Chaulagain Chakra P, Diacovo Maria-Julia, Van Amy, Martinez Felipe, Fu Chieh-Lin, Jimenez Jimenez Antonio Martin, Ahmed Wesam, Anwer Faiz
Department of Hematology and Oncology, Maroone Cancer Center, Myeloma and Amyloidosis Program, Cleveland Clinic Florida, Weston, FL, USA.
Department of Pathology, Cleveland Clinic Florida, Weston, FL, USA.
Clin Med Insights Blood Disord. 2021 Feb 26;14:2634853521999389. doi: 10.1177/2634853521999389. eCollection 2021.
Primary plasma cell leukemia (PCL) is a rare and aggressive variant of multiple myeloma (MM). PCL is characterized by peripheral blood involvement by malignant plasma cells and an aggressive clinical course leading to poor survival. There is considerable overlap between MM and PCL with respect to clinical, immunophenotypic, and cytogenetic features, but circulating plasma cell count exceeding 20% of peripheral blood leukocytes or an absolute plasma cell count of >2000/mm distinguishes it from MM. After initial stabilization and diagnosis confirmation, treatment of PCL in a fit patient typically includes induction combination chemotherapy containing novel agents typically, with proteasome inhibitors (such as bortezomib) and immunomodulatory drugs (eg, lenalidomide), followed by autologous hematopoietic stem cell transplant (HSCT) and multidrug maintenance therapy using novel agents post-HSCT. Long-term outcomes have improved employing this strategy but the prognosis for non-HSCT candidates remains poor and new approaches are needed for such PCL patients not eligible for HSCT. Here, we report a case of primary PCL, and a comprehensive and up to date review of the literature for diagnosis and management of PCL. We also present the findings of Positron Emission Tomography (PET) scan. Since PCL is often associated with extra-medulary disease, including PET scan at the time of staging and restaging may be a novel approach particularly to evaluate the extra-medullary disease sites.
原发性浆细胞白血病（PCL）是多发性骨髓瘤（MM）的一种罕见且侵袭性变体。PCL的特征是外周血出现恶性浆细胞浸润以及临床病程侵袭性强，导致生存率低。MM和PCL在临床、免疫表型及细胞遗传学特征方面有相当多的重叠，但外周血白细胞中循环浆细胞计数超过20%或绝对浆细胞计数>2000/mm可将其与MM区分开来。在初始病情稳定及确诊后，适合的PCL患者的治疗通常包括含新型药物的诱导联合化疗，通常使用蛋白酶体抑制剂（如硼替佐米）和免疫调节药物（如来那度胺），随后进行自体造血干细胞移植（HSCT）以及HSCT后使用新型药物的多药维持治疗。采用这种策略长期预后有所改善，但对于不适合HSCT的患者预后仍然很差，对于这类不适合HSCT的PCL患者需要新的治疗方法。在此，我们报告一例原发性PCL病例，并对PCL的诊断和管理进行全面且最新的文献综述。我们还展示了正电子发射断层扫描（PET）的结果。由于PCL常与髓外疾病相关，包括在分期和再分期时进行PET扫描可能是一种新方法，尤其用于评估髓外疾病部位。
