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CBL_7 Haematology Flashcards

1
Q

Normal hematopoiesis diagram

A
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2
Q

What’s the most preferred location for bone marrow biopsy?

A

upper pelvic bone (posterior iliac crest) -> no major blood vessels or organs are located nearby

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3
Q

Do malignant cells function normally?

A

No

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4
Q

Why we can see abnormal blood count in leukemia?

A

Leukaemia cells proliferating in the bone marrow will replace normal cells -> reduce other elements of the bone marrow -> abnormal blood counts

*as bone ,arrow will become ‘over-crowded’

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5
Q

What type of leukemia is more common in old age?

A

Acute Myeloid Leukemia (AML)

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6
Q

What type of leukemia is more common in children?

A

Acute Lymphoblastic Leukemia (ALL)

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7
Q

What’s the Philadelphia chromosome?

A

Chromosome 22

*in chronic myeloid leukemia: arms of chromosomes 22 and 9 switches the places (translocation)

This combination causes BCR-ABL genes to combine -> this switches Tyrosine Kinase -> uncontrolled proliferation

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8
Q

(2) characteristics of myelodysplastic syndrome

A
  • defective maturation of myeloid cells
  • build up of blasts (in the bone marrow)
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9
Q

What % of blasts is seen in:

A. Myelodysplastic syndrome

B. ALL

A
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10
Q

Risk factors for Acute Leukemia

A
  • Down Syndrome
  • exposure to radiotherapy (e.g. used in Rx for other malignancy)
  • exposure to alkylating chemotherapy
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11
Q

Symptoms of AML and ALL

A
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12
Q

Why is there hepatosplenomegaly and lymphadenopathy in leukemia?

A

Blasts (T cells) migrate to the organs

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13
Q

What does peripheral blood smear show in AML and what in ALL? (simply)

A
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14
Q

Features of myeloblasts in leukemia on peripheral blood smear

A

myeloblasts in leukemia = AML

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15
Q

Features of lymphoblasts in leukemia on peripheral blood smear

A

Lymphoblasts = ALL

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16
Q

What are (2) markers for lymphoblastic leukemia in immunophenotyping ?

A
  • CD10 -> surface marker for pre B cells
  • TdT -> DNA polymerase only in lymphoblasts
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17
Q

What is the key distinction between Acute and Chronic Leukemias?

A

Acute -> cells do not mature at all

Chronic -> cells mature only partially

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18
Q

What is a simple difference in pathophysiology of CML and CLL?

A
  • CML -> cells divide too quickly
  • CLL -> cells do not die as they should

Result of both: too many premature cells (as chronic leukemia = cells develop only partially)

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19
Q

Symptoms of Chronic Myeloid and Chronic Lymphoid Leukemia

A
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20
Q

The difference on a peripheral blood smear in CML and CLL

A
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21
Q

Clinical features of Acute Leukemias

A

–Tiredness, infection, bruising/bleeding

–High white cell count

–Low haemoglobin (anaemia)

–Low platelet count (thrombocytopenia)

–Low neutrophil count (neutropenia)

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22
Q

What type of cancer is it?

A

Auer Rod = Acute Myeloid Leukemia

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23
Q

What’s therapy for AML? (2)

A

AML

–3 to 4 cycles of combination chemotherapy

–Haemopoietic stem-cell transplant (HSCT) for some patients

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24
Q

What’s myelodysplasia?

A

Myelodysplasia -> an acquired neoplastic disorder of hematopoietic stem cells, they are pre-leukaemia and are most likely to progress to AML

They are more common with age, and they present with bone marrow failure

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25
In what type of cancer ***gum hypertrophy*** can be seen?
***Acute Myeloid Leukemia***
26
DIagnostic features of AML (3)
* WCC is often high, but this can be normal or low * Blast cells may be few in the peripheral blood * _**Auer Rods** are diagnostic of AML_
27
What's the supportive management of AML?
**_Supportive:_** * Blood/platelet transfusion * IV fluids. * Allopurinol and this is to prevent tumour lysis syndrome * Insert Hickman line for IV access (central venous catheter used for administration of chemo) * Walking can relieve the fatigue
28
Chemotherapy in ***AML*** - two main drugs used in chemo
* very intensive, resulting in long periods of marrow suppression with neutropenia and low platelets. **_Two main drugs used in chemo:_** Daunorubicin Cytarabine
29
What's **Myelodysplastic syndrome**?
30
What's the diagnosis of ***myelodysplastic syndrome***?
31
What's the management of ***myelodysplastic syndrome***?
32
Supportive Mx of ALL
* Blood/platelet transfusion * IV fluids * Allopurinol and this is to prevent tumour lysis syndrome * Insert Hickman line for IV access.
33
In what type of hematological cancer CNS involvement is common?
ALL
34
Investigations done in ALL
* Characteristic blast cells on b**lood film** and **bone marrow** * **WCC** are high * **CXR** and **CT** scan to look for mediastinal and abdominal lymphadenopathy. * **Lumbar puncture** should be done and that is to see if there is any CNS involvement.
35
Management of **infections** in ALL
Infections are dangerous and that is because of neutropenia caused by the disease * Immediate IV antibiotics * Prophylactic antibiotics, antivirals and antifungals * Neutropenic regimen (specific combination of ABx)
36
Steps in chemotherapy in ALL (3)
1. To induce **remission** (different chemo agents e.g. vinicristine) 2. **Consolidation**: medium dose chemo over few weeks 3. **Maintenance**: prolonged chemotherapy, mercaotopurine, methotrexate and vincristine and prednisolone for 2 years
37
What's given in terms of CNS protection in ALL?
ALL = often CNS involvement CNS prophylaxis: intrathecal or high dose IV methotrexate or CNS radiation
38
What condition overproduction of these mature blood cells may result in **Overproduction of:** **A. Neutrophils** **B. RBCs** **C. Platelets**
39
What's the most common leukemia seen in adults?
* **Chronic lymphocytic leukaemia** (CLL) is caused by a monoclonal proliferation of well-differentiated lymphocytes which are almost always B-cells (99%) * It is the most common form of leukaemia seen in adults
40
Features of CLL
* often none -\> found on blood test * constitutional: anorexia, weight loss * bleeding, infections * lymphadenopathy more marked than CML
41
What's ***Richter's transformation***?
**Chronic Lymphocytic Leukemia (CLL)** -\> possible transformation to high-grade lymphoma (Richter's transformation)
42
What is typical finding on blood smear in CLL?
***Smudge cells*** (aka smear cells)
43
What type of cancer is Philadelphia Chromosome mostly associated with?
CML (chronic myeloid leukemia)
44
Presentation of CML
Usually in 60-70 years * anaemia: lethargy * weight loss and sweating are common * splenomegaly may be marked →abdo discomfort * decreased leukocyte alkaline phosphatase * bone pain
45
Management of CML
* ***imatinib*** is now considered first-line treatment * hydroxyurea * interferon-alpha * allogenic bone marrow transplant ***Imatinib*** inhibitor of the tyrosine kinase associated with the BCR-ABL defect (Philadelphia chromosome) very high response rate in chronic phase CML
46
What's ***Imatinib***?
***Imatinib*** * inhibitor of the tyrosine kinase associated with the BCR-ABL defect (Philadelphia chromosome) * very high response rate in chronic phase CML
47
What's '***blast crisis***'?
blast crisis’ – i.e. transformation of CML to Acute Leukaemia (can either be ALL of AML)
48
What's myeloma? What's the peak incidence for myeloma?
Myeloma is neoplasm of a bone marrow Peak incidence 60-70 y
49
What are the clinical features of myeloma?
Clinical features * **bone disease:** bone pain, osteoporosis + pathological fractures (typically vertebral), osteolytic lesions * **lethargy** * **infection** * **hypercalcaemia** * **renal failure** * other features: amyloidosis e.g. Macroglossia, carpal tunnel syndrome; neuropathy; hyperviscosity
50
Investigations for myeloma (3)
* urine and bloods -\> characteristics of myeloma: monoclonal proteins (usually **IgG** or **IgA)** in the serum and urine ***(Bence Jones*** proteins) * Whole body MRI -\> to look for bone lesions * **X-rays**: '*rain-drop skull*' (random pattern of dark spots)
51
What are the characteristic findings in serum and urine in myeloma? (2)
* monoclonal proteins (serum) -\> usually **IgA**, **IgG** * ***Bence Jones*** proteins in urine
52
What are the diagnostic criteria for myeloma?
The diagnostic criteria for multiple myeloma requires **one major** and **one minor** criteria or **three minor criteria** in an individual who has signs or symptoms of multiple myeloma. _Major criteria_ * Plasmacytoma (as demonstrated on evaluation of biopsy specimen) * 30% plasma cells in a bone marrow sample * Elevated levels of M protein in the blood or urine _Minor criteria_ * 10% to 30% plasma cells in a bone marrow sample. * Minor elevations in the level of M protein in the blood or urine. * Osteolytic lesions (as demonstrated on imaging studies). * Low levels of antibodies (not produced by the cancer cells) in the blood.
53
Why is there hypercalcaemia in myeloma?
**_Hypercalcaemia in myeloma_** * primary factor: due primarily to **increased osteoclastic bone resorption caused by local cytokines** (e.g. IL-1, tumour necrosis factor) released by the myeloma cells * much less common contributing factors: impaired renal function, increased renal tubular calcium reabsorption and elevated PTH-rP levels
54
Treatment for myeloma
–Induction with combination chemotherapy –Autologous stem-cell transplant in first remission
55
What is the sign? What's the diagnosis?
'Rain drop' skull In multiple myeloma
56
What is the kidney failure a result of in multiple myeloma?
Deposition of abnormal proteins (e.g. abnormal monoclonal antibodies) produced by malignant cells
57
What's the mnemonic and meaning for the presentation of multiple myeloma?
**C constipation**, nausea, poor appetite -\> due to hypercalcaemia **R - Renal failure** (due to deposition of proteins produced by malignant plasma change) - may present with thirst **A- Anaemia** - may presence with fatigue and pallor (due to crowded bone marrow, depletion of RBCs) **B - bone lesions/ fractures/ Back pain** - as cytokines produced by malignant plasma cells increase bone turnover -\> more calcium into the blood -\> fragile bones
58
What's that called? What condition is it?
Rouleaux of RBCs -\> RBCs attached in chains Multiple myeloma
59
What (in general) lymphoma is?
* **Lymphomas** are disorders caused by malignant proliferation of lymphocytes * These accumulate in the lymph nodes caused lymphadenopathy, but may also be found in peripheral blood or infiltrate organs * Tumours of lymphatic system: lymph nodes, MALT, splee, bone marrow
60
Classification of lymphomas
61
What is (2) peak incidence for Hodgkin lymphoma?
* 15 - 30 * \>65
62
What are the cells characteristic of Hodkin's lymphomas?
The characteristics of Hodgkin's are cells with mirror-image nuclei, and these are called ***Reed-Sternberg*** cells
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64
Which lymphoma (Hodgkins or Non-Hodgkins) have a better survival?
Hodgkins have better survival
65
Symptoms for Hodgkins lymphoma
enlarged, painless, non-tender 'rubbery' superficial lymph nodes, typically the cervical, also the axillary and the inguinal The lymph nodes may grow bigger or decrease in size * Night sweats * Fever * Weight loss * Pruitis * Lethargy
66
What cancer may present with alcohol-induced lymph nodes pain?
Hodgkins lymphoma
67
What is the Ann-Arbor system used for?
**Ann-Arbor system** -\> in Hodgkins lymphoma, for prognosis and treatment
68
Stages on Ann-Arbor system
69
What are B symptoms in the Hodgkin lymphoma?
B symptoms - indicate worse prognosis: * weight loss in the last 6 months * unexplained fever \>38 C * night sweats (needing a change of clothes)
70
What is the difference between Hodgkin and Non-Hodgkin lymphoma?
There is an absence of Red Steenberg cell in Non-Hodgkin lymphoma
71
Treatment of Hodgkins lymphoma
both chemo and radiotherapy are used
72
What infection is linked with Hodgkin's lymphoma?
Infection with Ebstein Barr virus is linked to the condition (particularly mixed cellularity lymphoma
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74
What has better prognosis: T cell lymphoma or B cell lymphoma?
T-cell lymphomas tend to do less well then those with B-cell lymphomas (B cell lymphomas - better prognosis)
75
Types of stem cell transplants (2)
* **autogenous:** _from patient's own stem cells_, curative/ or extent remisison * **allogenic**: _from a donor_, curative intent (all types of hemo ca, but most often acute leukemia)
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Y3 CBLs (13 decks)

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