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Published on: February 21, 2018
Acute promyelocytic leukemia with marrow fibrosis at initial presentation: possible involvement of transforming
1Second Department of Internal Medicine, Hyogo College of Medicine, Hyogo, Japan.
Abstract:
Although the occurrence of marrow fibrosis in acute myeloid leukemia has been described, there have been no reports of acute promyelocytic leukemia (APL) associated with marrow fibrosis. Here we describe an APL patient with severe marrow fibrosis at initial presentation. He had the typical manifestations of APL, except for marrow fibrosis. Complete remission was achieved by treatment with all-trans retinoic acid plus chemotherapy, and his marrow fibrosis gradually improved concomitantly with the decrease in leukemic cells. To clarify the mechanism of marrow fibrosis in this patient, we investigated the expression of genes for several cytokines promoting fibrosis by the reverse transcriptase polymerase chain reaction methods. An overexpression of transforming growth factor-beta(1) was noted in his leukemic cells at initial presentation, whereas no increase in expression was observed at the time of relapse when he no longer had marrow fibrosis. These findings suggest that overexpression of transforming growth factor-beta(1) was involved in the development of marrow fibrosis in this APL patient.
Insights
This study reports the first case of acute promyelocytic leukemia (APL) with severe marrow fibrosis. Overexpression of transforming growth factor-beta(1) in leukemic cells correlated with fibrosis, suggesting its role in APL-associated marrow fibrosis.
Area of Science:
- Hematology
- Oncology
- Molecular Biology
Background:
- Marrow fibrosis is documented in acute myeloid leukemia but not previously reported in acute promyelocytic leukemia (APL).
- Acute promyelocytic leukemia is a distinct subtype of acute myeloid leukemia characterized by specific genetic mutations.
Observation:
- A case of APL presenting with severe marrow fibrosis is described.
- The patient exhibited typical APL manifestations alongside significant marrow fibrosis.
- Complete remission was achieved with all-trans retinoic acid and chemotherapy.
Findings:
- Marrow fibrosis gradually improved as leukemic cell counts decreased.
- Reverse transcriptase polymerase chain reaction analysis revealed overexpression of transforming growth factor-beta(1) in leukemic cells at initial presentation.
- This overexpression was absent at relapse when marrow fibrosis was no longer present.
Implications:
- These findings suggest that transforming growth factor-beta(1) overexpression plays a role in the development of marrow fibrosis in APL.
- This discovery may open new avenues for understanding and potentially treating APL-associated complications.
- Further research is warranted to confirm the mechanism and explore therapeutic strategies targeting TGF-β1.
