| Session: Therapy-related myeloid neoplasm |
| Case number: 188 |
Submitter(s): Carl L. Buckner, John Lazarchick.
Clinical history
The patient is a 63 y/o WM with a history of benign fibrohistiocytoma which was resected in 1975. He presented to the Medical University of South Carolina (MUSC) in 2002 with bilateral pulmonary nodules and a right gluteal mass. Biopsies revealed malignant fibrohistiocytoma, myxoid type. He was treated with surgical resection and radiation therapy. In May 2005, a CT scan showed soft tissue masses between the esophagus and base of the heart and below the left hemidiaphragm. In June 2005 the patient received ifosfamide and adriamycin for 6 cycles. He then received 6 cycles of ET743 that was completed in January 2006. During a follow-up visit in October 2006, the patient complained of easy bruisability and fatigue for six weeks. His WBC = 1.26K/mm3, hemoglobin = 10.7g/dL, hematocrit = 30.6%, and platelets = 19K/ mm3.
Details of gross/microscopic pathology:
Peripheral blood: RBCs show mild anisocytosis and poikilocytosis with scattered spherocytes present. There is a scarcity of leukocytes. Blasts are not identified. The platelets are markedly decreased. The differential WBC count is 9% polys, 2% monocytes, 89% lymphocytes, and 1% eosinophils.
Bone marrow aspirate: Cellular and adequate. The myeloid series is extremely left-shifted with predominance of promyelocytes and occasional blasts. Auer rods are not noted. The erythroid series shows progression through the full maturation sequence with no morphologic abnormalities. The M:E ration is 3.2:1. Megakaryocytes are extremely decreased in numbers. A differential cell count: 3% polys, 3% bands, 14% lymphocytes, 17% erythroid, 8% myelocytes, 46% promyelocytes, 6% blasts, and 3% plasma cells.
Bone marrow biopsy: Hypocellular with an overall cellularity of approximately 20%. The myeloid series is markedly left-shifted with rare terminally differentiated cells present. Prominent cytoplasmic granularity is present in the majority of these cells. Scattered erythrocyte precursors and rare megakaryocytes are identified.
Immunophenotype (flow cytometry/immunohistochemistry):
Bone marrow aspirate: Promyelocytes were identified by CD45 versus side scatter and comprise 57% of the non-erythroid marrow elements. These cells are positive for CD13, CD33, CD117, and CD38.
Cytogenetics:
46,XY,t(15;17)(q22;q21)[8]/46,XY[12]
Molecular analysis:
Fluorescence in situ hybridization (FISH) showed fusion of the PML and RARA signals in 113/252 (45%) of nuclei examined.
Interesting feature(s) of submitted case:
History of malignant fibrohistiocytoma treated with chemotherapy. Patient developed acute leukemia fifteen months later presenting with pancytopenia. Bone marrow aspirate and flow cytometry showed predominance of promyelocytes with prominent granularity and occasional blasts. Cytogenetics showed a 15;17 translocation and FISH showed fusion of PML/RARA probes in 45% of cells.
Proposed diagnosis:
Acute promyelocytic leukemia, therapy related (t-ANLL).
Panel diagnosis:
agree with proposed diagnosis
Comments:
PowerPoint:
Presentation Link
Images:
 |
Figure 1 |
 |
Figure 2 |
 |
Figure 3 |
 |
Figure 4 |
 |
Figure 5 |
 |
Figure 6 |
 |
Figure 7 |
Back to Top
Back to Cases by Session
Back to Cases by Contact Submitter