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Haematology > Malignant Haematology > Flashcards

Malignant Haematology Flashcards

1
Q

Causes of haematological malignancies

A
  1. Leukaemias
  2. lymphomas
  3. Myelodysplasias
  4. myeloproliferative disorders
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2
Q

Types of leukaemias

A

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

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3
Q

Acute lymphoblastic leukaemia (ALL) definition

A

malighnancy of lymphoblast cells (T & B cells)

“arresting maturation and uncontrolled proliferation of immature blast cells”

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4
Q

Causes of Acute lymphoblastic leukaemia (ALL)

A

Genetic (transloactions + Klinefletors)
+
env trigger

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5
Q

Presentation of Acute lymphoblastic leukaemia (ALL)

A

Tired, pale, bruised limping child !!

Anaemia: weakness and lethargy

Haemorrhage: purpura, bruising

Infiltration: lymphadenopathy, splenomegaly

decreased functioning WCC: infection

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6
Q

Investigations of Acute lymphoblastic leukaemia (ALL)

A

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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7
Q

FAB classification of Acute lymphoblastic leukaemia (ALL)

A

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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8
Q

Chemo Management of Acute lymphoblastic leukaemia (ALL)

A

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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9
Q

Supportive management of Acute lymphoblastic leukaemia (ALL)

A

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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10
Q

Chronic lymphocytic leukaemia definition

A

Clonal expansion of lymphocytes (mainly B cells)

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

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11
Q

Chronic lymphocytic leukaemia features

A

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

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12
Q

Presentation of Chronic lymphocytic leukaemia

A

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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13
Q

Investigations of Chronic lymphocytic leukaemia

A

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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14
Q

Staging of chronic lymphocytic leukaemia

A

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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15
Q

Management of chronic lymphocytic leukaemia

A

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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16
Q

Complications of chronic lymphocytic leukaemia

A

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)

17
Q

Prognosis of chronic lymphocytic leukaemia

A

Mainly incurable

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

18
Q

Acute myeloid leukaemia definition

A

Prolieration of immature NON-lymphoid cells

19
Q

Acute myeloid leukaemia Features

A

Presentation similar to ALL

Increases incidence with age

20
Q

Risk factors of Acute myeloid leukaemia

A

Myelodysplasia
Down Syndrome
Prior chemotherapy ± radiotherapy
Benzene exposure

21
Q

Investigations of Acute myeloid leukaemia

A

FBC
- increased WCC, pancytopenia

Blood smear
-Blasts

Bone marrow

  • Blasts, Auer rods
  • immunophenotyping & cytogenetics
22
Q

Management of acute myeloid leukaemia

A

Intensive chemotherapy ± SCT

65, 5 year survival

23
Q

Prognosis of acute myeloid leukaemia

A

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

24
Q

Define leukaemia

A

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

25
Q

Chronic myeloid leukaemia definition

A

uncontrolled proliferation of myeloid precursors, ability to differentiate remains intact

26
Q

Cause of chronic myeloid leukaemia

A

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

27
Q

Presentaiton of chronic myeloid leukaemia

A

insidious onset

  • weight decrease
  • fatigue
  • sweats
  • fever

abnormal discomfort (splenomegaly )

28
Q

Investigations of chronic myeloid leukaemia

A

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

29
Q

Phases of chronic myeloid leukaemia

A

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

30
Q

Treatment of chronic myeloid leukaemia

A

Imatinib

Tyrosine kinase inhibitors

31
Q

Prognosis of chronic myeloid

A

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

32
Q

Prognosis of ALL

A

5 year survival
90% in children
40% in adults

Cytogenetics
t(10:14) = favourable
Normal = intermediate
t(9:22) = adverse

33
Q

Myeloma Definition

A

haematological malignancy of plasma cells

34
Q

Causes of myeloma

A

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

35
Q

Presentation of myeloma

A

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
36
Q

Triad of myeloma

A
  1. Increased plasma cells in bone marrow
  2. Clonal immunoglobulin or paraprotein
  3. Lytic bone lesions
37
Q

Investigations of myeloma

A

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

38
Q

Myeloma treatment

A

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

Haematology (11 decks)

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