Blood Cancers Flashcards
1
Q
Acute myeloid leukaemia overview and prognostic factors:
A
- more common form of acute leukaemia
- primary or following secondary transformation of myeloproliferative disorder
poor prognosis:
- > 60yo
- > 20% blasts after first course chemo
- cytogenics: deletions of chromosome 5 or 7
2
Q
Features of acute myeloid leukaemia:
A
- anaemia: pallor, lethargy, weakness
- neutropenia: WCC may be high but functioning neutrophils low
- thrombocytopenia: bleeding
- splenomegaly
- bone pain
3
Q
Acute promyelocytic leukaemia M3
A
- associated with t(15;17)
- fusion of PML and RAR alpha genes
- presents younger
- Auer rods (myeloperoxidase stain)
- DIC or thrombocytopenia at presentation
- good prognosis
4
Q
Classification of acute myeloid leukaemia:
A
M0 - undifferentiated M1 - without maturation M2 - with granulocytic maturation M3 - acute promyelocytic M4 - granulocytic and monocytic maturation M5 - monocytic M6 - erythroleukaemia M7 - megakaryoblastic
5
Q
Burkitt’s lymphoma
A
- high grade B cell neoplasm
- endemic form (African) involving maxilla or mandible
- sporadic form: abdominal tumours and common in HIV
- c-myc gene translocation
- EBV implicated in African development
6
Q
Microscopy findings of Burkitt’s lymphoma:
A
- starry sky appearance
- lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells
7
Q
Management of Burkitt’s lymphoma:
A
- chemotherapy (may cause tumour lysis syndrome)
- Rasburicase often given before chemotherapy to reduce risk
- complications tumour lysis: hyperkalaemia, hyperphosphataemia, hypocalcaemia, hyperuricaemia, acute renal failure
8
Q
Complications of chronic lymphocytic leukaemia:
A
- anaemia
- hypogammaglobulinaemia (recurrent infections)
- warm AIHA
- transformation to high grade lymphoma (Richter’s transformation)
9
Q
What is Richter’s transformation?
A
- when leukaemia cells enter lymph node and change into high grade, fast growing non-hodgkin’s lymphoma
- become unwell very suddenly
- lymph node swelling, fever without infection, weight loss, night sweats, nausea, abdominal pain
10
Q
What is chronic lymphocytic leukaemia:
A
- monoclonal proliferation of well-differentiated lymphocytes almost always B cells
- most common form of leukaemia in adults
- often no symptoms (incidental lymphocytosis)
- anorexia, weight loss, bleeding, infections, lymphadenopathy more than chronic myeloid
11
Q
Investigations in chronic lymphocytic leukaemia:
A
- lymphocytosis, anaemia
- blood film: smudge cells (smear cells)
- immunophenotyping
12
Q
Chronic myeloid leukaemia:
A
- philadelphia chromosome
- BCR-ABL gene codes for fusion protein which has tyrosine kinase activity
- anaemia, lethargy, weight loss, sweating
- marked splenomegaly
- increase in granulocytes at different stages of maturation (with thrombocytosis)
- decreased leukocyte ALP
- may undergo blast transformation
13
Q
Management of chronic myeloid leukaemia:
A
- imatinib (inhibitor of tyrosine kinase, high response rate)
- hydroxyurea
- interferon alpha
- allogenic bone marrow transplant
14
Q
t(9;22)
A
- philadelphia chromosome
- > 95% in CML
- poor prognostic indicator
- BCR-ABL codes for fusion protein - increased tyrosine kinase activity
15
Q
t(15;17)
A
- acute promyelocytic leukaemia (M3)
- fusion of PML and RAR alpha genes
16
Q
t(8;14)
A
- Burkitt’s lymphoma
- MYC oncogene translocated to Ig gene
17
Q
t(11;14)
A
- Mantle cell lymphoma (non-hodgkin’s)
- deregulation of cyclin D1 (BCL-1) gene
18
Q
t(14;18)
A
- follicular lymphoma
- increased BCL-2 transcription
19
Q
Infections associated with blood cancer:
A
- EBV: Hodgkin’s, Burkitt’s, nasopharyngeal carcinoma
- HTLV-1: adult T cell leukaemia/lymphoma
- HIV-1: high grade B cell lymphoma
- H pylori: MALT gastric lymphoma
- malaria: Burkitt’s lymphoma
20
Q
What is Hodgkin’s lymphoma?
A
- malignant proliferation of lymphocytes
- characteristed by presence of Reed Sternberg cell
21
Q
Features of Hodgkin’s lymphoma:
A
- lymphadenopathy - painless, non-tender, asymmetrical
- systemic: weight loss, pruritus, night sweats, fever
- alcohol pain in HL
- normocytic anaemia, eosinophilia
- LDH raised
22
Q
Histological classifications in Hodgkin’s lymphoma:
A
- nodular sclerosing
- mixed cellularity
- lymphocyte predominant
- lymphocyte depleted
23
Q
Which histological classification is most and least common in Hodgkin’s lymphoma?
A
- most: nodular sclerosing
- least: lymphocyte depleted
24
Q
Which histological classification has the best and worst prognosis in Hodgkin’s lymphoma?
A
- best: lymphocyte predominant
- worst: lymphocyte depleted
25
Staging in Hodgkin's lymphoma:
I: single lymph node
II: 2 or more lymph nodes/regions on same side of diaphragm
III: nodes on both sides of diaphragm
IV: spread beyond lymph nodes
A - no systematic symptoms other than pruritus
B - weight loss >10% in 6 months, fever >38, night sweats (poor prognosis)
26
What is multiple myeloma?
- plasma cell proliferation
- genetic mutations which occur as B lymphocytes differentiate into mature plasma cells
- median age 70yo
27
Presentation of MM:
CRABBI
- Calcium - hypercalcaemia due to increased osteoclast activity, constipation, nausea, anorexia, confusion
- Renal - light chain deposition in renal tubules, renal damage, dehydration and thirst, also amyloidosis, nephrocalcinosis, nephrolithiasis
- Anaemia - suppresses erythropoiesis in bone marrow
- Bleeding - thrombocytopenia
- Bones - lytic bone lesions, pain, increased risk fragility fractures (raindrop skull)
- Infection - reduction in normal Ig production
28
MM investigations:
- bloods: anaemia, thrombocytopenia, raised U&E, hypercalcaemia
- rouleaux formation
- serum or urine protein electrophoresis (raised conc monoclonal IgA and IgG - Bence Jones proteins)
- bone marrow aspiration and trephine biopsy
- whole body MRI - bone lesions
29
Management of MM:
- begin treatment immediately
- chronic relapsing and remitting which is incurable
- autologous stem cell transplantation with Bortezomib and Dexamethasone induction therapy
- no stem cell induction therapy: thalidomide and alkylating agent and dexamethasone
30
Managing complications of MM:
- analgesia
- zolendronic acid for osteoporosis and fragility fractures
- annual influenza and may receive Ig replacement therapy
- VTE prophylaxis
- consider erythropoietin therapy
31
Why do you get hypercalcaemia in myeloma:
- primary factor: increased osteoclastic bone resorption caused by local cytokines released by myeloma cells
- much less common: impaired renal function, increased renal tubular calcium reabsorption and elevated PTH-rP
32
What is the difference between Hodgkin's and non-Hodgkin's lymphoma?
- Hodgkin's: Reed-Sternberg cells
| - non-Hodgkin's: every other type, affecting B or T cells, more common, typically elderly
33
Risk factors for non-Hodgkin's lymphoma:
- elderly
- caucasians
- Hx of viral infection
- FHx
- chemical agents
- Hx of chemotherapy or radiotherapy
- immunodeficiency
- Autoimmune disease
34
Symptoms of non-Hodgkin's lymphoma:
- painless lymphadenopathy (non-tender, rubbery, asymmetrical)
- systemic
- extranodal disease: gastric, bone marrow (pancytopenia, bone pain), lungs, skin, CNS
- palpable abdominal masses
- testicular mass
35
Distinguishing Hodgkin's from non-Hodgkin's:
- bone marrow biopsy
- lymphadenopathy in Hodgkin's can show alcohol induced pain
- B symptoms earlier in Hodgkin's
- extra nodal disease more common in Hodgkin's
36
Investigations for non-Hodgkin's:
- excisional biopsy
- CT chest, abdomen and pelvis for staging
- HIV test
- FBC and blood film (rule out leukaemia and find normocytic anaemia)
- ESR (prognostic indicator)
- LDH (marker of cell turnover, prognostic indicator)
37
Complications of Non-hodgkin's lymphoma:
- bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
- superior vena cava obstruction
- metastasis
- SCC
38
Low grade vs high grade non-hodgkin's:
high grade has worse prognosis but higher cure rate
39
Most common form of lymphoma in UK:
diffuse large B cell lymphoma
