haem malignancys - step 2 Flashcards
(69 cards)
chemo regimen for hodgkins lymphoma
AVBD
adriamycin (doxorubicin)
vincristine
bleomycin
dacarbazine
management of AML
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)
how does blood transfusions affect calcium levels
multiple blood transfusions can cause hypocalcaemia
blood products often contain citrate which calcium binds to = low free calcium
contraindication for the use of rasburicase for tumour lysis syndrome
G6PD deficiency
how can leukostasis syndrome in ALL/AML be managed
hydroxyurea +/- leukopharesis which decreases cell counts
leukostasis can occur when WCC is very high >100,000 resulting in CNS infarcts, pul hypertension, DIC etc
indicators of poor prognosis of ALL
age < 1 yr or >10 yr
increase in WBC >50,000
philadelphia chromosome t(9,22)
CNS involvement at diagnosis
indicators of poor prognosis for AML
age > 60yrs
elevated LDH
poor risk or complex karyotype
smudge cells on blood smear
CLL
(indicates crushing of fragile leukaemic cells)
flow cytometry markers diagnostic of CLL
CD5, CD23
most accurate diagnostic test for suspected CLL
flow cytometry - shows monoclonal B cells with CD5, CD23
most accurate investigation for diagnosing suspected AML or ALL
bone marrow biopsy with flow cytometry
management for CLL
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)
describe the natural course of untreated CML
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
in CML, what myeloid cell is found to be elevated which differentiates CML from normal leukaemoid reaction to infection
increase in basophils
most accurate test for diagnosing suspected CML
philadelphia chromosome by PCR or flouroscene insitu hybridization (FISH) analysis showing t(9;22) translocation
or
BCL/ABL PCR or northern blot
how can CML be diffeentiated from leukaemoid reaction
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
management of CML
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
chromosomal translocation associated with APL
T(15;17)
myeloperoxidase positive - myeloid or lymphoid origin ?
myeloid
terminal deoxynucleotidetransferase (Tdt) - myeloid or lymphoid origin ?
lymphoid
management for hairy cell leukaemia
best initial: Cladribine
other options: rituximab, pentostatin, interferon (INF-a)
‘TRAPped in a hairy situation with CLADia (cladribine)’
what cell type is hair cell leukaemia
well-differentiated B cell lymphocytes
patient presents with waxing and waning lymphadenopathy - most common haem malignancy that would present like this ?
follicular lymphoma
indolent course or low grade
t(14:18)
management of follicular lymphoma
most often requires surveillence
localised disease may be treated with radiotherapy