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Y4 Cancer Care > Haematological malignancy > Flashcards

Haematological malignancy Flashcards

1
Q

What sort of information should you provide patients with myeloma and their family members/carers?

A
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2
Q

What is beta-2 microglobulin?

A
  • beta-2 microglobulin tumour marker for multiple myeloma and lymphoma
  • if raised in CSF, suggestive of brain or spinal cord mets

Also Known As - B2M, B2M, β2-Microglobulin, Thymotaxin

Formal Name - Beta2 Microglobulin, Serum, Urine, or CSF

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3
Q

What are the laboratory investigations for people with suspected myeloma?

A
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4
Q

Give some laboratory investigations to provide prognostic information regarding myeloma.

A
  • FISH
  • immunophenotyping on bone marrow plasma cells
  • immunohistochemistry on trephine biopsy

- serum free light chain assay and serum-free-light-chain ratio to assess prognosis

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5
Q

Give some imaging investigations for people with suspected myeloma.

A

- whole-body MRI 1st line imaging

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6
Q

Give some imaging options for people with newly diagnosed myeloma.

A

- to look for myeloma-related bone disease and extra-medullary plasmacytomas

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7
Q

What sort of services should hospitals treating myeloma but not receiving inpatient chemotherapy or a transplant offer?

A
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8
Q

What is the first line treatment of newly diagnosed myeloma?

A

- Bortezomib with dexamethasone OR with dexamethasone and thalidomide

(for those eligible for high dose chemotherapy with haematopoietic stem cell transplantation)

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9
Q

How do you manage acute renal disease caused by myeloma?

A

- Bortezomib and dexamethasone immediately

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10
Q

Give some ways of preventing bone disease in myeloma patients.

A
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11
Q

Give some ways of managing non-spinal bone disease in myeloma patients.

A
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12
Q

Give some ways of managing spinal bone disease in myeloma patients.

A

- Zoledronic acid OR disodium pamidronate CR sodium clodronate

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13
Q

What are some ways of preventing infection in myeloma patients?

A
  • seasonal influenza vaccine
  • extending pneumococcal vaccine to under 65’s

- IV immunoglobulin if hypogammaglobilinaemic

- prophylactic aciclovir

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14
Q

How can you manage peripheral neuropathy in myeloma patients?

A
  • reduce dose
  • reduce to weekly doses
  • temporarily stop neuropathy-inducing myeloma tx
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15
Q

How can you manage fatigue in myeloma patients?

A

EPO analogues - only for symptomatic anaemia

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16
Q

What sort of monitoring is required for myeloma patients?

A

- serum immunoglobulins and serum protein electrophoresis

- serum-free-light-chain assay

- routine bloods

- bone profile

- whole body MRI

- whole body CT

- FDP-PET CT

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17
Q

How do you manage relapsed myeloma?

A
18
Q

Outline the subsequent therapy for myeloma.

A

NB RESOURCES:

  • MSD manual
  • NICE CKS
  • NICE
  • ZTF
19
Q

What are some causes of thrombocytosis?

A
  • CML
  • reactive
  • iron deficiency
  • essential thrombocythaemia - most common (often presents as incidental finding, causes thrombosis)
20
Q

What tests can you do for essential thrombocythaemia?

A
  • genetic testing for MPN
  • bone marrow testing (aspirate or trephine)
21
Q

What is myeloma?

A

- plasma cell disorder with production of abnormal monoclonal immunoglobulins (called paraproteins or M protein) that can affect many organ systems (kidneys, heart, bones..). Hence can present with chronic renal impairment, proteinuria..

  • can be IgG, IgM, IgA.. (these are the most common ones)
  • abnormal proteins produced
  • myeloma can cause amyloidosis (worse prognosis)
  • can detect amyloid with Congo ink stain
22
Q

What sort of drug Tx’s are used for myeloma? Give some side effects of these.

A
  • VTD chemotherapy regime
  • side effect of thalidomide = bilateral peripheral neuropathy in hands and feet (NB neuropathy due to CTS would be UNILATERAL not BILATERAL!)
  • can change thalidomide to cyclophosphamide if pt develops peripheral neuropathy, ie switch from VTD to VCD regime
  • cyclophosphamide once weekly chemo tablets
  • may consider BM transplant in responsive disease, young pt’s

VTD: injections into tummy

VCD: no injections

VTD is more effective regime than VCD slightly, but there is a lower risk of axonal neuropathy with VCD

Myeloma pt’s tend to suffer more from Tx toxicity than the myeloma itself

  • peripheral neuropathy secondary to chemo, NCTs showed neuro-axonal neuropathy

- monitor paraprotein and light chain for disease response

- 24hr urine protein to monitor proteinuria

  • thalidomide, dexamethasone.. ->common drugs for myeloma*
  • NB amyloidosis has a bad outlook if a patient has it concurrently with myeloma*
23
Q

How can you investigate proteinuria associated with myeloma?

A

24hr urine collection

- eg if pt complaining of frothy urine, or low eGFR/rising serum Cr

- make sure to measure serum Cr at the same time as the urine collection to get accurate result

  • if proteinuria persists, refer to renal
24
Q

What sort of questions should you ask when following up a patient with follicular NHL?

A

Ask about:

- fever

- night sweats

- significant unintentional weight loss (>10% over 6 months)

- any new lumps/swellings - ?lymphadenopathy

ie assess whether the patient has any symptoms of active disease or not

(maintenance with rituximab)

25
Q

Give some features of CLL.

A
  • ibrutinib is Tx (but can cause bleeding)
  • is common above 50, treatable but not curable

- lymphocytosis is key feature

- anaemia

- thrombocytopenia.. all as affects bone marrow

- hepatosplenomegaly

- lymphadenopathy

- can progress to myeloid leukaemia

  • fatigue is a s/e of any chemo
26
Q

Give some features of AHA.

A
  • autoimmune haemolytic anaemia
  • can be triggered by viral infection
  • if pale, breathlessness - get blood test by GP
  • ask about bruising, fever, night sweats (bruising as side effect of edoxaban)
  • assess reticulocytes - bone marrow response
  • LDH - cell turnonver/haemolysis

Other markers of haemolysis:

- bilirubin

- K+ - as most potassium is intracellular, thus is released on RBC breakdown

- haptoglobin - released by liver. Normally binds to globin chains (product of RBC haemolysis) as free globin chains are toxic. Thus, in increased haemolysis, the haptoglobin is used up … so..

INCREASED HAEMOLYSIS = LOW HAPTOGLOBIN LEVELS

- thus, haptoglobin is a test for AHA - as it mops up globin from haemolysed RBCs

27
Q

Give some features of Hodgkin’s lymphoma.

A
  • presents with large lymphadenopathy

- SOB

- is a B cell disorder

- is a disease of the lymph nodes

- typically affects younger pt’s, but also a peak in adults

  • treatable, but sometimes can be stubborn
28
Q

Outline the Ann Arbor staging for Hodgkin’s lymphoma.

A
29
Q

Outline the Ann Arbor staging for Hodgkin’s lymphoma.

A
30
Q

Outline the Ann Arbor staging for Hodgkin’s lymphoma.

A
31
Q

What is the key histological finding in Hodgkin’s lymphoma?

A

Reed Sternberg cell

32
Q

What questions should you ask when following up a patient with Hodgkin’s lymphoma?

A
  • any breathlessness (performance status)
  • stage 5, IPI score; classical Hodgkin’s lymphoma
  • PET CT
  • use contraception 3-4mths after chemo as may have preserved fertility (chemo may not have made a pt completely sterile).
33
Q

Outline the investigation and Tx of lymphoma.

A
  • s/e of chemo is cystitis (tx: trimethoprim)
  • ask about fever, night sweats, wt loss
  • lumps on any part of body
  • angio, anaplastic T cell lymphoma - more aggressive
  • ITP

- T cell lymphoma tends to recur (usually within 2 yrs)

- if low kidney function - advise to increase fluid intake to at least 2L/day

34
Q

Outline essential thrombocythaemia.

A
  • JAK2 mutation
  • is a MPD
  • can transform into leukaemia or myelofibrosis if left untreated (or may stay as ET for 20yrs)
  • Tx: hydroxycarbamide (cytoreductive therapy) tablets
  • side effect of hydroxycarbamide = hair thinning; may need to reduce dose
  • ask about nausea
  • take hydroxycarbamide 4 days a week 500 micrograms
  • Tx for ET doesn’t stop, but may reduce to 2 or 3 times a week
  • do FBC
35
Q

Give some features of a diffuse large B cell lymphoma.

A
  • stomach
  • with active B cell sub-type extending into the spleen
  • ?repeat PET CT - as some change in FDG PET
  • ask about appetite, swallowing difficulties, wt loss, fever, night sweats (stomach cancer) ie ask if any B Sxs?
  • take 20mg OD omeprazole as no Sx’s at the moment
  • bloods - routine + LDH
  • PET CT FDG -ve, no avidity ie whether stomach can take up the reagent or not
  • residual FDG was cleared
36
Q

Outline the Tx of ITP.

A
37
Q

Outline the features of ITP.

A
  • chronic, can have since childhood

- Romiplostim = Tx, sub cut injections, thrombopoietin receptor agonists

  • injections every 10 days
  • bloods - routine, U+E’s
  • Tx options for ITP: splenectomy, rituximab, steroids, immunoglobulins, thrombo-mimetics
38
Q

Outline haemochromatosis.

A
  • mutation in HFE gene
  • SF63G gene
  • (anaemia of chronic disease–> hepcidin–> DMT 2 transporter in proximal bowel)
  • hepcidin acts on bone marrow, kidneys, proximal bowel.. to reduce iron absorption. it causes involution of the divalent metal transporter in the proximal bowel (transporter for 2+ charge ions eg FE2+, Mg2+)
  • ?trial of hepcidin as Tx for haemochromatosis - but effects of giving hepcidin not known, but would work theoretically!
  • HFE gene carrier = heterozygous for HFE gene
  • homozygous for HFE gene mutation = has disease
  • ?C2A2Y mutation detected
  • alpha fetoprotein marker
39
Q

Outline monoclonal lymphocytosis of unknown significance.

A
  • not a Dx in itself - can either be a LPD or reactive
  • is a LPD (lymphoproliferative disorder)
  • monoclonal B cell lymphocytosis - a pre CLL condition, total WCC<5
  • ask: ?fever, wt loss, night sweats
  • ?lump/swelling in neck, groin, armpit..
40
Q

What is chronic lymphocytic leukaemia?

A

- a disease of older people

  • a malignant MONOCLONAL expansion of B lymphocytes with accumulation of abnormal lymphocytes in the blood, bone marrow, spleen, lymph nodes and liver
41
Q

Outline the causes of erythrocytosis.

A

Primary:

  • polycythaemia rubra vera (JAK2)

Secondary: usually because something affects EPO release from kidneys (drives red cell production by bone marrow):

  • chronic hypoxia - COPD, emphysema, smoking, congenital heart disease, obstructive sleep apnoea, inherited haemoglobinopathies (affect ability of red cells to carry O2), high altitudes
  • renal disease - cysts, tumours (RCC), renal artery stenosis
  • Tumours - RCC, cerebellar haemangioblastomas, brain, liver, endometrial ca
  • dehydration (burns, vomiting, diarrhoea, not drinking enough fluids)
  • diuretics - cause relative erythrocytosis
42
Q
A

Y4 Cancer Care (2 decks)

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