CC Sept 2015 Acute lymphoblastic leukemia (ALL)

echoecho's version from 2016-05-26 02:57


Question Answer
What is ALL?it is a malignancy in which the bone marrow lymphoid precursor cells are arrested in an early stage of development
What likely initiates the process?chromosomal translocations resulting in abnormal gene expression
What is the pathogenesis?lymphoid precursor cells (lymphoblasts) replace normal bone marrow
What happens the above happens?there is decreased production of red blood cells (anemia), platelets (thrombocytopenia) and normal WBCs (leukpenia)
These lymphoid precursor cells (lymphoblasts) may also be found outside of the marrow in what organs?1) spleen 2) liver 3) lymph nodes
ALL has a bimodal age of onset, with the initial peak occurring between ___ to ___ years of age and a second peak in adults > ____ years of age?2-5; 50
ALL is the most common type of leukemia in what age category (representing 3/4 of all leukemias in this age category)?children
ALL is the most common pediatric cancer (2,500 - 3,500 new cases annually), accounting for 1/4 of all pediatric cancers in patients < ____ years of age?14
About _______ new ALL cases were diagnosed in adults in 2015?6500
ALL is slightly more common in what gender?males
What is the most common presentation (which may be independent of infection)?fever
List other symptoms?1) anemia 2) fatigue 3) lightheadedness 4) palpitations
What lab finding could cause a presentation of abnormal bleeding?thrombocytopenia
List other symptoms?1) bone pain 2) palpable LAD 3) splenomegaly
What is the most common cause of death in patients with ALL? Because of this what symptom should be vigorously investigated and what should be ruled out?1) death 2) fever 3) infectious source
What is the initial work-up?1) lab studies (CBC, chem 13, blood cultures if fever is presents) 2) imaging (CT if LAD is present) 3) bone marrow aspiration and biopsy as well flow cytometry
Prognosis for ALL varies between individuals based on several factors. List these factors?1) females have better outcomes than males 2) whites (while more likely to develop ALL have a better prognosis than AA or Hispanics with the disease)
*** Between 1975 and 1990, the 5- year survival rate for ALL increased from ____% to ____% in children < 15 years of age and from ____% to > ____% in adolescents between 15 and 19 years of age?60; 90; 28; 75
The cure rate for ALL in children approaches ___%; whereas cure rates in adults are about _________ to ___% ?80; 20; 40
Patients who are > ____ years of age, have a WBC > ________cells/uL, adverse cytogenetics, translocations or fail to achieve remission w/in 4 weeks have a poorer prognosis?60; 100,000
A personal hx of what syndrome or family hx of what disorder also increases one's risk for developing ALL?1) Down syndrome 2) autoimmune disorders (e.g. Hashimoto's thyroiditis)
*** The translocation between chromosomes ___ and ___ is seen in 5% of pediatric ALL cases but 20% of adult cases. If this translocation is present, comment on treatment?9; 22; treatment is more difficult and prognosis is poorer
What is the name of the chromosome for ALL?Philadelphia
The translocation between 4 and 11 is seen in 4% of cases and is most common in infants < ____ months of age and also connotes a poor prognosis?12
Patients with a ____-cell lineage ALL has a poorer prognosis than those with ___-cell lineage?B; T
ALL that spreads to the CNS system also has worse outcomes, true or false?true
What type of chemotherapy using multiple drugs is typically initiated in an inpatient setting?induction
The more recent addition of what type of therapy has improved prognosis?tyrosine kinase inhibitory therapy
Among children with ALL, > _____% achieve remission; ____% of patients 1-18 years of age are newly diagnosed ALL who treated with current regimens are expected to survive long term?95%; 80%
Who have the higher remission rates, children or adults?children
Patient who do not respond in ___ to ___ weeks should be considered for enrollment into clinical trials?4; 6
Since recurrent ALL frequently involves the CNS in adults and children, current regimens include what type of treatment?intrathecal prophylactic chemotherapy
Younger, high-risk patients may be also be considered for what transplantation?bone marrow
Adjuvant blood products should be used to keep hgb > ____ g/dL and platelets > ______ to _______ cells / uL?8; 10,000; 20,000
*** SUMMARY = What causes abnormal gene expression that initiates the arrest of bone marrow lymphoid precursor cells in early stage development of acute lymphoblastic leukemia (ALL)?chromosome translocation
*** SUMMARY = ALL has a bimodal age of onset, with the initial peak occurring between ___ and ___ years of age and a second peak in adults > ____ years of age?2; 5; 50
*** SUMMARY = What is the most common presenting symptom?fever
*** SUMMARY = Prognosis dependes on multiple factors including what?age, race, sex, presence or absence of identified translocation