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Acute Leukemia of Ambiguous Lineage (ALAL)
Published in Dongyou Liu, Tumors and Cancers, 2017
Grouped under the myeloid neoplasms and acute leukemia category (which also includes myeloproliferative neoplasms [MPN], mastocytosis, myeloid/lymphoid neoplasms with eosinophilia and rearrangement, myelodisplastic/myeloproliferative neoplasms [MDS/MPN], myelodisplastic syndrome [MDS], acute myeloid leukemia [AML], blastic plasmocytoid dendritic cell neoplasms (BPDCN), B-lymphoblastic leukemia/lymphoma, and T-lymphoblastic leukemia/lymphoma), acute leukemia of ambiguous lineage (ALAL) consists of (i) acute undifferentiated leukemia, (ii) mixed phenotype acute leukemia (MPAL) with t(9;22)(q34.1;q11.2)—BCR-ABL1, (iii) MPAL with t(v;11q23.3)—KMT2A rearranged, (iv) MPAL-B/myeloid not otherwise specified (NOS), and (v) MPAL-T/myeloid NOS [1,2].
Outcomes of pediatric mixed phenotype acute leukemia treated with lymphoid directed therapy: Analysis of an institutional series from India
Published in Pediatric Hematology and Oncology, 2021
Shwetha Seetharam, Priyakumari Thankamony, Kaduveettil Gopinathan Gopakumar, Rekha Appukuttan Nair, Priya Mary Jacob, K. M. Jagathnath Krishna, Binitha Rajeswari, Manjusha Nair, C. S. Guruprasad, V. R. Prasanth
Mixed phenotype acute leukemia (MPAL) is a rare leukemia with a reported incidence of 1.7% to 4.4%.1–4 Although MPAL has been reported in all age groups, the survival of pediatric patients with MPAL has been documented as superior to that of adult MPAL.5,6 The low incidence makes it difficult to conduct randomized control trials or define treatment guidelines. Much of the evidence for MPAL has come from small institutional studies and multicentric non randomized studies.2–4,7–13 There is no clear consensus regarding the treatment of MPAL. Treatment strategies usually follow a lymphoid or myeloid-directed therapy or hybrid protocols involving the use of drugs active against both lymphoid and myeloid blasts with or without hematopoetic stem cell transplantation2–4,7–10
Applications and efficiency of flow cytometry for leukemia diagnostics
Published in Expert Review of Molecular Diagnostics, 2019
Maria Ilaria Del Principe, Eleonora De Bellis, Carmelo Gurnari, Elisa Buzzati, Arianna Savi, Maria Antonietta Irno Consalvo, Adriano Venditti
Once the presence of blasts has been identified, MFCI is the method of choice for determining the AL lineage. Through proper MFCI it is possible to identify within the two major groups of AL, myeloid and lymphoid lineage. For the latter evaluation, it is mandatory to investigate the presence of the normal antigens that characterize them such as cytoplasmic CD3 and CD79a: the first one having the strongest sensitivity and specificity for T-lineage, the other being an excellent B-lineage marker in ALL blasts [13,14]. Differently, AML blasts may express antigens characteristic of neutrophilic, monocytic, and rarely, erythroblastic and megakaryoblast differentiation, or antigens of immaturity (CD117), as detailed below [14–16]. They also may aberrantly express some antigens belonging to lymphoid differentiation as CD7, CD56, CD2, and CD19 [6]. Of note, the presence of aberrant nonspecific markers from different developmental lineages is not sufficient to define the diagnosis of mixed-phenotype acute leukemia (MPAL), which are effective entities with specific diagnostic criteria, as described below [14]. Finally, rare acute undifferentiated leukemia express no markers specific for myeloid or lymphoid lineage but CD45 at weak expression and CD34 positivity.
Early T-cell precursor acute lymphoblastic leukemia with KRAS and DNMT3A mutations and unexpected monosomy 7
Published in Baylor University Medical Center Proceedings, 2018
The definition of ETP-ALL relies on its early T-cell precursor immunophenotype, which is typically CD1a−, CD8−, CD5 − (dim), with expression of one or more of the following myeloid or stem cell markers: CD117, CD34, human leukocyte antigen–antigen D related, CD13, CD33, CD11b, or CD65.1,2 Importantly, absence of myeloperoxidase or monocytic expression is required to exclude classification as mixed phenotype acute leukemia. Not surprisingly, this entity is often found to overexpress genes associated with myeloid or stem cell profiles. Its mutational profile is also closer to myeloid leukemias than to other T-cell leukemias, with increased frequency of FLT3, RAS gene family, DNMT3A, IDH1, and IDH2 mutations.1 In keeping with this, our case demonstrated both KRAS and DNMT3A mutations. An unexpected finding was the presence of monosomy 7, an abnormality commonly seen in myeloid neoplasms and a subset of pediatric ALLs where they are associated with worse outcomes.3 However, there is limited literature information on monosomy 7 specifically in regards to ETP-ALL in adults.4 Altogether, ETP-ALL is uncommon and has been reported in 11% to 12% of childhood T-ALL/LBL and 7.4% of adult T-ALL/LBL.5