Factor VII Deficiency in Systemic Mastocytosis with an Associated Myeloid Neoplasm

Area of Science:

• Hematology
• Oncology
• Coagulation Disorders

Background:

• Factor VII (FVII) deficiency is a rare bleeding disorder, either congenital or acquired.
• Acquired FVII deficiency is typically linked to vitamin K deficiency, liver disease, or inhibitors.
• Isolated acquired FVII deficiency is uncommon and often associated with auto-antibodies.

Observation:

• A patient with polycythemia vera developed systemic mastocytosis and FVII deficiency concurrently.
• The FVII deficiency in this patient was not caused by inhibitors.
• The deficiency improved following antineoplastic treatment for the hematological neoplasms.

Findings:

• This case suggests a potential association between systemic mastocytosis with a hematological neoplasm (SM-AHN) and acquired FVII deficiency.
• Malignancies can deplete circulating FVII via cancer cell binding or proteolytic degradation.
• The findings indicate that pathological mast cells may influence clotting factor concentrations.

Implications:

• Further research is warranted to explore the relationship between systemic mastocytosis and acquired FVII deficiency.
• Patients with systemic mastocytosis should be evaluated for potential clotting factor abnormalities.
• Understanding this link may improve the management of bleeding complications in patients with hematological neoplasms.