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Published on: March 26, 2018
Plasma cell leukemia with myelofibrosis
T Murayama1, T Matsui, Y Hayashi
1Department of Medicine, Kobe University School of Medicine, Japan.
Insights
This case study highlights a potential link between plasma cell leukemia and myelofibrosis. Treatment of plasma cell leukemia with chemotherapy initially improved myelofibrosis, but relapse of leukemia also led to myelofibrosis recurrence.
Area of Science:
- Hematology
- Oncology
- Pathology
Background:
- Plasma cell leukemia (PCL) is a rare and aggressive malignancy of plasma cells.
- Myelofibrosis (MF) is a myeloproliferative neoplasm characterized by bone marrow fibrosis.
Observation:
- A 60-year-old woman presented with back pain and monoclonal hypergammaglobulinemia.
- Peripheral blood revealed approximately 40% plasma-cell-like cells, and bone marrow aspiration yielded a dry tap.
- A bone marrow biopsy confirmed a diagnosis of plasma cell leukemia with myelofibrosis.
Findings:
- Combination chemotherapy (vincristine, pirarubicin, dexamethasone) initially led to improvement in both PCL and MF.
- Resistance to chemotherapy in PCL was associated with the reappearance of myelofibrosis.
- This clinical course suggests a potential pathogenetic relationship between PCL and MF.
Implications:
- This case provides evidence supporting a shared or related etiology between plasma cell leukemia and myelofibrosis.
- Further research is warranted to elucidate the underlying mechanisms connecting these two hematologic malignancies.
- Understanding this relationship may lead to novel therapeutic strategies for patients with co-occurring PCL and MF.
Abstract:
We describe a case of plasma cell leukemia associated with myelofibrosis. A 60-year-old woman was admitted due to lumbago and monoclonal hypergammaglobulinemia. Peripheral blood showed about 40% of plasma-cell-like cells. A bone marrow aspiration was dry tap. The patient was diagnosed as having plasma cell leukemia with myelofibrosis by bone marrow biopsy. Plasma cell leukemia as well as myelofibrosis improved with combination chemotherapy using vincristine, pirarubicin, and dexamethasone. However, when plasma cell leukemia became resistant to chemotherapies, myelofibrosis also reappeared. This case strongly suggests the pathogenetic relationship between plasma cell leukemia and myelofibrosis.
