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primary plasma cell leukemia(A COMPREHENSIVE ANALYSIS OF 44 CASES)

Chinese Journal of Cancer Research 1991年第2期3卷 74-77页

作者：蔡则骥张国祯 Zhongshan Hospital Shanghai Medical Vniversity Zhongshan Hospital Shanghai Medical Vniversityour cases of primary plasma cell leukemia (PPCL) admitted to Zhongshan Hospital from 1959 to 1987 are reported with a review on additional 40 cases reported in China. Comparing with the 57 cases of multiple myeloma (MM) treated in our hospital the following features were observed in PPCL: (1) The age was younger with a mean of 45.2 years 34.1% of the patients were under 40 years. (2) Onset was abrupt. Duration from onset to diagnosis was 2 months or less in 77% patients but never beyond 6 months. (3) 81.8% patients had liver enlargement 59.1% splenomegaly and 61.4% sternum tenderness. (4) All patients showed marked anemia with an average hemoglobin of 65 g/L. BPC count was less than 100 × 109/L in 76% patients and WBC was more than 10×109/L in 77%. (5) Plasma cell number in the marrow was markedly increased with an average of 69% of which the blast cells and immature forms were predominant. (6) No destruction of bones was shown on X-ray film in 68.3% patients. (7) The response to chemotherapy was poor with a total response rate of 18% and a mean survival of 2 months. All the above-mentioned clinical features were significantly different from those of MM. In addition these two diseases were also different in cytology cytogenetics and ultrastructure. Therefore PPCL should be considered as a special type of acute leukemia distinct from MM. High dose of alkylating agents in combination with autologous bone marrow transplantation might improve the prognoses.

Four cases of primary plasma cell leukemia (PPCL) admitted to Zhongshan Hospital from 1959 to 1987 are reported with a review on additional 40 cases reported in China. Comparing with the 57 cases of multiple myeloma (MM) treated in our hospital, the following features were observed in PPCL: (1) The age was younger, with a mean of 45.2 years, 34.1% of the patients were under 40 years. (2) Onset was abrupt. Duration from onset to diagnosis was 2 months or less in 77% patients but never beyond 6 months. (3) 81.8% patients had liver enlargement, 59.1% splenomegaly and 61.4% sternum tenderness. (4) All patients showed marked anemia with an average hemoglobin of 65 g/L. BPC count was less than 100 × 109/L in 76% patients and WBC was more than 10×109/L in 77%. (5) plasma cell number in the marrow was markedly increased with an average of 69%, of which the blast cells and immature forms were predominant. (6) No destruction of bones was shown on X-ray film in 68.3% patients. (7) The response to chemotherapy was poor with a total response rate of 18% and a mean survival of 2 months. All the above-mentioned clinical features were significantly different from those of MM. In addition, these two diseases were also different in cytology, cytogenetics and ultrastructure. Therefore, PPCL should be considered as a special type of acute leukemia distinct from MM. High dose of alkylating agents in combination with autologous bone marrow transplantation might improve the prognoses.

关键词： PPCL primary plasma cell leukemia A COMPREHENSIVE ANALYSIS OF 44 CASES

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Venetoclax in combination with chidamide and dexamethasone in relapsed/refractory primary plasma cell leukemia without t(11;14):A case report

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World Journal of Clinical Cases 2021年第5期9卷 1175-1183页

作者： Yang Yang Li-Juan Fu Chun-Mei Chen Mei-Wei Hu Department of Hematology The Second Affiliated Hospital of Zhejiang Chinese Medical UniversityHangzhou 310014Zhejiang ProvinceChina

BACKGROUND Conventional therapies for primary plasma cell leukemia(pPCL)are usually ineffective,with a short remission time with the use of multiple myeloma medications,showing aggressiveness of pPCL.B-cell lymphoma-2 inhibitor venetoclax is usually used for relapsed/refractory multiple myeloma(RRMM)with t(11;14).There are very few studies published on the use of venetoclax in pPCL without t(11;14).Similarly,histone deacetylase inhibitors are considered effective for the treatment of RRMM,but there are no reports on their use in *** SUMMARY A 57-year-old woman with severe anemia,thrombocytopenia,multiple bone destruction,impaired renal function,and 42.7%of peripheral plasma cells is *** multiple chemotherapy regimens and chimeric antigen receptor Tcell treatment,the disease progressed *** patient had very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone *** The success of venetoclax-chidamide-dexamethasone combination therapy in achieving a very good partial response suggested that it can be used for refractory/relapsed pPCL patients who have been exhausted with the use of various drug combinations and had poor survival outcomes.

关键词： Relapsed/refractory primary plasma cell leukemia Venetoclax Chidamide Very good partial response Case report

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Haploidentical stem cell transplantation used in treating primary plasma cell leukemia: a case report

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Journal of Medical Colleges of PLA(China) 2010年第1期25卷 54-57页

作者： Ouyang Guifang Zhu Huiling Hong Yongwei Xu Kaihong Mu Qitian Le Jing Wu Wenmiao Lu Ying Gu Xuewei Ni Lifeng Department of Hematology the First People's Hospital of Ningbo Affiliated Medical College of Ningbo University Ningbo 315010 China

Here we report a successful protocol in treatment of a patient with primary plasma cell leukemia (PPCL) using haploidentical stem cell transplantation (hi-HSCT). During first complete remission after routine chemotherapy, the patient received autologous blood stem cell transplantation, but he had relapse later. He gained a second CR after chemotherapy and underwent hi-HSCT from his daughter, who had HLA mismatched at three loci. Recovery of hemopoiesis was found at day 14 and complete donor chimerism was confirmed by PCR-STR on day 34, 95 and 238. The patients have survived disease-free for 56 months since hi-HSCT, without serious graft-versus-host-disease.

关键词： primary plasma cell leukemia Stem cell transplantation Haploidentical

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