SH/EAHP 2007 Workshop - Case Display

SH/EAHP 2007 Workshop - Progress in T-cell and NK cell Malignancies - title graphic

Session: Ph- chronic myeloproliferative disease

Case number: 069

Submitter(s): Jean Coviello-Malle, Jeffrey Sawyer, Wlodek Szczarkowski, Judy Burroughs, Robert Lorsbach.

Clinical history
This 57-year old female presented with a few month history of dyspnea. Laboratory workup at that time revealed a WBC count of 3.4 K/ul, hemoglobin 6.4 g/dl, and platelets 93 K/ul. Bone marrow examination at an outside hospital revealed an infiltrate of blasts (71% of nucleated cells), consistent with acute leukemia (Images 1 & 2). The patient underwent induction and consolidation chemotherapy. However, 5 months later, she developed hyperleukocytosis and was referred to our institution for further management. At UAMS, her WBC count was 294 K/ul with a differential count of 89% neutrophils, 6% lymphocytes, 3% eosinophils, and 1% monocytes. A concurrent bone marrow biopsy revealed a myeloproliferative disorder with marked marrow hypercellularity and myeloid hyperplasia (Images 4-6). The patient subsequently underwent allogeneic BMT 2 months later. Her post-transplant course was complicated by CMV infection and mild graft-versus-host disease. Nearly 29 months post transplantation, cytogenetics revealed recurrence of the same karyotypic abnormality seen at initial diagnosis (Image 9). The patient subsequently developed left-shifted myelopoiesis and mild eosinophilia in the bone marrow (Images 7 & 8). At the time of case submission, the patient was undergoing treatment with donor lymphocyte infusion.

Details of gross/microscopic pathology:
Submitted H&E sections from bone marrow biopsy 5 months after diagnosis (corresponds to Image 4). Decalcified in RDO.

Immunophenotype (flow cytometry/immunohistochemistry):
At diagnosis, flow cytometric analysis identified two blast populations. The first (35% of analyzed events) showed the phenotype: CD45^mod, CD34⁺, CD19⁺, CD20^-/+, cytoplasmic CD22⁺, cytoplasmic CD79a⁺, HLA-DR⁺, TdT⁺, CD33^-/+, and CD13^-/+; negative for all other myeloid, monocytic, and T lymphoid markers tested. The second blast population (36% of analyzed events) was: CD45⁺, CD34^-/+, CD33⁺, CD13^-/+, CD11b⁺, CD7^dim, CD2^-/+, and HLA-DR⁺; negative for all other myeloid, monocytic, and lymphoid markers tested.

Cytogenetics:
46,XX,t(6;8)(q27;p11.2) was present as the sole cytogenetic aberration at initial diagnosis and in subsequent pre- and post-transplant marrow specimens.

Molecular analysis:
None.

Interesting feature(s) of submitted case:
1) The 8p11 myeloproliferative disorder is an uncommon group of myeloproliferative disorders; 2) Although the frequency of progression to acute leukemia is high in the 8p11 disorders, this case is unusual in that acute leukemia was present at initial diagnosis, highlighting the importance of cytogenetics in the workup of cases of 8p11 myeloproliferative syndrome; 3) The development of acute bilineal leukemia has only rarely been described in the setting of the 8p11 myeloproliferative syndrome.

Proposed diagnosis:
Acute bilineal leukemia arising in the background of the 8p11 myeloproliferative syndrome.

Panel diagnosis:
agree with proposed diagnosis

Comments:

Images:

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	Figure 9