| Session: Ph- chronic myeloproliferative disease |
| Case number: 208 |
Submitter(s): Louis Novoa-Takara, Steven H. Kroft.
Clinical history
This 66 y/o woman was noted 3.5 yrs ago to have an elevated WBC count in the range of 40-60 K/uL consisting nearly entirely of mature neutrophils, with normal Hb and platelet counts. The patient was asymptomatic at this time. Eighteen months ago, her WBC count increased to 84 K/uL, with a decrease in her Hb level from 12.2 to 11.1 g/dL. The platelet count remained in the normal range. A bone marrow revealed a 95% cellular marrow with no increase in blasts and an M:E ratio of 9.3:1. No dysplastic features were present, and plasma cells were not increased. Cytogenetics were normal. The patient remained asymptomatic. Because of the worsening leukocytosis she was started on hydroxyurea 1 year ago. Since that time she has had worsening anemia requiring transfusion, although her platelet count remained stable. Recently, she developed weight loss and fatigue. CBC reveals WBC of 81.7 k/uL with 93% segmented neutrophils, 3% bands, 1% lymphocytes, and 3% monocytes, Hb 8.3 g/dL, MCV 116.7 fL, and platelets 118 k/uL. By ultrasound, the spleen is enlarged (18.4 x 9.5 cm).
Details of gross/microscopic pathology:
The peripheral leukocytosis consisted nearly entirely of mature neutrophils with prominent toxic changes, including numerous large Dohle bodies. Tetraploid (twin) neutrophils were often seen. The marrow aspirate was highly cellular, with 21.4% promyelocytes and myelocytes, 54.6% metamyelocytes, bands, and segmented neutrophils, 6.2% erythroid precursors, 0.4% eosinophils, 0.6% monocytes, 3.2% lymphocytes, and 13.6% plasma cells. Neutrophilic precursors were left shifted with prominent granulation, but no dysplasia. Many of the plasma cells had distinct nucleoli. The core biopsy was 95% cellular with a marked predominance of granulocytes. Megakaryocytes were mildly decreased in number and morphologically unremarkable. A prominent increase in interstitial plasma cells was present.
Immunophenotype (flow cytometry/immunohistochemistry):
Immunohistochemistry revealed lambda light chain restriction in the plasma cells.
Cytogenetics:
Bone marrow karyotyping was normal and FISH for t(9;22) was negative.
Molecular analysis:
Interesting feature(s) of submitted case:
This patient had a 3.5 year history of marked neutrophilia with features of chronic neutrophiic leukemia (CNL). Neutrophilic proliferations with features of CNL are associated with underlying neoplastic processes in a significant minority of cases, most notably plasma cell myeloma. Bone marrow examination 1.5 years ago showed no evidence of plasma cell dyscrasia. Now, however, a clonal plasma cell proliferation constituting 14% of marrow cells is present. Serum immunoglobulin quantification currently shows IgA of 3116 mg/dL (normal 66-433), IgG of 498 mg/dL (normal 66-433), and IgM of 67 mg/dL (normal 45-281). Protein electrophoresis and skeletal survey are pending. Interestingly, both the blood and marrow showed features reminiscent of a G-CSF response.
Proposed diagnosis:
Chronic neutrophilia associated with occult plasma cell dyscrasia.
Panel diagnosis:
agree with proposed diagnosis
Comments:
Blasts represented <1% of the BM differential count.
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