Acute Promyelocytic Leukemia Presenting with Severe Marrow Fibrosis.

Harsh Shah,Hamid Sayar,Carol Bradford

doi:10.1155/2015/826894

Abstract

We report a case of acute promyelocytic leukemia (APL) presenting with severely fibrotic marrow. There are four other reports of similar cases in the literature. Our patient was treated with All-Transretinoic Acid- (ATRA-) containing induction chemotherapy, followed by consolidation and maintenance therapy. He achieved a complete morphologic remission with adequate count recovery in a timely fashion, and later a molecular remission was documented. The patient remains in molecular remission and demonstrates normal blood counts now more than 4 years after induction. Since the morphological appearance may not be typical and the bone marrow may not yield an aspirate for cytogenetic analysis, awareness of such entity is important to make a correct diagnosis of this potentially curable disease.

Highlights

Marrow fibrosis is thought to be secondary to activation of normal fibroblasts by cytokines secreted from CD34+/HLADR+ leukemic blasts, markers that are usually not expressed by acute promyelocytic leukemia (APL) cells [3]
Bone marrow fibrosis has only rarely been reported in patients with APL
It is unclear why the incidence of fibrosis is much smaller in APL compared to other subtypes of acute myeloid leukemia (AML), but overproduction of fibrosis may be related to cytokine effect of CD34- and HLA-DR-expressing blasts which are more abundant in other subtypes than APL

Summary

Introduction

Variable levels of focal or diffuse reticulin fibrosis are detected in the bone marrow samples of up to 30% of patients with acute leukemia [1]. There are only four case reports in the literature of acute promyelocytic leukemia (APL) presenting with severe reticulin bone marrow fibrosis [2,3,4,5]. It is important to differentiate APL with fibrosis from other types of fibrotic acute myeloid leukemia (AML) in order to provide the most appropriate therapy and proper surveillance. We report a case of 48-year-old previously healthy male who presented with APL and severe bone marrow reticulin fibrosis

Case Report

Discussion

Findings

Conclusion

Conflict of Interests