Clinical Characteristics and Survival Analysis of Patients with Plasma Cell Leukemia / 中国实验血液学杂志

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.

Effect of Circulating Plasma Cells on the Prognosis of Patients with Multiple Myeloma / 中国实验血液学杂志

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.

Leucemia plasmocitária primária: relato de caso e revisão sistemática sobre seus fatores prognósticos / Primary plasma cell leukemia: a case report and systematic review of prognostic factors / Leucemia primaria de células plasmáticas: reporte de caso y revisión sistemática de sus factores pronósticos

A leucemia de células plasmocitárias (LCP) é uma neoplasia hematológica rara, de caráter agressivo, ainda sem consenso quanto ao melhor esquema terapêutico e prognóstico da doença. Alguns estudos a consideram como uma variante do mieloma múltiplo. O objetivo deste estudo foi realizar um relato de caso e desenvolver uma revisão sistemática da literatura sobre os fatores prognósticos relacionados a essa enfermidade. Esta revisão sistemática seguiu o protocolo PRISMA, utilizou o PubMED como base de dados, buscando estudos que avaliassem fatores prognósticos da LCP. Os estudos selecionados foram posteriormente avaliados quanto a sua qualidade pelo protocolo STROBE. A primeira busca identificou 260 artigos. Após aplicado critério de exclusão, dez foram selecionados. Destes, oito avaliaram fatores prognósticos clínicos e laboratoriais; cinco avaliaram, além disso, fatores citogenéticos; e um avaliou apenas a idade como fator prognóstico. A qualidade dos estudos, avaliada pelo protocolo STROBE, apresentou uma classificação média de 79,1%. Nos estudos analisados, idade > 65 ou > 60 anos, performance status ECOG > 2, hipercalcemia, aumento de lactato desidrogenase (LDH), aumento da creatinina, plaquetopenia e hipoalbuminemia foram encontrados como preditores de prognóstico ruim. Foram também demonstradas alterações citogenéticas consideradas de alto risco, como as translocações e deleções de genes. Diversas condições clínicas, laboratoriais e citogenéticas parecem estar associadas ao pior prognóstico na LCP. O conhecimento desses fatores pode interferir na prática clínica. Entretanto, ainda são necessários estudos mais robustos, multicêntricos e com maior número amostral para aprofundar o conhecimento sobre essa patologia.

Plasma Cell Leukemia (PCL) is rare and aggressive hematologic disorder, with no consensus on the best therapeutic scheme or disease prognosis. Some studies consider it to be a variant of multiple myeloma. This research sought to report a PCL case and to present a systematic literature review on its prognostic factor related. Bibliographic search followed the PRISMA protocol, and was conducted on the PubMED database to identify studies on PCL prognostic factors. Of the 260 articles identified, only ten were included after application of exclusion criteria. Of these, eight evaluated clinical and laboratorial prognostic factors; five assessed cytogenetic factors; and only one investigated age as a prognostic factor. Quality of the selected studies was evaluated by STROBE protocol, presenting a median classification of 79.1%. Age >65 or >60 years old, status performance ECOG>2, hypercalcemia, increased DHL, increased creatinine, thrombocytopenia, and hypoalbuminemia were the factors identified as predictors of a bad prognosis. Studies also showed high-risk cytogenetic abnormalities such as genetic translocation and deletions. Many clinical, laboratorial and cytogenetic conditions seem to be related to a worse PCL prognosis. Since knowledge of these factor can interfere in the clinical practice, more robust studies are needed on this pathology.

La leucemia de células plasmáticas (LCP) es una neoplasia hematológica rara, de carácter agresivo, sin consenso sobre el mejor régimen terapéutico y pronóstico de la enfermedad. Algunos estudios la consideran como una variante del mieloma múltiple. El objetivo de este estudio fue realizar un reporte de caso y desarrollar una revisión sistemática de la literatura sobre los factores pronósticos relacionados con esta enfermedad. Esta revisión sistemática siguió el protocolo PRISMA, utilizó PubMED como base de datos para la búsqueda de estudios que evaluaran los factores pronósticos para LCP. Los estudios seleccionados fueron posteriormente evaluados por su calidad mediante el protocolo STROBE. La primera búsqueda identificó 260 artículos. Después de aplicados los criterios de exclusión, se seleccionaron diez. De estos, ocho evaluaron los factores pronósticos clínicos y de laboratorio, cinco evaluaron también los factores citogenéticos y uno evaluó la edad solo como factor pronóstico. La calidad de los estudios, evaluada por el protocolo STROBE, presentó una calificación promedio del 79,1%. En los estudios analizados, la edad > 65 o > 60 años, el estado funcional ECOG > 2, la hipercalcemia, el aumento de lactato deshidrogenasa (LDH), el aumento de creatinina, la trombocitopenia y la hipoalbuminemia fueron los predictores de mal pronóstico. También se demostraron alteraciones citogenéticas que se consideraron de alto riesgo, como translocaciones y deleciones de genes. Varias condiciones clínicas, de laboratorio y citogenéticas parecen estar asociadas con peor pronóstico en LCP. El conocimiento de estos factores puede interferir en la práctica clínica. Sin embargo, aún se necesitan estudios más robustos, multicéntricos y con mayor tamaño muestral para profundizar en el conocimiento sobre esta patología.

Screening and predication on tumor neoantigen for primary plasma cell leukemia / 中国肿瘤生物治疗杂志

@#Objective:To investigate the tumor-specific neoantigen for primary plasma cell leukemia (PCL) using gene sequencing technology combined with bioinformatic analysis. Methods: Peripheral blood samples of one patient with primary PCL during relapse and remission periods were collected. HLA molecular typing was performed using polymerase chain reaction with sequencing-based typing; whole-exome and transcriptome were sequenced by next-generation sequencing method; and bioinformatics software NetMHCpan was used to predict neoantigens. Results: Six tumor-specific missense mutations were found in the patient's peripheral blood during relapse period, located in genes FRG1, MLL3, SVIL, MYOM1, ZDHHC11 and RFPL4A.Considering patient's HLA sub-types, 43 neoantigens were predicted via bioinformatics. Considering that FRG1 and MLL3 had relatively high gene expression levels, 20 neoantigens derived from mutations of the two genes were preferentially selected, among which four neoantigens had high affinity with the patient's HLA molecules and thus had potential clinical application value. Conclusion: The study has completed a tumor neoantigen screen and prediction for primary PCL. This practice demonstrates that predicting neoantigen based on tumor-specific somatic mutation is feasible for primary PCL.

Diagnostic and differential diagnostic of primary plasma cell leukemia and lymphoma with increased plasma cell / 临床与实验病理学杂志

Purpose To investigate the diagnosis,differential diagnosis and clinical manifestation of primary plasma cell leukemia (PPCL) and lymphoma with increased plasma cell.Methods Through clinical data and cell morphology,flow cytometry (FCM),immunofixation electrophoresis and immunohistochemistry of EliVision two-step examination were used to analyze 7 cases of PPCL and 3 cases of lymphoma with increased plasma cell.Results All patients with PPCL and lymphoma with increased plasma cell presented with anemia,thrombocytopenia,fever,liver and spleen and lymph node swelling.The proportion of plasma cells in peripheral blood morphology were larger than 20％,accompanied by morphological abnormality.FCM of peripheral blood showed all 7 cases of PPCL expressed CD38 and CD138,CD56 expression in the 2 cases and CD20 in the 2 cases.The light chain (Lamda,Kappa) showed a monoclonal restricted expression,which was consistent with the diagnosis of PPCL.CD19 and CD45 were weakly positive in 3 cases of lymphoma with increased plasma cell,CD38 and CD138 were positive,and no restricted expression was found in light chain IgL,wich belonging to the immunophenotypes of normal plasma cells.Of 3 cases of light chain (Ig) without restrictive expression,2 of them were angioimmunoblastic T-cell lymphoma (ATCL) and 1 case was CD30-positive sinusoidal large B-cell lymphoma (CD30 + SLBCL) that confirmed by lymph node biopsy and pathological examination.Conclusion The PPCL and lymphoma with increased plasma cell have the same clinical manifestations and similar morphological characteristics of blood cells.The diagnosis of PPCL should be combined with immunoelectrophoresis and FCM,and the diagnosis of lymphoma with increased plasma cell needs to be confirmed by histological examination of lymph nodes.

Serum M protein and clinical efficacy of Bortezomib in secondary plasma cell leukemia / 中国生化药物杂志

Objective To evaluate the effect of Bortezomib in the treatment of secondary plasma cell leukemia in serum M protein and clinical efficacy.Methods20 cases of patients with secondary plasma cell leukemia were randomly divided into the control group and treatment group, 9 cases in the control group,11 cases in the treatment group.The control group was given Prednisolone acetate tablets 60mg/(m2·d),oral, d1~d4 + Melphalan tablets, 8mg/(m2·d),oral, d1~d4+Thalidomide 100~200mg/d, oral, bedtime;on the basis of the control group, the experimental group was treated with Bortezomib for Injection, 1.3mg/(m2·d), intravenous injection, d1、d4、d8、d11.Before and after treatment, compared between the two groups of patients with the ratio of bone marrow plasma cells, serum monoclonicity Immunoglobulin A (IgA), monoclonicity immunoglobulin G (IgG) levels, efficiency of treatment and safety.ResultsAfter treatment, compared with the control group,the plasma cell ratio, the serum levels of IgA and IgG were lower in the experimental group (P<0.05);the treatment efficiency of the experimental group (54.55%) was significantly higher than that of the control group (11.11%), the results were statistically significant.ConclusionThe Bortezomib can significantly reduce the ratio of bone marrow plasma cells and the serum levels of monoclonicity IgA、IgG in patients with secondary plasma cell leukemia, improve clinical efficacy, and the safety was high.

Primary plasma cell leukemia: A report of two cases of a rare and aggressive variant of plasma cell myeloma with the review of literature.

Plasma cell leukemia (PCL) is a rare and aggressive variant of myeloma accounting for 2-3% of all plasma cell dyscrasias characterized by the presence of circulating plasma cells. The diagnosis is based on the % (≥20%) and absolute number (≥2x109/L) of plasma cells in the peripheral blood. The incidence of primary PCL (pPCL) is very rare and reported to occur in <1 in a million. It is classified as either pPCL occurring at diagnosis or as secondary PCL in patients with relapsed/refractory myeloma. pPCL is a distinct clinicopathological entity with different cytogenetic and molecular findings. The clinical course is aggressive with short remissions and survival duration. We report two cases of pPCL, both having acute onset of illness, varied clinical presentation with one of them showing “hairy cell morphology,” with rapidly progressing renal failure, and was not suspected to be plasma cell dyscrasia clinically. A detailed hematopathological evaluation clinched the diagnosis in this case. It is recommended that techniques such as immunophenotyping by flow cytometry and protein electrophoresis must be performed for confirmatory diagnosis. A detailed report of two cases and a review of PCL are presented here.

Plasma cell leukemia in North India: retrospective analysis of a distinct clinicohematological entity from a tertiary care center and review of literature

BACKGROUND: Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm. In PCL, clonal plasma cells comprise ≥20% of the peripheral blood (PB) leukocytes and/or the absolute clonal PB plasma cell count is ≥2×10(9)/L. Primary PCL (PPCL) originates de novo, whereas, secondary PCL (SPCL) evolves from pre-existing multiple myeloma. METHODS: Clinicohematological features, immunophenotypic profile, and survival of PCL patients were analyzed retrospectively. RESULTS: Between January 2007 and December 2014, ten PPCL and four SPCL patients were investigated (8 PPCLs and 3 SPCLs had complete clinical data). All were North Indians, sharing common geography and ethnicity. Our cohort showed less frequent renal failure, more frequent hepatomegaly, and non-secretory type disease. In contrast to western literature, flow cytometric immunophenotyping of our cohort revealed altered expression of CD138 (67%), CD56 (33%), and CD20 (0%). With novel therapeutic agents, these PPCL patients had a median overall survival of 15 months. CONCLUSION: We highlight that our PPCL patients from North India had distinct clinicohematological and immunophenotypic profiles. The significance of our findings must be tested in a larger patient cohort and must be supported by molecular and cytogenetic investigations to unmask possible significant effects on pathogenesis.

Primary plasma cell leukemia- A case report.

Plasma cell leukemia is a rare variant of multiple myeloma and this term is applied when the absolute plasma cell count is greater than 2x10⁹ /L or the relative plasma cell number is greater than 20% of the peripheral white blood cells. PCL may be primary or secondary. A 55 year old male presented to our outpatient department with complaints of lower back pain for four months, low grade fever off and on for two months, generalised weakness and loss of appetite for 20 days without prior history of multiple myeloma. His peripheral blood smear showed 26% plasmacytoid cells and bone marrow revealed predominantly plasma cell population (80%) with binucleate and trinucleate forms. Chemotherapy was started but unfortunately patient had expired. This case is presented due to its rare occurrence, presence of organomegaly and poor outcome.

Primary plasma cell leukemia in 32 years old male A case report.

Plasma cell leukemia (PCL) is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. Histogenetically, plasma cell leukemia is derived from terminally differentiated B cells. It is diagnosed by presence of absolute plasma cell count >2000 per cm or >20% circulating plasma cells. Here we report a case of plasma cell leukemia, who presented with easy fatigability, weakness and high grade fever since 1 month. Hematological investigation revealed leukocytosis with plasmacytosis (7420/ mm3). On bone marrow examination, >45% plasma blasts were seen. Biochemical analysis showed high LDH level (4236 U/L) and serum calcium level was also raised (12.3 mg/dl). Final diagnosis of plasma cell leukemia was made. As PCL is rare disease and it is even rarer to find them in a 32 years old. Here we are able to find and document the typical features of PCL.

Plasma cell leukemia - A comprehensive analysis of clinical & pathological features of 7 cases.

Background: Plasma cell leukemia (PCL) is a rare, yet aggressive plasma cell (PC) neoplasm, variant of multiple myeloma (MM), characterized by high levels of PCs circulating in the peripheral blood. PCL can either originate de novo (primary PCL) or as a secondary leukemic transformation of MM (secondary PCL) and is characterized by circulating PCs >2×109/L in peripheral blood and a peripheral blood plasmacytosis >20%. Aims & Objective: Present study was undertaken to analyze the main clinical & pathological features of PCL. For diagnostic purpose the morphological appearances and confirmation by immunophenotyping are emphasized rather than more sophisticated testing methods that may not be widely available. Material and Methods: A descriptive study was carried out in the department of Pathology, in a tertiary care teaching hospital, Ahmedabad, India during year 2009-2013. We investigated the important clinical characteristics, pathological, biochemical & radiological features, immunophenotype, & prognostic factors of 7 patients of PCL. Results: Common clinical features at diagnosis were anaemia, renal insufficiency, bone pain, splenomegaly or hepatomegaly. Anaemia, leucocytosis, thrombocytopenia & plasmacytosis were seen in peripheral blood. Plasma cell marker - CD 38 & CD 138 were expressed in all cases. Serum β2-microglobulin, serum LDH were increased & serum albumin was decreased in all 7 cases & were associated with poor prognosis. The median survival time from diagnosis was 9 months. Conclusion: Plasma cells have characteristic morphological features which can be easily identified on peripheral blood & bone marrow examination.CD 38 & CD 138 are excellent plasma cell markers. Increased serum β2-microglobulin & serum LDH & decreased serum albumin are potent poor prognosis factors. PCL is aggressive neoplasm with poor response to chemotherapy & low median survival time from diagnosis.

Plasma cell leukemia: A retrospective study of 10 cases.

BACKGROUND: Plasma cell leukemia (PCL) is a rare aggressive variant of multiple myeloma (MM) characterized by a fulminant course and poor prognosis. Flow cytometry (FCM) is very useful in the diagnosis of the plasma cell leukemia. Herein, we present 10 cases of PCL. MATERIALS AND METHODS: We retrospectively studied immunophenotypic profile of 10 cases of PCL from Jan 2009 to Dec 2013 using 5 parameters, 6 color flow cytometric analysis. We also studied their clinical presentation and other laboratory findings. RESULTS: Common clinical features at presentation were weakness, bone pain, anemia, thrombocytopenia and osteolytic lesions. Plasma cell population were identified by strong expression of CD38 and co‑expression of CD38 and CD138. CD56 was expressed in 20% cases. CD19 and CD117 were negative in all cases. CONCLUSIONS: Immunophenotyping is highly useful to differentiate PCL from other chronic lymphoproliferative disorders with plasmacytoid morphology as well as from non‑neoplastic reactive plasma cells. Co‑expression of CD38 and CD138 is a best combination to identify the plasma cells by using FCM.

Diagnosis and treatment of primary plasma cell leukemia / 肿瘤

Primary plasma cell leukemia (PPCL) is a rare, highly aggressive plasma cell disease characterized with a poor prognosis and a low response rate to conventional therapy. It occurs de novo in the absence of a prior history of plasma cell myeloma. Recently, the use of novel drugs such as bortezomib and the approaches such as stem cell transplantation may improve the outcomes of PPCL. This review summarizes the diagnosis and treatment of PPCL in order to provide reference information for clinical practice. Copyright© 2012 by TUMOR.

Immunophenotypic profile of plasma cell leukemia: A retrospective study in a reference cancer center in India and review of literature.

Background: Plasma cell leukemia (PCL) is a rare but aggressive subtype of plasma cell dyscrasia. It is known to present with highly variable morphological features and may mimic with other lymphoid neoplasms. Multicolor flow cytometry (MFC) with availability of newer markers is highly useful in the diagnosis of the plasma cell leukemia. We present an immunophenotypic profile in ten cases of PCL along with their clinical and laboratory findings. Materials and Methods: We retrospectively studied immunophenotypic profile of 10 cases of plasma cell leukemia (out of 4615 cases of hematolymphoid neoplasms) using five parameter, three color flow cytometric analysis. We also studied their clinical presentation and other laboratory findings. Results: Common clinical features at presentation were weakness, bone pain, anemia, thrombocytopenia and osteolytic lesions. Plasma cell population was identified on strong expression of CD38 and co-expression of CD38 and CD138. CD56 was expressed in 44% cases. CD19 and CD20 were negative in all cases. Surface light chain restriction was seen in 50% cases and in remaining 50% cases revealed cytoplasmic light chain restriction. CD117 was expressed in one out of two cases studied. Conclusions: MFC immunophenotyping is highly useful to differentiate Plasma cell leukemia from other chronic lymphoproliferative disorders with plasmacytoid morphology as well as from non-neoplastic reactive PC and co-expression of CD38 and CD138 is a best combination to identify the plasma cells by MFC.

Immunophenotypic profile of plasma cell leukemia: A retrospective study in a reference cancer center in India and review of literature.

Background: Plasma cell leukemia (PCL) is a rare but aggressive subtype of plasma cell dyscrasia. It is known to present with highly variable morphological features and may mimic with other lymphoid neoplasms. Multicolor flow cytometry (MFC) with availability of newer markers is highly useful in the diagnosis of the plasma cell leukemia. We present an immunophenotypic profile in ten cases of PCL along with their clinical and laboratory findings. Materials and Methods: We retrospectively studied immunophenotypic profile of 10 cases of plasma cell leukemia (out of 4615 cases of hematolymphoid neoplasms) using five parameter, three color flow cytometric analysis. We also studied their clinical presentation and other laboratory findings. Results: Common clinical features at presentation were weakness, bone pain, anemia, thrombocytopenia and osteolytic lesions. Plasma cell population was identified on strong expression of CD38 and co-expression of CD38 and CD138. CD56 was expressed in 44% cases. CD19 and CD20 were negative in all cases. Surface light chain restriction was seen in 50% cases and in remaining 50% cases revealed cytoplasmic light chain restriction. CD117 was expressed in one out of two cases studied. Conclusions: MFC immunophenotyping is highly useful to differentiate Plasma cell leukemia from other chronic lymphoproliferative disorders with plasmacytoid morphology as well as from non-neoplastic reactive PC and co-expression of CD38 and CD138 is a best combination to identify the plasma cells by MFC.

A rare case of plasma cell leukemia in a 35 year old.

Plasma cell leukemia is a rare, aggressive form of multiple myeloma. A 35-year-old male presented with backache, generalized weakness, and facial puffiness. His complete blood count showed anemia and a high WBC count with atypical cells on peripheral smear. Bone marrow examination showed more than 90% of atypical plasma cells, confirming a diagnosis of plasma cell leukemia. Patient also had azotemia, hypercalcemia, and hyperuricemia. The patient was started on chemotherapy along with supportive care. Patient improved dramatically and he was discharged on regular follow-up.

Nervous system relapse in primary plasma cell leukemia / 基础医学与临床

Objective To improve the anderstanding of clinical profile of primary plasma cell leukemia.Methods Case report and literature review.Results A rare case of nervous system relapse in primary plasma cell leukemia was reported.Six patients were identified from the literature.The type of immunoglobulin included IgG(3 patients),IgD(2 patients).Clinical manifestations of nervous system were variable.The average interval from initial diagnosis to the development of nervous system relapse was 16.5 months.Plasma cells were found in cerebrospinal fluid in 4 patients.The mean surviaval time was 6.7 months after nervous system relapse.Conlusion Nervous system relapse in primary plasma cell leukemia is rare with poor prognosis.

A Case with Plasma Cell Leukemia and Anaplastic Large T cell Lymphoma / 대한혈액학회지

The association of B-cell and T-cell disorders is rare. We report a case with coexisting plasma cell leukemia and anaplastic large T cell lymphoma. It is believed to be the first case reported in the literature. A 49-year man was admitted to the hospital because of indigestion. On the gastrofibroscopic examination, a huge, ulcerofungating mass was detected in the stomach, and Ki-1 positive anaplastic large T-cell lymphoma was diagnosed on the biopsy. On the peripheral blood smear and bone marrow study performed as a part of the staging work-up, plasma cell leukemia was diagnosed. The protein electrophoresis and immunofixation of serum and urine revealed IgG, lamda type monoclonal gammopathy. But in the pleural effusion, the involvement of anaplastic large T-cell lymphoma was noted.

1 Case of Primary Plasma Cell Leukemia associated with Esophageal Cancer / 대한임상병리학회지

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.

A Case of Non-secretory Plasma Cell Leukemia with Complex Chromosomal Abnormalities / 대한혈액학회지

We report a case of non-secretory plasma cell leukemia with complex chromosomal abnormalities including t (11;14)(q13;q32). A 57-year-old man was admitted to hospital due to anemia, thrombocytopenia and renal insufficiency. Bone marrow examination and peripheral blood smear revealed a large number of immature plasma cells with positivity for CD38. Monoclonal gammopathy or abnormal paraproteins were not observed in serum protein electrophoresis and immunofixation. The cytogenetic analysis showed complex chromosomal abnormalities [45, XY, -1, t (11;14)(q13;q32), t (12;17)(p13;q21)]. He was died of adult respiratory distress syndrome on the 6th hospital day.