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B-cell acute lymphoblastic leukemia with t(4;11)(q21;q23) in a young woman: evolution into mixed phenotype acute leukemia with additional chromosomal aberrations in the course of therapy.

Carulli G, Marini A, Ferreri MI, Azzarà A, Ottaviano V, Lari T, Rocco M, Giuntini S, Petrini M - Hematol Rep (2012)

Bottom Line: Karyotype showed the co-existence of three cell lines, with persistence of t(4;11)(q21;q23) and appearance of +8,+12,+13 and two der(4).The patient died because of disseminated intravascular coagulation.This finding suggests a blast cell derivation from a common lymphoid/monocytic precursor leading to a final bilineal acute leukemia.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology, Department of Clinical and Experimental Medicine, University of Pisa;

ABSTRACT
About 5% of adult B-cell acute lymphoblastic leukemias (B-ALL) are characterized by t(4;11)(q21;q23), which confers peculiar features to this B-ALL subtype, including a very immature immunophenotype and poor prognosis. We describe the case of a 21-year-old female who presented with B-ALL carrying the t(4;11)(q21;q23) and blasts positive for CD19, TdT, CD79a, CD38, HLA-DR. Before completing the Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) therapy regimen, the B-cell leukemic clone still was detected, but an additional leukemic clone appeared, with morphology and immunophenotype (CD13, CD33, CD64, CD38, CD56, CD15, CD4(dim)) compatible with derivation from the myeloid/monocytic lineage. Karyotype showed the co-existence of three cell lines, with persistence of t(4;11)(q21;q23) and appearance of +8,+12,+13 and two der(4). The patient died because of disseminated intravascular coagulation. Our report describes a rare, possible evolution of such a subtype of B-ALL, with transformation into mixed phenotype acute leukemia in the course of therapy. This finding suggests a blast cell derivation from a common lymphoid/monocytic precursor leading to a final bilineal acute leukemia.

No MeSH data available.


Related in: MedlinePlus

Morphology of blast cells at diagnosis (A) and in the course of fatal evolution (B,C). A) blasts appear with lymphoid morphology; B,C) blasts consist of two different clones. Blasts with lymphoid morphology (arrows) show smaller size. The additional blast cell population consists of cells of larger size and more abundant cytoplasm (long arrows). Some monocytoid cells (arrowheads) and one myeloblast with evident cytoplasmic granulations (B) are also shown. Peripheral blood, May-Grünwald-Giemsa staining (1000×).
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Figure 1: Morphology of blast cells at diagnosis (A) and in the course of fatal evolution (B,C). A) blasts appear with lymphoid morphology; B,C) blasts consist of two different clones. Blasts with lymphoid morphology (arrows) show smaller size. The additional blast cell population consists of cells of larger size and more abundant cytoplasm (long arrows). Some monocytoid cells (arrowheads) and one myeloblast with evident cytoplasmic granulations (B) are also shown. Peripheral blood, May-Grünwald-Giemsa staining (1000×).

Mentions: Manual WBC differential count of peripheral blood showed 90% blasts without morphologic differentiation (Figure 1A), 2% neutrophils, 8% small lymphocytes. Blasts resulted negative for myeloperoxidase stain. Flow cytometric analysis was therefore performed using a wide monoclonal antibody panel and a six-color method: blasts were positive for CD19, TdT, CD79a, CD38, CD58, HLA-DR (Figure 2A–F).


B-cell acute lymphoblastic leukemia with t(4;11)(q21;q23) in a young woman: evolution into mixed phenotype acute leukemia with additional chromosomal aberrations in the course of therapy.

Carulli G, Marini A, Ferreri MI, Azzarà A, Ottaviano V, Lari T, Rocco M, Giuntini S, Petrini M - Hematol Rep (2012)

Morphology of blast cells at diagnosis (A) and in the course of fatal evolution (B,C). A) blasts appear with lymphoid morphology; B,C) blasts consist of two different clones. Blasts with lymphoid morphology (arrows) show smaller size. The additional blast cell population consists of cells of larger size and more abundant cytoplasm (long arrows). Some monocytoid cells (arrowheads) and one myeloblast with evident cytoplasmic granulations (B) are also shown. Peripheral blood, May-Grünwald-Giemsa staining (1000×).
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3475937&req=5

Figure 1: Morphology of blast cells at diagnosis (A) and in the course of fatal evolution (B,C). A) blasts appear with lymphoid morphology; B,C) blasts consist of two different clones. Blasts with lymphoid morphology (arrows) show smaller size. The additional blast cell population consists of cells of larger size and more abundant cytoplasm (long arrows). Some monocytoid cells (arrowheads) and one myeloblast with evident cytoplasmic granulations (B) are also shown. Peripheral blood, May-Grünwald-Giemsa staining (1000×).
Mentions: Manual WBC differential count of peripheral blood showed 90% blasts without morphologic differentiation (Figure 1A), 2% neutrophils, 8% small lymphocytes. Blasts resulted negative for myeloperoxidase stain. Flow cytometric analysis was therefore performed using a wide monoclonal antibody panel and a six-color method: blasts were positive for CD19, TdT, CD79a, CD38, CD58, HLA-DR (Figure 2A–F).

Bottom Line: Karyotype showed the co-existence of three cell lines, with persistence of t(4;11)(q21;q23) and appearance of +8,+12,+13 and two der(4).The patient died because of disseminated intravascular coagulation.This finding suggests a blast cell derivation from a common lymphoid/monocytic precursor leading to a final bilineal acute leukemia.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology, Department of Clinical and Experimental Medicine, University of Pisa;

ABSTRACT
About 5% of adult B-cell acute lymphoblastic leukemias (B-ALL) are characterized by t(4;11)(q21;q23), which confers peculiar features to this B-ALL subtype, including a very immature immunophenotype and poor prognosis. We describe the case of a 21-year-old female who presented with B-ALL carrying the t(4;11)(q21;q23) and blasts positive for CD19, TdT, CD79a, CD38, HLA-DR. Before completing the Hyper-CVAD (hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone) therapy regimen, the B-cell leukemic clone still was detected, but an additional leukemic clone appeared, with morphology and immunophenotype (CD13, CD33, CD64, CD38, CD56, CD15, CD4(dim)) compatible with derivation from the myeloid/monocytic lineage. Karyotype showed the co-existence of three cell lines, with persistence of t(4;11)(q21;q23) and appearance of +8,+12,+13 and two der(4). The patient died because of disseminated intravascular coagulation. Our report describes a rare, possible evolution of such a subtype of B-ALL, with transformation into mixed phenotype acute leukemia in the course of therapy. This finding suggests a blast cell derivation from a common lymphoid/monocytic precursor leading to a final bilineal acute leukemia.

No MeSH data available.


Related in: MedlinePlus