OBJECTIVE: to analyze the effect of circulating plasma cells(CPC) on the prognosis of patients with multiple myeloma(MM) in the era of new drugs, and to explore the new definition standard of primary plasma cell leukemia(pPCL). METHODS: The clinical data of 321 patients with newly diagnosed MM and 21 patients with pPCL admitted to our hospital from January 2014 to May 2022 were retrospectively analyzed. According to the proportion of CPC in peripheral blood smears, all patients were divided into 4 groups: CPC 0% group(211 cases), CPC 1%-4% group(69 cases), CPC 5%-19% group(41 cases) and CPC≥20% group(21 cases). The clinical features of patients in each group were compared and the prognosis fators was analyzed. RESULTS: The median OS of the four groups were 44.5,21.3,24.6 and 12.8 months, respectively. Among them, 295 patients(86.3%) were treated with new drugs, and the median OS of the four groups were not reached, 26.7, 24.6 and 14.9 months, respectively. As the survival curves of CPC 5%-19% group and CPC≥20% group were similar, the patients were divided into CPC<5% group and CPC≥5% group, the median OS of CPC<5% group was better than that in CPC≥5% (43.5 vs 22.3 months, P<0.001). In addition, the median OS of patients in the CPC 1%-4% group was also significantly lower than that in the CPC 0% group and similar to that in the CPC≥5% group. Multivariate analysis showed that 1%-4% CPC was an independent risk factor for the OS of patients with CPC<5%. The patients with CPC<5% were stratified by R-ISS staging, and the OS of R-ISS stage Ⅰ or stage Ⅱ with 1%-4% CPC was similar to that of R-ISS stage Ⅲ. The newly defined pPCL patients showed increased tumor load and obvious invasive characteristics. Multivariate analysis showed no independent prognostic factors for pPCL, and high-risk cytogenetic abnormalities(HRCA) had no significant effect on the prognosis. CONCLUSION The validity of IMWG's new pPCL definition standard was verified, and it was found that the survival of MM with 1%-4% CPC also is poor and the prognosis is very close to pPCL. In addition, the newly defined pPCL has unique clinical and biological characteristics.
Humans ; Multiple Myeloma/pathology* ; Plasma Cells/pathology* ; Retrospective Studies ; Prognosis ; Leukemia, Plasma Cell/diagnosis*

OBJECTIVE: To investigate the clinical characteristics, therapeutic response and prognosis of patients with plasma cell leukemia (PCL) and improve the understanding of this disease. METHODS: The clinical manifestations, laboratory tests and treatment response of 27 patients with plasma cell leukemia treated in The Second Hospital of Shanxi Medical University from December 2010 to August 2019 were analyzed retrospectively, and their clinical characteristics were summarized. Kaplan-Meier method was used for survival analysis. RESULTS: There were 18 cases of primary plasma cell leukemia (pPCL) and 9 cases of secondary plasma cell leukemia (sPCL). The male to female ratio was 1.7∶1. The median age was 62 years old. The first manifestations were bone pain, fatigue, fever, splenomegaly and bleeding, and a large number of plasma cell infiltration was observed in the morphological examination of peripheral blood and bone marrow cells. 13 cases were detected by immunotyping and all of them expressed CD38/CD138. 8 cases underwent karyotype analysis, and 3 cases were normal, clonal abnormalities occurred in 5 cases. FISH detection was performed in 12 cases, of which 8 cases were abnormal. In 17 cases of bortezomib based chemotherapy, the ovevall response rate was 52.9%, which was higher than that in the non-bortezomib group, but there was no significant difference between the two groups (P =0.242). The overall median survival time of 27 patients was 6.4 months, the median progression-free survival time was 3.5 months, and the median survival time of patients with pPCL and sPCL was 8.2 months and 2.4 months, respectively, the difference between the two groups was statistically significant (P =0.031). CONCLUSION PCL is highly invasive and has diverse clinical manifestations, and is not sensitive to traditional chemotherapy. The median survival time of patients with pPCL is relatively longer than that of patients with sPCL. The chemotherapy regimen based on bortezomib improves the treatment effectiveness and prolongs the survival time of PCL patients.
Male ; Female ; Humans ; Leukemia, Plasma Cell/diagnosis* ; Retrospective Studies ; Bortezomib/therapeutic use* ; Prognosis ; Survival Analysis

He died after 2 months from diagnosis due to massive bleeding in esophageal lesion with complication. He died after 2 months from diagnosis due to massive bleeding in esophageal lesion with complication. We report one case of plasma cell leukemia associated with esophageal cancer. A 71-year-old man was admitted due to dysphagia and diagnosed as undifferentiated squamous cell cancer based on esophagogram and biopsy. In peripheral blood smear, large parcent of plasma cell like cells are found, so bone marrow examination was done and 52.5% of plasma cells are found with unusual morphology such as convoluted, multilobulated nuclei. Immunochemical stain and immunophenotypic features of these cells were suggestive of plasma cell origin with positivity for methylgreen pyronin positivity and CD38, CD56 positivity. Serum rotein electrophoresis and immunoelectrophoresis showed monoclonal gammopathy of Ig G ,k type. This patient had no history of previous multiple myeloma or other maligancy. He died after 2 months from diagnosis due to massive bleeding in esophageal lesion with complication.
Aged ; Biopsy ; Bone Marrow Examination ; Deglutition Disorders ; Diagnosis ; Electrophoresis ; Esophageal Neoplasms* ; Hemorrhage ; Humans ; Immunoelectrophoresis ; Leukemia, Plasma Cell* ; Multiple Myeloma ; Neoplasms, Squamous Cell ; Paraproteinemias ; Plasma Cells* ; Plasma*

A case of plasmacytoma of the ovary and breast, which developed in a patient with a solitary plasmacytoma in the lumbar vertebra for nine months, was diagnosed cytologically and histologically. Enlargement of the right ovary and multiple palpable masses in the right and left breast were already present at six months after the diagnosis of vertebral solitary plasmacytoma. At eight months, plasma cell leukemia developed, and nine months the enlarged both ovaries, replaced by yellowish-gray solid tumors showed infiltration of immature plasma cells. The cytologic features of the ovarian tumors were same with those of the breast tumor. The tumor cells were of predominantly immature plasma cells with one or more nuclei. Some mature plasma cell had an eccentric nucleus with single nucleolus and peripherally clumped chromatin. Binucleated or multinucleated giant cells were often present. Histologically, sheets of poorly differentiated plasmacytoid tumor cells were separated by strands of hyaline fibrous tissue. On immunohistochemical stains, the tumor cells showed strong reactivity for lambda-light chain but no reaction for kappa-light chain, cytokeratin, or leukocyte common antigen.
Antigens, CD45 ; Breast Neoplasms ; Breast* ; Chromatin ; Coloring Agents ; Diagnosis ; Female ; Giant Cells ; Humans ; Hyalin ; Keratins ; Leukemia, Plasma Cell ; Ovary* ; Plasma Cells ; Plasmacytoma* ; Spine*

BACKGROUND: Mutation in the p53 gene occurs frequently in a wide spectrum of human malignancies and the mutant protein may prove to be a useful diagnostic or prognostic marker for acute leukemia. The aim of this study was evaluation of the clinical significance of plasma p53 levels in acute leukemia. METHODS: Plasma p53 protein were measured by an enzyme linked immunosorbent assay in 136 acute leukemic patients and the results were compared with immunohistochemistry on the paraffin-embedded section in 78 cases. In 28 leukemia cases showing increased plasma p53 protein levels at diagnosis, follow-up plasma p53 levels were analysed and compared with morphologic findings of the bone marrow at the same time. RESULTS: (1) In immunohistochemistry, 22 of 78 cases (28%) revealed positive results for p53 protein. (2) Initially positive plasma p53 protein levels were detected in 40 out of 136 (29.4%). There was a moderate agreement (kappa factors 0.3-0.4) between the immunohistochemistry and ELISA results. All 13 cases in which initial positive p53 results were converted to negative at follow-up analysis revealed complete remission. Positive plasma p53 protein levels were converted to negative before the follow-up bone marrow examination in all six cases (acute myeoid leukemia 4 cases, acute lymphocytic leukemia 2 cases) which were analysed serially. (3) The poor prognostic factors reveal no statistically significant difference between the positive and negative plasma p53 groups. CONCLUSION: The results suggest that analysis of plasma p53 level in acute leukemia is a useful test to predict complete remission in addition to the bone marrow examination. Especially, we may expect that all the acute leukemia cases showing positive plasma p53 level at diagnosis and converted to negative level during chemotherapy will enter complete remission.
Bone Marrow ; Bone Marrow Examination ; Diagnosis ; Drug Therapy ; Enzyme-Linked Immunosorbent Assay ; Follow-Up Studies ; Genes, p53 ; Humans ; Immunohistochemistry ; Leukemia* ; Mutant Proteins ; Plasma* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma

Chronic lymphocytic leukemia (CLL) and multiple myeloma (MM) are both lymphoproliferative disease occurring in different stages of B cell oncogeny. An increased incidence of secondary malignancies in patients with CLL is well recognized, however, the coexistence of both disorders in the same patient was very rare. Furthermore, clonal relationship between these diseases has not been clearly established. We report the occurrence of MM during the course of CLL. A 68-year-old patient was presented with general weakness and bone marrow aspiration showed a hypercellular marrow with 80% mature lymphocytes. At 5 months after diagnosis of CLL, bone marrow of the patient showed increased immature plasma cells. Serum protein electrophoresis showed monoclonal gammopathy and serum immunoelectrophoresis IgG kappa type monoclonality. The patient received six cycles of VAD (vincristine, adriamycin, dexamethasone) chemotherapy, but died of pneumonia and sepsis.
Aged ; Bone Marrow ; Diagnosis ; Doxorubicin ; Drug Therapy ; Electrophoresis ; Humans ; Immunoelectrophoresis ; Immunoglobulin G ; Incidence ; Leukemia, Lymphocytic, Chronic, B-Cell* ; Lymphocytes ; Multiple Myeloma* ; Paraproteinemias ; Plasma Cells ; Pneumonia ; Sepsis

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

Although neutrophilia can manifest from various causes, it is important to be able to distinguish chronic neutrophilic leukemia (CNL) from neutrophilic leukemoid reactions (NLR). In this paper, we describe four cases of leukocytosis with neutrophilia, including one case of CNL with a T618I mutation in colony stimulating factor 3 receptor (CSF3R) and three cases of NLR associated with malignancy or sepsis, which were initially suspected as CNL. Of the three NLR cases, one was associated with ovarian cancer, one with monoclonal gammopathy of undetermined significance and one with multiple myeloma with sepsis. This study demonstrated that confirming the clonality of myeloid cells with CSF3R T618I could contribute to making an accurate differential diagnosis between CNL and NLR in patients with solid cancers or plasma cell neoplasms caused by paraneoplastic syndromes and/or infection.
Colony-Stimulating Factors ; Diagnosis, Differential ; Humans ; Leukemia, Neutrophilic, Chronic* ; Leukemoid Reaction* ; Leukocytosis ; Monoclonal Gammopathy of Undetermined Significance ; Multiple Myeloma ; Myeloid Cells ; Neoplasms, Plasma Cell ; Neutrophils* ; Ovarian Neoplasms ; Paraneoplastic Syndromes ; Sepsis

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