What chemotherapy can be used for blood malignancies affecting the brain?
- Methotrexate crosses the BBB
- Intrathecal Chemo
- Irradiate brain
Incidence of leukaemia per 100 000?
Which sex is more likely to get leukaemia?
China has a low incidence of which leukaemia in particular?
Is it acute or chronic leukaemia:
Proliferation of all primitive stem cells
Is it acute or chronic leukaemia:
Malignant clonal differentiation that can't differentiate or mature
Mature cells accumulate?
In acute leukaemia, what percentage of peripheral blood cells are blasts?
What is the incidence of AML:ALL in
What are the signs of leukaemia?
Signs of bone marrow failure
What if there are auer rods in cells?
Myelin lasted leukaemia
What kind of anaemia is seen in acute leukaemia?
What is the prognosis of acute leukaemia?
- With treatment: 80% achieve remission
- Without: 5 days
Three stages of treatment of acute leukaemia?
- Remission induction: chemo
- Remission consolidation: chemo, radio, BMT
- Remission maintenance
CML is proliferation of all myeloid haematopoeitic cells but which line in particular?
Age of onset of CML?
Incidence of CML per 100 000?
CML counts for what percentage of leukaemias?
CML is caused by a part translocation of which chromosome, creating the BCR proto oncogene?
Philadelphia chromosome from 22->9
What percentage of CML are asympto?
What organs/lymph nodes are enlarged in CML?
Hepatosplenomegaly (esp spleen)
Symptoms of chronic leukaemia?
- Bone marrow failure
What kind of anaemia do chronic leukaemias cause?
What is the commonest leukaemia (30% of them)?
Sex and age of onset of CLL?
Which leukaemia will have a monoclonal B cell band with CD19 and CD23 antigens and aberrant T cell antigen CD25?
Staging of CLL?
- A) no anaemia/thrombocytopenia and <3 lymphadenopathy
- B) no anaemia/thrombocytopenia and >3 lymphadenopathy
- C) anaemia or thrombocytopenia
What chemo do you use for CLL?
- Cyclophosphamide with flu daring
When would you do splenectomy for CLL?
- If its massive
- Or RBC count is v low
Prognosis for stage B or CLL?
What can be the difference between prolymphocytic leukaemia and CLL?
- 25 % of Prolymphocytic is a variant with T cells
- More likely to have massive splenomegaly than lymphadenopathy
What is hairy cell leukaemia?
- Type of CLL
- B cells
- Neutropenia with hairy cells
- Always splenomegaly never lymphadenopathy
Sex ratios for hair cell CLL?
6 female : 1 male
What is a hairy cell?
- B cell with
- CD25 and CD103
- Stains with CID phosphatase
- Resistant to tartare staining
Treatment and relative CLL prognosis of hairy cell leukaemia?
Chemo: cladribine and deoxycoformycin
Prog is better - yrs
What is myelodysplastic syndrome?
Clonal haematopoetic proliferation
Myelodysplastic syndromes are because of mutations in which 2 chromosomes?
Name a cause
5 or 7
how many people in 100 000 over 70yrs have myelodysplastic syndromes?
what does the blood film of MDS show?
- macrocytic dysplastic RBC anaemia
- hypogranular neutropenia
- +/- blasts in 20%
treatment for MDS?
- NO CURE
- give RBC and PLT infusions, G-CSF and epo
what is RARS?
refractory anaemia with ringed sideroblasts
what is a sideroblast?
a nucleated RBC that cannot encorporate it's iron into Hb so it's granulated in a ring around the nucleus
what is 5q syndrome?
- 5q long arm deletion that leads to MDS
- occurs in females with age
are all classed as what syndromes
what is lymphoma?
what is Hodgekin's lymphoma?
- B cell lymphoma:
- with Reed Steinberg B cells surrounded by:
- nornal T cells
- plasma cells
how many people per 100 000 get lymphoma?
what is the sex ration of lymphoma?
age of onset?
- 1.5 male: 1 female
- middle class
what viruse is assoc in the PMH of lymphoma?
what is the commonest type of hodgekin's lymphoma?
- nodular sclerosing
- (classical type)
what causes painless, rubbery cervical lymphadenopathy?
what causes a dry cough and SOB in hodgekin's lymphoma?
treatment for hodgekin's lymphoma?
- chemo: doxy, bleo, vinblastin, dacarbazine
- autologous BMT
prognosis of hodgekin's lymphoma?
what is the Ann Arbor classification of HL?
- I: HL with one other site or one node involved
- II: HL with 2 other sites or nodes on the same side of diaphragm
- III: nodes on both sides of diaphragm involved
- IV: diffuse involvement of nodes
what does A or B mean in classes of lymphoma?
- A - no systemic symptoms
- B - weight loss, drenching sweats
what cell types can non-hodgekin's lymphoma be?
- 70% are B cell
- 300% are T cell
what is the incidence of non-hodgekin's lymphoma in 100 000 over 75 yrs?
which type of non-hodgekin lymphoma is potentially curable: high or low grade?
treatment for high grade nonhodgekin lymphoma?
- chemo: cyclophos, doxorubicin, vincristine, pred
- autologous BMT
(underlined is different to HL)
treatment for low grade non hodgekin lymphoma?
- radio if stage 1 ann arbor
- chemo: chlorambucil
what is non hodgekin lymphoma assoc with?
- HIV and it's co infections
- gastric lymphoma is assoc with h. pylori
tests for leukaemia?
- blood film
- bone marrow aspirate
- immunophenotyping for leukocytes antigens
- uric acid level
which is likely to be widely disseminated at presentation?
non hodgekin lymphoma
non hodgekin lymphoma
which is likely to present with weight loss, sweats, itching?
non hodgekin lymphoma
low grade lymphoma survival?
10yrs of relapsing/remitting
what is paraproteinaemia?
- clonal proliferation of antibodies
- can occur without underlying disease
how do you detect paraproteinaemias?
name some causes of polyclonal paraproteinaemia?
name some causes of monoclonal paraproteinaemias?
- polymyalgia rheumatica
what is MGUS?
monoclonal gammopathy of uncertain significance
aka benign monoclonal gammopathy
what percentage of those over 80yrs have MGUS?
why do you monitor these people?
monitor because 20% will get myeloma
MGUS is assoc with which malignancy?
what is waldenstrom macroglobulinaemia?
- lymphoplasma cytoid lymphoma
- IgM paraprotein
- low grade
what is the treatment for waldenstrom macroglobulinaemia?
- plasmaphoresis of IgM to reduce blood viscosity
- alkylating chemo eg chlorabucil
how many people per 100 000 get multiple myeloma?
what is the sex ratio and the age of onset of multiple myeloma?
which race does it affect the most?
- 2 male:1 female
- afrocaribbean race
what is multiple myeloma?
- malignant proliferation of
- B cells (derived from plasma cells)
what monoclonal antibodies do myeloma B cells produce?
(normal B cells produce polyclonal Abs)
- 55% IgG
- 21% IgA
- 20% no paraproteinaemia
other than paraproteinaemia, what do myeloma B cells produce?
what is Bence Jones proteinuria?
when myeloma light chains are in the urine
they are nephrotoxic!
why is there bone lysis in myeloma?
- myeloma B cells live in bone
- cause increase osteoclast action
- therefore bone resorption increases
clinical picture of mutliple myeloma?
- bone pain from lysis
- bone marrow failure symptoms
diagnosis of multiple myeloma needs 2 of what 3 things?
- malignant plasma cells in bone marrow
- serum/urinary paraproteinaemia
- skeletal lytic lesions
which LFT is raised in multiple myeloma?
what is the 10yr survival rate for multiple myeloma?
amyloidosis and myeloma?
amyloidosis is due to deposits of light chains
name some causes of renal failure associated with myeloma?
- bence jones proteinuria
what is urate nephropathy?
uric acid crystals block renal tubules
- seen in leukaemia due to tumour cell lysis (myeloma) and chemo
- or gout
treatment for hyperuricaemia?
why give GCs in myeloma?
they reduce antibody production
what chemo is used in myeloma?
- alkylating agents
treatment for bone lysis pain in myeloma?
- dental review - jaw osteocronosis
what causes failure of pluripotent stem cells and thus bone marrow hypoplasia and pancytopenia?
primary idiopathic acquired apalastic anaemia
where in the world is idiopathic acquired aplastic anaemia commonest?
rare in europre
what kind of anaemia is seen in aplastic anaemia?
- low reticulocyte count
what is a cure for young people with aplastic anaemia?
- allogenic BMT
- 90% if matched
what percentage of people with aplastic anaemia die in the first year?
complications of aplastic anaemia?
treatment for older people with acquired aplastic anaemia?
- cyclosporin immunosuppression
- anti-thymocyte globulin
commonest cause of secondary aplastic anaemia?
name three types of myeloproliferative disorders?
- essential thrombocytopenia
- polycaethaemia ruba vera (PRV)
age of onset of:
c) essential thrombocythaemia?
what happens in myeolofibrosis?
- hypercellular bone marrow
- increased megakaryoctes -> increased GF
- -> inc fibroblasts
symptoms of myelofibrosis?
- weight loss
- night sweats
- splenomeg - painful due to infarcts
prognosis of myelofibrosis?
4 yr survival
treatment for myelofibrosis?
- RBCs for anaemia
- folic acid
- cytotoxics: hydroxycarbamide
what gene mutation is assoc with myeloproliferative disease?
what causes essential thrombocytopenia?
- prolif of megakaryoctes
- JAK2 mutation
- risk factors: DM, HTN
treatment for essential thrombocythaemia?
hydroxycarbamide/anagrelide to stop megakaryocyte maturation
what is the PLT count like in essential thrombocythaemia?
how do you diagnose PRV?
- haematocrit increases
how do you measure plasma vol?
what is the prognosis of PRV?
what could it lead to?
- 65% -> myelofibrosis
- also: MI, CVA