Haematological malingnacy summaries Flashcards

1
Q

There are four main types of acute and chronic leukaemia:

A
  • Acute myeloid leukaemia (AML)
  • Acute lymphoblastic leukaemia (ALL)
  • Chronic lymphocytic leukaemia
  • Chronic myeloid leukaemia
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2
Q

Acute vs Chronic leukaemia

A

The difference between acute and chronic leukaemia is that:
- Acute leukaemia is a result of impaired cell differentiation, resulting in large numbers of malignant precursor cells in the bone marrow;
- Chronic leukaemia is the result of excessive proliferation of mature malignant cells, but cell differentiation is unaffected.

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3
Q

Myeloid vs Lymphocytic leukaemia

A

The difference between myeloid leukaemia and lymphocytic leukaemia is that:
- Myeloid leukaemia commonly arises from a myeloid precursor cell, such as neutrophils.
- Lymphocytic leukaemia arises from a lymphoid precursor, such as B-cells.

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4
Q

Acute myeloid leukaemia (AML) background

A
  • AML is the most common acute leukaemia in adults. It is associated with myelodysplastic syndromes.
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5
Q

presentation of AML

A

Symptoms of bone marrow failure:
- anaemia
- bleeding
- infections
Signs of infiltration, including:
- hepatomegaly
- splenomegaly
- gum hypertrophy.

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6
Q

diagnosis of AML

A
  • Elevated white blood cells (leucocytosis) (can be normal or low)
  • Bone marrow biopsy: Auer rods
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7
Q

Management of AML

A

Chemotherapy regimens or bone marrow transplant.

The chemo drugs used most often to treat AML are a combination of:
- Cytarabine (cytosine arabinoside or ara-C)
- An anthracycline drug, such as daunorubicin (daunomycin) or idarubicin

Phases of treatment:
1) Induction is the first phase of treatment. It is short and intensive, typically lasting about a week. The goal is to clear the blood of leukemia cells (blasts) and to reduce the number of blasts in the bone marrow to normal.

2) Consolidation is chemo given after the patient has recovered from induction. It is meant to kill the small number of leukemia cells that are still around but can’t be seen (because there are so few of them). For consolidation, chemo is given in cycles, with each period of treatment followed by a rest period to allow the body time to recover.

3) Maintenance (or post-consolidation), low doses of chemo (or other treatments) are given for months or years after consolidation is finished.

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8
Q

Acute lymphoblastic leukaemia (ALL)

A

ALL is the most common cancer of childhood. It is caused by the abnormal clinical proliferation of lymphoid progenitor cells.

These lymphoid precursors infiltrate normal haematopoietic cells of the bone marrow and other organs of the body.

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9
Q

presentation of ALL

A

Marrow failure
- anaemia including fatigue, abnormal
- bleeding/bruising caused by low platelets
- infections caused by low white cells

Symptoms may also be caused by organ infiltration, such as bone pain.

Signs
- include painless lymphadenopathy,
- hepatosplenomegaly,
- CNS involvement (e.g. cranial nerve palsies, meningism)
- testicular infiltration (resulting in painless unilateral testicular enlargement).

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10
Q

Diagnosis of ALL

A

Blood results show leucocytosis and blast cells on blood film and bone marrow analysis.

Hand mirror

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11
Q

management of ALL

A
  • Multi-drug chemotherapy
    o E.g. Vincristine, doxorubicin, cyclophosphamide, methotrexate
  • Steroids
  • Targeted cancer drugs
    o Rituximab – monoclonal antibody
  • Immunotherapy e.g. CAR T cell therapy
  • Radiotherapy
  • Stem cell or bone marrow transplants
  • Phases of treatment
    o Induction
    o Consolidation
    o Intensification
    o Maintenance
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12
Q

chronic myeloid leukaemia

A

CML is most common in middle-aged patients, with males slightly more affected.

It is classically associated with the Philadelphia chromosome.

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13
Q

Clinical features of CML

A

CML usually presents with weight loss, tiredness, fever, and sweating.

Common signs:
- include massive splenomegaly (>75%)
- bleeding (due to thrombocytopenia),
- gout

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14
Q

diagnosis of CML

A

Blood tests commonly show leucocytosis, in particular raised myeloid cells which include:
- neutrophils
- monocytes
- basophils
- eosinophils.

Bone marrow analysis shows similar findings.

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15
Q

Management of CML

A

CML is commonly treated with chemotherapy regimens

e.g. - Imatinib – tyrosine kinase inhibitors

other TKIs can be tried if one does work

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16
Q

Chronic lymphocytic leukaemia (CLL) pathophysiology

A

CLL is most common in male patients over the age of 60. It is caused by the proliferation of functionally incompetent malignant B cells.

17
Q

CLL presentation

A

CLL typically presents asymptomatically. May present with
- non-tender lymphadenopathy,
- hepatosplenomegaly
- B symptoms (weight loss, night sweats, and fever).

Features of marrow failure are less common than in the acute leukaemias.
- infection
- anaemia,
- bleeding

18
Q

Diagnosis of CLL

A

The most common initial blood result is an incidental lymphocytosis. Subsequent blood film shows smudge cells, which are cells damaged as they lack a cytoskeletal protein. Flow cytometry is then used to measure particular markers expressed by the malignant cells. Other tests include bone marrow biopsy.

19
Q

Management of CLL

A

CLL is commonly treated with chemotherapy regimens.

Tyrosine Kinase Inhibitors e.g Ibrutinib, acalabrutinib- Bruton’s tyrosine kinase inhibitor. Blocking BTK inhibits the B-cell receptor pathway, which is often aberrantly active in B cell cancers.

20
Q

Hodgkins lymphoma

A

are malignant lymphomas characterised by the presence of Reed-Sternberg cells.

21
Q

RF for Hodgkin lymphoma

A

Epstein Barr Virus
HIV
Immunosuppression
Cigarette smoking

22
Q

Hodgkin lymphoma presentation

A

It typically presents in young adults with cervical or supraclavicular non-tender lymphadenopathy, though the location of diseased nodes can vary. Alcohol-induced pain is a suggestive symptom. There may be symptoms caused by compression of surrounding structures e.g. shortness of breath or abdominal pain. B symptoms (fever, night sweats, and weight loss), occur in 30% of patients. It is important to examine the patient for lymphadenopathy, and to check for hepatomegaly or splenomegaly.

23
Q

diagnosis of Hodgkin lymphoma

A

Lymph node biopsy with evidence of Reed Sternberg cells is diagnostic. Blood results can predict a poor prognosis via low haemoglobin and raised LDH, as they indicate high red cell turnover. Staging scans are required to elucidate the extent of disease.

24
Q

management of Hodgkin lymphoma

A
  • Steroids to reduce lymph node mass
  • Radiation +
  • Chemotherapy

ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Dacarbazine

25
Q

Non-Hodgkin lymphoma

A

Non-Hodgkin’s lymphoma is an umbrella term for a group of malignancies affecting the lymphoid system.

It is defined as all lymphomas without Reed-Sternberg cells.

26
Q

types of non-hodgkin lymphoma

A

There are over 60 different types, and they may be classified according to cell of origin (B-cell or T-cell) or pathological grade (high-grade or low-grade).

27
Q

associations of non-hodgkins lymphoma

A

Non-Hodgkin’s lymphoma is commonly associated with viral infection and immunodeficiency.

  • Helicobacter pylori with gastric MALT (mucosa-associated lymphoma tissue).
  • Epstein Barr virus with Burkitt’s lymphoma and AIDS-related CNS lymphoma.
  • Hepatitis C virus with diffuse large B-cell lymphoma and splenic marginal zone lymphoma.
  • Human T cell lymphotropic virus type 1 with T-cell lymphoma.

Other aetiological associations include:
* Immunodeficiency states e.g. HIV/AIDS and post-organ transplant.
* Autoimmune disorders e.g. Sjogren’s syndrome and coeliac diseas

28
Q

presentation of NH lymphoma

A

Common presenting symptoms include painless lymphadenopathy. In contrast to Hodgkin’s lymphoma, the lymphadenopathy is more likely to be symmetrical, at multiple sites, and spread discontinuously across nodal sites. B symptoms (fever, night sweats, and weight loss) are more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma, and are a poor prognostic marker.

29
Q

examination findings NH lymphoma

A

On physical examination there is typically non-tender, firm lymphadenopathy affecting the cervical, axillary, and/or inguinal lymph nodes. There may be splenomegaly and hepatomegaly. The two most common sites of extranodal disease are the gut and skin.

Extra-nodal disease (such as skin lesions in cutaneous T-cell lymphoma or neurological disease) is more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma.

30
Q

diagnosing NH lymphoma

A

The main blood result to remember is an elevated LDH, which is a poor prognostic marker as it reflects greater red cell turnover. The blood film typically reveals nucleated red cells and left shift (the presence of early white blood cell precursors) - particularly in the high grade cancers. This occurs due to marrow involvement and increased marrow turnover. Blood film may also reveal circulating lymphoma cells (i.e. abnormal lymphocytes). Other investigations include bone marrow biopsy and staging scans (eg. CT CAP, PET).

31
Q

diagnosing NH lymphoma

A

The main blood result to remember is an elevated LDH, which is a poor prognostic marker as it reflects greater red cell turnover. The blood film typically reveals nucleated red cells and left shift (the presence of early white blood cell precursors) - particularly in the high grade cancers. This occurs due to marrow involvement and increased marrow turnover. Blood film may also reveal circulating lymphoma cells (i.e. abnormal lymphocytes). Other investigations include bone marrow biopsy and staging scans (eg. CT CAP, PET).

32
Q

management of NH lymphoma

A
  • Watchful waiting
  • Steroids to reduce mass of lymph nodes
  • Chemotherapy (CHOP)
    o Cyclophosphamide
    o Doxorubicin hydrochloride (hydroxydaunorubicin)
    o Vincristine sulfate (Oncovin)
    o Prednisone.
  • Monoclonal antibodies such as rituximab
  • Radiotherapy
  • Stem cell transplantation
33
Q

low grade

A

may not require treatment - watch and wait approach

uncurable

34
Q

high grade lymphoma

A

will require prompt treatment and is potentially curable