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Published on: April 1, 2015
Primary myelofibrosis terminated in basophilic leukemia and successful allogeneic bone marrow transplantation
Naoshi Sugimoto1, Takayuki Ishikawa, Saori Gotoh
1Department of Hematology and Clinical Immunology, Kobe City General Hospital, Kobe, Japan.
Abstract:
Transformation of primary myelofibrosis (PMF) to basophilic leukemia is very rare. We report the case of a 44-year-old man who had had PMF for 6 years. His hematopoiesis deteriorated with marked splenomegaly, requiring multiple red blood cell and platelet transfusions. Soon after splenectomy, progressive basophilia (32.3 x 10(9)/L) developed, infiltrating the skin as well as the bone marrow. The patient underwent allogeneic bone marrow transplantation with cells from an HLA-matched sibling. Despite the presence of hyperhistaminemia (99.1 ng/mL) after conditioning with cyclophosphamide, the pregrafting and post-grafting periods were uneventful. Prophylactic administration of both H1 and H2 receptor antagonists and sufficient hydration appeared to be important.
Insights
Transformation of primary myelofibrosis to basophilic leukemia is rare. This case highlights successful allogeneic bone marrow transplant management for a patient with advanced disease and hyperhistaminemia.
Area of Science:
- Hematology
- Oncology
- Stem Cell Transplantation
Background:
- Primary myelofibrosis (PMF) is a chronic myeloid neoplasm.
- Transformation to acute leukemia, particularly basophilic leukemia, is exceptionally rare.
- This case involves a patient with a 6-year history of PMF and disease progression.
Observation:
- The patient presented with deteriorating hematopoiesis and significant splenomegaly.
- Splenectomy was followed by the rapid development of marked basophilia (32.3 x 10(9)/L).
- Basophilic infiltration affected both the skin and bone marrow.
Findings:
- The patient underwent successful allogeneic bone marrow transplantation from an HLA-matched sibling.
- Conditioning with cyclophosphamide resulted in hyperhistaminemia (99.1 ng/mL).
- The pre- and post-transplant periods were uneventful with prophylactic H1/H2 antagonists and hydration.
Implications:
- This case demonstrates a rare transformation of PMF to basophilic leukemia.
- Successful management involved allogeneic stem cell transplantation.
- Prophylactic histamine receptor blockade and hydration are crucial in managing post-transplant hyperhistaminemia.
