Abstract

Reactive myelofibrosis (MF) may be associated with infectious, inflammatory, or neoplastic conditions. Extensive myelofibrosis and indolent course are frequent in acute megakaryoblastic leukemia, but these findings are rarely reported in acute promyelocytic leukemia (APL). Patients with neoplastic disorders associated with MF are reported to have an inferior outcome. However, the effect of this association is not apparent in APL patients. This report presents a case of APL in a pediatric patient in association with significant reticulin fibrosis and osteosclerosis. The case highlights that fibrosis in the marrow can make it more challenging to diagnose acute leukemia. The PML-RARA transcripts can be present with subtle clinical or hematological findings, emphasizing the importance of molecular studies in unexplained cytopenias. Furthermore, in the case of APL, as opposed to other myeloid neoplasms, fibrosis does not appear to be associated with a worse prognosis and can resolve after treatment.

Highlights

  • Reactive myelofibrosis (MF) may be associated with infectious, inflammatory, or neoplastic conditions, such as Epstein-Barr virus (EBV), systemic lupus erythematosus, myelodysplastic syndrome (MDS) or myeloproliferative neoplasms, acute lymphoblastic leukemia (ALL), and acute myeloid leukemia (AML) [1]

  • Extensive myelofibrosis and indolent course are frequent in acute megakaryoblastic leukemia subtype, but these findings are rarely reported in acute

  • This report presents a case of acute promyelocytic leukemia (APL) in a pediatric patient in association with significant reticulin fibrosis and osteosclerosis

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Summary

Introduction

Reactive myelofibrosis (MF) may be associated with infectious, inflammatory, or neoplastic conditions, such as Epstein-Barr virus (EBV), systemic lupus erythematosus, myelodysplastic syndrome (MDS) or myeloproliferative neoplasms, acute lymphoblastic leukemia (ALL), and acute myeloid leukemia (AML) [1]. This report presents a case of APL in a pediatric patient in association with significant reticulin fibrosis and osteosclerosis. A second BM aspiration and biopsy were performed, revealing 62% abnormal promyelocytes, which contained abundant dysplastic granules and Auer rods, suggesting APL in a medium cellularity bone marrow (Fig. 1). Flow cytometric evaluation of the bone marrow shows 56% atypical cell population with high side scatter characteristics, strongly suggestive of APL, expressing CD13/CD33/CD117/myeloperoxidase (MPO), lacking CD34/HLA-DR (Fig. 2).

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