| Session: Ph- chronic myeloproliferative disease |
| Case number: 066 |
Submitter(s): Dong Chen, Curtis A. Hanson.
Clinical history
A 47 year old man with acute chest pain was noted to have significant leukocytosis due to a marked neutrophilia. The patient was afebrile and lacked other symptoms of infection. Splenomegaly was not present on examination. Serum protein electrophoresis studies were normal. Although multiple blood cultures were negative for organisms, he was empirically treated with antibiotics. Subsequently, his chest pain gradually resolved, but his leukocytosis persisted. A bone marrow biopsy was performed.
CBC: Hgb: 12.1 g/dL; WBC: 22.6 x10(9)/L; PLT: 73 x10(9)/L
WBC Differential (%): Neutrophils 87; lymphocytes 5; monocytes 1; eosinophils 1; basophils 1; metamyelocytes 4; myelocytes 1.
Details of gross/microscopic pathology:
The peripheral blood smear showed a marked absolute neutrophilia with a minimal left shift. Dohle bodies and toxic granulation were absent. There was no basophilia or monocytosis. The bone marrow aspirate and biopsy (B5-fixed; acid decal) were markedly hypercellular for age (95%). There was a marked granulocytic hyperplasia with left-shifted maturation, but with no increase in blasts. Megakaryocytes were normal. Myelodysplastic features were not identified. Reticulin fibrosis was absent.
Cytochemical stains:
Myeloperoxidase, chloroacetate esterase, and butyrate esterase cytochemical studies showed normal staining without any increase in monocytes.
Immunophenotype (flow cytometry/immunohistochemistry):
Immunohistochemical stains were performed on paraffin-embedded bone marrow using antibodies against the following antigens: myeloperoxidase (MPO), CD61, CD34, and CD68. The myeloperoxidase stain confirmed the granulocyte predominance in the marrow. Normal numbers of monocytes and macrophages were identified by CD68. Megakaryocytes appeared unremarkable with anti-CD61. Only scattered CD34 positive cells were present.
Cytogenetics:
Cytogenetic studies of bone marrow showed a normal male karyotype: 46,XY.
Molecular analysis:
FISH studies of bone marrow cultured cells showed no fusion signal of BCR and ABL (500 cells were analyzed). Genomic DNA was extracted and a quantitative, allele-specific polymerase chain reaction (PCR) assay used to evaluate for the point mutation causing JAK2-V617F. No JAK2-V617F mutation was detected.
Interesting feature(s) of submitted case:
Chronic neutrophilic leukemia (CNL) is a rare chronic myeloproliferative disorder that is a diagnosis based on exclusion of other entities. Acute and chronic infectious etiologies and persistent neutrophilias associated with plasma cell malignancies need to be excluded. Myelodysplastic changes and features of classic myeloproliferative disorders, such as significant megakaryocyte abnormalities and clustering, are not identified. Reticulin fibrosis is minimal. Molecular studies are necessary to exclude bcr-abl and JAK2 abnormalities.
Proposed diagnosis:
Chronic myeloproliferative disorder, chronic neutrophilic leukemia.
Panel diagnosis:
No definitive evidence of chronic neutrophilic leukemia. Differential diagnosis includes toxic myelopathy.
Comments:
Panel comments: Occasional clusters of plasma cells. Stains performed by the panel: reticulin 1+, CD34 within normal limits, CD42b highlights increased dysplastic megakaryocytes, kappa and lambda immunostains show polyclonal plasma cells. Additional information from the author: MCV 83.9 fL.
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