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High-Risk Microgranular Acute Promyelocytic Leukemia with a Five-Way Complex Translocation Involving PML-RARA.

Powers B, Persons D, Rao D, Woodroof J, Lin TL - Case Rep Hematol (2015)

Bottom Line: Given his elevated white blood cell count at presentation, intrathecal chemotherapy for central nervous system prophylaxis was also given.The patient remains on maintenance therapy and remains in remission.This is the first such report of a 5-way chromosomal translocation leading to APL.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology/Oncology, Department of Internal Medicine, University of Kansas School of Medicine, 3901 Rainbow Boulevard, Kansas City, KS 66210, USA.

ABSTRACT
Acute promyelocytic leukemia (APL) is classically characterized by chromosomal translocation (15;17), resulting in the PML-RARA fusion protein leading to disease. Here, we present a case of a 50-year-old man who presented with signs and symptoms of acute leukemia with concern for APL. Therapy was immediately initiated with all-trans retinoic acid. The morphology of his leukemic blasts was consistent with the hypogranular variant of APL. Subsequent FISH and cytogenetic analysis revealed a unique translocation involving five chromosomal regions: 9q34, 17q21, 15q24, 12q13, and 15q26.1. Molecular testing demonstrated PML/RARA fusion transcripts. Treatment with conventional chemotherapy was added and he went into a complete remission. Given his elevated white blood cell count at presentation, intrathecal chemotherapy for central nervous system prophylaxis was also given. The patient remains on maintenance therapy and remains in remission. This is the first such report of a 5-way chromosomal translocation leading to APL. Similar to APL with chromosomal translocations other than classical t(15;17) which result in the typical PML-RARA fusion, our patient responded promptly to an ATRA-containing regimen and remains in complete remission.

No MeSH data available.


Related in: MedlinePlus

(a) Peripheral smear revealing blasts that contained bilobed nuclei with very fine granules (100x). (b) Bone marrow biopsy showing a hypercellular marrow with 64% blasts and decreased trilineage hematopoiesis (40x).
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fig1: (a) Peripheral smear revealing blasts that contained bilobed nuclei with very fine granules (100x). (b) Bone marrow biopsy showing a hypercellular marrow with 64% blasts and decreased trilineage hematopoiesis (40x).

Mentions: Upon transfer, the peripheral smear revealed nearly 100% blasts with no Auer rods or coarse granularity. Many of the blasts contained dumbbell-shaped nuclei with very fine granules concerning the hypogranular variant of APL (Figure 1(a)). He was immediately started on ATRA and dexamethasone given his elevated WBC count, as well as prophylactic allopurinol and antibiotics. He was given cryoglobulin to maintain fibrinogen >150 mg/dL and platelet transfusions to goal 50 K/UL.


High-Risk Microgranular Acute Promyelocytic Leukemia with a Five-Way Complex Translocation Involving PML-RARA.

Powers B, Persons D, Rao D, Woodroof J, Lin TL - Case Rep Hematol (2015)

(a) Peripheral smear revealing blasts that contained bilobed nuclei with very fine granules (100x). (b) Bone marrow biopsy showing a hypercellular marrow with 64% blasts and decreased trilineage hematopoiesis (40x).
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4321077&req=5

fig1: (a) Peripheral smear revealing blasts that contained bilobed nuclei with very fine granules (100x). (b) Bone marrow biopsy showing a hypercellular marrow with 64% blasts and decreased trilineage hematopoiesis (40x).
Mentions: Upon transfer, the peripheral smear revealed nearly 100% blasts with no Auer rods or coarse granularity. Many of the blasts contained dumbbell-shaped nuclei with very fine granules concerning the hypogranular variant of APL (Figure 1(a)). He was immediately started on ATRA and dexamethasone given his elevated WBC count, as well as prophylactic allopurinol and antibiotics. He was given cryoglobulin to maintain fibrinogen >150 mg/dL and platelet transfusions to goal 50 K/UL.

Bottom Line: Given his elevated white blood cell count at presentation, intrathecal chemotherapy for central nervous system prophylaxis was also given.The patient remains on maintenance therapy and remains in remission.This is the first such report of a 5-way chromosomal translocation leading to APL.

View Article: PubMed Central - PubMed

Affiliation: Division of Hematology/Oncology, Department of Internal Medicine, University of Kansas School of Medicine, 3901 Rainbow Boulevard, Kansas City, KS 66210, USA.

ABSTRACT
Acute promyelocytic leukemia (APL) is classically characterized by chromosomal translocation (15;17), resulting in the PML-RARA fusion protein leading to disease. Here, we present a case of a 50-year-old man who presented with signs and symptoms of acute leukemia with concern for APL. Therapy was immediately initiated with all-trans retinoic acid. The morphology of his leukemic blasts was consistent with the hypogranular variant of APL. Subsequent FISH and cytogenetic analysis revealed a unique translocation involving five chromosomal regions: 9q34, 17q21, 15q24, 12q13, and 15q26.1. Molecular testing demonstrated PML/RARA fusion transcripts. Treatment with conventional chemotherapy was added and he went into a complete remission. Given his elevated white blood cell count at presentation, intrathecal chemotherapy for central nervous system prophylaxis was also given. The patient remains on maintenance therapy and remains in remission. This is the first such report of a 5-way chromosomal translocation leading to APL. Similar to APL with chromosomal translocations other than classical t(15;17) which result in the typical PML-RARA fusion, our patient responded promptly to an ATRA-containing regimen and remains in complete remission.

No MeSH data available.


Related in: MedlinePlus