Telek Béla, Méhes Leonóra, Batár Péter, Kiss Attila, Udvardy Miklós
Debreceni Egyetem, Orvos- és Egészségtudományi Centrum, Altalános Orvostudományi Kar II. Belgyógyászati Klinika, Hematológiai Tanszék Debrecen Pf. 20. 4012.
Orv Hetil. 2008 Oct 12;149(41):1957-9. doi: 10.1556/OH.2008.28432.
The most aggressive and rare manifestation of multiple myeloma is plasma cell leukaemia (PCL). While secondary form of PCL represents those heavily pretreated cases when leukaemic transformation develops terminally after intensive chemotherapy in patients with multiple myeloma, primary cases are characterized by leukaemic symptoms present at diagnosis. The secondary form has a rapid progression. The management of PCL is still unsolved. The authors present a case of a patient with non-secretory multiple myeloma who had developed plasma cell leukaemia after peripheral stem cell transplantation. PAD (bortezomib, doxorubicin, dexamethasone) treatment resulted in complete remission and 9-month survival of the patient. Previous case reports in the literature and our experience have revealed PAD protocol to be well tolerated and effective in PCL. Combination of PAD treatment with autologous and/or allogenic stem cell transplantation might further improve patients' outcome.
多发性骨髓瘤最具侵袭性且罕见的表现形式是浆细胞白血病(PCL)。PCL的继发性形式指的是那些在多发性骨髓瘤患者接受强化化疗后,白血病转化在终末期发生的经过大量前期治疗的病例,而原发性病例的特征是在诊断时就出现白血病症状。继发性形式进展迅速。PCL的治疗仍然没有解决办法。作者报告了一例非分泌型多发性骨髓瘤患者,该患者在接受外周干细胞移植后发生了浆细胞白血病。PAD(硼替佐米、阿霉素、地塞米松)治疗使患者完全缓解并存活了9个月。文献中先前的病例报告以及我们的经验表明,PAD方案在PCL中耐受性良好且有效。将PAD治疗与自体和/或异基因干细胞移植相结合可能会进一步改善患者的预后。