Haematological lecture Flashcards

1
Q

What are the three main functions of blood?

A
  1. Regulation
  2. Transportation
  3. Protection.
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2
Q

What are the main components of blood?

A

1- Plasma (extracellular matrix that contains dissolved substances)
2- formed elements (cells and fragments) - WBCs RBCs and platelets.

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3
Q

What is the function of RBC, WBC and Platelets

A
  • RBCs - carry the o2 via haemoglobin
  • WBCs - fight infection (2 main types; neutrophils and lymphocytes
  • Platelets are for clotting and preventing/stopping bleeding.
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4
Q

What is haematopoiesis?

A
  • Process of creating blood cells

- all blood cells come from step cells in the bone marrow.

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5
Q

What is leukaemia?

A
  • neoplastic proliferation of WBCs
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6
Q

What is the function of different WBCs?

A

monocyte - mobile and phagocytic
lymphocyte - t-cell, immunity
granulocyte - neutrophil, protects against foreign material

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7
Q

What are the 3 types of haematological malignancies?

A
  • leukamia
  • polycythemia rubra vera
  • multiple myeloma
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8
Q

How is leukaemia characterised?

A
  • Leukaemia cells take up all of the space in the marrow so no room left for normal cells to grow
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9
Q

What are the four types of leukaemia and what groups do they affect?

A

ALL - Acute Lymphoblastic Leukaemia

  • occurs mainly in children/ rarely in adults
  • develops quickly/ appears suddenly

AML - Acute Myeloid Leukaemia
- occurs mainly in adults although can occur in children and adolescents

CLL - Chronic Lymphocytic Leukaemia
- Occurs in adults, does not occur in children

CML - Chronic Myeloid Leukaemia

  • Occurs at any age but rare >20yo
  • appears gradually and develops slowly.
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10
Q

What is ALL? + Pathology

A
  • Occurs mainly in children, rarely in adults
  • too many stem cells become lymphocytes = infection, easy bleeding and anaemia.

Pathology = increased number of immature lymphoblasts in the reticular-endothelial system.

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11
Q

What are the risk factors for ALL?

A
  • DOWN SYNDROME
  • Environmental exposure to benzene
  • Mother smoked marijuna/ was exposed to benzene during pregnancy
  • young age
  • some viral causes
  • blooms syndrome (causes chromosome breakage)
  • rad exposure as a child – ionising rad is leukomongic
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12
Q

What are the signs and symptoms of ALL?

A
  • shortness of breath
  • weight loss
  • fever due to infection
  • bone marrow failure leading to leucopenia and thrombocytopenia which case bruising and infection
  • splenomegaly
  • palpable liver
  • petechiae
  • peripheral lymphadeneopathy.

a child will generally present:

  • pale
  • few weeks of malaise
  • tender bones esp. over sternum
  • bone pain particularly in the long bone
  • could be menenigeal involvement leading to headaches, stiff neck and vomiting and vomiting
  • haemorrhages in the eye
  • mucosal ulceration and gingivitis.
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13
Q

What are some of the adverse prognostic indicators for ALL?

A
  • Philadelphia chromosome present
  • T-cell phenotype
  • CNS involvement at presentation
  • testicular relapse
  • adverse cytogenic markers
  • early marrow relapse
  • WBC count >20000
  • if it spreads to the spinal cord or the brain
  • whether it is a relapse that is being treated.
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14
Q

What are some of the clinical investigations that can be done for ALL? (8)

A
  • clinical examination – get pts history and look for anything unusual
  • cytogenic analysis – look for abnormalities in chromosomes using bone marrow/ blood samples. e.g. the Philadelphia chromosome.
  • Blood count - looking at the counts of everything
  • Peripheral blood smear - look for the presence of blast cells, platelets, and changes in the shape of WBCs.
  • Liver examination
  • Immunophenotyping
  • radiography
  • Bone marrow aspiration or biopsy - a needle is inserted through the hip/pelvic bones from skin to marrow and a small sample of bone marrow is taken. Done under general anaesthesia in the ward, takes 15mins. Looks for anything abnormal. Sometimes need a core of Bone marrow (trephine biopsy).
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15
Q

What staging system is used for ALL?

A
  • No specific staging system used.
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16
Q

What is AML characterised by?

A
  • Occurs in mainly adults can occur in younger people but rare under 20.
  • over production of white blood cells called myeoblasts / leukaemic blasts.
  • thought to be associated with damage to the chromosome that cause blood development.
17
Q

What are the risk factors for AML?

A
  • No links to environmental agents
  • those who have had rad exposure and some chemos
  • downs syndrome
  • pts who have some specific blood disorders e.g. myodisplastic syndrome
  • CHROMOSOMAL ABNORMALITIES
18
Q

What are the signs and symptoms (aetiology) of AML?

A
  • infection
  • decreased platelet count/ healthy WBC/ RBC
  • anemia causing them to feel tired and look pale
  • bone marrow failure (lc)
  • malaise
  • UNUSUAL BLEEDING AND BRUISING
  • ACHING BONES AND JOINTS
  • bone pain (lc)
  • leukostasis (lc)
19
Q

What are some of the clinical investigations done for AML?

A
  • Radiography to see the extent of infection
  • clinical examination
  • CHROMOSOME ANALYSIS
  • BIOCHEMISTRY – shows abnormal liver function
  • full blood count
20
Q

What is the classification used for AML?

A

-FAB (French-American-British)

21
Q

What are the characteristics of CML?

A
  • common in adults, rare >20
  • excess amount of granulocytes
  • chronic period of 2-4 years, acute period of 3-9 months
22
Q

What is the only cure for CML?

A
  • Stem cell transplant
23
Q

CML risk factors

A
  • unknown
  • Philadelphia chromosome in most cases
  • some cases due to exposure to ionising radiation
24
Q

CML signs and symptoms

A
  • splenomegaly
  • ITCHING
  • lymphadectomy
  • increased WBC count
  • womens periods become heavier
  • weight loss
  • petechiae
  • bone pain
  • fevers
  • UNUSUAL EXCESSVE BLEEDING
25
Q

What are some investigations that may be done for CM

A
  • ultrasound to confirm hepatomegaly and splenomegaly
  • bone marrow biopsy/ aspiration – show BCR - ABL gene
  • blood cell count
26
Q

What are the three phases of CML?

A

chronic phase

  • tx usually performed as outpatient who has regular blood tests
  • rarely symptoms
  • usually lasts 4-5 years
  • disease progresses slowly

accelerated phase

  • disease accelerates
  • inpatient
  • increased symptoms
  • not everyone goes through this phase
  • patient will become very tired
  • increase in the number of blast cells

blast phase

  • disease becomes more like acute leukaemia
  • increase in the blast cells (much of the bone marrow replaced by these)
  • blast crisis = enlarged spleen, increased temp and fevers
  • myelofibrosis = doesn’t occur in every patient but due to fibrosis bone marrow can’t produce new cells.
27
Q

For CML what is considered remission and what is complete remission

A

revision - when the number of blast cells returns to normal.
complete remission - philideliphia chromosome not found by even the most sensitive test. (PCR test)

28
Q

What characterises CLL?

A

Chronic lymphocytic leukaemia

  • damage to dna causing uncontrolled growth and production of B lymphocytic cells.
  • important distinction from ALL is that it doesn’t impede the production of blood when it accumulates in the marrow as much.
29
Q

What are the risk factors for ALL?

A
  • NOT associated with radiation exposure like the rest of them
  • maybe fam history
  • not really known though
30
Q

What are the signs and symptoms of ALL?

A
  • peripheral lymphadenopathy
  • splenomegaly
  • hepatomegaly
  • anemia
  • RECURRANT INFECTIONS OF THE KIDNEY AND SKIN
  • fever
  • sweats
  • weight loss
31
Q

Investigations

A
  • Immunophenotyping
  • Bone marrow biopsy to show excess lymphoid cells
  • blood count
32
Q

What is the prognosis for CLL?

A
  • remission easily achieved but pts never really cured.

- pt can live many years in remission

33
Q

What is hairy cell leukaemia?

A
  • over production of b-lymphocytic cells with hair like projections.
  • these cells get caught in the spleen causing it to become enlarged, which also further worsens conditions like anaemia etc.
  • don’t know why it happens
  • predominantly occurs in 40-60yo
  • develops slowly and may not cause signs and symptoms for a long time.
34
Q

How is multiple myeloma characterised?

A
  • increased production of immature abnormal plasma cells and monoclonal immunoglobins.
35
Q

What are the risk factors in multiple myeloma

A
  • race
  • increasing age
  • BRACA 1 / 2 gene
  • rad exposure
36
Q

What are the signs and symptoms for multiple myeloma?

A
  • depends on how far the immunoglobin involved
  • pts can be assymtomatic for years
  • diffuse bone = major complaint
  • skeletal abnormalities - leads to pathological fractures and vertebral crush
  • weakness and fatigue
  • anorexia and weight loss
  • anemia
  • hypercalcemia
  • paraprotein = leads to renal failure