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.

Malignant pleural effusion in multiple myeloma: a case report / Derrame pleural malign em mieloma múltiplo: relato de caso

Multiple myeloma (MM) is a malignant neoplasm of monoclonal plasma cells that accumulate in bone marrow (BM). Malignant pleural effusions (MPE), as part of multiple myeloma clinical presentation, are unusual. Is even more rare as the first sign of presentation, occurring in less than 1% of the cases. The most common associated immunoglobulin with malignant pleural effusions is IgA subtype (80%). This condition carry a poor prognosis. We aim to describe a refractory case of multiple myeloma with extensive disease that presented with extramedullary relapse with malignant pleural effusions , besides discussing the importance of differential diagnosis.

O mieloma múltiplo (MM) é uma neoplasia maligna de células plasmáticas monoclonais que se acumulam na medula óssea (MO). Os derrames pleurais malignos (EPM), como parte da apresentação clínica do mieloma múltiplo, são incomuns. É ainda mais raro como primeiro sinal de apresentação, ocorrendo em menos de 1% dos casos. A imunoglobulina associada mais comum a derrames pleurais malignos é o subtipo IgA (80%). Esta condição carrega um mau prognóstico. Nosso objetivo é descrever um caso refratário de mieloma múltiplo com doença extensa que apresentou recidiva extramedular com derrame pleural maligno, além de discutir a importância do diagnóstico diferencial

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.

A Case of Central Nervous System Myelomatosis with Complex Chromosome Aberrations / 대한진단검사의학회지

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.

Linfoma difuso de grandes células B com diferenciação plasmoblástica: caracterização clínica, morfológica e imunofenotípica / Diffuse large B-cell differentiation with plasmoblástica: clinical, morphological and immunophenotypic

OS linfomas B difusos de grandes células plasmobásticos (LBDGC-LP) constituem um grupo heterogêneo de doenças com curso clínico agressivo, descritos inicialmente em pacientes HIV+. O LP tem sido associado à infecção pelo Herpes vírus tipo 8 em pacientes HIV+ e esses linfornas têm sido observados em pacientes HIV(-). Várias entidades tais como plasmocitoma anaplásico (PA) e pouco diferenciado (PPD) exibem uma superposição morfológica e imuno-histoquímica com os LP, principalmente quando estão em localização extra-ossea. O objetivo deste trabalho é contribuir para o diagnóstico diferencial das neoplasias linfóides com diferenciação plasmocitoide. Foram revisados 154 casos diagnosticados como Plasmocitomas, LBDGC, LBDGC-PL e Linfomas de pequenas células com diferenciação plasmocitoíde (LPC-P) dos arquivos de um centro de referência em oncologia. Foram selecionados 34 casos com padrão plasmocitoíde / plasmoblástico, confirmados por imunoistoquímica. Foram utilizados os seguintes marcadores: CD20, VS38c, CD56, CD79a, Mib, Lana-1 (HHV-8), Kappa e Lambda. Os casos foram analisados e classificados em quatro grupos morfológicos: Plasmocitomas bem diferenciados (PBD), PPD, PAlLP, LPC-P. Os PBD tinham localização óssea predominante e em 2/3 destes havia Mieloma associado, enquanto os LP tiveram apresentação nodal e visceral em 82 por cento dos casos. A positividade para CD56 ocorreu predominantemente nos PBD (p=O,04). Em um caso de LP/PA houve positividade para CD56 forte. A sobrevida foi significativamente menor nos casos de LP/PA. Nenhum dos casos mostrou positividade para LNA-1 (HHV-8). A associação com HIV foi observada em um caso de LP. A forte expressão de CD56 nos plasmocitomas sugere que este seja útil no diagnóstico diferencial. A sobrevida foi significativamente menor nos LP. A expressão de Lana-1 (HHV-8) foi negativa em todos os casos de Linfomas e Plasmocitomas, refutando a hipótese de que alguns Linfomas Plasmoblásticos representem a variante sólida do Linfoma de Efusão Primário na nossa série.

[Primary plasma cell leukemia: effective treatment with intermediate dose melphalan]

Primary Plasma cell Leukemia [PCL] is a rare disease with no standard treatment, although, combination chemotherapy, BMT and intermediate dose melphalan have been shown to be effective, in some case reports. Patients usually present with anemia, thrombocytopenia, hypercalcemia, and renal failure. Diagnosis is confirmed by peripheral and bone marrow examination. We recently had a case in our department. A 43 years old gentleman presented with history of fatigue, weakness, weight loss, dyspnea and bone pain. Diagnosis of PCL was confirmed by PBS and bone marrow exam. The patient was treated using single intermediate dose melphalan [60 mg/m2/ IV] plus Dexamethasone with G-CSF support; after 3 weeks, complete remission was achieved. In the last visit, done 9 months after treatment, he was doing well clinically and his Lab data were normal. This case report confirmed the efficacy of intermediate dose of melphalan in the management of plasma cell leukemia

Plasma cell leukemia with pleomorphic plasma cells--a case report.

A case of plasma cell leukemia showing mostly pleomorphic plasma cells in the form of convoluted and multilobated nuclei with some having bilobed nuclei and internuclear bridges is being reported for its rarity of occurrence. Patient presented with congestive cardiac failure and features of nephropathy. There were no lytic lesions in the bone. Serum electrophoresis did not show any M-band while urine electrophoresis demonstrated Bence Jones protein confirming a light chain only type of myeloma. Patient is in remission 5 months after diagnosis. The significance of recognising such pleomorphic plasma cells is discussed.

Plasma cell leukemia masquerading as ALL-L3--a case report.

Plasma cell leukemia (PCL) is a rare type of plasma cell dyscrasia. It is diagnosed when circulating plasma cells (PC) are more than 20%. We present a case of PCL in a 62-year-old female. Peripheral smear revealed more than 80% atypical vacuolated plasma cells (Mott cells) almost mimicking Burkitt cells of Acute Lymphoid Leukemia-L3 (ALL-L3). Bone marrow aspirate revealed few mature myeloma cells for which a diagnosis of PCL was thought of. Serum electrophoresis showed a positive M-band and X-ray revealed lytic lesions over femur & pelvic bones. A final diagnosis of PCL was given.

Plasma cell leukaemia--a report of two cases.

Two cases of plasma cell leukaemia--a rare form of leukaemia are described. Both cases presented with anaemia and hepatosplenomegaly. Investigations revealed leucocytosis with increased plasma cells (> 20%). Skeletal survey revealed a few osteolytic lesions in both cases.

Leucemia plasmocitária primária de cadeias lambda: relato de um caso / Primary plasmacytic leukemia of lambda chain: report of a case

Um caso raro de leucemia plasmocitária primária com excreçäo de cadeias leves do tipo lambda é relatado como uma discrasia plasmática, caracterizada por plasmocitose no sangue e medula óssea, escassa lesäo osteolítica e quadro clínico de insuficiência cardíaca e renal. A morfologia das células apresentava diferentes estágios de diferenciaçäo e maturaçäo. Foi identificada excreçäo de proteína de Bence-Jones tipos lambda na urina e no soro. A paciente foi tratada com quimioterapia (esquema M2) e sobreviveu por 33 meses, indicando resposta a terapêutica

Primary plasma cell leukaemia.

A rare form of plasma cell dyscrasia, primary plasma cell leukemia is presented. The clinical picture resembled an acute leukaemia with a fulminant course and a rapidly fatal outcome.