Malignant Haematology Flashcards by Taryn Miller

Causes of haematological malignancies

Leukaemias
lymphomas
Myelodysplasias
myeloproliferative disorders

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Types of leukaemias

Acute lymphoblastic leukaemia (ALL)
Acute myeloid leukaemia (AML)
Chrninic lymphocytic leukaemia (CLL)
Chronic myeloid leukaemia (CML)

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Acute lymphoblastic leukaemia (ALL) definition

malighnancy of lymphoblast cells (T & B cells)

“arresting maturation and uncontrolled proliferation of immature blast cells”

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Causes of Acute lymphoblastic leukaemia (ALL)

Genetic (transloactions + Klinefletors)
+
env trigger

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Presentation of Acute lymphoblastic leukaemia (ALL)

Tired, pale, bruised limping child !!

Anaemia: weakness and lethargy

Haemorrhage: purpura, bruising

Infiltration: lymphadenopathy, splenomegaly

decreased functioning WCC: infection

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Investigations of Acute lymphoblastic leukaemia (ALL)

FBC
- WCC and pancytopenia

Peripheral blood film

immature lymphoblasts
FAB classification

BM biopsy

Blast infiltrate >20%
cytogenetics

CT and CXR
-lymphoblastic lymphoma

Lumar puncture
-CNS involvement

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FAB classification of Acute lymphoblastic leukaemia (ALL)

FAB classification:
L1 – small uniform blast cells with scanty cytoplasm
L2 – large heterogenous blast cells with nucleoli and low nuclear-cytoplasmic ratio
L3 – basophilic vacuolated blast cells

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Chemo Management of Acute lymphoblastic leukaemia (ALL)

Complex, specialist units

Often in clinical trial

Prednisolone, cyclophosphamide, anthracycline, vincristine, etoposide

1) Remission Induction: achieve complete remission
2) Consolidation Therapy: reduce risk of relapse
3) CNS Prophylaxis: irradiation / intrathecal
4) Maintenance Therapy: 1-2 years (or SCT)

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Supportive management of Acute lymphoblastic leukaemia (ALL)

Tranfusion – if symptomatic

Fresh frozen plasma – for coagulopathy or DIC

Platelet transfusion – if bleeding/bruising and during fever/sepsis/DIC

Allopurinol and good urine output – to prevent tumour lysis syndrome:
-occurs in leukaemias with high WCC due to tumour necrosis (from treatment) causing renal, cardiac and neurological complications; allopurinol prevent hypercalcaemia and good urine output prevents hyperkalaemia

Antibiotics – prophylaxis against neutropenia; also should have antifungal, antiviral, good mouthcare, avoid certain foods (cream, paté, raw foods)

Growth factors – if neutropenic

Granulocytes – if unresponsive to treatment of infection

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Chronic lymphocytic leukaemia definition

Clonal expansion of lymphocytes (mainly B cells)

Accumulate in blood, lymph nodes, bone marrow, spleen and liver

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Chronic lymphocytic leukaemia features

males> females (2;1)
Elderly
mean age is 67

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Presentation of Chronic lymphocytic leukaemia

50% incidental
- Lymphocytosis on FBC

Symptomatic

Anaemia: lethargy, pallor, dyspnoea
Thrombocytopenia: bruising/bleeding

Lymphadenopathy, splenomegaly, hepatomegaly

Recurrent infections

B symptoms

Fever
Night sweats
Weight loss

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Investigations of Chronic lymphocytic leukaemia

FBC
-Lymphocytosis (>5x109/L), pancytopenia

Blood film
-Larger normal mature lymphocytes & smear cells

Bone marrow biopsy

Increased numbers of lymphocytes
Nodular or diffuse infiltrate

Immunophenotyping
Via flow cytometry: CD5, 19, 20, 23 +ve

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Staging of chronic lymphocytic leukaemia

Binet staging system (or RAI)

Stage
A- 3 LN involved no anaemia or thrombocytopenia 5 years
C - Anemia or thrombocytopenia 2 years

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Management of chronic lymphocytic leukaemia

Stage A:
- Observation only

Stage C or symptomatic:

Fludarabine, Cyclophosphomide, Rituximab
Allogeneic SCT

Palliative:

Minimum toxicity chemotherapy
Radiation to refractory, bulky lymphadenopathy

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Complications of chronic lymphocytic leukaemia

Immune complications

Autoimmune haemolytic anaemia
Autoimmune thrombocytopenia
Manage with steroids

Infections
-As likely to die of infection as from CLL
-Pulmonary infections common
(Bacterial, viral, pneuomocystis, fungal)

Prognosis of chronic lymphocytic leukaemia

Mainly incurable

-5 year survival 18 months-10 years
-Poor prognosis from cytogenetics
-17p and 11q deletions
(Loss of p53)- Chemo can’t work

Acute myeloid leukaemia definition

Prolieration of immature NON-lymphoid cells

Acute myeloid leukaemia Features

Presentation similar to ALL

Increases incidence with age

Risk factors of Acute myeloid leukaemia

Myelodysplasia
Down Syndrome
Prior chemotherapy ± radiotherapy
Benzene exposure

Investigations of Acute myeloid leukaemia

FBC
- increased WCC, pancytopenia

Blood smear
-Blasts

Bone marrow

Blasts, Auer rods
immunophenotyping & cytogenetics

Management of acute myeloid leukaemia

Intensive chemotherapy ± SCT

65, 5 year survival

Prognosis of acute myeloid leukaemia

5 year survival
Children: 60-70%
Adults (60): 10-20%

Prognostic factors
t(15:17) = favourable
Normal = intermediate
t(6:9), 2° AML, relapsed AML, elderly = unfavourable

Define leukaemia

Malignancy of haematopoietic stem cells resulting in the accumulation of abnormal cells in the bone marrow ± peripheral circulation

Chronic myeloid leukaemia definition

uncontrolled proliferation of myeloid precursors, ability to differentiate remains intact

Cause of chronic myeloid leukaemia

t (9:22)- reciricol transloaction between long arm of 9 and long arm of 22 forms fusion gene with BCR-ABL has tyrosine kinase acitivty KNOWN AS THE PHILIDELPHIA CHROMOSOME

Presentaiton of chronic myeloid leukaemia

insidious onset - weight decrease - fatigue - sweats - fever abnormal discomfort (splenomegaly )

Investigations of chronic myeloid leukaemia

BM -hypercellular FBC - increased WCC (often > 100x 10 9) - neutrophils, basophils, eosinophills meylocytes - decreased hb or normal - Plts variable - urate and B12 Cytogenetics -look for philly chromosome

Phases of chronic myeloid leukaemia

Chronic -few symptoms can last years Accelerated: -increaseing symptoms and splenic size- symptoms form cellular accumulation Acute - blast trasnformations from; symptoms from bone marrow failure

Treatment of chronic myeloid leukaemia

Imatinib | Tyrosine kinase inhibitors

Prognosis of chronic myeloid

1/3 never rpogress 1.3 progress slowly 1/3 progress rapidly

Prognosis of ALL

5 year survival 90% in children 40% in adults Cytogenetics t(10:14) = favourable Normal = intermediate t(9:22) = adverse

Myeloma Definition

haematological malignancy of plasma cells

Causes of myeloma

Abnormal prolferation of a single clone of plasma or lymphocytes Leads to secretion of monoclonal (paraprotein) Ig light chain (igG - most common, IgA second most common) Accumulates in blood Arises in BM Microenvironment produces cytokines - osteoclastogenesis

Presentation of myeloma

Majority of new cases have non-specific symptoms - backache or rib pain (60%) - fatigue - symptoms from hyper Ca++ (30%) - recurrent infections - renal impairment (25-30%) ``` CRAB C- hypercalcamia R- renal dysfunction A- anaemia B- bone pain ```

Triad of myeloma

1. Increased plasma cells in bone marrow 2. Clonal immunoglobulin or paraprotein 3. Lytic bone lesions

Investigations of myeloma

Blood tests - FBC - anaemia or thrombocytopenia - U and Es - increased Ca2+, increased urea, + creatiie Urinalysis - to look for M-protein in urine – “Bence-Jones protein - Red cell casts Bone marrow aspirate Imaging Serum and urine electrophesis M band

Myeloma treatment

Fit for autograft? Chemotherapy – novel therapies eg thalidomide, bortezomib Radiotherapy BMT – autograft if fit; allograft rare Supportive therapy - biphosphonates - blood transfusion/EPO - surgery - interventional radiology