ما هو العلاج الخطي الأول لورم الخلايا التغصنية البلازمية الانفجارية؟

References

DOI: 10.6004/jnccn.2023.7026

• Treatment is continued until disease progression or unacceptable toxicity.
• Hypoalbuminemia is the most consistent predictor of CLS, and hence before initiation of therapy, a serum albumin level of ≥3.2 g/dL with adequate cardiac function is required.
• Based on retrospective studies, ALL-based regimens have resulted in better response rates compared with AML-based or CHOP-based regimens.
• CNS prophylaxis is essential for patients who receive conventional intensive chemotherapy.
• In this trial, Pemmaraju et al first described in detail the clinical response criteria for BPDCN: normalization of peripheral blood counts; bone marrow blasts (<5%); regression of nodal, spleen, and liver masses; and 100% clearance of all skin lesions with no new lesions.
• At a median follow-up of 34 months, the median time to response was 39 days and median duration of response was 24.9 months.