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Cancer care > Haematological malignancies > Flashcards

Haematological malignancies Flashcards

1
Q

Symptoms of bone marrow failure

A

Anaemia - fatigue, dyspnoea, dizziness
Thrombocytopenia
Neutropenia - recurrent/atypical infections

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2
Q

Constitutional symptoms

A

Unintentional WL >10% over 6m, drenching night sweats, fever, pruritis

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3
Q

Symptoms of hypercalcaemia

A

Fatigue, abdo pain N+V, confusion, constipation, headache, polydipsia, polyuria

Mostly a/w myeloma + high grade lymphomas

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4
Q

Symptoms of hyperviscosity

A

Rare, mostly myeloma - headache, somnolence, visual disturbance, ischaemic events

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5
Q

Outline the steps of the lymphoreticular examination

A
  • Inspection - cachexia, rashes, bleeding
  • Lymphadenopathy: cervical, axillary, epitrochlear, inguinal
  • Abdo exam
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6
Q

How can you identify splenomegaly on abdomen examination?

A
  • LUQ
  • Palpable splenic notch
  • Moves infero-medially with inspiration
  • Not ballotable
  • Cannot get above it
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7
Q

Causes of lymphadenopahty

A
  • Local: bacterial/viral infection, skin conditions like eczema, malignancy (mets or lymphoma)
  • General: systemic infections (e.g. TB, HIV, endocarditis), malignancy (lymphoma, lymphoid leukaemias), inflammatory (sarcoid, CT disease), generalised allergic conditions
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8
Q

Red flags for malignancy in a neck lump

A
  • Hard fixed mass
  • > 35y
  • Mucosal lesion in H+N
  • Persistent hoarseness/dysphagia
  • Trismus
  • Ear pain (Referred from tongue base)
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9
Q

Differentials for a midline neck lump

A
  • LN
  • Lipoma: painless smooth
  • Dermoid cyst: along lines of embryological fusion, don’t move with tongue protrusion, more in children/YA
  • Sebaceous cyst
  • Thyroid gland: below thyroid cartilage; thyroid nodules: adenoma/cysts/malignancy
  • Thyroglossal cysts: painless/smooth/cystic, rise on tongue protrusion
  • Laryngocele: reducible tense mass
  • Cystic hygroma: toddlers, trans-illuminates, soft fluctuant mass
  • Brachial cyst - doesn’t transillimuninate
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10
Q

Differentials for an anterior triangle neck lump

A
  • LN
  • Lipoma, sebaceous cyst
  • Salivary gland swelling (doesn’t move on swallow)
  • Branchial cyst: from birth, in early adulthood presents as infected neck lump
  • Carotid artery aneurysm: pulsatile mass, bruit on auscultation
  • Carotid body tumour: transmits pulsation, can be moved sideways but not vertically cos of carotid sheath
  • Laryngocele
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11
Q

Differentials for a posterior triangle neck lump

A
  • LN
  • Lipoma, sebaceous cyst
  • Subclavian artery aneurysm
  • Pharyngeal pouch
  • Cystic hygroma: often on left, fluctuant, transilluminates
  • Branchial cyst
  • Tail of parotid mass
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12
Q

When would you refer someone with lymphadenopathy?

A
  • If any red flag symptoms

* If not gone down within 6w

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13
Q

What are the clinical features of lymphadenopathy?

A
  • Benign <1cm, smooth, round, non-tender, mobile
  • Reactive smooth, round, tender, mobile
  • Haem malignancy localised/generalised, rubbery
  • Mets: hard/firm/irregular/tethered
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14
Q

Abnormal lymphocyte counts?

A
  • Lymphocytosis: some viral infections, some bacterial e.g. pertussis, lymphoid neoplasia
  • Lymphopenia: viral infection like HIV, CT disease, lymphoma, severe BM failure immunosuppressive drugs
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15
Q

What does the spleen do?

A

Makes antibodies, processes antigens, phagocytosis (esp encapsulated bacteria), sequesters red cells, removes nuclear remnants (Howell-Jolly bodies), haemopoiesis in early fetus

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16
Q

Mild, moderate and massive splenomegaly?

A
  • Mild: just palpable 1-3cm below costal margin. E.g. haem cancers, portal HTN, EBV, haemolytic anaemia, SLE
  • Moderate: between CM+umbilicus, 4-8cm below CM. E.g. haem cancers like CML/lymphoma, amyloidosis
  • Massive: crosses umbilicus + midline, >8cm. E.g. CML, myelofibrosis, tropical infections, HIV, hereditary spherocytosis
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17
Q

Indications for bone marrow aspiration

A

Unexplained cytopenias, suspected BM infiltration (with cancer or storage disorders), suspected infections like Leishmaniasis/TB

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18
Q

What is immunophenotyping (flow cytometry)?

A

Looks at the protein expressed by cells by labelling cells with an antibody directed against surface proteins and passing them through a flow cytometer

Helps show lineage in leukaemias by seeing if surface proteins are myeloid or lymphoid

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19
Q

Outline the differentiation from a multipotent haematopoietic stem cell

A
  • Common myeloid precursor – megakaryocytic (make thrombocytes), erythrocytes, mast cells, myeloblast (makes basophil, neutrophil, eosinophil, monocytes the latter which make macrophages)
  • Common lymphoid precursor – NK cells and small lymphocytes (make T and B lymphocytes)
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20
Q

What is the significance of blast cells in the blood film?

A

They are precursors of myeloid cell (myeloblasts) or lymphoid cells (lymphoblasts) - bigger, immature nucleus, atypical appearance

Suggests an acute leukaemia or a chronic disorder beginning to become acute e.g. CML blast crisis

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21
Q

What is the difference between leukaemia and lymphoma?

A

Lots of crossover but leukaemias mostly affect the bone marrow and may have circulating neoplastic cells in the blood, whereas lymphomas mostly affect nodes/organs

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22
Q

What is leukaemia?

A

Increased WBC

  • Myeloid vs lymphoid
  • Acute - more immature cells, v proliferative
  • Chronic - more differentiated mature cells
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23
Q

Aetiology of leukaemia

A
  • Activation of oncogenes causing prolfieration
  • Deletion of TSGs like p53
  • Altered apoptosis control
  • Epigenetic changes

Due to genetics, chemicals, irradiation and viruses

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24
Q

Describe how acute leukaemias tend to present

A
  • Marrow failure e.g. pallor, cardiac flow murmur, fever, ulcers, sepsis, petechiae, gingival bleeding, fundal haemorrhage
  • High WCC: hypoxia, pulmonary infiltrates, retinal vein dilation, papilloedema, confusion
  • Tissue infiltration: bone pain, hepatosplenomegaly, lymphadenopathy, gum hypertrophy (AML), violaceous skin deposits (AML), testicular enlargement (ALL), mediastinal masses (ALL)
  • Substance release: DIC (AML), hyperuricaemia (acute gout or TLS)
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25
Q

Typical Ix findings in acute leukaemias

A 
Low Hb
Raised WCC
Low platelets
Blood film - blast cells, Auer rods suggests AML
BMA - more cells, less erythropoiesis
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26
Q

How are acute leukaemias managed?

A
  • Curative intent: induction of complete remission then a long consolidation phase
  • Supportive (cure + palliative): reduce anaemia, prevent bleeding, FFP for clotting abnormalities, leucophoresis to reduce WCC, infection, control hyperuricaemia with hydration + allopurinol
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27
Q

Acute lymphoblastic leukaemia

A
  • Proliferation of lymphoid blasts (B/T cells), commonest cancer in children + young adults
  • Sometimes a/w Philadelphia chromosome
  • ALL when BM involvement, or localised disease as lymphoblastic leukaemia (but same tumour cells)
  • CF: typically rapid deterioration with fever, BM failure, T cell versions can cause mediastinal mass, meningeal signs if CNS involvement, hepato/splenomegaly
  • M: 2-3y of chemo, induce remission then maintenance with chemo or allogeneic transplant + intrathecal chemotherapy
  • Children have a v good prognosis
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28
Q

Acute myeloid leukaemia (nb no longer adequate label as so many subtypes)

A
  • Proliferation of myeloid blasts (whereas CML is mature cells), Auer rods in film
  • Typically affects more elderly people
  • CF include gum hypertrophy, splenomegaly and ecchymosis
  • M: chemo (intensive if <60 as high chance of remission, often with a monoclonal antibody e.g. gemtuzumab)
  • Often offered clinical trials
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29
Q

What affects prognosis in AML?

A

Improve: t(8;21) and t(15;17), APML subtype

Reduce: age, failure to respond after 2 induction cycles, complex genetics, comorbidities, high WCC on presentation

30
Q

Chronic myeloid leukaemia

A

*Presence of Philadelphia chromosome t(9;22) translocation
*Peak 40-60y
*High WCC (esp neuts + basophils) + circulating immature cells
*CF: asymptomatic, or marrow failure, WL, night sweats (hypermetabolic state) + splenomegaly (extra medullary haemopoiesis)
*Slowly progressive - if not cured eventually blast crisis transformation to AML/myelofibrosis
*Blood film shows mix of mature + immature cells - indicates chronic
*Management: imatinib (a TKi that blocks the BCR-ABL fusion protein), allogeneic SCT
Resistant can try a 2nd gen like nilotinib

31
Q

Chronic lymphocytic leukaemia

A
  • Commonest type of leukaemia mostly occurs in later life
  • Clonal expansion of mature lymphocytes, alters T cell immunity so can get autoimmune things like AIHA and ITP
  • Often a chance finding on FBC, early diagnosis years survival or if late can be low survival
  • CF: usually asymptomatic, may be predisposed to infections like VZV, lymphadenopathy, hepato/splenomegaly causing discomfort, bleeding/petechiae/fatigue, tonsillar enlargement
  • Ix: low/normal Hb, high WCC, film shows small/medium lymphocytes or smudge cells {(no immature blasts)
  • Median survival 10y
  • Management: supportive (e.g. steroids for autoimmune haemolysis, EPO, immunoglobulin replacement, allopurinol), may need chemo if BM failure
  • Richters transformation
32
Q

Hairy cell leukaemia

A

Clonal proliferation of abnormal B cells in the BM + spleen (usually p/w splenomegaly)

33
Q

What is lymphoma?

A

B+T cell neoplasms

They occur at different stages at development so different subtypes, more aggressive if of proliferating cells

34
Q

What do investigations typically show in lymphoma?

A
  • FBC: anaemia, plt abnormalities
  • CXR: mediastinal widening, hilarity LN
  • LDH non specific but often rises in haem cancers
35
Q

What is Hodgkin lymphoma and how does it present?

A

A distinct tumour of lymphatic system with multinucleated giant cells (Reed-Sternberg cells); there is also a nodular type with a ‘popcorn’ cell (rarer)

Bimodal age dist most common 20s + 60s

Lymphadenopathy (75%) - painless, non-tender, asymmetrical
systemic (25%): weight loss, pruritus, night sweats, fever (Pel-Ebstein)
alcohol pain in HL
normocytic anaemia, eosinophilia
LDH raised

CF: painless rubbery cervical lymphadenopathy often asymmetrical/spreading (75%), mediastinal mass (cough, sob), hepatosplenomegaly, B symptoms (25%-WL, night sweats, fever) pruritus, alcohol-related pain at the LN

Ix often show a normocytic anaemia, eosinophilia and raised LDH

Prognosis based on albumin (lower=worse), WCC, Hb and ESR

36
Q

Staging of Hodgkin lymphoma

A

Lugano staging (I + II limited, III + IV advanced), basically same as Ann-Arbor, done via PET scan (no need for BM biopsy as it shows up on scan)

I: one node/group of adjacent nodes
II: two or more groups on same side of diaphragm
III: nodes on both sides, or just above diaphragm with spleen involvement
IV: diffuse involvement of one/more extra-nodal organs or tissue

37
Q

Management of Hodgkin lymphoma

A

Aimed towards cure

  • Chemotherapy then radiation usually thing for cure
  • Try to avoid irradiating chest area but sometimes needed
  • Often in stage I or II may do expectant management as no progression for many years, or rituximab to slow progression
  • Stage 3+4 also often curable
38
Q

What is non-Hodgkin lymphoma and how does it present?

A

A group of lymphoproliferative malignancies with different behaviours, much greater extra-nodal spread than HL, majority B-cell malignancies

CF include painless LN, sx of LN mass, soft tissue masses, sx at BM/spleen/bone/lung/skin/testicular/gastric/CNS

39
Q

NHL aetiology

A
  • EBV (endemic Burkitt’s lymphoma, transplants, also to a lesser extent a/w HL)
  • HIV
  • H pylori (gastric MALT)
  • HTLV-1 (adult T cell lymphoma/leukaemia)
  • HCV (splenic marginal zone lymphoma)
  • Inherited syndromes e.g. Wistkott-Aldrich syndrome
  • Immunosuppressive drugs
40
Q

Types of NHL

A
  • High grade: usually aggressive with high mortality early on but good cure rate with intensive treatment . Either B or T cell. E.g. DLBCL, Burkitts
  • Low grade: usually incurable but treatable for a long time. Usually B cell. E.g. follicular lymphoma, marginal zone lymphoma, Waldenstrom’s macroglobulinaemia
  • Primary extra-nodal NHL: anywhere there there is lymphoid tissue, e.g. in CNS (SOL sx), gastric (a/w H pylori), skin (e.g. Sezary syndrome with erythematous lesions)
41
Q

Why does lymphoma not involve metastasis?

A

Talk of progression not mets due to behaviour of disease

May mean transformation to a high grade type or involvement of BM

42
Q

What is the main MAB used in NHL & why?

A

Rituximab

Is MAB of CD20, which is expressed on lots of B cells esp in DLBCL

43
Q

What is myeloma?

A

Clonal proliferation of BM plasma cells due to a single clone of immunoglobulin that secretes terminally-differentiated B cells

44
Q

Presentation of myeloma

A

Typically >60

  • Bone disease: lytic lesions in spine/skull/long bones/ribs, vertebral collapse, bone pain
  • BM infiltration: anaemia, neutropenia, thrombocytopenia, paraproteinaemia, rarely-hyperviscosity
  • Kidney injury (light chains in renal tubules): hypercalcaemia, hyperuricaemia
  • Recurrent infection: due to reduced levels of normal immunoglobulins
45
Q

Investigations in myeloma typically show

A
  • BM suppression on FBC
  • High ESR
  • FIlm may show rouleaux formation cos of paraprotein
  • U+E AKI
  • Bone profile - hypercalcaemia
  • Serum protein electrophoresis - monoclonal band
  • Skeletal survey for lytic lesions (not bone scans as these only show osteoblastic changes which cause sclerotic lesions [which are what most mets cause])
  • BM aspirate/trephine: plasma cell infiltration >10% (‘fried egg’ appearance), flow cytometry/protein electrophoresis to establish clonality
46
Q

Management of myeloma

A
  • Incurable
  • Supportive: EPO for anaemia, infections, RT/dexamethasone/bisphosphonates for bone pain
  • Palliative chemo may be used e.g. proteasome inhibitors
47
Q

What is MGUS?

A

Monoclonal gammopathy of uncertain significance, more common with age

Is when there’s low paraprotein and BM plasma cells are <10% but there is no myeloma-related organ/tissue damage

Risk of progression to myeloma 1% per year so follow up but don;t need treatment

48
Q

What are myeloproliferative neoplasms?

A

Clonal stem cell disorders causing uncontrolled proliferation of one or more cell lines in BM, can lead to AML

Basically too many cells cos apoptosis isn’t effective

49
Q

Essential thrombocythaemia

A
  • Persistently raised platelets due to JAK2 mutation
  • Causes thrombosis, haemorrhage, splenomegaly
  • Can transform to myelofibrosis, PRV or leukaemia
  • Exclude reactive causes
  • M: hydroxycarbamide
50
Q

Polycythaemia vera

A
  • Primary increase in RBC/myeloid/megakaryocytes despite EPO not being made; red cells most affected
  • Leads to thrombosis, neuro sequelae, pruritus, plethora, dusky cyanosis, gout, splenomegaly
  • Usually in >60y, JAK2 mutation
  • Risk of leukaemia + myelofibrosis
  • M: venesection (iron def will reduce erythropoiesis), chemo, low dose aspirin, anagrelide (limits megakaryocytic differentiation)
51
Q

Myelofibrosis

A
  • Clonal disorder of haemopoietic stem cells, abnormal cells make cytokines leading to BM fibrosis
  • CF: debilitating, causes lethargy weakness WL splenomegaly, bone pain + gout, extra medullary haemopoeisis, BM failure, tear drop forms of RBCs
  • M: supportive e.g. transfusion, cytotoxics like JAK2 inhibitors, splenectomy if massive
52
Q

What is myelodysplastic syndrome

A

Heterogenous group of acquired BM disorders due to a defect in stem cells – cells incorrect rather than too many

CF: elderly with pancytopenia (fail in at least one cell line)

~30% transform to AML

M: supportive e.g. EPO, immunosuppression or allogeneic SCT for younger, if evidence of AML chemo

53
Q

What is BM failure and how does it present?

A

Pancytopenia + low cellularity of the BM (usually v v low retics), without any malignant cells - may be absent or ineffective production

CF include severe anaemia, thrombocytopenia (e.g. ecchymoses), leukopenia, lymphopenia

54
Q

What are the types of BM failure?

A
  • Specific lineages like red cell aplasia, agranulocytosis
  • Aplastic anaemia - all 3 lineages affected, idiopathic, prob linked to immune. Supportive like plt + red cell transfusions, reduce infection. V severe if untreated, risk of AML, BMT in young pt
  • Congenital causes like Diamond-Blackfan syndrome, Fanconi anaemia
  • Acquired - COMMONEST REASON
55
Q

What is acute promyelocytic leukaemia?

A

APML
A variant of AML with translocation t(15;17)
Causes coagulopathy so risk of presenting with life threatening DIC, but high chance of cure

56
Q

What is the role of imatinib in CML?

A
  • CML: the Philadelphia chromosome makes a BCR-ABL oncogene (BCR from chromosomal 22 fuses with ABL on chromosome 9) –> abnormal oncogene on Ch 22 –> abnormal tyrosine kinase –> changes in cell adhesion in marrow + increased proliferation
  • Imatinib: tyrosine kinase inhibitor specifically for the BCR-ABL protein so mostly jut targets cancer cells
  • If have good response just keep taking with a normal lifespan
57
Q

What is Richters transformation?

A

When CLL transforms into a high grade lymphoma (usually diffuse large B cell)

58
Q

Which types of lymphoma are a/w development of autoimmune diseases?

A

Low grade

59
Q

Diffuse large B cell lymphoma

A

Commonest type, high grade

P/w painless LN or intra-abdoimanl disease

Rapidly progressive but good response to RCHOP + radio

60
Q

Follicular lymphoma

A

2nd commonest type, low grade, usually adults p/w painless LN

In some it can be relapsing-remitting

About 1/4 transform to DLBCL (worse prognosis) - get increase in B symptoms

M: expectant if well, radiotherapy or chemoimmunotherpay (RCHOP_

61
Q

What is RCHOP?

A

Common regime in lymphoma with chemo drugs (CHOP) + rituximab

62
Q

Burkitts lymphoma

A

A high grade NHL with v rapid proliferation

Commonest childhood malignancy in developing world - endemic type (always EBV-ass related to malaria), sporadic or AIDs-related

CF: jaw tumour, abdo mass, BM suppression, CNS/kidney/testis involvement

High risk of TLS

63
Q

Waldenstrom’s macroglobulinaemia?

A

A low grade NHL that’s a/w IgM paraprotein + BM infiltration

CF: older, LN/anaemia/hyperviscosity syndrome

64
Q

Explain paraproteins in myeloma

A

BM has many plasma cells - in myeloma a specific type predominates - the antibody this produces is more prevalent than the others - monoclonal paraprotein

Usually IgG or IgA paraprotein

cf HIV, where all immunoglobulins are raised

65
Q

What are Bence Jones protein?

A

Light chains excreted in urine when there is paraproteinaemia

Not checked anymore as just check paraprotein in blood

66
Q

Causes of acquired bone marrow failure

A
  • Infection: EBV, HIV, HCV, parvovirus
  • Drugs: chloramphenicol, co-trimoxazole, sulfasalazine, carbamazepine, phenytoin, NSAIDs, chemo drugs
  • Immune-mediated
  • Radiation
  • Vitamin def: B12, folate, anorexia nervosa
  • Infiltration with tumours or storage diseases like Gaucher’s
  • Idiopathic
67
Q

What is the blood count + film like in the different types of leukaemia?

A
  • AML - blasts
  • ALL - blasts
  • CML - raised granulocytes at different stages of maturation, +/- thrombocytosis (high plt)
  • CLL - raised lymphocytes
68
Q

Essential thrombocytosis

A

Very high plt usually >450

May also have raised WCC

69
Q

Aplastic anaemia

A

Peak age 30y

  • normochromic, normocytic anaemia
  • leukopenia, with lymphocytes relatively spared
  • thrombocytopenia

may be the presenting feature acute lymphoblastic or myeloid leukaemia

a minority of patients later develop paroxysmal nocturnal haemoglobinuria or myelodysplasia

70
Q

Features of myeloma?

A

CRAB:

  • Calcium high
  • Renal insufficiency - abnormal U+E, infection susceptibility as production of normal antibodies is impaired
  • Anaemia - BM infiltration
  • Bone pain/#/lytic lesions
71
Q

First line Ix in suspected myeloma?

A

Serum protein electrophoresis - immunoglobulin produced by dysplastic cells shows up as a monoclonal band

72
Q

Polycyathaemia vera

A

Myeloprolifeative. Increase in red cell volume, often a/w high neuts + plts. Mutation in JAK2 in majority. Peak in 50s. CF include hyper viscosity, pruritus, splenomegaly, haemorrhage, plethoric appearance, HTN, low ESR. Test FBC + film, JAK2 mutation, ferritin, U+E, LFT. If JAK2 negative do BMA + trephine + cytogenetics. Management involves aspirin, venesection, hydroxyurea, phosphorus-32 therapy. Comps are thrombotic events, 5-15% progress to myelofibrosis or acute leukaemia

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