[Acquired platelet dysfunction with severe bleeding tendency in triple-negative myelofibrosis]

Area of Science:

• Hematology
• Oncology
• Internal Medicine

Background:

• Acquired platelet dysfunction is an uncommon complication in myelodysplastic syndromes (MDS) and myeloproliferative neoplasms (MPN).
• The precise frequency and underlying mechanisms of platelet dysfunction in these conditions remain poorly understood.
• Patients with MDS/MPN may present with bleeding tendencies, necessitating further investigation into hemostatic abnormalities.

Observation:

• A 50-year-old man presented with leukocytosis, anemia, splenomegaly, and bleeding tendency.
• Bone marrow biopsy revealed hypercellularity, multilineage dysplasia, and fibrosis, consistent with triple-negative myelofibrosis.
• The patient experienced massive hematomas post-biopsy, despite normal coagulation factors, indicating significant platelet dysfunction.

Findings:

• Platelet aggregation studies showed impaired responses to ADP, collagen, and epinephrine.
• Treatment with hydroxyurea and ruxolitinib (JAK inhibitor) was ineffective.
• The patient died 144 days post-hospitalization, suggesting aggressive disease progression.

Implications:

• This case highlights the potential for acquired platelet dysfunction in myelofibrosis, possibly due to somatic genetic events.
• Further research is needed to elucidate the frequency and mechanisms of platelet dysfunction in MDS/MPN.
• Understanding these mechanisms could lead to improved diagnostic and therapeutic strategies for bleeding complications.