BACKGROUND Plasma cell myeloma(PCM)is characterized by hypercalcemia,renal impairment,anemia,and bone destruction.While pleural effusion,ascites,abdominal pain,and bloody stool are common manifestations of lung diseas...BACKGROUND Plasma cell myeloma(PCM)is characterized by hypercalcemia,renal impairment,anemia,and bone destruction.While pleural effusion,ascites,abdominal pain,and bloody stool are common manifestations of lung disease or gastrointestinal disorders,they are rarely observed in patients with PCM.CASE SUMMARY A 66-year-old woman presented with complaints of recurrent chest tightness,wheezing,and abdominal bloating accompanied by bloody stools.Computed tomography revealed pleural effusion and ascites.Pleural effusion tests showed inflammation,but the T-cell spot test and carcinoembryonic antigen were negative.Endoscopy showed colonic mucosal edema with ulcer formation and local intestinal lumen stenosis.Echocardiography revealed enlarged atria and reduced left ventricular systolic function.The diagnosis remained unclear.Further testing revealed elevated blood light chain lambda and urine immunoglobulin levels.Blood immunofixation electrophoresis was positive for immunoglobulin G lambda type.Smear cytology of the bone marrow showed a high proportion of plasma cells,accounting for about 4.5%.Histopathological examination of the bone marrow suggested PCM.Flow cytometry showed abnormal plasma cells with strong expression of CD38,CD138,cLambda,CD28,CD200,and CD117.Fluorescence in situ hybridization gene testing of the bone marrow suggested 1q21 gene amplification,but cytogenetic testing showed no clonal abnormalities.Colonic mucosa and bone marrow biopsy tissues were negative for Highman Congo red staining.The patient was finally diagnosed with PCM.CONCLUSION A diagnosis of PCM should be considered in older patients with pleural effusion,ascites,and multi-organ injury.展开更多
Refractory and relapsed multiple myeloma (RRMM) with plasma-cell leukemia (PCL) transformation is highly aggressive and resistant to conventional therapy. Novel therapeutics are needed for RRMM-transformed PCL. Seline...Refractory and relapsed multiple myeloma (RRMM) with plasma-cell leukemia (PCL) transformation is highly aggressive and resistant to conventional therapy. Novel therapeutics are needed for RRMM-transformed PCL. Selinexor [an oral exportin 1 (XPO1) inhibitor], carfilzomib (a second-in-class proteasome inhibitor), pomalidomide (third generation of immunomodulatory drug) are usually used for RRMM, but there are no reports on their application in PCL transformation. We describe a 62-year-old male initially diagnosed with MM IgD-lambda type with complex karyotype and extramedullary plasmacytoma in 2020, and relapsed after five months of autologous stem cell transplantation. Despite the use of various therapies, the patient rapidly developed into PCL over a 4-month period. The patient was started on selinexor, carfilzomib, pomalidomide, and dexamethasone (XKPd) combination as a salvage regimen in July 2021. He achieved fast response in first cycle. Then, he fulfilled third cycle of consolidation treatment and got four-month remission. The success of XKPd therapy in achieving a good response suggests its utility in RRMM transformed-PCL patients, who have exhausted various combinations of drug regimens and have historically poor survival outcomes.展开更多
Objectives: To determine the multiple myeloma (MM) prognostic formulas applicable in Congolese with many aberrant and/or immature plasma cells according to GOASGUEN’s plasma cell morphology algorithm (PMA). Methods: ...Objectives: To determine the multiple myeloma (MM) prognostic formulas applicable in Congolese with many aberrant and/or immature plasma cells according to GOASGUEN’s plasma cell morphology algorithm (PMA). Methods: The prospective, observational and descriptive study was carried out at the Ngaliema Clinic in Kinshasa from July 2016 to December 2019. The variables studied were the plasma cell types and the prognosis groups according to GOASGUEN and ZANDECKI. The nucleolus, the chromatin and the N/C ratio of 2 for each plasma cell encountered in multiple myeloma are examined in order by asking three successive questions as follows: 1) Is the nucleolus present? If yes, we denote 1;if not, note 0;2) Is the chromatin thin? If yes, we score 1;if not we score 0;3) The N/C ratio is greater than 6, if so, 1 is noted;if not, we note 0. We obtain a number with 3 successive digits preceded by the letter P for each plasma cell. Results: The overall rate of immature plasma cells and aberrant plasma cells in MM among Congolese was high at 26.6%. Their integration in the prognostic formulas showed for group A" = 22 patients, group B" = 5 patients and group C" = 32 patients. Examination of plasma cell types in the deceased showed that 14/17 had a P001 plasma cell count ≥ 15%. Conclusion: The high rate of aberrant and/or immature plasma cells, the P001 plasma cell type at a rate ≥ 15%, found mostly in group C" with poor prognosis, are responsible for the aggressive nature of MM in Congolese Blacks.展开更多
Multiple myeloma (MM) is both a complex and heterogeneous disease. Cytogenetic and molecular abnormalities lead to resistance to treatment and transformation to plasma cell leukemia, which is defined by the presence i...Multiple myeloma (MM) is both a complex and heterogeneous disease. Cytogenetic and molecular abnormalities lead to resistance to treatment and transformation to plasma cell leukemia, which is defined by the presence in circulating blood of plasma cells over 2 G/L, or more than 20% of leukocytes. It is an uncommon hematological malignancy with a poor prognosis. Against this backdrop, we report an observation of multiple myeloma transformed into plasma cell leukemia diagnosed at the Hôpital Principal de Dakar (HPD) that occurred on a 64-year-old man with a history of thyroidectomy followed for multiple myeloma presenting with Salmon et Durie stage IIIA and ISS stage I. Despite a marked improvement in management strategy, myeloma remains an almost invariably incurable disease. However, the development of genetic and molecular biomarkers is necessary to improve its prognosis.展开更多
BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small ...BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.CASE SUMMARY Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search(Pub Med database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma(EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males(67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach(41%), followed by the duodenum(15%). The most common presenting complaint was melena(44%). In the majority of cases, the EMPs were a secondary manifestation(63%) at the background of multiple myeloma(26%), plasmablastic myeloma(7%) or high-grade plasma cell myeloma(4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.CONCLUSION Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.展开更多
Gastrointestinal involvement in plasma cell neoplasms,either as primary localizations(extramedullary plasma-cytomas) or as secondary involvement in systemic multiple myeloma, is a well-known event. Accurate histologic...Gastrointestinal involvement in plasma cell neoplasms,either as primary localizations(extramedullary plasma-cytomas) or as secondary involvement in systemic multiple myeloma, is a well-known event. Accurate histological examination is crucial in defining the diag-nosis. In this report, an uncommon case of duodenal localization of myeloma with plasmablastic features is described, with emphasis on the role of clinical data and findings from ancillary immunostaining techniques to avoid misdiagnosis.展开更多
BACKGROUND Chronic myeloid leukemia(CML)is a clonal hematopoietic stem cell disorder.Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders.The co-occurrence of CML and plasma cell dyscrasias...BACKGROUND Chronic myeloid leukemia(CML)is a clonal hematopoietic stem cell disorder.Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders.The co-occurrence of CML and plasma cell dyscrasias in the same patient is an extremely rare incident and has been reported in several cases in the literature.CASE SUMMARY In the present report,we described a rare case of the co-occurrence of CML and plasma cell dyscrasias in a 48-year-old man,and we discussed the reason why monoclonal gammopathy of undetermined significance progressed to smoldering multiple myeloma and eventually to multiple myeloma while being treated with dasatinib for CML.The tyrosine kinase inhibitor treatment and cytogenetic change may contribute to this phenomenon,and clonal hematopoiesis of indeterminate potential may lead to both CML and multiple myeloma cells in a patient.Future studies are warranted to further explain the hidden reasons.CONCLUSION This case highlights that gene translocation may contribute to initiation and sustainability of clonal proliferation.Moreover,the treatment with tyrosine kinase inhibitor and cytogenetic change may contribute to progression from monoclonal gammopathy of undetermined significance to smoldering multiple myeloma and eventually to multiple myeloma.展开更多
Plasma cell neoplasms comprise a spectrum of diseases that include monoclonal gammopathy of undetermined signi-ficance (MGUS) and multiple myeloma (MM). Flow cytometric immunophenotyping has become an invaluable tool ...Plasma cell neoplasms comprise a spectrum of diseases that include monoclonal gammopathy of undetermined signi-ficance (MGUS) and multiple myeloma (MM). Flow cytometric immunophenotyping has become an invaluable tool as an ancillary and diagnostic test for hematologic malignancies and is being used with increasing frequency in the diag-nosis and monitoring of plasma cell neoplasms. As multiparameter flow cytometry has evolved, faster fluidics and detection systems facilitate the screening of a large number of events and the detection of multiple antigens simultaneously. This review addresses the approaches used to evaluate clonal plasma cell neoplasms and describes different surface and cytoplasmic markers and techniques that are important for the study of these diseases.展开更多
Introduction: Plasma cell leukemia (PL) is a rare lymphoproliferative disorder characterized by the monoclonal proliferation of plasma cells in the marrow and blood peripheral. It is defined by a blood plasmacytosis g...Introduction: Plasma cell leukemia (PL) is a rare lymphoproliferative disorder characterized by the monoclonal proliferation of plasma cells in the marrow and blood peripheral. It is defined by a blood plasmacytosis greater than 2 G/l or a plasma cell level greater than 20% of leukocytes. It can be primitive or secondary to multiple myeloma (MM). We reported 3 cases of PL. Observations: Case 1: A 59 years old woman with fever, anemia with 7 g/dl, hyperleukocytosis 9200/mm<sup>3</sup>, thrombopenia 86 G/l inflammatory biological syndrome with CRP at 129 mg/l, hypercalcemia at 120 mg/l, renal failure with serum creatinine at 35 mg/l, urea at 0.85 g/l and 24-hour proteinuria at 0.98 g/24h. Β2 microglobulin at 10.34 mg/l. The blood smear shows dysmorphic plasma cells at 68% and the bone marrow at 79% of dysmorphic plasma cells. The immunophenotyping of blood cells, the electrophoretic serum protein, shows PL CD38+, secondary of a MM LAMBDA. Case 2: A 65-year-old man with type 2 diabetes presented, right femoral neck, anemia, hyperleukocytosis at 22 G/l, and thrombocytopenia at 99 G/l. There was no hypercalcemia, or kidney failure. The blood smear showed 28% of plasma cells and 9% of blasts. On the myelogram, the marrow was normal richness with significant medullary plasmacytosis (31%) made up of dysmorphic plasma cells. The CT scan showed a settling of the body of D5 with heterogeneous osteocondensation. The patient was transferred to hematology where she was treated with polychemotherapy. The evolution was unfavorable following a death due to malignant hypercalcemia. Case 3: A 62-year-old woman who had a 5-year follow-up of Ig G kappa multiple myeloma was treated with Melphalan, Prednisone, and thalidomide with a therapeutic break for 2 months. She came back to the Internal Medicine department with: severe global dehydration, anemia with externalized bleeding gingivorrhagia, pain in mechanical bones of the ribs, lower limbs, and pelvis, bilateral pneumonia. The biology found hyperleukocytosis at 99 G/l, anemia at 4.7 g/dl, thrombocytopenia at 31 g/l, hypercalcemia at 190 mg/l, renal failure with creatinine at 34 mg/L, and urea at 1.08 g/l, a biological inflammatory syndrome with CRP 294 mg/l. The smeared blood had shown 93% blood plasma cells and immunophenotyping showed CD38+. The patient died before specific treatment for the disease. Conclusion: Plasma cell leukemia is a rare atypical variant, complicating essentially multiple light chain myeloma. She must be suspected especially when there are cytological abnormalities such as major leukocytosis or thrombocytopenia, which are unusual in classical myeloma. Evolution is usually a very bad prognosis, with a median survival of 12 to 14 months for the form primary and 2 to 3 months for the secondary form.展开更多
Purpose: To report a retro-orbital localization of Multiple Myeloma (MM) describing its treatment and clinical result. Case report: A 50-years-old male patient with Magnetic Resonance Imaging (MRI) evidence of a retro...Purpose: To report a retro-orbital localization of Multiple Myeloma (MM) describing its treatment and clinical result. Case report: A 50-years-old male patient with Magnetic Resonance Imaging (MRI) evidence of a retro-orbital mass with exophthalmos, due to the pathological diagnosis of MM, was referred for Radiation Therapy (RT). Discussion: The orbital involvement in Multiple Myeloma is rare and few cases are reported in the literature. The treatment of choice is RT alone with a prescribed dose ranging between 40 Gy and 45 Gy. In our patient the retro-orbital lesion, measuring 26 × 16 mm, was treated with Intensity Modulated Radiotherapy Technique (IMRT) delivering 4400 cGy with conventional fractionation. The treatment was well tolerated, the patient experienced a complete regression of the exophthalmos without any significant side effect.展开更多
Multiple myeloma is characterized by the accumulation of clonal, malignant plasma cells in the bone marrow. Multiple lytic skeletal lesions in some tumor patients with multiple myeloma are easily considered as bone me...Multiple myeloma is characterized by the accumulation of clonal, malignant plasma cells in the bone marrow. Multiple lytic skeletal lesions in some tumor patients with multiple myeloma are easily considered as bone metastases secondary to tumors, resulting in a missed diagnosis of multiple myeloma. Herein, we report a rare case, in which rectal cancer with multiple myeloma was initially misdiagnosed with bone metastases secondary to rectal cancer, due to the symptoms of multiple lytic sketetal lesions, and ignoring the abnormal plasma cells in the peripheral circulating blood smear. The patient was finally diagnosed with coexistence of rectal cancer and multiple myeloma. The case focuses on the importance of the peripheral circulating blood smear detection.展开更多
目的探讨根据国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准不同浆细胞病分型患者骨髓浆细胞数量及免疫表型的差异。方法回顾性分析2019年6月12日至2023年9月5日在复旦大学附属中山医院厦门医院诊治的浆细胞病患者...目的探讨根据国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准不同浆细胞病分型患者骨髓浆细胞数量及免疫表型的差异。方法回顾性分析2019年6月12日至2023年9月5日在复旦大学附属中山医院厦门医院诊治的浆细胞病患者血清学及骨髓流式结果。结果纳入102例浆细胞病患者,男性63例,女性39例,发病年龄22~85岁,其中意义未明的单克隆丙种球蛋白血症46例,冒烟型骨髓瘤5例,多发性骨髓瘤39例,轻链淀粉样变性12例。所有患者均伴有M蛋白,包含IgG型58例、非IgG型44例。所有患者骨髓均可检测到浆细胞,其中79例患者骨髓检测到异常浆细胞,63例患者骨髓检测到正常浆细胞,40例患者骨髓同时检测到正常及异常浆细胞。52例患者骨髓的异常浆细胞表达CD56,12例患者骨髓异常浆细胞表达CD117。不同疾病组间的性别、年龄差异无统计学意义。不同疾病组间的M蛋白类型及浓度、血清受累/非受累游离轻链、骨髓总浆细胞(包括正常及异常浆细胞)/有核细胞、骨髓异常浆细胞/有核细胞、骨髓异常浆细胞/骨髓总浆细胞、骨髓正常浆细胞/骨髓总浆细胞的差异均有统计学意义(P均<0.05)。不同疾病组中异常浆细胞CD56的表达存在统计学差异(P=0.009),而CD117的表达差异无统计学意义。结论轻链淀粉样变性患者骨髓克隆性浆细胞的比例、克隆性浆细胞/骨髓总浆细胞、异常浆细胞CD56表达比例与意义未明单克隆丙种球蛋白血症相仿,而与多发性骨髓瘤患者有显著差异。展开更多
文摘BACKGROUND Plasma cell myeloma(PCM)is characterized by hypercalcemia,renal impairment,anemia,and bone destruction.While pleural effusion,ascites,abdominal pain,and bloody stool are common manifestations of lung disease or gastrointestinal disorders,they are rarely observed in patients with PCM.CASE SUMMARY A 66-year-old woman presented with complaints of recurrent chest tightness,wheezing,and abdominal bloating accompanied by bloody stools.Computed tomography revealed pleural effusion and ascites.Pleural effusion tests showed inflammation,but the T-cell spot test and carcinoembryonic antigen were negative.Endoscopy showed colonic mucosal edema with ulcer formation and local intestinal lumen stenosis.Echocardiography revealed enlarged atria and reduced left ventricular systolic function.The diagnosis remained unclear.Further testing revealed elevated blood light chain lambda and urine immunoglobulin levels.Blood immunofixation electrophoresis was positive for immunoglobulin G lambda type.Smear cytology of the bone marrow showed a high proportion of plasma cells,accounting for about 4.5%.Histopathological examination of the bone marrow suggested PCM.Flow cytometry showed abnormal plasma cells with strong expression of CD38,CD138,cLambda,CD28,CD200,and CD117.Fluorescence in situ hybridization gene testing of the bone marrow suggested 1q21 gene amplification,but cytogenetic testing showed no clonal abnormalities.Colonic mucosa and bone marrow biopsy tissues were negative for Highman Congo red staining.The patient was finally diagnosed with PCM.CONCLUSION A diagnosis of PCM should be considered in older patients with pleural effusion,ascites,and multi-organ injury.
文摘Refractory and relapsed multiple myeloma (RRMM) with plasma-cell leukemia (PCL) transformation is highly aggressive and resistant to conventional therapy. Novel therapeutics are needed for RRMM-transformed PCL. Selinexor [an oral exportin 1 (XPO1) inhibitor], carfilzomib (a second-in-class proteasome inhibitor), pomalidomide (third generation of immunomodulatory drug) are usually used for RRMM, but there are no reports on their application in PCL transformation. We describe a 62-year-old male initially diagnosed with MM IgD-lambda type with complex karyotype and extramedullary plasmacytoma in 2020, and relapsed after five months of autologous stem cell transplantation. Despite the use of various therapies, the patient rapidly developed into PCL over a 4-month period. The patient was started on selinexor, carfilzomib, pomalidomide, and dexamethasone (XKPd) combination as a salvage regimen in July 2021. He achieved fast response in first cycle. Then, he fulfilled third cycle of consolidation treatment and got four-month remission. The success of XKPd therapy in achieving a good response suggests its utility in RRMM transformed-PCL patients, who have exhausted various combinations of drug regimens and have historically poor survival outcomes.
文摘Objectives: To determine the multiple myeloma (MM) prognostic formulas applicable in Congolese with many aberrant and/or immature plasma cells according to GOASGUEN’s plasma cell morphology algorithm (PMA). Methods: The prospective, observational and descriptive study was carried out at the Ngaliema Clinic in Kinshasa from July 2016 to December 2019. The variables studied were the plasma cell types and the prognosis groups according to GOASGUEN and ZANDECKI. The nucleolus, the chromatin and the N/C ratio of 2 for each plasma cell encountered in multiple myeloma are examined in order by asking three successive questions as follows: 1) Is the nucleolus present? If yes, we denote 1;if not, note 0;2) Is the chromatin thin? If yes, we score 1;if not we score 0;3) The N/C ratio is greater than 6, if so, 1 is noted;if not, we note 0. We obtain a number with 3 successive digits preceded by the letter P for each plasma cell. Results: The overall rate of immature plasma cells and aberrant plasma cells in MM among Congolese was high at 26.6%. Their integration in the prognostic formulas showed for group A" = 22 patients, group B" = 5 patients and group C" = 32 patients. Examination of plasma cell types in the deceased showed that 14/17 had a P001 plasma cell count ≥ 15%. Conclusion: The high rate of aberrant and/or immature plasma cells, the P001 plasma cell type at a rate ≥ 15%, found mostly in group C" with poor prognosis, are responsible for the aggressive nature of MM in Congolese Blacks.
文摘Multiple myeloma (MM) is both a complex and heterogeneous disease. Cytogenetic and molecular abnormalities lead to resistance to treatment and transformation to plasma cell leukemia, which is defined by the presence in circulating blood of plasma cells over 2 G/L, or more than 20% of leukocytes. It is an uncommon hematological malignancy with a poor prognosis. Against this backdrop, we report an observation of multiple myeloma transformed into plasma cell leukemia diagnosed at the Hôpital Principal de Dakar (HPD) that occurred on a 64-year-old man with a history of thyroidectomy followed for multiple myeloma presenting with Salmon et Durie stage IIIA and ISS stage I. Despite a marked improvement in management strategy, myeloma remains an almost invariably incurable disease. However, the development of genetic and molecular biomarkers is necessary to improve its prognosis.
文摘BACKGROUND Plasma-cell neoplasms rarely involve the gastrointestinal tract and manifest as gastrointestinal bleeding. Plasmablastic myeloma is an aggressive plasma cell neoplasm associated with poor outcomes. A small number of cases with gastrointestinal involvement is reported in the literature and therefore high index of suspicion is essential for avoiding delays in diagnosis and treatment.CASE SUMMARY Our aim is to present our experience of a 70-year-old patient with a secondary presentation of plasmablastic myeloma manifesting as unstable upper gastrointestinal bleeding and to review the literature with the view to consolidate and discuss information about diagnosis and management of this rare entity. In addition to our case, a literature search(Pub Med database) of case reports of extramedullary plasma cell neoplasms manifesting as upper gastrointestinal bleeding was performed. Twenty-seven cases of extramedullary plasmacytoma(EMP) involving the stomach and small bowel presenting with upper gastrointestinal bleeding were retrieved. The majority of patients were males(67%). The average age on diagnosis was 62.7 years. The most common site of presentation was the stomach(41%), followed by the duodenum(15%). The most common presenting complaint was melena(44%). In the majority of cases, the EMPs were a secondary manifestation(63%) at the background of multiple myeloma(26%), plasmablastic myeloma(7%) or high-grade plasma cell myeloma(4%). Oesophagogastroscopy was the main diagnostic modality and chemotherapy the preferred treatment option for secondary EMPs.CONCLUSION Despite their rare presentation, upper gastrointestinal EMPs should be considered in the differential diagnosis of patients with gastrointestinal bleeding especially in the presence of systemic haematological malignancy.
文摘Gastrointestinal involvement in plasma cell neoplasms,either as primary localizations(extramedullary plasma-cytomas) or as secondary involvement in systemic multiple myeloma, is a well-known event. Accurate histological examination is crucial in defining the diag-nosis. In this report, an uncommon case of duodenal localization of myeloma with plasmablastic features is described, with emphasis on the role of clinical data and findings from ancillary immunostaining techniques to avoid misdiagnosis.
文摘BACKGROUND Chronic myeloid leukemia(CML)is a clonal hematopoietic stem cell disorder.Plasma cell dyscrasias are a rare heterogeneous group of hematological disorders.The co-occurrence of CML and plasma cell dyscrasias in the same patient is an extremely rare incident and has been reported in several cases in the literature.CASE SUMMARY In the present report,we described a rare case of the co-occurrence of CML and plasma cell dyscrasias in a 48-year-old man,and we discussed the reason why monoclonal gammopathy of undetermined significance progressed to smoldering multiple myeloma and eventually to multiple myeloma while being treated with dasatinib for CML.The tyrosine kinase inhibitor treatment and cytogenetic change may contribute to this phenomenon,and clonal hematopoiesis of indeterminate potential may lead to both CML and multiple myeloma cells in a patient.Future studies are warranted to further explain the hidden reasons.CONCLUSION This case highlights that gene translocation may contribute to initiation and sustainability of clonal proliferation.Moreover,the treatment with tyrosine kinase inhibitor and cytogenetic change may contribute to progression from monoclonal gammopathy of undetermined significance to smoldering multiple myeloma and eventually to multiple myeloma.
文摘Plasma cell neoplasms comprise a spectrum of diseases that include monoclonal gammopathy of undetermined signi-ficance (MGUS) and multiple myeloma (MM). Flow cytometric immunophenotyping has become an invaluable tool as an ancillary and diagnostic test for hematologic malignancies and is being used with increasing frequency in the diag-nosis and monitoring of plasma cell neoplasms. As multiparameter flow cytometry has evolved, faster fluidics and detection systems facilitate the screening of a large number of events and the detection of multiple antigens simultaneously. This review addresses the approaches used to evaluate clonal plasma cell neoplasms and describes different surface and cytoplasmic markers and techniques that are important for the study of these diseases.
文摘Introduction: Plasma cell leukemia (PL) is a rare lymphoproliferative disorder characterized by the monoclonal proliferation of plasma cells in the marrow and blood peripheral. It is defined by a blood plasmacytosis greater than 2 G/l or a plasma cell level greater than 20% of leukocytes. It can be primitive or secondary to multiple myeloma (MM). We reported 3 cases of PL. Observations: Case 1: A 59 years old woman with fever, anemia with 7 g/dl, hyperleukocytosis 9200/mm<sup>3</sup>, thrombopenia 86 G/l inflammatory biological syndrome with CRP at 129 mg/l, hypercalcemia at 120 mg/l, renal failure with serum creatinine at 35 mg/l, urea at 0.85 g/l and 24-hour proteinuria at 0.98 g/24h. Β2 microglobulin at 10.34 mg/l. The blood smear shows dysmorphic plasma cells at 68% and the bone marrow at 79% of dysmorphic plasma cells. The immunophenotyping of blood cells, the electrophoretic serum protein, shows PL CD38+, secondary of a MM LAMBDA. Case 2: A 65-year-old man with type 2 diabetes presented, right femoral neck, anemia, hyperleukocytosis at 22 G/l, and thrombocytopenia at 99 G/l. There was no hypercalcemia, or kidney failure. The blood smear showed 28% of plasma cells and 9% of blasts. On the myelogram, the marrow was normal richness with significant medullary plasmacytosis (31%) made up of dysmorphic plasma cells. The CT scan showed a settling of the body of D5 with heterogeneous osteocondensation. The patient was transferred to hematology where she was treated with polychemotherapy. The evolution was unfavorable following a death due to malignant hypercalcemia. Case 3: A 62-year-old woman who had a 5-year follow-up of Ig G kappa multiple myeloma was treated with Melphalan, Prednisone, and thalidomide with a therapeutic break for 2 months. She came back to the Internal Medicine department with: severe global dehydration, anemia with externalized bleeding gingivorrhagia, pain in mechanical bones of the ribs, lower limbs, and pelvis, bilateral pneumonia. The biology found hyperleukocytosis at 99 G/l, anemia at 4.7 g/dl, thrombocytopenia at 31 g/l, hypercalcemia at 190 mg/l, renal failure with creatinine at 34 mg/L, and urea at 1.08 g/l, a biological inflammatory syndrome with CRP 294 mg/l. The smeared blood had shown 93% blood plasma cells and immunophenotyping showed CD38+. The patient died before specific treatment for the disease. Conclusion: Plasma cell leukemia is a rare atypical variant, complicating essentially multiple light chain myeloma. She must be suspected especially when there are cytological abnormalities such as major leukocytosis or thrombocytopenia, which are unusual in classical myeloma. Evolution is usually a very bad prognosis, with a median survival of 12 to 14 months for the form primary and 2 to 3 months for the secondary form.
文摘Purpose: To report a retro-orbital localization of Multiple Myeloma (MM) describing its treatment and clinical result. Case report: A 50-years-old male patient with Magnetic Resonance Imaging (MRI) evidence of a retro-orbital mass with exophthalmos, due to the pathological diagnosis of MM, was referred for Radiation Therapy (RT). Discussion: The orbital involvement in Multiple Myeloma is rare and few cases are reported in the literature. The treatment of choice is RT alone with a prescribed dose ranging between 40 Gy and 45 Gy. In our patient the retro-orbital lesion, measuring 26 × 16 mm, was treated with Intensity Modulated Radiotherapy Technique (IMRT) delivering 4400 cGy with conventional fractionation. The treatment was well tolerated, the patient experienced a complete regression of the exophthalmos without any significant side effect.
文摘Multiple myeloma is characterized by the accumulation of clonal, malignant plasma cells in the bone marrow. Multiple lytic skeletal lesions in some tumor patients with multiple myeloma are easily considered as bone metastases secondary to tumors, resulting in a missed diagnosis of multiple myeloma. Herein, we report a rare case, in which rectal cancer with multiple myeloma was initially misdiagnosed with bone metastases secondary to rectal cancer, due to the symptoms of multiple lytic sketetal lesions, and ignoring the abnormal plasma cells in the peripheral circulating blood smear. The patient was finally diagnosed with coexistence of rectal cancer and multiple myeloma. The case focuses on the importance of the peripheral circulating blood smear detection.
文摘目的探讨根据国际骨髓瘤工作组(International Myeloma Working Group,IMWG)标准不同浆细胞病分型患者骨髓浆细胞数量及免疫表型的差异。方法回顾性分析2019年6月12日至2023年9月5日在复旦大学附属中山医院厦门医院诊治的浆细胞病患者血清学及骨髓流式结果。结果纳入102例浆细胞病患者,男性63例,女性39例,发病年龄22~85岁,其中意义未明的单克隆丙种球蛋白血症46例,冒烟型骨髓瘤5例,多发性骨髓瘤39例,轻链淀粉样变性12例。所有患者均伴有M蛋白,包含IgG型58例、非IgG型44例。所有患者骨髓均可检测到浆细胞,其中79例患者骨髓检测到异常浆细胞,63例患者骨髓检测到正常浆细胞,40例患者骨髓同时检测到正常及异常浆细胞。52例患者骨髓的异常浆细胞表达CD56,12例患者骨髓异常浆细胞表达CD117。不同疾病组间的性别、年龄差异无统计学意义。不同疾病组间的M蛋白类型及浓度、血清受累/非受累游离轻链、骨髓总浆细胞(包括正常及异常浆细胞)/有核细胞、骨髓异常浆细胞/有核细胞、骨髓异常浆细胞/骨髓总浆细胞、骨髓正常浆细胞/骨髓总浆细胞的差异均有统计学意义(P均<0.05)。不同疾病组中异常浆细胞CD56的表达存在统计学差异(P=0.009),而CD117的表达差异无统计学意义。结论轻链淀粉样变性患者骨髓克隆性浆细胞的比例、克隆性浆细胞/骨髓总浆细胞、异常浆细胞CD56表达比例与意义未明单克隆丙种球蛋白血症相仿,而与多发性骨髓瘤患者有显著差异。
