Journal List > Korean J Hematol > v.42(2) > 1032716

Kim, Park, Lee, Jang, Chi, and Seo: A Case of Chronic Neutrophilic Leukemia with Multiple Myeloma and an Increased Function of Her Neutrophils

Abstract

We describe here a 64-year-old woman who simultaneously presented with chronic neutrophilic leukemia (CNL) and multiple myeloma (MM). The patient presented with mature neutrophilic leukocytosis, hepatosplenomegaly, the absence of Philadelphia chromosome and the BCR-ABL fusion gene, along with IgG kappa type monoclonal gammopathy in her serum and urine. The bone marrow aspirates showed hypercellularity with marked granulocytic hyperplasia and an increase in immature plasma cells. The neutrophil function tests showed increased phagocytosis, chemotaxis and respiratory burst activity, but there was normal microbial killing activity. The patient was treated with dexamethasone and pamidronate for MM and with hydroxyurea for CNL, and she was discharged from the hospital in an improved condition.

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Fig. 1
Peripheral blood and bone marrow findings in the patient showing coexistence of chronic neutrophilic leukemia and multiple myeloma. (A) Neutrophilia, toxic changes of neutrophils and rouleaux formation of red cells in peripheral blood (Wright stain, ×400). (B) Markedly increased granulocytic series and frequent plasmablasts and plasma cells in bone marrow aspirate (Wright stain, ×400). (C) Hypercellular marrow with granulocytic hyperplasia and increased plasmablasts and plasma cells (H&E stain, ×400).
kjh-42-151f1.tif
Fig. 2
Immunofixation electrophoresis shows IgG kappa type monoclonal gammopathy in serum (A) and IgG kappa and free kappa type Bence-Jones proteinuria in urine (B). SPE, serum protein electrophoresis; UPE, concentrated urine electrophoresis.
kjh-42-151f2.tif
Table 1.
Neutrophil function in the patient with coexisting chronic neutrophilic leukemia and multiple myeloma
Chemotaxis Neutrophil No./Boyden chamber well (8mm2)
    Stimulants Patient Normal control Ratio (Patient/Normal control)
    Phosphate buffered saline (random motility) 46,080 16,896 2.73
    FMLP∗ 10?8 M/L 83,968 52,224 1.61
    Heat-inactivated normal plasma 73,728 35,840 2.06
    Zymosan activated normal plasma 100,352 72,704 1.38
Phagocytosis Candida albicans Patient Normal control Ratio (Patient/Normal control)
    Median channel of fluorescence 178 1,863 440 4.23
Respiratory burst activity Mean channel of fluorescence (H2O2 production)
    Stimulants Patient Normal control Ratio (Patient/Normal control)
    Rest (no stimulant) 1,106 166 6.66
    PMA 200ng/mL 7,032 3,020 2.33
Microbial killing Patient Normal control
    % of C. albicans killing by neutrophils 40.8% 43.1%

∗Formyl-methionyl-leucyl-phenylalanine,

Phorbol myristate acetate. Compared with the normal control neutrophils, the patient neutrophils show increased chemotaxis, phagocytosis and respiratory burst activity, especially increased in no stimulation state, but normal microbial killing activity.

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