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Bone Marrow
Published in Wojciech Gorczyca, Atlas of Differential Diagnosis in Neoplastic Hematopathology, 2014
Blasts and other immature cells can be easily visualized in the BM by immunohistochemistry, using CD34, CD117, terminal deoxynucleotide transferase (TdT), CD123, and/or CD133. Other markers that are often helpful include CD68 and muramidase for monocytic precursors; CD71, hemoglobin A, and GPHA for erythroid precursors; CD4, CD123, and CD56 for BPDCN; CD10, CD19, CD22, and CD79a for B-ALL; CD3 for T-ALL; and CD61 for megakaryocytic lineage. The differential diagnosis of the increased numbers of blasts in the BM includes regenerating marrow after therapy or toxic insult, treatment with Neupogen, refractory anemia with excess blasts (RAEB; Figure 2.9A), chronic myelomonocytic leukemia-2 (CMML-2; Figure 2.9B), CML (Figure 2.9C), and other MPNs in accelerated phase (AP) or blast crisis, APL (Figure 2.9D), AML (Figure 2.9E), BPDCN (Figure 2.9F), B-ALL (Figure 2.9G), and T-ALL (Figure 2.9H). The normal BM shows less than 2%–3% blasts. Based on the number of blasts in the BM, RAEB is divided into type 1 (RAEB-1; 5%–9% blasts) and type 2 (RAEB-2; 10%–19% blasts) [2]. The Revised International Prognostic Scoring System (IPSS-R) for patients with MDS uses the following cutoff points of blasts as one of the prognostic parameters: ≤2%, >2%–≤5%, 5%–10%, and >10% [6]. CMML with <10% of blasts in the BM is classified as type 1 and CMML with 10%–19% blasts as type 2. The current WHO classification of the hematopoietic tumors defines AML by the presence of ≥20% blasts in the blood or BM [2]. AML with certain chromosomal (molecular) changes may show <20% blasts. Based on the WHO classification, AP of CML is defined by the presence of 10%–19% myeloblasts in the BM or blood (among other parameters), but recent reports of patients treated with imatinib (e.g., MD Anderson Cancer Center, International Bone Marrow Transplant Registry, European LeukemiaNet) define AP by the presence of 15%–29% blasts in the blood or BM, or >30% blasts and promyelocytes in the blood or BM (blasts <30%) [7–13]. In the WHO classification, the blast phase (BP) of CML is defined by the presence of (1) ≥20% blasts in the blood or BM or (2) extramedullary blast proliferation. MD Anderson Cancer Center defines BP by the presence of ≥30% blasts in the blood or BM or extramedullary disease with localized immature blasts [13]. Patients with LBL/ALL with <25% BM involvement are classified as having lymphoma, whereas those with ≥25% BM blasts are diagnosed with ALL [2].
De nove Philadelphia chromosome-positive myelodysplastic syndromes with complex karyotype and p230 BCR::ABL fusion transcript: a case report with a literature review
Published in Hematology, 2023
Jinlong Ma, Jiaheng Guan, Baoan Chen
In the literature review, 38 cases of MDS were reported with Ph-positive. In addition, 18/38 (47.4%) of these cases acquired secondary Ph during transformation to higher stage/leukemias. Four cases of de nove Ph-positive MDSs were excluded: an infant of MDS [11] and three cases of MDS-refractory anemia with excess blasts (RAEBt), which were AML according to the new WHO classification [12–14] This case, together with other 16 adult cases with de nove Ph-positive MDS in literature, was enrolled in this study. A total of 17 cases included 10 males and seven females with a median age of 67 (30–85), as summarized in Table 1. Median WBC, hemoglobin and platelets were 4.2 (1.7–15.7) × 109/l, 82 (42–125) g/l and 98 (10–325) × 109/l, respectively.
A review of FDA-approved acute myeloid leukemia therapies beyond ‘7 + 3’
Published in Expert Review of Hematology, 2021
Alexandre Bazinet, Sarit Assouline
HMAs were initially established as effective drugs for higher risk myelodysplastic syndrome (MDS). In the landmark azacitidine CALGB and AZA-001 trials, it was noted that a subset of patients with a diagnosis of refractory anemia with excess blasts in transformation (RAEB-T, 20–30% bone marrow blasts), which would be considered AML by modern World Health Organization (WHO) criteria, derived a survival benefit from AZA [26,27]. This prompted a phase III RCT of AZA versus conventional care regimens (CCRs) in older AML patients with over 30% bone marrow blasts [28]. Survival was improved in the AZA arm, but only reached statistical significance when patients who received subsequent AML therapy after stopping AZA were censored. Similarly, DAC has demonstrated efficacy in low (≤ 30%) or higher (> 30%) blast count AML [29–31]. In the landmark DACO-16 trial, DAC was compared to best supportive care or low-dose cytarabine (LDAC) in older AML patients and was associated with improved response rates, although the improvement in OS was not statistically significant [31]. The FDA approved AZA in 2004 and DAC in 2006 for AML with ≤ 30% blasts (the entity formerly referred to as RAEB-T). In contrast, the European Medicines Agency (EMA) approved AZA and DAC for AML patients over age 65 ineligible for aHSCT, regardless of blast count. No randomized trial has directly compared AZA and DAC.
Cord blood transplantation for acute leukemia
Published in Expert Opinion on Biological Therapy, 2020
Mattia Algeri, Stefania Gaspari, Franco Locatelli
A collaborative study between the European Working Group on childhood MDS (EWOG-MDS), CIBMTR, and Eurocord–EBMT examined factors influencing the outcome of 70 children with MDS (refractory cytopenia (n = 33), refractory anemia with excess blasts (n = 28), and refractory anemia with excess blasts in transformation (n = 9) at diagnosis), who received UCBT after a myeloablative regimen [33]. At day +60, the probability of neutrophil recovery was 76%; a TNC >6 × 107/kg, transplantation of HLA-matched or 1-locus mismatched UCB cells, and an irradiation-containing conditioning regimen were associated with faster engraftment. The 3-year DFS was 50% for children transplanted after 2001, as compared with 27% for children given UCBT in the earlier period (P = 0.018). This difference may be explained by the fact that, after 2001, UCBT was performed within 6 months after diagnosis and using UCB units containing a higher cell dose [33].