymphoma?
chemo radiotherapy combined chemo then RT haemotopoietic cell transplantation - relapsed/ refractory hodgkins
40
what is differenced between non hodgkins and hodgkins?
hodgkins - reed sternberg non- hodgkins - everything else
41
what is non-hodgkins lymphoma?
malignant proliferation of lymphocytes in lymph nodes or other organs can affect B/T cells
42
what is the 6th most common cancer in the UK?
non-hodgkins
43
who is affected more in non-hodgkins?
men more then females
44
what are RF for non-hodgkins?
elderly cauasians EBV FamHx chemical exposure - pesticides, solvents previous chemo/ RT immunodeficiency - transplant, HIV, DM autoimmune conditions - sjogrens, SLE, coeliac
45
how does non-hodgkins present?
non tender painless lymphadenopathy - rubbery, asymmetrical B symptoms other systems - gastric - abdo pain, dsyphagia, bone marrow - bone pain, pancytopenia testicular masses fever
46
what investigations are needed within non-hodgkins?
excisional biopsy CT TAP ESR - prognostic indicator HIV test LDH FBC - normocytic anaemia PET CT
47
what is LDH?
lactate dehydrogenase cell turnover - prognostic indicator
48
how is non-hodgkns managed?
specific to sub type rituximab in combo neutropenia - prophylactic ABx flu/ pneumococcal vax
49
whoch grades have best prognosis in non-hodgkins?
low grade - better prog high grade - worse prog but better cure rate
50
what are complications of non-hodgkins?
bone marrow infilitration spinal cord compression Superior vena cava obstruction side effects of chemo mets
51
what is ALL?
acute lymphoid leukaemia neoplastic malignant proliferation of lymphoblasts - immature lymphoblasts accumulate in bone marrow usually B cell
52
what are RF for ALL?
young age B cell - peaks at 3yrs T cells - peak at 15-20 down syndrome radiation exposure
53
what are signs of ALL?
abrupt onset anaemia - fatigue, SoB, pallor neutropenia - fever, sepsis, pneumonia bone pain hepatosplenomegaly lymphadenopathy
54
how is ALL diagnosed?
FBC/ blood film: more lymphoblasts high WCC BM film: lymphoblast dominance - starry sky, mitotic figures immunophenopathy- +ve nuclear staining
55
how is ALL distinguised from ALL on the investigations?
ALL - immunophenotyping is +ve AML: -ve
56
how is ALL managed?
aggressive chemo - more successful in kids if brain mets: intrathecal chemo/ RT tyrosine inhib: imatinib, dasatinib
57
what is the ALL/ AML ABCDEF ?
A - acute B- blast prodinate C: children D: drastic course E; elderly F: fair few WBC and fever
58
what is AML?
neoplastic monoclonal prolif of myeloblasts in BM - accumulate of myeloblast
59
what are RF of AML?
adults - peaks at 60yrs radiation/ chemo down syndrome - <5yrs myeloproliferative disorders
60
what are complications of AML?
DIC
61
what are signs of AML?
abrupt onset anaemia thrombocytopenia neutropenia leukaemia cutis gum swelling
62
what is leukaemia cutis ?
skin infiltration of aml
63
how is AML diagnosed?
FBC/ blood film - high leucocytes, anaemia BM smear: high myeblasts. auer rods
64
how is aml managed?
chemo promyelotic - all trans retonic acid treatment BM transplant
65
what is CLL?
chronic lymphoid leukaemia neoplastic monoclonal proliferation og fucntionally abnormal B cells
66
what can cause CLL?
chromosome abnormalities mutations in tyrosine kinase pathways
67
what are RF of CLL?
fam history agent orange exposure
68
what are symptoms of CLL?
late onset anaemia thrombocytopenia neutropenia neoplastic infilitration
69
what are complications of CLL?
abnormal iG secretion - hypogammaglobulinemia, autoimmunity ritcher syndrome - progress to aggressive lymphoma
70
how is CLL diagnosed?
FBC/ blood film - lymphocytosis, smudge cells immunophenotyping - CD5, CD20, CD23 lymph node biopsy - small round lymphocyte infilitration
71
what are smudge cells ?
disruption of fragile membranes of abnormal lymphocytes
72
how is CLL managed?
chemo/ RT BM transplant immunotherapy
73
what is CML?
chronic myeloid leukaemia monoclonal proliferation of mature granulocytes/ precurosrs
74
what are granulocytes?
eosinophils basophils neutrophils
75
what Chr is linked to CML?
philadeplphia cheese - C milk - M
76
what are RF for CML?
adult - above 40 radiation exposure benzene exposure
77
what are the three phases of CML?
chronic accelerated blast crisis
78
what occurs within chronic phase of CML?
mainly neutophil dominance B symptoms
79
what occurs within accelerated phase of CML?
more basophils anaemia hepatosplenomegaly lymphadenopathy recurrent infections bleeding petechiae ecchymose
80
what is ecchymose?
bruises
81
what occurs within blast crisis of CML?
rapid progression - poor prog more myeblasts bone pain fever significant splenomegaly
82
how is CML diagnosed?
more granulocytes BMM biopsy: hypercellularity of myeloid
83
how do you manage CML?
tyrosine kinase inhib: imatinib, dasatinib nilotinib BM transplant
84