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.

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.