haem malignancys - step 2 Flashcards

(69 cards)

1
Q

chemo regimen for hodgkins lymphoma

A

AVBD
adriamycin (doxorubicin)
vincristine
bleomycin
dacarbazine

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2
Q

management of AML

A

ALL (non-acute promyelogenic subtype): chemotherapy +/- bone marrow transplant

APL: all trans retinoic acid with arsenic trioxide

(ATRA is a vitamin A derivative that promotes differentiation of immature malignant promyelocytes into mature granulocytes)

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3
Q

how does blood transfusions affect calcium levels

A

multiple blood transfusions can cause hypocalcaemia
blood products often contain citrate which calcium binds to = low free calcium

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4
Q

contraindication for the use of rasburicase for tumour lysis syndrome

A

G6PD deficiency

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5
Q

how can leukostasis syndrome in ALL/AML be managed

A

hydroxyurea +/- leukopharesis which decreases cell counts

leukostasis can occur when WCC is very high >100,000 resulting in CNS infarcts, pul hypertension, DIC etc

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6
Q

indicators of poor prognosis of ALL

A

age < 1 yr or >10 yr
increase in WBC >50,000
philadelphia chromosome t(9,22)
CNS involvement at diagnosis

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7
Q

indicators of poor prognosis for AML

A

age > 60yrs
elevated LDH
poor risk or complex karyotype

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8
Q

smudge cells on blood smear

A

CLL
(indicates crushing of fragile leukaemic cells)

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9
Q

flow cytometry markers diagnostic of CLL

A

CD5, CD23

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10
Q

most accurate diagnostic test for suspected CLL

A

flow cytometry - shows monoclonal B cells with CD5, CD23

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11
Q

most accurate investigation for diagnosing suspected AML or ALL

A

bone marrow biopsy with flow cytometry

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12
Q

management for CLL

A

is asymptomatic then follow-up to monitor every 3-6 months

if symptomatic:
- Ibrutinib (BTK inhibitor)
- rituximab (anti-CD20 monoclonal Ab)
- venetoclax (anti-BCL 2 therapy)

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13
Q

describe the natural course of untreated CML

A

3 phases;
(1) chronic phase: typically lasts 3.5 - 5 yrs, asymptomatic

(2) accelerated phase: transition towards blast crisis, with rapid increase in peripheral and bone marrow blast counts. suspected when there is an abrupt increase in basophils and thrombocytopenia

(3) acute phase: resembles AML, survival 3-6 months

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14
Q

in CML, what myeloid cell is found to be elevated which differentiates CML from normal leukaemoid reaction to infection

A

increase in basophils

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15
Q

most accurate test for diagnosing suspected CML

A

philadelphia chromosome by PCR or flouroscene insitu hybridization (FISH) analysis showing t(9;22) translocation

or

BCL/ABL PCR or northern blot

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16
Q

how can CML be diffeentiated from leukaemoid reaction

A

leukaemoid reaction = normal reaction in response infection etc

in CML
- leukocyte alkaline phosphatase (LAP) will be absent/low
- elevated basophils (basophillia)

in leukaemoid reaction
- LAP elevated
- normal basophils

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17
Q

management of CML

A

tyrosin kinase inhibitors i.e. Imatinib

if young then consider allogenic stem cell transplant if matched biling donor available

if blast crisis then treat as acute leukaemia with chemotherapy

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18
Q

chromosomal translocation associated with APL

A

T(15;17)

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19
Q

myeloperoxidase positive - myeloid or lymphoid origin ?

A

myeloid

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20
Q

terminal deoxynucleotidetransferase (Tdt) - myeloid or lymphoid origin ?

A

lymphoid

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21
Q

management for hairy cell leukaemia

A

best initial: Cladribine
other options: rituximab, pentostatin, interferon (INF-a)

‘TRAPped in a hairy situation with CLADia (cladribine)’

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22
Q

what cell type is hair cell leukaemia

A

well-differentiated B cell lymphocytes

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23
Q

patient presents with waxing and waning lymphadenopathy - most common haem malignancy that would present like this ?

A

follicular lymphoma

indolent course or low grade
t(14:18)

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24
Q

management of follicular lymphoma

A

most often requires surveillence

localised disease may be treated with radiotherapy

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25
how does diffuse large B cell commonly present and the genetics found
single rapidly growing mass BCL2, BCL6, MYC
26
management of diffuse large B-cell lymphoma
most common non-hodgkins lymphoma good survival rate with R-CHOP rituximab, cyclophosphamide, doxorubicin (hydroxydonomycin), vincristine (oncovin) and prednisolone
27
adolescent boy immigrated from africa presents with growing mass on the jaw ?diagnosis
burkitts lymphoma presents with abdominal mass in non-immigrants associated with EBV
28
stary sky appearance on bone marrow biopsy
burkitts lymphoma
29
treatment for burkitts lymphoma
chemotherapy
30
t(11:14) + CD5 positive
mantle cell lymphoma
31
best initial test for suspected lymphoma
excisional lymph node biopsy
32
blood values of tumour lysis syndrome
hyperuricaemia hyperkalaemia hyperphosphataemia hypocalcaemia
33
female patient is chemo + radiotherapy for lymphoma. what further imaging should be undertaken ?
mammogram to screen for breast cancer as a treatment complication
34
in general terms, what is the treatment approach for non-hodgkins lymphoma
NHL can be treated with chemo and/or radiotherapy low grade - palliative high grade - chemotherapy (i.e. R-CHOP) with curative approach
35
most common type of hodgkins lymphoma in younger (30s) vs older (60s)
bimodal age distrubution younger age - nodular sclerosing type older age - lymphocyte-depleted type
36
in the treatment of hodgkins lymphoma the use of radiotherapy increases the risk of what conditions
hodgkins typically occurs above the diaphgram so radiotherapy would cause hypothyroidism, premature coronary artery disease and solid tumours i.e. lung, breast + thyroid
37
hodgkins lymphoma stage II vs stage III
stage II - nodal disease localised to one side of the diaphragm once nodal involvement infradiaphragmatic = disseminated disease
38
CD type found in multiple myeloma
monoclonal CD138 + plasma cells on bone marrow biopsy
39
gamma gap in multiple myeloma
gamma gap = total serum protein - albumin elevated in MM elevated if > 4
40
what conditions can increase M protein
multiple myeloma MGUS CLL lymphoma amyloidosis
41
why might skeletal survey (bone scan) in the investigation of MM be negative
bone scans detect osteoblast activity. MM bone disease is causes by osteoclast process therefore has a high false negative rate
42
management of MM
chemotherapy + bone marrow transplant - cyclophosphamide - daratummumab (anti-CD 136) - dexamethasone - bortexomib - lenalidmoide
43
features of waldenstrom macroglobinaemia
clonal disorder of B cells that leads to malignant monoclonal gammopathy elevated IgM which leads to; - hyperviscosity syndrome - coagulation abnormalities - cryoglobinaemia - cold agglutins (leading to AIHA) - amyloidosis
44
MGUS vs waldenstrom macroglobinaemia
both clonal disorder of B cells leading to monoclonal gammopathy MGUS becomes WM when symptoms/complications arise such as anaemia, cryoglobinaemi etc
45
management of waldenstrom macroglobinaemia
rituximab + chemotherapy for symptomatic disease plasmaphoresis to remove excess immunoglobulin in patients who present with hyperviscosity syndrome
46
bone marrow shows Dutcher bodies on PAS stain
waldenstrom macroglobinaemia plasma cells with Dutcher bodies (PAS positive for IgM deposits around the nucleus)
47
65 year old patient presents with tiredness, fatigue, blue hands on exposure to the cold, peripheral neuropathy. o/e: palpable spleen and engorged eye vessels. blood shows elevated IgM, ESR, LDH and uric acid. ?next step ?differential
bone marrow bipsy waldenstrom macroglobinaemia pt showing signs of; - neuropathy - anaemia - hyperviscosity syndrome - cryoglobinaemia bone marrow will show plasma cells with Dutcher bodies (PAS positive IgM deposits around nucleus) manage with rituximab and chemo
48
most accurate test for diagnosing amyloidosis
tissue biopsy with congo red staining showing apple green perfringence under polarised light
49
treatment of amyloidosis
primary amyloidosis (AL): chemotherapy + autologous stem cell transplant secondary amyloidosis (AA): treat underlying cause
50
autologous vs allogenic stem cell transplant
autologous - stem cells from self allogenic (allo = other) - stem cells from donor
51
what type of amyloidosis is associated with myeloma and waldenstrom macroglobinaemia
primary amyloidosis - plasma cell dyscrasia with deposition of monoclonal light chain fragments - treat with chemo +/or autologous stem cell transplant
52
what conditions is secondary amyloidosis associated with
deposition of acute phase reactant serum amyloid A autoimmune i.e. rheumatoid arthritis infection neoplasma
53
deposition of what substance is found in dialysis related amyloisosi
B2 microglobulin
54
deposition of what substance is found in secondary amyloidosis
acute phase reactant serum amyloid A
55
deposition of what substance is found in primary amyloidosis
monoclonal light chain fragments
56
deposition of what product is found in hereditary amyloidosis
deposition of abnormal gene products i.e. transythrin also known as prealbumin
57
deposition of what product is found in senile amyloisosi
deposition of otherwise normal tranythrin
58
syngeneic stem cell transplant
stem cells from genetically identical twin
59
how does graft vs host disease present
can be acute < 100 days or chronic GII problems cholestatic liver dysfunction skin changes obstructive lung disease
60
management of graft vs host disease
high dose corticosteroids
61
antibiotic regimen to prevent infection in the immunosuppressed post-transplant patient
co-trim, ganciclovir + fluconazole
62
immunosuppression regimen for post transplant patient
prednisolone mycophenilate mofitil (MMF) FK506 (tacrolimus) co-trim, ganciclovir + fluconazole
63
what is the graft vs leukaemia affect
subtype of graft vs host disease in which patients who receive allogenic stem cell transplant have reduced relapse rate in comparison to autologous stem cell transplants thought to be caused by recognition of leukaemia by the donor T cells
64
management of hyperacute, acute and chronic transplant rejection
hyperacute: cytotoxic agents acute: confirm with biopsy, steroids, additional immunosuppression chronic: biopsy to rule out treatable acute reaction
65
12 month old infant presents with fever, rash, lethargy and poor intake. O/E: hepatomegaly. bloods show pancytopenia, elevated Scd25 and CXCL9. ?next step ?diagnosis ?management
bone marrow biopsy; haemophagocytosis diagnosis; haemophagocytic lymphohistocytosis management: - dexamethasone + etoposide - antibiotics - supportive care i.e. fluid resuscitation, transfusion etc
66
patient presents with red/brown maculopapular cutaneous lesions, fatigue and pallor. O/E: splenomegaly. Biopsy shows elevated tryptase >20. what ther finding would you expect on biopsy given the likely diagnosis ? diagnosis ?management
mastocytosis biopsy with KIT stain shows elevated mast cells, KIT mutation and tryptase >20 bone marrow biopsy may also show cytopenias due to mast cell crowding of marrow treatment aimed at preventing mast cell degranulation with antihistamines, cromolyn and anti-leukotrienes
67
IgM (M protein) level suggestive of MGUS
< 3
68
Sezary syndrome
diffuse erythroderma, will have history of erythematous plaques/tumours of the skin (mycosis fungoides). Associated with end-stage mycosis fungoides manage with total skin electron bean radiotherapy (TSEBT)
69
most common lymphoma type CLL can transform into
diffuse large B-cell (Ritcher syndrome)