| Session: Therapy-related myeloid neoplasm |
| Case number: 014 |
Submitter(s): Jeremy C. Wallentine, Sherrie L. Perkins.
Clinical history
The patient is a 54 year old Hispanic male with diffuse large B-cell lymphoma (1983) treated with 4 cycles of CHOP and local radiation treatment. The patient relapsed later in 1983 and was treated with cyclophosphamide, doxorubicin, and etoposide (ProMACE). In 2004 he relapsed again and received 2 cycles of rituximab,cyclophosphamide, doxorubicin, high-dose methotrexate/ifosfamide, etoposide, and high-dose cytarabine (CODOX-M/IVAC) with no further evidence of disease.
In 2006 the patient experienced progressive fatigue, and weakness. A CBC and bone marrow study performed in June of 2006 showed findings consistent with therapy related myelodysplastic syndrome (MDS) in transformation (fig. 1-4, 9-10). In October of 2006 a second bone marrow study was performed as part of an evaluation for bone marrow transplant. The CBC at that time was remarkable for a WBC of 1,260/uL, a hematocrit of 22.1% and a platelet count of 9,000/uL. The bone marrow study showed an acute myeloid leukemia (AML) with an extensive erythroid component (fig. 5-8).
Details of gross/microscopic pathology:
June, 2006:
The peripheral smear showed circulating dyspoietic red blood cells, an absolute leukopenia and a left shift with myeloid dyspoiesis (fig. 1-2). The bone marrow showed trilineage dysplasia with 0.6% myeloblasts (fig. 3-4). The M:E ratio was 0.6.
October, 2006:
The peripheral smear showed circulating dyspoietic red blood cells, an absolute neutropenia and lymphocytopenia, and circulating myeloblasts (6%) (fig. 5). The bone marrow aspirate showed increased MPO+ myeloblasts (24.1%, 92.4% of myeloid cells), and increased numbers of markedly dyspoietic erythroid precursors (12.2% pronormoblasts, 9.6% basophilic normoblasts, and 42.7% polychromatophilic normoblasts, total erythroids are 66.2%) (fig. 6-8).
Immunophenotype (flow cytometry/immunohistochemistry):
Initial flow showed 4% CD34+ myeloblasts with weak CD13 expression and lacking CD10 (fig. 9-10). No flow was performed in October of 2006.
Cytogenetics:
June, 2006:
Results: 45,XY,add(1)(q32),add(4)(p11),-5,-7,del(14)(q13q32),+mar[15]/46,XY[1]
October, 2006:
Results: 43~48,X,-Y,add(1)(q32),add(4)(p11),del(5)(p13),-14,add(15)(p11.1),add(16)(q24),-19,+1~3mar,+r[cp20].
Molecular analysis:
N/A.
Interesting feature(s) of submitted case:
The case describes an example of AML/MDS arising secondary to alkylating, radiation, and topoisomerase II inhibitor therapy for the treatment of primary and recurrent diffuse large B-cell lymphoma. Interesting features include the complex cytogenetic abnormalities resulting in rapid disease transformation. This case, if classified according to the FAB criteria, would be classified as an acute erythroleukemia (erythroid/myeloid FAB M6) that comprise approximately 5-6% of AML cases. However, since it occurred as a result of therapy and arose out of MDS it is classified as AML/MDS, therapy related by the WHO classification.
Proposed diagnosis:
AML/MDS, therapy related.
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 |
 |
Figure 8 |
 |
Figure 9 |
 |
Figure 10 |
Back to Top
Back to Cases by Session
Back to Cases by Contact Submitter