Clinical correlates of serum pro-major basic protein in a spectrum of eosinophilic disorders and myelofibrosis

Ching Liang Ho, Hirohito Kito, Diane L. Squillace, Terra L. Lasho, Ayalew Tefferi

Research output: Contribution to journalArticle

Abstract

Background/Aims: Eosinophil cytoplasmic granules contain major basic protein (MBP), which is primarily translated from its precursor, proMBP. In this study, we evaluated the plasma level of proMBP in eosinophilic and chronic myeloproliferative disorders (MPN). Methods: The levels of plasma proMBP were measured by radioimmunoassay in 25 healthy controls, 23 patients with systemic mastocytosis (SM), 11 patients with idiopathic eosinophilia (IE) and a cohort of 170 patients with MPN which included 76 patients with de novo myelofibrosis, 42 with polycythemia vera (PV), 17 with postpolycythemic myeloid metaplasia (Post-PV MF), 21 with essential thrombocythemia (ET) and 14 with postthrombocythemic myeloid metaplasia (Post-ET MF). Results: The plasma proMBP level was significantly higher in patients with SM with eosinophilia (p < 0.001), IE (p < 0.001) and MPN with eosinophilia (p = 0.002) than in healthy controls. The median proMBP level of Post-PV MF and Post-ET MF patients was significantly higher than in PV and ET patients (p < 0.001 and p = 0.009, respectively). In IE patients, elevated proMBP was significantly correlated with the presence of splenomegaly (p < 0.05). In 76 de novo myelofibrosis patients, the proMBP level was correlated with spleen size and the presence of hypercatabolic symptoms. Conclusion: The significantly elevated levels of proMBP in myelofibrosis patients implies that proMBP could be an important stromal cytokine in bone marrow fibrosis.

Original languageEnglish (US)
Pages (from-to)158-164
Number of pages7
JournalActa Haematologica
Volume120
Issue number3
DOIs
StatePublished - Jan 2009

Fingerprint

Primary Myelofibrosis
Eosinophilia
Essential Thrombocythemia
Serum
Polycythemia Vera
Proteins
Systemic Mastocytosis
Cytoplasmic Granules
Myeloproliferative Disorders
Splenomegaly
Eosinophils
Radioimmunoassay
Spleen
Cytokines

Keywords

  • Eosinophilia
  • Myeloproliferative disorder
  • Pro-major basic protein
  • Systemic mastocytosis

ASJC Scopus subject areas

  • Hematology

Cite this

Clinical correlates of serum pro-major basic protein in a spectrum of eosinophilic disorders and myelofibrosis. / Ho, Ching Liang; Kito, Hirohito; Squillace, Diane L.; Lasho, Terra L.; Tefferi, Ayalew.

In: Acta Haematologica, Vol. 120, No. 3, 01.2009, p. 158-164.

Research output: Contribution to journalArticle

Ho, Ching Liang ; Kito, Hirohito ; Squillace, Diane L. ; Lasho, Terra L. ; Tefferi, Ayalew. / Clinical correlates of serum pro-major basic protein in a spectrum of eosinophilic disorders and myelofibrosis. In: Acta Haematologica. 2009 ; Vol. 120, No. 3. pp. 158-164.
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N2 - Background/Aims: Eosinophil cytoplasmic granules contain major basic protein (MBP), which is primarily translated from its precursor, proMBP. In this study, we evaluated the plasma level of proMBP in eosinophilic and chronic myeloproliferative disorders (MPN). Methods: The levels of plasma proMBP were measured by radioimmunoassay in 25 healthy controls, 23 patients with systemic mastocytosis (SM), 11 patients with idiopathic eosinophilia (IE) and a cohort of 170 patients with MPN which included 76 patients with de novo myelofibrosis, 42 with polycythemia vera (PV), 17 with postpolycythemic myeloid metaplasia (Post-PV MF), 21 with essential thrombocythemia (ET) and 14 with postthrombocythemic myeloid metaplasia (Post-ET MF). Results: The plasma proMBP level was significantly higher in patients with SM with eosinophilia (p < 0.001), IE (p < 0.001) and MPN with eosinophilia (p = 0.002) than in healthy controls. The median proMBP level of Post-PV MF and Post-ET MF patients was significantly higher than in PV and ET patients (p < 0.001 and p = 0.009, respectively). In IE patients, elevated proMBP was significantly correlated with the presence of splenomegaly (p < 0.05). In 76 de novo myelofibrosis patients, the proMBP level was correlated with spleen size and the presence of hypercatabolic symptoms. Conclusion: The significantly elevated levels of proMBP in myelofibrosis patients implies that proMBP could be an important stromal cytokine in bone marrow fibrosis.

AB - Background/Aims: Eosinophil cytoplasmic granules contain major basic protein (MBP), which is primarily translated from its precursor, proMBP. In this study, we evaluated the plasma level of proMBP in eosinophilic and chronic myeloproliferative disorders (MPN). Methods: The levels of plasma proMBP were measured by radioimmunoassay in 25 healthy controls, 23 patients with systemic mastocytosis (SM), 11 patients with idiopathic eosinophilia (IE) and a cohort of 170 patients with MPN which included 76 patients with de novo myelofibrosis, 42 with polycythemia vera (PV), 17 with postpolycythemic myeloid metaplasia (Post-PV MF), 21 with essential thrombocythemia (ET) and 14 with postthrombocythemic myeloid metaplasia (Post-ET MF). Results: The plasma proMBP level was significantly higher in patients with SM with eosinophilia (p < 0.001), IE (p < 0.001) and MPN with eosinophilia (p = 0.002) than in healthy controls. The median proMBP level of Post-PV MF and Post-ET MF patients was significantly higher than in PV and ET patients (p < 0.001 and p = 0.009, respectively). In IE patients, elevated proMBP was significantly correlated with the presence of splenomegaly (p < 0.05). In 76 de novo myelofibrosis patients, the proMBP level was correlated with spleen size and the presence of hypercatabolic symptoms. Conclusion: The significantly elevated levels of proMBP in myelofibrosis patients implies that proMBP could be an important stromal cytokine in bone marrow fibrosis.

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KW - Systemic mastocytosis

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