Leukaemia (AML, ALL, CML, CLL) Flashcards
What is ALL.
It is a haematological malignancy of lymphoid cells, affecting B or T lymphocyte cell lines, arresting maturation and promoting uncontrolled proliferation of immature blast cells with marrow failure and tissue infiltration.
Who tends to get ALL.
Commoner in children.
What is the peak age for ALL.
4 years.
What are the ages of presentation of ALL in adults. (2)
Between 15-25 years.
Above 75 years.
What are the signs and symptoms of ALL. (12)
Marrow failure symptoms: bleeding (bruising, menorrhagia, epistaxis), anaemia (low Hb), infection (low WCC).
Infiltration symptoms: hepatosplenomegaly, lymphadenopathy, orchidomegaly.
Lethargy.
Shortness of breath.
Arthralgia.
Malaise.
CNS infiltration (cranial nerve palsies, meningism).
What are the complications of ALL. (3)
Haemorrhage.
Thrombosis.
Tumour lysis syndrome (hyperuricaemia and renal failure) secondary to treatment.
What is AML.
A haematological malignancy resulting in the overproduction of immature myeloid WBCs.
This neoplastic proliferation of blast cells is derived from marrow myeloid elements.
What is the peak age of onset of AML.
70.
Who is AML particularly rare in.
Those under 20.
What are the risk factors for developing AML. (4)
Risk of transformation from CML.
Myelodysplasia.
Myelofibrosis.
Polycythaemia rubra vera (PRV).
What is the prognosis for ALL. (2)
Good for children.
Poor for adults.
What are the symptoms of AML. (10)
Bone marrow failure: anaemia, infection, bleeding.
DIC occurs in a specific subtype of AML.
Infiltrative symptoms: hepatosplenomegaly, gum hypertrophy, skin involvement.
Malaise.
Lethargy.
CNS involvement is rare in AML.
What are the physical signs of AML. (5)
Purpura. Organomegaly. Lymphadenopathy. Splenomegaly. DIC associated with M3 subtype.
What is seen on a FBC in a patient with AML. (3)
Low Hb.
Normal platelets.
WCC high or normal or even low.
What is seen in a bone marrow biopsy in patients with AML.
Auer cells.
What is CLL.
A monoconal malignancy resulting in functionally incompetent lymphocytes.
Accumulation of mature B cells that have escaped programmed cell death and undergone cell-cycle arrest in the G0/G1 phase - this is the hallmark of CLL.
Who gets CLL.
Those aged over 60.
What are the symptoms of CLL. (8)
Often none - presents as a surprise finding on FBC. Recurrent infections. Anorexia. If severe: Sweating. Weight loss. Malaise. Abdominal discomfort. Bleeding.
What are the physical signs of CLL. (5)
Lymphadenopathy (rubbery and non-tender). Splenomegaly. Hepatomegaly. Petechiae. Pallor.
What is seen in the FBC of a patient with CLL. (4)
Lymphocytosis (initial finding). Later, marrow infiltration leading to: Anaemia (low Hb). Thrombocytopenia (low platelets). Low neutrophils.
What is seen in the blood film of a patient with CLL.
Smudge cells.
What are the complications of CLL. (6)
Increased risk of second malignancy.
Autoimmune haemolytic anaemia.
Increased infection due to hypogammaglobinaemia, bacterial, viral (especially herpes zoster).
Idiopathic thrombocytopenic purpura (ITP).
Bone marrow failure.
What is CML.
A malignancy of granulocytes which leads to an increased production of myeloid precursors, with their differentiation ability still intact.
It is characterized by an uncontrolled clonal proliferation of myeloid cells.
It is a myeloproliferative disorder.
What is CML associated with.
Philadelphia chromosome (80% of those with CML).
What is the Philadelphia chromosome.
A translocation between 9 and 22 resulting in the bcr-abl gene that produced tyrosine kinase.
Who gets CML. (4)
Occurs in middle aged and elderly people.
Between 40-60 years.
It has a slight male predominance.
It is rare in childhood.
What are the symptoms of CML. (11)
Most of the symptoms are chronic and insidious. Anaemia. Weight loss. Tiredness. Lassitude. Anorexia. Abdominal discomfort (splenomegaly). Sweating. Gout (due to purine breakdown). Bleeding (platelet dysfunction). Priapism. Low grade fever.
What are the physical signs of CML. (5)
Bruising. Petechiae. Anaemia. Splenomegaly (>75%). Hepatomegaly.
What is seen in the FBC of a patient with CML. (4)
Raised WCC.
Whole spectrum of myeloid cells elevated (raised neutrophils, raised myelocytes, raised basophils, raised eosinophils).
Low or normal Hb.
Variable platelet levels.
What are the leukaemias. (4)
Acute lymphoblastic leukaemia.
Acute myeloid leukaemia.
Chronic lymphocytic leukaemia.
Chronic myeloid leukaemia.
What is the most important thing to remember about patient’s with leukaemia.
They fall ill suddenly and deteriorate quickly.
Take non-specific symptoms and drowsiness/confusion seriously.
What are the three main causes for sudden deterioration in leukaemia. (3)
Infection.
Bleeding.
Hyperviscosity.
What other dangers are there in leukaemia (besides the common three). (2)
Tumour lysis syndrome.
DIC.
What is tumour lysis syndrome caused by.
Caused by a massive destruction of cells, leading to raised potassium, raised urate and renal injury.
What are the risk factors for developing tumour lysis syndrome. (4)
Raised LDH.
Raised creatinine.
Raised urate.
Raised WCC >25.
When does hyperviscosity occur.
When WCC >100 thrombi may form in the brain, lung and heart (leukostasis).
What are the risk factors for ALL. (4)
Genetic susceptibility (eg with translocations).
Environmental triggers.
Ionizing radiation during pregnancy (Xrays).
Down’s syndrome.
How common is CNS involvement in ALL.
Very common.
What are the three classification systems for ALL. (3)
Morphological classification by microscopic appearance.
Immunological classification based on surface markers.
Cytogenic chromosomal analysis (detectable in 85% of cases).
What are the common sites of infection in ALL. (4)
Chest.
Mouth.
Perianal.
Skin.
What is normally seen on a blood film in ALL. (2)
Blast cells on blood film (and in bone marrow).
Very high WCC.
What tests should be performed in ALL. (4)
Blood film.
Bone marrow biopsy.
CXR and CT to look for mediastinal and abdominal lymphadenopathy.
Lumbar puncture to look for CNS involvement.
What is the cure rate for children with ALL.
70-90%.
What is the cure rate for adults with ALL.
40%.
What are poor prognostic factors in ALL. (7)
Male. Adult. Philadelphia chromosome. Presentation with CNS involvement. Low Hb. High WCC. B cell ALL.
What are the characteristics of blast cells. (6)
Immature precursor of myeloid cells or lymphoid cells.
Bigger than normal counterparts.
Immature nucleus.
Cytoplasmic appearances often atypical.
Not seen in normal individuals.
If seen, highly suggestive of ALL or a chronic disorder that is beginning to transform into an acute disease.
What are some risk factors for leukaemia. (5)
Ionising radiation. Cytotoxic drugs. Retroviruses. Genetic. Immunological.
What is the prognosis for patients with AML. (2)
Death occurs in 2 months if left untreated.
20% 3year survival after treatment.
What is the incidence of AML. (2)
It is the commonest acute leukaemia of adults.
1/10,000/year.
What is AML associated with. (4)
A long term complication of chemotherapy (eg for lymphoma).
Myelodysplastic states.
Radiation.
Syndromes (eg Down’s).
How is AML diagnosed.
Bone marrow biopsy.
What are the major complications in AML. (3)
Infection (be alert to signs of septicaemia).
Chemotherapy causes increased plasma urate levels (from tumor lysis) so give allopurinol with chemo.
Leukostasis may occur if very high WCC.
What is the mortality rate of AML.
10%.
What percentage of leukaemias are CML.
15%.
What percentage of CML cases are detected by chance.
30%.
What are the 3 stages of the natural history of the disease. (3)
Chronic (lasts months or years, with few, if any, symptoms).
Accelerated phase (with increasing symptoms, spleen size and difficulty in controlling counts.
Blast transformation with features of acute leukaemia and eventually death.
What is the male:female ratio of CLL.
2:1.
What is the incidence of CLL.
4/100,000.
What increases the risk of CLL. (4)
Mutations, trisomies and deletions (del17p13) influence risk.
Pneumonia may be a triggering event.
What is the natural history of CLL. (2)
Some remain in status quo for years, or even regress.
Usually nodes slowly enlarge (with or without lymphatic obstruction).
What is death usually due to in CLL. (2)
Infection.
Transformation to aggressive lymphoma (Richter’s syndrome).
What are the common causes of infection in CLL. (5)
Pneumococcus. Haemophilus. Meningiococcus. Candida. Aspergillosis.
