Haem- malignancy Flashcards by Elise Giblin

what is leukaemia

a group of blood cancers with an increase in white blood cells

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what is chronic myeloid leukaemia

Proliferation of myeloid cells - granulocytes and their precursors, other lineages (platelets)

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what genetic abnormality is characteristic of chronic myeloid leukaemia (CML)

Philadelphia chromosome- t(9:22)

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Philadelphia chromosome results in a new gene-

BCR-ABL1

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what are the three phases of CML

chronic phase
accelerated phase
blast phase

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how long can the CML chronic phase last and how is it often discovered

can last around 5 years
often asymptomatic discovered incidentally through raised WBC count

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CML accelerated phase

high proportion of the cells in the bone marrow and blood (10-20%)
patients become more symptomatic, develop anaemia, thrombocytopenia, and become more immunocompromised

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CML blast phase

high proportion of blast cells (>30%)
This phase has severe symptoms and pancytopenia
It is often fatal

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CF of CML

asymptomatic
splenomegaly
hyper metabolic symptoms
gout

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treatment of CML

tyrosine kinase inhibitors e.g. imatinib

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MOA of tyrosine kinase inhibitors e.g. imatinib

Prevents the action of theBCR-ABL fusion protein i.e.this is the abnormal protein produced by the Ph mutation

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what is acute myeloid leukaemia

malignant disease of primitive myeloid cells (excess of myeloblasts)

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acute myeloid leukaemia common age range

> 60 years

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what is acute lymphoblastic leukaemia

Malignant disease of primitive lymphoid cells (lymphoblasts)

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what is the most common childhood cancer

acute lymphoblastic leukaemia

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what syndrome is ALL often associated with?

Down syndrome

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CF of ALL

marrow failure- anaemia, infections, bleeding
leukaemic effects
bone pain

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abnormal cells ‘blasts’ features

large size
high nuclear:cytoplasmic ratio
prominent nucleolus

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blood film specific to AML

Auer rod- Blast cells have rods inside their cytoplasm

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diagnostic tool required for definite diagnosis of AML/ALL

immunophenotyping

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multi-agent chemotherapy in AML

intensive chemo (3-4 cycles)
prolonged hospitalisation

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multi-agent chemotherapy in ALL

can last up to 2-3 years
different phases of treatment at varying intensity

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what is a Hickman line

used to provide long-term central venous access

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complications of acute leukaemia

anaemia
neutropenia
thrombocytopenia

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neutropenia complication

infections- increased severity and duration gram negative bacteria can cause life threatening sepsis in neutropenic patients prolonged neutropenia makes patients susceptible to fungal infections

thrombocytopenia complication

bleeding- purport, petechiae

complications of anti-leukaemic treatment

nausea and vomitting hair loss liver, renal dysfunction tumour lysis syndrome infection late affects (eg infertility, cardiomyopathy)

single most important prognostic factor in AML

cytogenic type

females/males have better prognosis in ALL?

females

what is chronic lymphocytic leukaemia

chronic proliferation of a single type of well differentiated lymphocyte, usually mature B-lymphocytes

common age range for CLL

>55 years

CF of CLL

asymptomatic non-specific (night sweats etc) lymph node/spleen enlargement infections bleeding warm autoimmune haemolytic anaemia

blood film findings in CLL

>lymphocytes smear cells

what does the BCR-ABL1 gene code for

tyrosine kinase

first and second line treatment for chronic myeloid leukaemia

first line- imatinib second line- dasatinib

at what age does acute lymphoblastic leukaemia typically peak

2-4 years

Is Acute Lymphoblastic Leukaemia (ALL) associated with any chromosomal abnormalities?

Yes, the Philadelphia chromosome (t(9:22) translocation) is associated with ALL in 30% of adults and 3-5% of children

what type of lymphocytes are usually involved in ALL

B-lymphocytes

by definition, how many 'blasts' are required in the peripheral blood or bone marrow for a diagnosis of acute leukaemia

at least 20% blasts in either peripheral blood or bone marrow

what cytogenetic abnormalities are commonly found in chronic lymphocytic leukaemia (CLL)

deletion of 13q (most common) trisomy 12

what are the absolute indications for treatment in chronic lymphocytic leukaemia

weight loss of over 10% over 6 months night sweats for more than 1 month progressive marrow failure (anaemia or thrombocytopenia)

what is a major complication of chronic lymphocytic leukaemia

can transform into a high-grade lymphoma

what is Richter's transformation

development from chronic lymphocytic leukaemia into aggressive lymphoma, usually diffuse large B-cell lymphoma

which type of leukaemia is often associated with myelodysplastic syndromes but can arise ‘de novo’

acute myeloid leukaemia

what is lymphoma

group of cancers that affect the lymphocytes inside the lymphatic system; cancerous cells proliferate within lymph nodes and cause lymph nodes to become abnormally large

what percentage of lymphomas does Hodgkins lymphoma account for

1/5 of all lymphomas

cause of Hodgkins lymphoma

proliferation of lymphocytes

what are the two age peaks of Hodgkins lymphoma

age 20 age 75

risk factors of Hodgkins lymphoma

immunosuppression (eg HIV) autoimmune disorders (eg rheumatoid arthritis, sarcoidosis) epstein-barr virus (EBV) family history

what is Burkitt lymphoma associated with

epstein-barr virus malaria HIV

what is MALT lymphoma and what is it associated with

affects the mucosa-associated lymphoid tissue, usually around the stomach associated with H.pylori infection

How does Diffuse large B cell lymphoma typically present in patients over 65 years?

rapidly growing painless mass

risk factors for non-hodgkins lymphoma

infections- HIV, EBV, H.pylori hepatitis b or c infection Exposure to pesticides and a specific chemical called trichloroethylene used in several industrial processes family history

clinical features of lymphoma

enlarged lymph nodes- non tender, rubbery, may be painful when drinking alcohol systemic symptoms itch without rash

what is the significance of LDH in blood tests for Hodgkins lymphoma

LDH often raised in Hodgkins lymphoma, but it is not specific and can be elevated in other cancers and many non-cancerous diseases

what blood results are associated with a poor prognosis in Hodgkins lymphoma

elevated ESR and/or decreased haemoglobin

what is the key diagnostic test for Hodgkins lymphoma

lymph node biopsy

what is the key finding in a lymph node biopsy for hodgkins lymphoma

the reed-sternberg cell- abnormally large b cells with multiple nuclei and nucleoli inside them

what is the Ann-arbor system

used for staging Hodgkins lymphoma, compromising four key stages (I-IV) with possible subcategories (A and B)

What does Stage I in the Ann-Arbor staging system represent?

presence of single lymph node involved

What does Stage II in the Ann-Arbor staging system represent?

Stage II involves two or more lymph nodes/regions on the same side of the diaphragm

What does Stage III in the Ann-Arbor staging system represent?

Stage III involves nodes on both sides of the diaphragm

What does Stage IV in the Ann-Arbor staging system represent?

spread beyond the lymph nodes to other organs or tissues

what is the primary treatment of Hodgkins lymphoma

multi-agent chemotherapy with/without radiation

what does the acronym ABVD stand for in Hodgkin lymphoma treatment

adriamycin bleomycin vinblastine dacarbazine

what potential side effect is associated with bleomycin in Hodgkin lymphoma treatment

pneumonitis

good/bad cure rates in Hodgkins lymphoma?

good, especially in younger patients

What does the acronym R-CHOP stand for in Non-Hodgkin lymphoma treatment?

Rituximab cyclophosphamide hydroxydaunorubicin vincristine (oncovin) prednisolone

what are some risks associated with R-CHOP treatment of non-hodgkins lymphoma

neutropenia cardiotoxicity

which type of lymphoma causes a classical 'starry sky appearance' on lymph node aspirate histology

burkkitts lymphoma

which chromosomal translocation is associated with burkitts lymphoma

translocation between chromosomes 8 and 14

what is the median age of diagnosis for polycythaemia vera (PV)

~65, but can affect younger patients

what genetic mutation is present in over 95% of PV patients

JAK2 kinase mutation (substitution)

what is polycythaemia vera (PV)

rare blood cancer where bone marrow produces too many red blood cells

which type of haematological malignancy is associated with aquagenic pruritus

polycythaemia vera (PV)

target haematocrit in PV management

<0.45

what procedure is used to reduce haematocrit in PV patients

venesection

what is an example of a cytotoxic oral chemotherapy used in PV

hydroxycarbamide

JAK2 mutations in approx how many patients with essential thrombocytaemia

50-60%

what is idiopathic myelofibrosis

healthy bone marrow replaced by fibrosis, resulting in a lack of production of normal cells

what mutations are associated with idiopathic myelofibrosis

JAK2 CALR MPL gene

teardrop shaped RBCs in peripheral blood characteristic of-

idiopathic myelofibrosis