| Session: Therapy-related myeloid neoplasm |
| Case number: 156 |
Submitter(s): Sarah L. Lott, Magdalena Czader.
Clinical history
A 49 year old female with a long history of Wegener's granulomatosis, currently on prednisone, presented for evaluation of progressive deterioration of blood counts. The patient had previously been treated with Methotrexate and Cytoxan. She received her last dose of Methotrexate two months prior to the bone marrow exam. The CBC concurrent to the bone marrow exam was: WBC 10.7 k/cumm, hemoglobin 7.4 g/dl, MCV 95.7 fl, platelet count 6 k/cumm, absolute monocyte count 5.2 k/cumm. (CBC one month prior was: WBC 3k/cumm, hemoglobin 10.4 g/dl, platelet count 66k/cumm). Physical exam showed no evidence of lymphadenopathy or organomegaly. There was no history of exposure to radiation or toxins. Shortly after the diagnosis the patient developed ARDS and died before receiving treatment.
Details of gross/microscopic pathology:
Bone marrow (BM) biopsy was hypercellular with 80% cellularity and showed granulocytic hyperplasia with a left shift and focal eosinophilia. The erythroid series was well-represented and showed dysplastic features. Megakaryocytes were decreased with dysmegakaryopoiesis.[figure1]BM aspirate smear showed 10% blasts, a left shift in the granulocytic series, erythroid hyperplasia (55%) with marked dyserythropoiesis, and increased monocytes and their precursors.[figure3]
Immunophenotype (flow cytometry/immunohistochemistry):
CD34 immunostain performed on BM biopsy showed increased CD34+ cells (approximately 10%) with focal ALIP-like aggregates.[figure2]Flow cytometric immunophenotyping performed on peripheral blood showed 8% of myeloid blasts, 54% of monocytes negative for CD15 and with partial CD2 co-expression and hypogranulation of granulocytic series.
Cytogenetics:
Cytogenetic studies showed 46,XX,-7,+21[20]
No evidence of BCR/ABL1 fusion or deletion of 9q34 by FISH
No evidence of inversion of chromosome 16 by FISH.
Molecular analysis:
Interesting feature(s) of submitted case:
Significant peripheral blood monocytosis and increased monocytes and their precursors present in the bone marrow raise the differential diagnosis of therapy-related chronic myelomonocytic leukemia. Rare cases of therapy-related CMML have been described in the literature (Singh Z, AJCP. Brichard B, Leuk Res. Oo T, AJH). However, the recent study by Singh et al demonstrated that morphologic subclassification and even the number of blasts (<5% vs. 5-19%) are of no clinical significance in patients with therapy-related myeloid neoplasms, as all these cases demonstrate uniformly poor outcome. Similarly, only a borderline difference in median survival was found between patients with initial diagnosis of t-MDS and t-AML.
Proposed diagnosis:
MDS/AML, therapy related.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Images:
 |
Figure 1 |
 |
Figure 2 |
 |
Figure 3 |
Back to Top
Back to Cases by Session
Back to Cases by Contact Submitter