| Session: Ph- chronic myeloproliferative disease |
| Case number: 045 |
Submitter(s): C. V. Curry, J. L. Jorgensen, C. C. Chang.
Clinical history
A 51-year-old male presented with pneumonia. The CBC showed RBC 3.10 M/uL, Hgb 9.9 g/dL, Hct 31.6%, WBC 14.16 K/uL [Differential count: 41% neutrophils, 15% lymphocytes, 3% monocytes, 41% eosinophils (5,806/uL)], and platelets 163 K/uL. A bone marrow biopsy showed a hypercellular marrow with pronounced eosinophilia. At the time, the diagnosis of hypereosinophilic syndrome was rendered and the patient was treated with hydrea and steroids. Hypereosinophilia, however, was not resolved.
Nine months later, the patient presented with swelling of left neck and persistent hypereosinophilia. The CBC showed RBC 3.2 M/uL, Hgb 10.4 g/dL, Hct 32.4%, WBC 7.1 K/uL [differential count: 56% neutrophils, 8% lymphocytes, 6% monocytes, 30% eosinophils (2130/uL)], and platelets 77 K/uL. An ultrasound-guided biopsy of the left neck mass was performed and "precursor T lymphoblastic lymphoma" was diagnosed. The patient was treated with hyperCVAD and intrathecal AraC.
Details of gross/microscopic pathology:
Bone marrow: (B-plus fixative) The bone marrow biopsy was hypercellular (85% cellularity), showing trilineage hematopoiesis with marked eosinophilia and myeloid expansion (M:E ratio 5:1). Striking hypereosinophilia was appreciated in both the bone marrow sections and the aspirate smear. Hybrid (basophilic) granules were noted in many eosinophils and their precursors. [Figures 1-4]
Differential cell count: 1.5% myeloblasts, 8% promyelocytes, 20.5% myelocytes, 10.5% metamyelocytes, 15% band granulocytes, 10% segmented granulocytes, 16% eosinophils, 0.5% monocytes, 14.5% nucleated erythrocytes, and 2.5% lymphocytes and 1% plasma cells.
Neck mass: (10% formalin fixation) The biopsy cores were diffusely infiltrated by small to medium-sized neoplastic lymphoid cells having a high nuclear:cytoplasmic ratio, and round to oval to irregularly shaped nuclei with dispersed chromatin without prominent nucleoli. Several mitotic figures were present. Eosinophils were not appreciated. [Figures 5-7]
Immunophenotype (flow cytometry/immunohistochemistry):
Bone marrow: No evidence of precursor T lymphoblastic leukemia by flow cytometry.
Neck mass: Immunohistochemistry showed the neoplastic lymphoid cells were positive for TdT [Figure 8], CD1a, CD3, CD5, CD8, CD43 and negative for CD34 and CD20.
Cytogenetics:
Cytogenetic studies of the bone marrow showed add (12)(p12.3).
Molecular analysis:
Interesting feature(s) of submitted case:
The 8p11 myeloproliferative disorder, a chronic myeloproliferative disorder frequently presenting with eosinophilia and associated T-cell lymphoblastic lymphoma, was recently described; the most common recurrent translocation is t(8;13)(p11;q12). Our patient presented with typical manifestations of 8p11 myeloproliferative syndrome. However, the classic 8p11 translocation was not identified by cytogenetics.
Proposed diagnosis:
Myeloproliferative disorder with eosinophilia/precursor T lymphoblastic lymphoma, lacking a demonstrable 8p11 translocation.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Additional information from the submitter: The cytogenetic studies [add(12)(p12.3)] were performed on the initial bone marrow with hypereosinophilic syndrome. The bone marrow examination concurrent to the examination of the neck mass was also performed and showed essentially similar findings to the initial bone marrow, i.e. hypercellular marrow with pronounced eosinophilia. The cytogenetics of this concurrent bone marrow also demonstrated [add(12)(p12.3)].
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