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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

Immunophenotype of peripheral blood blasts during the terminal phase of disease. A) CD45/SSC dot-plot. B–E) Pattern of expression of myeloid markers (CD13,CD33,CD64), of CD34, CD19 and CD56. F) Different SSC properties of lymphoid and myeloid blasts. G) CD15 positivity pattern of myeloid blasts. H) Different FSC and SSC properties of lymphoid and myeloid blasts. P1: global blast population. Green dots and P2: lymphoid (CD19-positive) blasts.
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Figure 3: Immunophenotype of peripheral blood blasts during the terminal phase of disease. A) CD45/SSC dot-plot. B–E) Pattern of expression of myeloid markers (CD13,CD33,CD64), of CD34, CD19 and CD56. F) Different SSC properties of lymphoid and myeloid blasts. G) CD15 positivity pattern of myeloid blasts. H) Different FSC and SSC properties of lymphoid and myeloid blasts. P1: global blast population. Green dots and P2: lymphoid (CD19-positive) blasts.

Mentions: The observation of peripheral blood smears showed blasts 95%, which consisted of two different clones, the former being represented by blast cells with lymphoid appearance (about 10%), the latter being represented by cells with a larger size, abundant cytoplasm, giant nucleus with irregular profile (about 90%) (Figures 1B and 1C). Some cells were classified as atypical monocytoid cells (Figure 1C) and very few blasts showed cytoplasmic granulations (Figure 1B). Immunophenotyping of circulating blasts showed a peculiar CD45/SSC dot-plot, with the presence of two distinct blast cell populations. Blasts with small forward scatter (FSC) and side scatter (SSC), which accounted for 10%, showed the phenotypic characteristics observed at diagnosis and were classified as belonging to the B-cell lineage. The majority of blasts (90%) were characterized by larger FSC and SSC and appeared to derive from a myeloid/monocytic clone, being positive for CD13, CD33, CD64, CD15, CD56 and CD4dim (Figure 3).


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)

Immunophenotype of peripheral blood blasts during the terminal phase of disease. A) CD45/SSC dot-plot. B–E) Pattern of expression of myeloid markers (CD13,CD33,CD64), of CD34, CD19 and CD56. F) Different SSC properties of lymphoid and myeloid blasts. G) CD15 positivity pattern of myeloid blasts. H) Different FSC and SSC properties of lymphoid and myeloid blasts. P1: global blast population. Green dots and P2: lymphoid (CD19-positive) blasts.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 3: Immunophenotype of peripheral blood blasts during the terminal phase of disease. A) CD45/SSC dot-plot. B–E) Pattern of expression of myeloid markers (CD13,CD33,CD64), of CD34, CD19 and CD56. F) Different SSC properties of lymphoid and myeloid blasts. G) CD15 positivity pattern of myeloid blasts. H) Different FSC and SSC properties of lymphoid and myeloid blasts. P1: global blast population. Green dots and P2: lymphoid (CD19-positive) blasts.
Mentions: The observation of peripheral blood smears showed blasts 95%, which consisted of two different clones, the former being represented by blast cells with lymphoid appearance (about 10%), the latter being represented by cells with a larger size, abundant cytoplasm, giant nucleus with irregular profile (about 90%) (Figures 1B and 1C). Some cells were classified as atypical monocytoid cells (Figure 1C) and very few blasts showed cytoplasmic granulations (Figure 1B). Immunophenotyping of circulating blasts showed a peculiar CD45/SSC dot-plot, with the presence of two distinct blast cell populations. Blasts with small forward scatter (FSC) and side scatter (SSC), which accounted for 10%, showed the phenotypic characteristics observed at diagnosis and were classified as belonging to the B-cell lineage. The majority of blasts (90%) were characterized by larger FSC and SSC and appeared to derive from a myeloid/monocytic clone, being positive for CD13, CD33, CD64, CD15, CD56 and CD4dim (Figure 3).

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