Symposium on neoplastic hematology and medical oncologyAdult Acute Lymphoblastic Leukemia
Section snippets
Epidemiology and Etiology
ALL has a bimodal distribution with the first peak occurring in individuals around 5 years of age and the second peak at around 50 years of age. It is mainly considered a pediatric leukemia with 80% of cases occurring in children and 20% occurring in adults.4, 5, 6, 7 The median age at diagnosis is 14 years, and approximately 60% of patients are diagnosed at younger than 20 years of age, 25% at around 45 years of age, and 11% at around 65 years of age.8 ALL is relatively uncommon during late
Clinical Presentation and Laboratory Abnormalities
The clinical presentation of ALL is nonspecific, and thus, patients can present with an array of ailments such as “B symptoms” (ie, fever, unexpected weight loss, night sweats), infection, easy bruising/bleeding, dyspnea, and fatigue due to low blood cell counts.8 Patients may exhibit petechiae, pallor, and ecchymosis on physical examination, but children may present with only joint pain.19
Approximately 20% of patients will have leukemic infiltration in the spleen and/or liver leading to
Diagnostic Evaluation
The diagnosis of ALL requires the presence of 20% or more lymphoblasts in the bone marrow.8 Further assessment by flow cytometry, morphological studies, immunophenotyping, and cytogenetic testing is important. Historically, the diagnosis of ALL was based on the French-American-British morphological criteria that described 3 subtypes of ALL (L1, L2, and L3) based on cell size, cytoplasm, nucleoli vacuolation, and basophilia.21 Because of this system’s lack of prognostic value, a classification
Treatment
The structure of adult ALL treatment is similar to that for pediatric ALL; the chemotherapy consists of induction, consolidation, and long-term maintenance therapy along with CNS prophylaxis interwoven during the first year of treatment. The purpose of this multidrug treatment approach is to eradicate the disease and restore normal hematopoiesis, provide prophylaxis to “sanctuary sites,” and prevent an upsurge of resistant clones that may lead to relapse.19
Treatment Approach
A bone marrow examination must be performed to confirm the diagnosis of ALL. In addition, a complete immunophenotypic, cytogenetic, and molecular panel should be conducted because it is crucial for risk stratification and to determine targetable mutations. This will help determine the most optimal treatment regimen.
Conclusion
Major progress has been made in revising prognostic factors, understanding the impact of MRD, and the development of novel targeted therapies for adult patients with ALL. The addition of rituximab to conventional chemotherapy in the treatment of B-cell ALL has substantially improved survival. The incorporation of TKIs in the treatment of Ph-positive ALL has drastically improved outcomes, but their role after transplant is still to be determined. Blinatumomab and inotuzumab have marked activity
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Potential Competing Interests: Drs Elias J Jabbour and Hagop M Kantarjian have research grants from Pfizer, Amgen, Ariel, and Bristol-Myers Squibb.
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