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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?

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
Give some features of CLL.
- 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
Give some features of AHA.
- 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
Give some features of Hodgkin's lymphoma.
- 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
Outline the Ann Arbor staging for Hodgkin's lymphoma.
29
Outline the Ann Arbor staging for Hodgkin's lymphoma.
30
Outline the Ann Arbor staging for Hodgkin's lymphoma.
31
What is the key histological finding in Hodgkin's lymphoma?
## Footnote **Reed Sternberg cell**
32
What questions should you ask when following up a patient with Hodgkin's lymphoma?
- 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
Outline the investigation and Tx of lymphoma.
- 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
Outline essential thrombocythaemia.
- 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
Give some features of a diffuse large B cell lymphoma.
- 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
Outline the Tx of ITP.
37
Outline the features of ITP.
- 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
Outline haemochromatosis.
- 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
Outline monoclonal lymphocytosis of unknown significance.
- 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
What is chronic lymphocytic leukaemia?
**- 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
Outline the causes of erythrocytosis.
**_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

Y4 Cancer Care (2 decks)

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