| Session: Therapy-related myeloid neoplasm |
| Case number: 034 |
Submitter(s): Gerald M. Penn.
Clinical history
The patient is a 61 y.o. male with prev. hx of Lt. cheek follicular lymphoma in 19994, Rx observation. In 5/99, he had a recurrence in Lt. neck, Rx CHOP-R and GM-CSF. A staging CT of abdomen and chest in 7/03 showed a mediastinal mass. A CBC showed: WBC 6.4K/uL, MCV 88.1 fL, Hgb 15.6 g/dL, and PLT 208K/uL. Differential was normal with no atypical cells. BM showed erythroid hyperplasia with no lymphoma. Flow cytometry and cytogenetics were normal. Rx was Rituxin until he developed Lt. axillary (3/04) and cervical lymph node (4/04) involvement. Rx was changed to Fludara. In 6/04, he developed a hemolytic anemia (combs -); no PNH testing was performed. A Rt. eyebrow lymphoma was demonstrated in 7/05 and Rx changed to COP-R until 1/06 when he developed pancytopenia; WBC 2.4K/uL, Hgb 8.9 g/dL, MCV 86.3 fL, and PLT 33K/uL with 46.2% granulocytes, 32% lymphocytes, 21.4% monocytes, and 0.4% blasts.
Details of gross/microscopic pathology:
The bone marrow aspirate from 8/03 demonstrated 46% erythroids with increased erythroblasts and <1% myeloblasts. Iron stain showed increased sideroblasts, but no ringed sideroblasts.[figure1]The peripheral blood smear of 3/06 demonstrated hypogranular-pelgeroid PMNs and occasional blasts.[figure2]Megaloblastoid NRBC were also noted. The bone marrow aspirate was hypocellular with an inverted M/E and 10% blasts.[figure3]Iron stain showed multinucleated and dysplastic sideroblasts.[figure4]The bone marrow biopsy was 95% cellular and contained numerous immature precursors with increased reticulin.[figure5]
Immunophenotype (flow cytometry/immunohistochemistry):
Special stains on the bone marrow biopsy of 8/03 demonstrated the erythroid hyperplasia and confimed the lack of significant reticulum and myeloblasts.[figure6][figure7]The latter observation was confirmed by flow cytometry. (not shown).
In contrast, the flow cytometry of the 3/06 bone marrow aspirate showed hypogranular myeloid cells and increased blast cells with an aberrant phenotype; CD7+, CD13+, CD33-, CD34+, CD117+, and HLA-Dr.[figure8][figure9]Immunohistochemistry of the biopsy core demonstrates the numerous blasts and immature erythroid cells.[figure10]
Cytogenetics:
Cytogenetics showed:
45,XY, del(7)(q22), add (11)(q25), -21[3]
45,idem,t(13;19)(q14;p13.3)[2]
46,XY[2].
Molecular analysis:
FISH analysis confirmed deletion of 7q.
Interesting feature(s) of submitted case:
This case illustrates the need for molecular markers to define erythroid dysplasia, as well as the use of phenotypic markers other than sideroblasts for demonstrating an evolving erythroleukemia. (such as CD55, CD59, Hgb H, Maturation of NRBC by FLOW).
Proposed diagnosis:
MDS/AML therapy related.
Panel diagnosis:
agree with proposed diagnosis
Comments:
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