Primary plasma cell leukemia in Latin America: demographic, clinical, and prognostic characteristics. A study of GELAMM group.

Primary plasma cell leukemia (pPCL) is an infrequent and aggressive plasma cell disorder. The prognosis is still very poor, and the optimal treatment remains to be established. A retrospective, multicentric, international observational study was performed. Patients from 9 countries of Latin America (LATAM) with a diagnosis of pPCL between 2012 and 2020 were included. 72 patients were included. Treatment was based on thalidomide in 15%, proteasome inhibitors (PI)-based triplets in 38% and chemotherapy plus IMIDs and/or PI in 29%. The mortality rate at 3 months was 30%. The median overall survival (OS) was 18 months. In the multivariate analysis, frontline PI-based triplets, chemotherapy plus IMIDs and/or PI therapy, and maintenance were independent factors of better OS. In conclusion, the OS of pPCL is still poor in LATAM, with high early mortality. PI triplets, chemotherapy plus IMIDs, and/or PI and maintenance therapy were associated with improved survival.

[Prevalence investigation of plasma cell leukemia in China: a calculation based on national urban medical insurance in 2016].

Objective: To analyze the epidemiological features of patients with plasma cell leukemia (PCL) and calculate the prevalence of PCL in urban China in 2016. Methods: Calculation in this study was based on China's urban basic medical insurance from 23 provinces between January 1, 2016 and December 31, 2016. The identification of the patients with PCL was based on the disease names and codes in the claim data. Subgroup analyses were carried out by sex, region, and age. To test the robustness of the results, we performed sensitivity analyses. Age-adjusted prevalence was calculated, based on the 2010 Chinese census data. Results: The prevalence of PCL in urban China in 2016 was 0.11 per 100 000 population (95% CI 0.05-0.19) , and the male prevalence and female prevalence were 0.12 per 100 000 population (95% CI 0.06-0.21) and 0.10 per 100 000 population (95% CI 0.04-0.19) , respectively. The prevalence of PCL peaked at 70-79 years old. Sensitivity analyses proved the robustness of the primary result. The age-adjusted prevalence based on 2010 Chinese census data was 0.12 per 100 000 population (95% CI 0.11-0.13) . Conclusion: This study firstly analyzed the epidemiological characteristics of PCL in China, which can provide evidence for the research and policies regarding PCL.

[Primary plasma cell leukemia. Report of five cases]. / Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno.

BACKGROUND: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). AIM: To report the features of patients with pPCL. MATERIAL AND METHODS: Review of databases of the Hematology Department and the Hematology laboratory. RESULTS: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. CONCLUSIONS: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.

Epidemiology of Plasma Cell Leukemia in the Czech Republic.

BACKGROUND: Plasma cell leukemia (PCL) is a rare but most aggressive form of monoclonal gammopathies. PCL is characterized by the presence of clonal plasma cells in peripheral blood. There are two forms of PCL - primary which presents de novo in patients with no evidence of previous multiple myeloma and secondary which is a leukemic transformation of relapsed or refractory dis-ease in patients with previously recognized multiple myeloma. MATERIALS AND METHODS: This is the first study to provide information on PCL epidemiology in the Czech population us-ing The Czech National Cancer Registry (CNCR) as the basic source of data for the population-based evaluation of PCL epidemiology. RESULTS: Accord-ing to CNCR data, there were on average six newly dia-gnosed cases of PCL and four deaths caused by PCL each year in the Czech Republic in the period 2000- 2015. PCL incidence in the Czech Republic was reported at 0.57 per million in 2000- 2015. We suppose that most reported cases of PCL are primary PCL because secondary PCL is a relapse of a previously reported myeloma and, in most cases, is not coded as an independent dia-gnosis in the CNCR. CONCLUSION: Data from registries such as the CNCR can provide useful information on epidemiology of various dis-eases. These data, however, have several limitations, such as dia-gnostic criteria and proper cod-ing of not only the dis-ease itself, but also its various forms. These limitations have to be taken into account dur-ing the process of results interpretation. Key words plasma cell leukemia -  epidemiology -  Czech National Cancer Registry (CNCR) -  Czech Republic.

Leucemia de células plasmáticas primaria: experiencia en un hospital público chileno / Primary plasma cell leukemia: report of five cases

Background: Primary plasma cell leukemia (pPCL) is uncommon, aggressive and has a different biology than multiple myeloma (MM). Aim: To report the features of patients with pPCL. Material and Methods: Review of databases of the Hematology Department and the Hematology laboratory. Results: Of 178 patients with monoclonal gammopathies, five (2.8%) patients aged 33 to 64 years (three females) had a pPCL. The mean hemoglobin was 7.3 g/dL, the mean white blood cell count was 52,500/mm3, with 58% plasma cells, and the mean platelet count was 83,600/mm3. The mean bone marrow infiltration was 89%, LDH was 2,003 IU/L, serum calcium was 13 mg/dL, and creatinine 1.5 mg/dL. Two patients had bone lesions. Three were IgG, one IgA lambda and one lambda light chain. CD20 was positive in one, CD56 was negative in all and CD117 was negative in 3 cases. By conventional cytogenetic analysis, two had a complex karyotype. By Fluorescence in situ Hybridization, one was positive for TP53 and another for t (11; 14). One patient did not receive any treatment, three patients received VTD PACE and one CTD. None underwent transplant. Three patients are alive. The mean survival was 14 months. Conclusions: These patients with pPCL were younger and had a more aggressive clinical outcome than in multiple myeloma.

Risk of acute myeloid leukemia and myelodysplastic syndrome after autotransplants for lymphomas and plasma cell myeloma.

BACKGROUND: Exposures to DNA-damaging drugs and ionizing radiations increase risks of acute myeloid leukemia (AML) and myelodysplastic syndrome (MDS). METHODS: 9028 recipients of hematopoietic cell autotransplants (1995-2010) for Hodgkin lymphoma (HL; n = 916), non-Hodgkin lymphoma (NHL; n = 3546) and plasma cell myeloma (PCM; n = 4566), reported to the CIBMTR, were analyzed for risk of subsequent AML or MDS. RESULTS: 335 MDS/AML cases were diagnosed posttransplant (3.7%). Variables associated with an increased risk for AML or MDS in multivariate analyses were: (1) conditioning with total body radiation versus chemotherapy alone for HL (HR = 4.0; 95% confidence interval [1.4, 11.6]) and NHL (HR = 2.5 [1.1, 2.5]); (2) ≥3 versus 1 line of chemotherapy for NHL (HR = 1.9 [1.3, 2.8]); and (3) subjects with NHL transplanted in 2005-2010 versus 1995-1999 (HR = 2.1 [1.5, 3.1]). Using Surveillance, Epidemiology and End Results (SEER) data, we found risks for AML/MDS in HL, NHL and PCM to be 5-10 times the background rate. In contrast, relative risks were 10-50 for AML and approximately 100 for MDS in the autotransplant cohort. CONCLUSIONS: There are substantial risks of AML and MDS after autotransplants for HL, NHL and PCM.

Incidence, characteristics, and outcome of solitary plasmacytoma and plasma cell leukemia. Population-based data from the Swedish Myeloma Register.

Solitary plasmacytoma (SP) and plasma cell leukemia (PCL) are uncommon (3-6%) types of plasma cell disease. The risk of progression to symptomatic multiple myeloma (MM) is probably important for the outcome of SP. PCL is rare and has a dismal outcome. In this study, we report on incidence and survival in PCL/SP, and progression to MM in SP, using the prospective observational Swedish Multiple Myeloma Register designed to document all newly diagnosed plasma cell diseases in Sweden since 2008. Both solitary bone plasmacytoma (SBP) (n=124) and extramedullary plasmacytoma (EMP) (n=67) have better overall survival (OS) than MM (n=3549). Progression to MM was higher in SBP than in EMP (35% and 7% at 2 years, respectively), but this did not translate into better survival in EMP. In spite of treatment developments, the OS of primary PCL is still dismal (median of 11 months, 0% at 5 years). Hence, there is a great need for diagnostic and treatment guidelines as well as prospective studies addressing the role for alternative treatment options, such as allogeneic stem cell transplantation and monoclonal antibodies in the treatment of PCL.

Plasma cell leukemia: Single institution experience.

BACKGROUND: The first case of plasma cell leukemia (PCL) was recognized by Gluzinski and Reichentein. It is the most aggressive among the monoclonal gammopathies. It is diagnosed by the presence of more than 20% plasma cells in the peripheral blood or an absolute plasma cell count of> 2000/mm3. Because of the relatively low incidence, most data come from case reports and retrospective studies. No prospective series have been published, and only seven reports including more than twenty patients have been identified. We report a retrospective series of 18 patients identified as PCL. AIM: To study the clinical features and outcome of patients with PCL. MATERIALS AND METHODS: A retrospective study was conducted from the year 2006 to 2015 wherein all the patients diagnosed with PCL were identified. Complete clinical and treatment details and outcome were obtained from the records. RESULTS: There were total 18 cases of PCL (3.7% of cases with multiple myeloma) diagnosed between the year 2006 and 2015. 16 cases (84%) were primary PCL, and two cases were secondary PCL. Twelve patients were males and six were females. The median age was 56.5 years. All patients had aggressive clinical course and median overall survival even with immunomodulatory agents was only 3 months. CONCLUSION: PCL is a very aggressive disease, and no prospective trials have been conducted. Patients with PCL require induction with immunomodulators, proteasome inhibitors, and further trials are needed to evaluate the role of autologous stem cell transplant in this disease.

Plasma cell leukemia: Clinicopathologic, immunophenotypic and cytogenetic characteristics of 4 cases.

Plasma cell leukemia (PCL) is a rare hematologic malignancy with very poor outcome. It is defined by the presence of >2 × 10(9)/L plasma cells or >20% plasmacytosis of the differential white cell count in the peripheral blood. Primary PCL is first diagnosed in the leukemic phase, while secondary PCL corresponds to the leukemic transformation of a previously diagnosed multiple myeloma (MM). The incidence of PCL ranges between 2-4% of patients with MM and 0.9% of patients with acute leukemia. In this case series, we describe the clinicopathologic, immunophenotypic, and cytogenetic findings of four patients diagnosed with PCL within a ten-year period (2002-2012) at King Faisal Specialist Hospital and Research Centre (General Organization), Riyadh, Saudi Arabia.

Plasma cell leukaemia and HIV co-infection: profile of patients and experience at Universitas Academic Hospital in Bloemfontein, South Africa.

Plasma cell leukaemia (PCL) is a rare condition with high mortality. HIV-positive patients have a propensity to develop malignancy; however, the occurrence of PCL with HIV infection in South Africa has not been documented. We describe patients with PCL in Universitas Hospital in Bloemfontein, South Africa, and report two new cases of HIV infection concurrent with PCL. A retrospective case series of PCL patients (2006-2012) seen at our Clinical Haematology unit is reported. Patient files were used to obtain information. The median age of patients (n = 9) was 51 years, and 66.7 % of cases were of African ethnicity. The condition was equally distributed between genders. Two patients were HIV positive. Both received combination antiretroviral therapy. The diagnosis of PCL was usually made as an incidental finding, subsequently confirmed on bone marrow aspirate and trephine. Deranged haematological and biochemical parameters, including severe anaemia, hypoalbuminaemia, and hyper-cellular bone marrow, were observed. Only one patient improved markedly on treatment, and remains alive at the time of writing. PCL shows poor response to treatment and predominates among Africans. The small sample size made it difficult to determine whether co-infection with HIV was a coincidental finding or the two diseases are pathophysiologically linked.

Primary plasma cell leukemia: a Surveillance, Epidemiology, and End Results database analysis between 1973 and 2004.

BACKGROUND: Primary plasma cell leukemia (PCL) is a rare plasma cell disorder, and current knowledge regarding survival in this disease is limited to small series of patients. Although there has been significant improvement in the survival of patients with multiple myeloma (MM) over the past few decades, it is not known whether there has been a similar trend for PCL. METHODS: The authors analyzed the Surveillance, Epidemiology, and End Results (SEER) database to evaluate the characteristics and survival of patients who had PCL compared with those of patients who had MM. RESULTS: Among 291 patients with PCL, the median age was 67 years (range, 19-98 years), the distribution of men and women was nearly equal, and the majority of patients were white (79.4%). The median overall survival (OS) was 4 months and the median disease-specific survival (DSS) was 6 months for patients with PCL; the 1-year, 2-year, and 5-year OS rates were 27.8%, 14.1%, and 6.4%, respectively. There were no survival differences noted according to sex or race. Patients aged<60 years were found to have a better median OS compared with patients aged>or=60 years (median OS, 7 months vs 3 months; P=.007), although the 5-year OS was equally poor in both groups (6.3% vs 6.4%, respectively). During the same period, 49,106 patients with MM were identified. Unlike MM, in which there has been a modest but statistically significant improvement in OS and DSS noted over time, no significant improvement was evident for PCL. CONCLUSIONS: The poor long-term outcome and the lack of survival improvement in PCL suggest the need for better therapeutic options for these patients.

Plasma cell leukemia--a study of 28 cases from India.

Plasma cell leukemia (PCL) is a rare neoplasm that has not been comprehensively reported in an Indian population. We report the clinico-pathological features of 28 cases studied during 1999-2008. Organomegaly and bleeding tendency was common in primary PCL but not in secondary. Misdiagnosis as acute leukemia or the leukemic phase of lymphoma on the initial peripheral blood smear examination was frequent (31.4% cases) in the primary form of PCL. This is best addressed by an emphasis on the morphological appearances and confirmation by simple serum electrophoresis rather than by more sophisticated testing that may not be widely available. Response to treatment is poor and PCL has a poor prognosis, a situation that may be amenable to improvement by a better understanding of the biology of the disease.

Age 40 years and under does not confer superior prognosis in patients with multiple myeloma undergoing upfront autologous stem cell transmplant.

Multiple myeloma (MM) rarely occurs in patients 40 years of age and younger. This young age has been reported to correlate with improved survival in patients with MM. The objective of this study is to describe presenting features and outcomes of patients < or =40 years of age with MM who undergo autologous stem cell transplantation (ASCT) as first-line treatment, and compare overall survival (OS) and progression free survival (PFS) to patients aged 41-65 years. We performed a retrospective institutional review of all patients < or =40 years of age and 41-65 years of age at the time of diagnosis of MM who had undergone upfront ASCT from January 1, 1990, to July 31, 2007. Thirty-eight patients < or =40 years of age and 608 patients aged 41-65 were identified. There was a high rate of plasma cell leukemia (PCL) in young patients at 11% compared to the reported rate of 2%-4%. At diagnosis, there was an increased rate of renal failure in the young cohort compared to patients aged 41-65 years at 25% versus 16% and Bence Jones proteinuria at 81% versus 51%. The rate of complete or partial response was similar between the groups at 79% and 83% in the young and older cohorts, respectively. Median PFS post-ASCT was 22.0 months (95% confidence interval [CI]: 16.1, 28.0), versus 26.9 months (95% CI: 24.0, 29.8) for patients aged 41-65 years (P = .66). Median OS from date of ASCT was also similar to those over 40 years: 68.1 months (95% CI: 39.0, 97.2) versus 80.7 months (95% CI: 68.1, 93.4); P = .90. Treatment-related mortality (TRM) was low at 2.6% and 2.3% in the young and older cohorts, respectively. Despite previous reports that young age is a positive prognostic marker, our study found OS post-ASCT is equivalent to those aged 41-65 years. This study emphasizes the importance of developing strategies to better the outcomes of young patients with MM.

Molecular cytogenetic aberrations in 21 Chinese patients with plasma cell leukemia.

Plasma cell leukemia (PCL) is a rare malignant plasma cell disorder. Cytogenetic studies performed on plasma cell disorders are scarce and difficult because of the low proliferation rate of plasma cells (PCs). Fluorescence in situ hybridization (FISH) analysis is an attractive alternative for evaluation of chromosomal changes in PCL. To explore the molecular cytogenetic abnormalities in Chinese patients with PCL, interphase FISH studies with three probes for the regions containing 13q14.3 (D13S319), 14q32 (IGHC/IGHV) and 1q12(CEP1) were retrospectively performed in 21 PCL patients. FISH with LSI IGH/CCND1 and LSI IGH/FGFR3 probes were used to detect t(11;14)(q13;q32) and t(4;14)(p16;q32) in patients with 14q32 rearrangement. Among 21 PCL patients, molecular cytogenetic aberrations were found in 18 (81.8%) patients, four (19.0%) patients simultaneously had 13q14 deletion, illegitimate IgH translocation and 1q abnormality. 13q14 deletion was detected in 13 (61.9%) cases and illegitimate 14q32 rearrangement in 16 (76.2%) including six with t(11;14) and three with t(4;14). Chromosome 1 abnormality was found in seven (33.3%) patients, one with deletion of 1q, six with at least three copies amplifications of 1q12 (Amp1q12). 14q32 rearrangement and 13q14 deletion were found concurrently in 11 (52.4%) cases. It was showed that most PCL had chromosomal abnormalities, 14q32 rearrangement, 13q14 deletion and chromosome 1 abnormality are the frequent abnormalities, and over half of the 14q32 rearrangement were t(11;14) or t(4;14). t(4;14) and 13q14 deletion were correlated in PCL. FISH is a highly sensitive technique at detecting molecular cytogenetic aberrations in PCL and should be used in the routine evaluation of PCL.

Plasma cell leukemia: a report on 15 patients.

Plasma cell leukemia (PCL) can be considered the leukemic variant of multiple myeloma. The diagnosis is based on hematological features, including a plasmacytosis exceeding 2 x 10(9)/l and any evidence of a clonal plasma cell proliferation. There are two forms of PCL: the primary form occurring in individuals without preceding multiple myeloma, and the secondary form arising as a late manifestation in patients with multiple myeloma. From 1974 to 1988 we diagnosed 8 primary PCL cases out of a total 301 multiple myeloma cases (incidence, 2.6%) and a total of 847 acute leukemia cases (incidence, 0.9%). During the same period we observed in 7 multiple myeloma patients a terminal PCL, for an incidence of PCL in myeloma of 2.3%. Most clinical characteristics were similar in both types of plasma cell leukemia. In particular we found no difference in the average age and in the incidence of bone pain, hepatosplenomegaly, lytic bone lesions. None of our cases showed a clinically relevant lymphadenopathy either as presenting symptom or during the course of the disease. The values for hemoglobin, leukocytes, plasma cells, serum creatinine and calcium did not differ significantly between the two groups of patients. The median survival was 7 months for patients with primary PCL and 1 month for patients with secondary PCL. 5 of the 8 patients with primary PCL obtained a response to conventional myeloma therapy including single alkylating agents, with a duration ranging from 7 to 44 months. Only 1 of the patients with secondary PCL had a partial response after combination chemotherapy.

Plasma cell leukaemia. Diagnostic problems in our experience with 11 cases.

11 patients with plasma cell leukaemia (PCL) are reported. Diagnostic clinical, haematological, immunological, biochemical and electron microscopical (TEM) data were analysed and compared to the largest series of PCL cases reported in the literature. Special attention was paid to four facets of this disease: (a) the clinical picture at admission; (b) the frequency of PCL; (c) the production of M components in relation to the maturity and type of the asynchronous plasma cells, and (d) the diagnostic problems of this entity of acute leukaemia of the afferent limb of the B lymphocyte transformation. In this series PCL emerges as a distinct clinical entity: patients are severely anaemic, hepatosplenomegaly is prominent, bone lessions are uncommun, but if present are usually non-osteolytic, and the response to treatment with an alkylating agent and glucocorticoid is poor. The diagnosis is difficult since the circulating plasma cells may have morphological features which only allows the diagnosis to be made after the TEM examination. If the peripheral blood of cases of acute leukaemias and immunocytic dyscrasias is routinely examined by TEM, PCL appears to be a not uncommon variant of plasma cell dyscrasia--in the present study it was 11%.