Leukemias Flashcards
Acute Leukemia
MOA
Cause
Malignancy of the hematopoietic progenitor cell
- progenitor cells proliferate in an uncontrolled fashion and replace normal bone marrow
- the cells spill out into the blood where they are seen in large numbers
- these cells may also infiltrate the liver, spleen, and LNs causing enlargement
Cause is unknown- certain exposures(chemicals, radiation) and dysregulation of genes may increase incidence
How are leukemias classified?
by their cell type and if they are acute or chronic
Acute Lymphocytic Leukemia (ALL)
Peak incidence in what age group and gender?
What give you an increased risk?
Between 2-5 years of age, usually boys
Incidence higher in hispanic whites
Genetic and immunodeficiency syndromes at greater risk
ALL What is it? Signs and symptoms Diagnostics Treatment
ALL contains a group of neoplasms composed of B or T lymphocytes referred to as lymphoblasts
Signs and symptoms
- Early- fever, bleeding/bruising (from thrombocytopenia), bone pain (bones filling up and so many cells are made they just spill out), lymphadenopathy
- MS pain (bone pain to long bone)
- HA
- Lymphadenopathy (50% of cases)(non-tender, firm, rubbery, and matted)
- testicular enlargement
- mediastinal mass
- peripheral blood abnormalities (anemia, thrombocytopenia)
Dx
- referral to pediatric cancer center
- BM bx
- peripheral blood
Tx
- Remission induction-kill off cancer cells with chemo
- consolidation/intensification-do bx, look at blood, if any cancer cells remain, kill those too
- maintenance-after they have killed them all, give a low dose of chemo to make sure they don’t make anymore
- -can use chemo, stem cell transplant, targeted therapy
AML What is it? Signs and symptoms Diagnostics Treatment
What is it?
- a group of hematopoetic neoplasms involving myeloid precursor cells
- characterized by clonal proliferation of myeloid precursors with reduced capacity to differentiate into more mature cellular elements
- cancer is at myeloblast stage
Signs and symptoms
- generally present w/ symptoms related to complications of pancytopenia
- fever (infection)
- Pallor (anemia)
- petechiae or ecchymoses (thrombocytopenia)
- eyes (pale conjunctiva, hemorrhages or whitish plaques)
- Gingival hypertrophy
- hepatosplenomegaly
- Polyarthritis/arthralgia
- palpable adenopathy in uncommon
Diagnostics
- normocytic normochromic anemia
- circulating myeloblasts seen in peripheral smear
- Auer rods
- BM bx and aspirate (hypercellular)
Dx requires both: blast forms account for 20% of totally cells AND leukemic cells must be of myeloid origin
Treatment 2 phases -Induction of remission -post remission therapy 4 types -chemo -radiation -stem cell transplant -drug therapy (arsenic trioxide, all-trans retioic acid ATRA)
Acute Myelogenous Leukemia (AML)
Peak incidence in what age group and gender?
What give you an increased risk?
*most common acute leukemia in adults
65yo, males
Risk
- environmental factors
- genetic abnormalities
- benign and malignant hematologic diseases
Complications of Acute leukemia
Leukostasis- increase blast count leads to emboli that go into the kidneys and the lungs and the brain
Hyperuricemia- (excess of uric acid in the blood) breakdown of purine nucleotides secondary to chemo from leukemic cell death
Tumor lysis syndrome- necrosis of malignant cells during initial phase of chemo, leads to life threatening metabolic disorder
Chronic Leukemia is malignancies involving proliferation of what cells?
more fully differentiated myeloid and lymphoid cells
Chronic Lymphocytic Leukemia (CLL)
Peak incidence in what age group and gender?
What give you an increased risk?
70 yo men
Risk
- benzene and heavy solvent exposure
- rubber manufacturing workers
- multiple episodes of pneumonia
CLL What is it? Signs and symptoms Diagnostics Treatment
What is it?
- a clonal malignancy of B lymphocytes
- most common form of leukemia in adults
Signs and symptoms
- painless swelling of lymphnodes
- fatigue
- hepatosplenomegaly
- macules, papules, nodules, ulcers, or blisters on skin
- exaggerated rxn to insect bites
Diagnostics
- lymphocytosis
- cytopenias
- “smudge” cells (really fragile cells that are ruptured from smearing the blood in the slide)
- BM bx and aspirate are not required
Treatment 5 types -watchful waiting -radiation -chemo -surgery (splenectomy) -targeted therapy (monoclonal antibody or tyrosine kinase inhibitor-TKI)
Chronic Myelogenous Leukemia (CML)
Peak incidence in what age group and gender?
What give you an increased risk?
15-20% of leukemia in adults
50-60 yo males
CML What is it? Signs and symptoms Diagnostics Treatment
- myeloproliferative neoplasm characterized by dysregulated production and uncontrolled proliferation of mature granulocytes with fairly normal differentiation
- fusion of two genes
- -BCR (chromosome 22)
- -ABL1 (Chromosome 9)
Signs and symptoms
- 20-50% are asymptomatic
- fatigue
- malaise
- weight loss
- excessive sweating
- abdominal fullness
Diagnostics
- Leukocytosis
- absolute basophilia and eosinophilia of granulocytes
- platelet count can be normal or elevated
- BM bx and aspiration not necessary
- Confirm by: demonstration of Philadelphia Chromosome
Treatment 3 phases -chronic (fewer than 10% blasts in BM) --Use tyrosine kinase inhibitor (TKI) (imatinib mesylate) --Interferon or chemotherapy --stem cell transplant -accelerated (10-20%) --use higher doses of drugs -blastic (greater than 20%) --palliative therapy
Why would we use a tyrosine kinase inhibitor?
Tyrosine kinase is what tells our cells to replacate over and over again
Hairy Cell Leukemia (HCL)
Peak incidence in what age group and gender?
What give you an increased risk?
Pretty rare
52 yo males
risk- caucasians
HCL What is it? Signs and symptoms Diagnostics Treatment
What is it?
- chronic B cell lymphoproliferative disorder characterized by accumulation of small mature B cell lymphoid cell with abundant cytoplasm and “hairy” projections
- results in splenomegaly and reduction in normal red cells, platelets, mature granulocytes, and monocytes
Signs and symptoms
- splenomegaly
- cytopenias
- liver enlarged in 50% of cases
- lymphadenopathy is uncommon
Diagnostics
- “hairy cell” in peripheral blood
- BM bx -dry tap
Treatment
- if asymptomatic can observe
- -purine analog drugs (cladribine and pentostatin)
- return of leukemia
- -purine analog drugs
- -monoclonal ab (rituximab) or interferon alpha
- splenectomy
