OBJECTIVE: To evaluate the clinical characteristics, genetic abnormalities, treatment efficacy and prognostic factors in patients with plasma cell leukemia(PCL). METHODS: 30 patients diagnosed as PCL in our hospital from January 1993 to December 2019 were enrolled, and the clinical characteristics, laboratory findings, therapeutic regimes, and survival data of the patients were retrospectively analyzed. RESULTS: The median age of the 30 patients was 56.5 (28-80) years old, among them, 25 patients were primary plasma cell leukemia, and 5 patients were secondary plasma cell leukemia. Complex karyotypes and subdiploids were most common in cytogenetic abnormalities. Among the 20 cases of chromosome G banding, 11 (55%) cases were complex karyotypes and 8 (40%) cases were hypodiploid. Fluorescent in situ hybridization (FISH) test showed that among 11 cases, 6 cases showed 17p13 deletion, 8 cases showed at least two kinds of abnormalities, which including t (14; 16), t (8; 14), t (11;14), 17p13 deletion, and 13q14 deletion. The median overall survival (OS) time was 10.5 months for all patients. The median OS time of the patients in ECOG score ≤ 2 group was 21.5 months, which was significantly longer than those in the ECOG score>2 group(1.2 months) (P=0.017). The median OS time of the patients treated with novel agents (including proteasome inhibitor and/or immunomodulator) was 24.9 months, which was significantly longer than the patients treated with traditional chemotherapy group(10.5 months) (P＜0.001). For the patients treated with novel agents, the median OS time of patients accepted two novel agents combination was 30.9 months, which was longer than those of single novel agent(11.5 months) (P=0.021). The effect of genetic abnormolity to the OS of the patients showed no statistical difference. Multivariate statistical analysis showed that ECOG score>2 was the independent prognostic factor of plasma cell leukemia patients. There were two patients underwent allogeneic hematopoietic stem cell transplantation in the study,but died due to the pulmonary infection within 6 months after transplantation. CONCLUSION In the era of novel agents, ECOG score is an independent prognostic factor of plasma cell leukemia. Multiple novel agents treatment should be underwent as soon as possible to improve the prognosis of the patients. Pulmonary infection is a common factor that cause the death of the patients after allogeneic hematopoietic stem cell transplantation.
Aged ; Aged, 80 and over ; Hematopoietic Stem Cell Transplantation ; Humans ; In Situ Hybridization, Fluorescence ; Leukemia, Plasma Cell/genetics* ; Middle Aged ; Patients ; Prognosis ; Retrospective Studies

No abstract available.
Aged ; Humans ; Leukemia, Plasma Cell/complications/*diagnosis/pathology ; Leukocytosis ; Lymph Nodes/pathology ; Lymphoma, T-Cell/complications/*diagnosis/pathology ; Male ; Paraproteinemias/complications ; Polymerase Chain Reaction ; Receptors, Antigen, T-Cell, gamma-delta/genetics/metabolism ; Tomography, X-Ray Computed

This study was aimed to investigate the expression of plasma miR-223 in pediatric acute lymphoblastic leukemia (ALL) in different treatment time point. A total of 64 pediatric ALL samples were selected from patients treated in Beijing Children's Hospital from May 2005 to January 2012, including 30 samples at new diagnosis (ND), 30 samples at complete remission (CR) and 4 samples at relapse. Without RNA extraction, the miR-223 levels in plasma were directly detected by a reverse-transcription quantitative real-time PCR assay. The results indicated that the expression of plasma miR-223 in pediatric ALL was lower at ND but elevated after CR. The miR-223 expression in plasma of relapse patients didn't show significant difference probably due to a few cases of relapse. The miR-223 levels in plasma had not displayed significant difference between TEL-AML1 positive patients and no fusion gene B lineage ALL patients either at ND or at CR. It is concluded that the plasma miR-223 decreases at ND and increases in CR of children with ALL. miR-223 may act as an anti-oncogene and may be taken as a potential predictive biomarker for evaluating the therapeutic effect of leukemia.
Child ; Child, Preschool ; Female ; Humans ; Male ; MicroRNAs ; blood ; genetics ; Plasma ; metabolism ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; blood ; genetics ; Reverse Transcriptase Polymerase Chain Reaction ; methods

Involvement of the central nervous system is very uncommon in multiple myeloma, observed in approximately 1% of the multiple myeloma patients. We report a case of central nervous system myelomatosis with complex chromosome aberrations in a 62-yr-old female patient, who had previously been diagnosed as multiple myeloma. Fluorescent in situ hybridization revealed 13q deletion, p53 gene deletion and IGH/FGFR3 rearrangement and chromosomal study showed complex chromosome aberrations. After four cycles of chemotherapy, the patient was admitted to the hematology department with severe headache. Plasma cells were found in the cerebrospinal fluid (CSF), and CSF immunoelectrophoresis revealed abnormal precipitin arcs against anti-IgG and anti-lambda antisera. She was given systemic chemotherapy and eight courses of intrathecal chemotherapy, which cleared plasma cells in the CSF. Two months later, she was given autologous stem cell transplantation. Three months after stem cell transplantation, central nervous system myelomatosis progressed to plasma cell leukemia and two months later,the patient expired.
Antineoplastic Agents/therapeutic use ; Central Nervous System Neoplasms/*diagnosis/drug therapy/genetics ; Cerebrospinal Fluid/cytology ; *Chromosome Deletion ; Combined Modality Therapy ; Disease Progression ; Female ; Gene Deletion ; Humans ; Immunoelectrophoresis ; In Situ Hybridization, Fluorescence ; Leukemia, Plasma Cell/diagnosis ; Middle Aged ; Multiple Myeloma/*diagnosis/drug therapy/genetics ; Plasma Cells/pathology ; Precipitins/metabolism ; Receptor, Fibroblast Growth Factor, Type 3/genetics ; Stem Cell Transplantation ; *Translocation, Genetic ; Transplantation, Autologous ; Tumor Suppressor Protein p53/genetics

This study was aimed to investigate the deletion features of chromosome 13 in plasma cell leukemia (PCL) and its relationship with clinical features. The dual-color fluorescence in situ hybridization (FISH) with two different specific probes for the regions containing 13q14 and 13q31 was used to delet the chromosome in 21 patients with PCL. The results showed that 13 (61.9%) out of 21 PCL had 13q14 deletion. The number of 13q14 deletion cells ranged from 52% to 98%. 12 patients (57.4%) had 13q31 deletion. The number of cells with 13q31 deletion ranged from 50% to 98%. Out of 13 cases with (13q14) deletion, 12 cases had (13q31) deletion, but only 1 out of 12 cases was negative. It is concluded that chromosome 13 deletion may be involved in initiation of a subset of PCL, and the region of loss at chromosome 13 locates between 13q14 and 13q31 in PCL. FISH is a more rapid, exact and sensitive technique in analysis of chromosome 13 deletion in PCL.
Adult ; Aged ; Chromosome Deletion ; Chromosomes, Human, Pair 13 ; Female ; Humans ; In Situ Hybridization, Fluorescence ; methods ; Leukemia, Plasma Cell ; genetics ; Male ; Middle Aged ; Sequence Deletion