Haem - Myeloma, Lymphoma, DIC Flashcards by Alex Hanson

Myeloma - what is it?

Haematological malignancy that arises due to plasma cell proliferation

Cancer of the plasma cells - type of B lymphocyte that produce antibodies

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Myeloma - what is multiple myeloma?

Is when the myeloma affects multiple areas of the body

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Myeloma - pathophysiology?

Plasma cells are B cells (B lymphocytes) of the immune system that have become activated to produce a certain antibody
Called B cells because they are found in the bone marrow
Myeloma is a cancer of a specific type of plasma cell where there is a genetic mutation causing it to rapidly and uncontrollably multiply
These plasma cells produce one type of antibody, and antibodies are also called immunoglobulins
They are complex molecules made up of two heavy chains and two light chains arranged in a Y shape
Help the immune system recognise and fight infections by targeting specific proteins on the pathogen
They come in 5 main types: A, G, M, D and E
When you measure immunoglobulins in a patient with myeloma, one of those types will be significantly abundant
More than 50% of the time this is immunoglobulin type G (IgG)
This single type of antibody that is produced by all the identical cancerous plasma cells can be called a monoclonal paraprotein
This means a single type of abnormal protein

The “Bence Jones protein” that can be found in the urine of many patients with myeloma is actually a part (subunit) of the antibody called the light chains

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Myeloma - what is the median age at presentation?

70 years old

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Myeloma - what are the clinical features?

CRABBI

Calcium (elevated) - due to increased osteoclast activity

Renal failure - due to light chain deposition within renal tubules

Anaemia (normocytic, normochromic) - bone marrow crowding suppresses erythropoiesis

Bone pain/lesions - bone marrow infiltration by plasma cells

Bleeding - bone marrow crowding also results in thrombocytopenia, so increased risk of bleeding/bruising

Infection - reduction in the production of normal immunoglobulins results in increased susceptibility to infection

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Myeloma - what area does bone pain commonly present at?

Presents as back pain commonly

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Myeloma - what are the blood results on investigation?

Bloods:

FBC - show anaemia and thrombocytopenia (because of A and B in CRABBI)

U&E - raised urea and creatinine (because of renal failure)

Raised calcium

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Myeloma - what are the other investigations you can do, and what are their results?

Serum or urine protein electrophoresis: raised concentrations of monoclonal IgA/IgG proteins will be present in the serum
In the urine, they are known as Bence Jones proteins
Bone marrow aspiration and trephine biopsy: confirms the diagnosis if the number of plasma cells is significantly raised
Whole-body MRI (or CT if MRI is not suitable) is used to survey the skeleton for bone lesions

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Myeloma - what is a common XRAY finding?

‘Rain-drop’ skull

Numerous randomly placed dark spots seen on X-ray which occur due to bone lysis

‘Raindrops keep falling on MYeloma head’ - way to remember finding

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Myeloma - what three factors is symptomatic multiple myeloma defined at diagnosis by?

Symptomatic multiple myeloma is defined at diagnosis by the presence of the following three factors:

Monoclonal plasma cells in the bone marrow >10%
Monoclonal protein within the serum or the urine (as determined by electrophoresis)
Evidence of end-organ damage e.g. hypercalcaemia, elevated creatinine, anaemia or lytic bone lesions/fractures

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Myeloma - management?

No Cure

Initial treatment:

If suitable for autologous stem cell transplant (ASCT) - Bortezomib + Dexamethasone
If UNsuitable for ASCT - Thalidomide + an Alkylating agent + Dexamethasone

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Myeloma - what do you give if the patient relapses after initial treatment?

Bortezomib monotherapy

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Myeloma - what are the complications and how do you manage them?

Pain: treat with analgesia (using the WHO analgesic ladder)
Pathological fracture: Zoledronic acid is given to prevent and manage osteoporosis and fragility fractures
Infection: patients receive annual influenza vaccinations, may also receive Immunoglobulin replacement therapy

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Lymphoma - what is it?

Lymphomas are a group of cancers that affect the lymphocytes inside the lymphatic system
These cancerous cells proliferate within the lymph nodes
Cause lymph nodes to become abnormally large (lymphadenopathy)

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Lymphoma - what are the definitions of Hodgkin’s lymphoma and non-Hodgkin’s lymphoma?

Hodgkin’s lymphoma is a specific disease

Non-Hodgkins lymphoma encompasses all the other lymphomas

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Hodgkin’s Lymphoma - what is it caused by?

Caused by the proliferation of lymphocytes

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Hodgkin’s Lymphoma - what is the age distribution?

Bimodal age distribution

20 years old

75 years old

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Hodgkin’s Lymphoma - what are the risk factors?

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HIV

Family History

EBV

Autoimmune conditions like sarcoidosis or RA

Hodgkin’s Lymphoma - what is the presentation?

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Key presenting symptom - lymphadenopathy

These lymph nodes are characteristically non-tender and feel rubbery

Some patients will feel pain in lymph nodes when they drink alcohol

Hodgkin’s Lymphoma - what are the B symptoms?

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Systemic symptoms of lymphoma:

Weight loss

Night sweats

Fever

Hodgkin’s Lymphoma - what are other symptoms patients might experience?

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SoB

Fatigue

Cough

Itching

Recurrent infections

Hodgkin’s Lymphoma - what is a blood test you can do and what is it’s finding?

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Lactate Dehydrogenase (LDH) - RAISED, but not specific

Hodgkin’s Lymphoma - what is the key diagnostic test and key finding?

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Lymph Node Biopsy

Reed-Sternburg cells, look like an owl on histology

Hodgkin’s Lymphoma - what system do you use to stage?

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Ann Arbor Staging

Hodgkin's Lymphoma - what are the stages of Ann-Arbor?

* * Stage 1: Confined to **one region** of lymph nodes * Stage 2: In more than **one region** but on the **same side** of the **diaphragm** (either above or below) * Stage 3: Affects **lymph nodes both above and below** the diaphragm * Stage 4: **Spread beyond** lymph nodes, such as to the lungs or liver Each stage may be subdivided into A or B A = no systemic symptoms other than pruritus **B** = weight loss \> 10% in last 6 months, fever \> 38c, night sweats (poor prognosis), or just **presence of B symptoms**

Hodgkin's Lymphoma - management?

Aim to cure with: ***Chemotherapy*** and ***radiotherapy***

Hodgkin's Lymphoma - what are the potential side effects of chemo and radiotherapy?

***Chemotherapy*** creates a risk of ***leukaemia*** and ***infertility*** ***Radiotherapy*** creates a risk of ***cancer***, damage to tissues and hypothyroidism

Non-Hodgkin's Lymphoma - what is it?

Non-Hodgkins lymphoma is a group of lymphomas

Non-Hodgkin's Lymphoma - what cells does it affect?

B or T cells

Non-Hodgkin's Lymphoma - what are three examples?

***Burkitt lymphoma*** - associated with EBV, malaria, HIV ***MALT lymphoma** -* affects the ***mucosa-associated lymphoid tissue***, usually around the ***stomach,*** associated with ***H. pylori*** infection ***Diffuse large B cell lymphoma** -* often presents as a rapidly growing painless mass in patients over 65 years

Non-Hodgkin's Lymphoma - what are some risk factors?

HIV Epstein-Barr Virus Family history Autoimmune disorders - SLE, Sjogren's H. pylori (MALT lymphoma) Hepatitis B or C infection Exposure to pesticides and a specific chemical called ***trichloroethylene*** used in several industrial processes

Non-Hodgkin's Lymphoma - what is the clinical presentation?

Very similar to Hodgkin's Lymphoma - only way to **differentiate** is by **lymph node biopsy** * Painless lymphadenopathy (non-tender, rubbery, asymmetrical) * Constitutional/B symptoms (fever, weight loss, night sweats, lethargy) * Above similar to HL * Extranodal Disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)

Non-Hodgkin's Lymphoma - how do you differentiate between HL and NHL?

only way to **differentiate** is by **lymph node biopsy**

Non-Hodgkin's Lymphoma - while biopsy is occurring, what aspects of the clinical presentation can help point towards Hodgkin's lymphoma or non-Hodgkin's lymphoma?

* Lymphadenopathy in **Hodgkin's lymphoma** can experience **alcohol-induced pain** in the node * **'B' symptoms** typically occur **earlier** in **Hodgkin's lymphoma** and later in non-Hodgkin's lymphoma * **Extra-nodal disease** is much more **common** in n**on-Hodgkin's lymphoma** than in Hodgkin's lymphoma

Non-Hodgkin's Lymphoma - what investigations can you do?

* **Excisional node biopsy** is the diagnostic investigation of choice - certain subtypes have classical appearance, **Burkitt's lymphoma**, **'starry sky'** appearance * CT chest, abdomen and pelvis - assess staging * FBC and blood film - normocytic anaemia and can help rule out other haematological malignancy such as leukaemia * ESR - useful as a prognostic indicator * LDH - a marker of cell turnover, useful as a prognostic indicator

Non-Hodgkin's Lymphoma - what is the staging system and stages?

Ann Arbor system Same as Hodgkin's Lymphoma

Non-Hodgkin's Lymphoma - management?

Management dependent on specific sub-type of non-Hodgkin's lymphoma and will typically take the form of **watchful waiting, chemotherapy or radiotherapy** All patients will receive flu/pneumococcal vaccines Patients with neutropenia may require antibiotic prophylaxis

DIC - what is it?

It is the inappropriate activation of the clotting cascade, resulting in thrombus formation and depletion of clotting factors and platelets

DIC - what are the causes?

Sepsis Burns Trauma Malignancy Severe obstetric complications

DIC - what is a typical blood picture?

* **↓ platelets**, due to them all being used up in thromboses * **↓ fibrinogen**, due to them all being used up in thromboses * **↑ PT & APTT**, due to decreased platelets and fibrinogen, so can't clot as quickly * **↑ fibrinogen degradation products**, fibrinogen, broken down to make fibrin for clots, and then fibrin broken down through fibrinolysis, to make sure clot isn't too big that it doesn't need to be * **Schistocytes** due to microangiopathic haemolytic anaemia, way to think about it is that the microclots that from in the blood veseels due to DIC, shred the RBCs as they pass by, leads to schistocytes

DIC - how does it present?

Patients present with **excess bleeding** e.g. **epistaxis**, **gingival bleeding**, **haematuria**, bleeding from cannula sites Patients may also present with **fever**, confusion, or coma Physical signs include **petechiae**, confusion, and **hypotension**

DIC - what leukaemia is associated with DIC?

**Acute promyelocytic leukemia (APL) is an uncommon subtype of acute myelogenous leukemia that is associated with DIC**

DIC - what is FDP?

fibrin degradation products

Haem - Myeloma, Lymphoma, DIC Flashcards

(43 cards)