Clinical characteristics of plasma cell leukemia and progress in its diagnosis and treatment / 白血病·淋巴瘤

Plasma cell leukemia (PCL) is a plasma cell proliferative disorder with strong invasiveness, rapid progression and poor prognosis. The incidence of PCL is about (0.04-0.05)/100 000 per year. According to the multiple myeloma (MM) history, PCL can be divided into primary plasma cell leukemia (PPCL) and secondary plasma cell leukemia (SPCL). PPCL accounts for about 60% of PCL, and it is in the stage of leukemia at diagnosis and has no history of MM. SPCL accounts for the remaining 40% of PCL, and mostly shows as the MM end-stage manifestation, but also can be secondary to Waldenstrom macroglobulinemia, B-cell lymphoma, chronic lymphoblastic leukemia, amyloidosis, etc. Patients who progress from MM to SPCL account for 2%-4% of all MM patients. Due to the low incidence and strong clinical heterogeneity of PCL, the evidence-based medicine about PCL is relatively lacking, this article reviews the clinical characteristics of PCL and progress in its diagnosis and treatment.

Progress of bcl-2 inhibitors in treatment of plasma cell disorders with t(11;14) / 白血病·淋巴瘤

Plasma cell disorders are a group of heterogeneous diseases originating from plasma cells, including multiple myeloma, plasma cell leukemia and light-chain amyloidosis, etc. Monoclonal plasma cells are detected in bone marrow and affected tissues, monoclonal immunoglobulin or components are detected in serum or urine, and some end-organs are injured. Plasma cell disorders accompanied by t(11;14) have unique biological characteristics and unsatisfactory response to proteasome inhibitors. With t(11;14) translocation, the expressions of cyclin D1 and anti-apoptotic protein bcl-2 are relatively high, which lead to the occurrence of plasma cell disorders and have implications for the prognosis of disease. Venetoclax is a bcl-2 inhibitor, and its single agent or combined with other drugs has achieved good efficacy in treatment of plasma cell disorders with t(11;14). This article reviews the progress of bcl-2 inhibitors in treatment of plasma cell disorders.

Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno / Primary plasma cell leukemia: report of five cases

Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.

Update of diagnosis and treatment of plasma cell leukemia / 白血病·淋巴瘤

Plasma cell leukemia (PCL) is a rare and aggressive malignant plasma cell tumor that is clinically susceptible to extramedullary lesions. It is classified into two types: primary PCL (pPCL) and secondary PCL (sPCL), of which 60 % are pPCL. The treatment of new drugs such as bortezomib and lenalidomide is important for improving the overall survival and disease-free survival of PCL, especially after induction of bortezomib-based chemotherapy,combined with autologous hematopoietic stem cell transplantation (auto-HSCT) can improve the survival of patients. Whether to adopt the next step of allogeneic hematopoietic stem cell transplantation (allo-HSCT) still needs further investigation. For young and suitable patients, early new drug-based regimen chemotherapy combined with auto-HSCT can be used, and if a suitable donor is available, the further allo-HSCT consolidation therapy is feasible. For elderly patients (≥65 years old), a new drug-based regimen can be used to induce chemotherapy, and further followed consolidation therapy plus maintenance therapy. Whether to take auto-HSCT after early induction chemotherapy depends on the individualized factors of the patient. The next generation anti-plasma cells drugs, monoclonal antibodies and other immunotherapies or new drugs in clinical trials are also worth exploring.

Advances in clinical treatment and biological features of primary plasma cell leukemia / 白血病·淋巴瘤

Primary plasma cell leukemia (pPCL) is a rare and aggressive plasma cell disorder with distinct clinical and biological features.Thanks to the application of novel agents such as proteasome inhibitors (e.g.bortezomib) and immunomodulatory drugs (e.g.thalidomide and lenalidomide),along with stem cell transplantation,the outcome of pPCL has been improved a lot.Despite therapeutic advances,the current understanding of pPCL biology is still limited due to the lack of availability of clinical samples.This review provides the recent progress of the diagnostic and therapeutic options in pPCL.

Radiographic features of plasma cell leukemia in the maxilla: A case report

Plasma cell leukemia (PCL) is an aggressive form of multiple myeloma where there is hematogenous spread of abnormal plasma cells into the periphery. This is opposed to multiple myeloma, where the abnormal plasma cells stay in the bone marrow. PCL is more common in males than females, and is also more common in African-Americans than Caucasians. Signs and symptoms of PCL include, but are not limited to, renal insufficiency, hypercalcemia, anemia, lytic bone lesions, thrombocytopenia, hepatomegaly, and splenomegaly. Here, we discussed a case of a 71-year-old Caucasian female recently diagnosed with primary PCL with radiographic features of this disease throughout the body, with an emphasis on the maxillofacial skeleton and relevance from a dental standpoint.