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Histopathology. 2017 Dec;71(6):897-908. doi: 10.1111/his.13309. Epub 2017 Sep 28.

Prognostic significance of a comprehensive histological evaluation of reticulin fibrosis, collagen deposition and osteosclerosis in primary myelofibrosis patients.

Author information

1
Division of Pathology, Department of Pathophysiology and Transplantation, University of Milan and IRCCS Ca' Granda, Maggiore Policlinico Hospital Foundation, Milan, Italy.
2
Hematology Division, IRCCS Ca' Granda, Maggiore Policlinico Hospital Foundation, Milan, Italy.
3
Department of Oncology and Hemato-oncology, University of Milan, Milan, Italy.
4
Laboratory GA Maccacaro, Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy.
5
Division of Pathology, Department of Health Sciences, University of Milan and San Paolo Hospital, Milan, Italy.

Abstract

AIMS:

To evaluate whether a comprehensive histological evaluation of reticulin fibrosis, collagen deposition and osteosclerosis in bone marrow trephine biopsies (BMBs) of primary myelofibrosis (PMF) patients may have prognostic implications.

METHODS AND RESULTS:

Reticulin fibrosis, collagen deposition and osteosclerosis were graded from 0 to 3 in a series of 122 baseline BMBs. Then, we assigned to each case a comprehensive score [reticulin, collagen, osteosclerosis (RCO) score, ranging from 0 to 9] that allowed us to distinguish two groups of patients, with low-grade (RCO score 0-4) and high-grade (RCO score 5-9) stromal changes. Of 122 patients, 88 displayed a low-grade and 34 a high-grade RCO score. The latter was associated more frequently with anaemia, thrombocytopenia, peripheral blood blasts and increased lactate dehydrogenase levels. The RCO score was correlated strictly with overall mortality (P = 0.013) and International Prognostic Scoring System (IPSS) risk categories, and was able to discriminate the overall survival of both low- and high-grade patients (log-rank test: P < 0.001). Moreover, it proved to be more accurate than the European Consensus on Grading of Bone Marrow Fibrosis (ECGMF grade) in identifying high-risk patients with poor prognosis. Finally, a combined analysis of RCO scores and IPSS risk categories in an integrated clinical-pathological evaluation was able to increase the positive predictive value (PPV) for mortality in high-risk patients.

CONCLUSION:

The comprehensive RCO score, obtained by histological evaluation of reticulin fibrosis, collagen deposition and osteosclerosis was prognostically significant and more accurate than ECGMF grade in identifying high-risk patients and improved PPV when applied in addition to IPSS.

KEYWORDS:

bone marrow morphology; collagen deposition; osteosclerosis; primary myelofibrosis; reticulin fibrosis

PMID:
28710830
DOI:
10.1111/his.13309
[Indexed for MEDLINE]

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