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
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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
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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
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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
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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
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6
Q

Microscopy findings of Burkitt’s lymphoma:

A
  • starry sky appearance

- lymphocyte sheets interspersed with macrophages containing dead apoptotic tumour cells

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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
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8
Q

Complications of chronic lymphocytic leukaemia:

A
  • anaemia
  • hypogammaglobulinaemia (recurrent infections)
  • warm AIHA
  • transformation to high grade lymphoma (Richter’s transformation)
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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
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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
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11
Q

Investigations in chronic lymphocytic leukaemia:

A
  • lymphocytosis, anaemia
  • blood film: smudge cells (smear cells)
  • immunophenotyping
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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
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13
Q

Management of chronic myeloid leukaemia:

A
  • imatinib (inhibitor of tyrosine kinase, high response rate)
  • hydroxyurea
  • interferon alpha
  • allogenic bone marrow transplant
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14
Q

t(9;22)

A
  • philadelphia chromosome
  • > 95% in CML
  • poor prognostic indicator
  • BCR-ABL codes for fusion protein - increased tyrosine kinase activity
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15
Q

t(15;17)

A
  • acute promyelocytic leukaemia (M3)

- fusion of PML and RAR alpha genes

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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
Q

Staging in Hodgkin’s lymphoma:

A

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
Q

What is multiple myeloma?

A
  • plasma cell proliferation
  • genetic mutations which occur as B lymphocytes differentiate into mature plasma cells
  • median age 70yo
27
Q

Presentation of MM:

A

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
Q

MM investigations:

A
  • 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
Q

Management of MM:

A
  • 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
Q

Managing complications of MM:

A
  • analgesia
  • zolendronic acid for osteoporosis and fragility fractures
  • annual influenza and may receive Ig replacement therapy
  • VTE prophylaxis
  • consider erythropoietin therapy
31
Q

Why do you get hypercalcaemia in myeloma:

A
  • 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
Q

What is the difference between Hodgkin’s and non-Hodgkin’s lymphoma?

A
  • Hodgkin’s: Reed-Sternberg cells

- non-Hodgkin’s: every other type, affecting B or T cells, more common, typically elderly

33
Q

Risk factors for non-Hodgkin’s lymphoma:

A
  • elderly
  • caucasians
  • Hx of viral infection
  • FHx
  • chemical agents
  • Hx of chemotherapy or radiotherapy
  • immunodeficiency
  • Autoimmune disease
34
Q

Symptoms of non-Hodgkin’s lymphoma:

A
  • painless lymphadenopathy (non-tender, rubbery, asymmetrical)
  • systemic
  • extranodal disease: gastric, bone marrow (pancytopenia, bone pain), lungs, skin, CNS
  • palpable abdominal masses
  • testicular mass
35
Q

Distinguishing Hodgkin’s from non-Hodgkin’s:

A
  • 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
Q

Investigations for non-Hodgkin’s:

A
  • 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
Q

Complications of Non-hodgkin’s lymphoma:

A
  • bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
  • superior vena cava obstruction
  • metastasis
  • SCC
38
Q

Low grade vs high grade non-hodgkin’s:

A

high grade has worse prognosis but higher cure rate

39
Q

Most common form of lymphoma in UK:

A

diffuse large B cell lymphoma