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...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 pPCL.CASE SUMMARY A 57-year-old woman with severe anemia,thrombocytopenia,multiple bone destruction,impaired renal function,and 42.7%of peripheral plasma cells is reported.After multiple chemotherapy regimens and chimeric antigen receptor Tcell treatment,the disease progressed again.The patient had very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone therapy.CONCLUSION 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.展开更多
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 (...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.展开更多
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 chemother...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.展开更多
<span style="font-family:Verdana;">Plasma cell leukemia is a rare malignant hemopathy, characterized by a peripheral plasma cell proliferation of more than 20% of the leukocyte formula. A few rare case...<span style="font-family:Verdana;">Plasma cell leukemia is a rare malignant hemopathy, characterized by a peripheral plasma cell proliferation of more than 20% of the leukocyte formula. A few rare cases have been described. From which we report a case of plasma cell leukemia in a 52-year-old Nigerien subject initially in a coma, whose anemia led to the diagnosis in the Onco-Hematology department of the National Hospital of Niamey and the evolution was marked by clinical and biological remission after 3 cures of CTD, but died in an infectious picture after 6 cures.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
Bortezomib, a proteasome inhibitor, is an established therapy against plasma cell leukemia—a variant of plasma cell dyscrasias. Its most frequent side effects have been listed as peripheral neuropathy, neuropathic pa...Bortezomib, a proteasome inhibitor, is an established therapy against plasma cell leukemia—a variant of plasma cell dyscrasias. Its most frequent side effects have been listed as peripheral neuropathy, neuropathic pain, thrombocytopenia, and gastrointestinal problems. Allergic skin reaction is a rarely documented side effect in patients receiving bortezomib-based chemotherapy. A combination therapy consisting of intravenous bortezomib, oral Revlimid tablets, and oral dexamethasone tablets has been prescribed for the patient after his recent diagnosis of plasma cell leukemia. While receiving his third treatment cycle, he developed an allergic reaction (skin rash) involving the neck, and wrists, and mild bilateral eye swelling. The infusion was stopped immediately and then ciprofloxacin ophthalmic solution and oral diphenhydramine 25 mg were prescribed to the patient with significant improvement in his clinical condition. He was temporarily taken off bortezomib. At a follow-up visit a week later, a significant improvement was noticed in his condition. Rash had reduced on neck and wrists, and eye swelling had reduced as well. As of the time of writing this case report, he has been temporarily taken off bortezomib, but other medications in the treatment regimen were continued as prescribed.展开更多
BACKGROUND Plasma cell leukemia(PCL) is diagnosed by the presence of an absolute plasma cell count of > 2 × 109/L or 20% plasma cells in the peripheral blood. Because the incidence of PCL is relatively low, ou...BACKGROUND Plasma cell leukemia(PCL) is diagnosed by the presence of an absolute plasma cell count of > 2 × 109/L or 20% plasma cells in the peripheral blood. Because the incidence of PCL is relatively low, our case report study presents a rare opportunity to describe the clinical and pathological characteristics of this leukemia, as well as different modalities of treatment and outcomes of primary PCL(pPCL).CASE SUMMARY A 56-year-old male with a history of hypertension complained of pain in the left flank area which started four months prior to admission. On admission, his vital signs were stable, and physical examination was completely benign. Laboratory evaluation showed hemoglobin of 5.1 g/dL, white blood cell count of 6.6 cells per cubic millimeter with 16% atypical lymphocytes, and platelet count of 51000 per microliter. Peripheral smear showed more than 10%-15% of plasma cells(Figure1), and flow cytometry of peripheral blood confirmed PCL with 24% plasma cells CD138+. Bone marrow biopsy demonstrated 80% plasma cells(38+, 138+, 117+,10-, 19-, 20-, 56-) with 90% cellularity. The Oncology team was consulted, and VCD therapy was started. After completing therapy at 1, 4, 8, and 11 d, the patient was discharged home. The patient was being considered for a bone marrow transplant evaluation within two months of discharge.CONCLUSION PCL is a rare and aggressive form of leukemia with a poor prognosis. Multicenter studies and clinical trials should be conducted to develop accurate criteria for the initial diagnosis and prompt treatment of this disease.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
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
文摘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 pPCL.CASE SUMMARY A 57-year-old woman with severe anemia,thrombocytopenia,multiple bone destruction,impaired renal function,and 42.7%of peripheral plasma cells is reported.After multiple chemotherapy regimens and chimeric antigen receptor Tcell treatment,the disease progressed again.The patient had very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone therapy.CONCLUSION 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.
文摘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.
文摘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.
文摘<span style="font-family:Verdana;">Plasma cell leukemia is a rare malignant hemopathy, characterized by a peripheral plasma cell proliferation of more than 20% of the leukocyte formula. A few rare cases have been described. From which we report a case of plasma cell leukemia in a 52-year-old Nigerien subject initially in a coma, whose anemia led to the diagnosis in the Onco-Hematology department of the National Hospital of Niamey and the evolution was marked by clinical and biological remission after 3 cures of CTD, but died in an infectious picture after 6 cures.
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Bortezomib, a proteasome inhibitor, is an established therapy against plasma cell leukemia—a variant of plasma cell dyscrasias. Its most frequent side effects have been listed as peripheral neuropathy, neuropathic pain, thrombocytopenia, and gastrointestinal problems. Allergic skin reaction is a rarely documented side effect in patients receiving bortezomib-based chemotherapy. A combination therapy consisting of intravenous bortezomib, oral Revlimid tablets, and oral dexamethasone tablets has been prescribed for the patient after his recent diagnosis of plasma cell leukemia. While receiving his third treatment cycle, he developed an allergic reaction (skin rash) involving the neck, and wrists, and mild bilateral eye swelling. The infusion was stopped immediately and then ciprofloxacin ophthalmic solution and oral diphenhydramine 25 mg were prescribed to the patient with significant improvement in his clinical condition. He was temporarily taken off bortezomib. At a follow-up visit a week later, a significant improvement was noticed in his condition. Rash had reduced on neck and wrists, and eye swelling had reduced as well. As of the time of writing this case report, he has been temporarily taken off bortezomib, but other medications in the treatment regimen were continued as prescribed.
文摘BACKGROUND Plasma cell leukemia(PCL) is diagnosed by the presence of an absolute plasma cell count of > 2 × 109/L or 20% plasma cells in the peripheral blood. Because the incidence of PCL is relatively low, our case report study presents a rare opportunity to describe the clinical and pathological characteristics of this leukemia, as well as different modalities of treatment and outcomes of primary PCL(pPCL).CASE SUMMARY A 56-year-old male with a history of hypertension complained of pain in the left flank area which started four months prior to admission. On admission, his vital signs were stable, and physical examination was completely benign. Laboratory evaluation showed hemoglobin of 5.1 g/dL, white blood cell count of 6.6 cells per cubic millimeter with 16% atypical lymphocytes, and platelet count of 51000 per microliter. Peripheral smear showed more than 10%-15% of plasma cells(Figure1), and flow cytometry of peripheral blood confirmed PCL with 24% plasma cells CD138+. Bone marrow biopsy demonstrated 80% plasma cells(38+, 138+, 117+,10-, 19-, 20-, 56-) with 90% cellularity. The Oncology team was consulted, and VCD therapy was started. After completing therapy at 1, 4, 8, and 11 d, the patient was discharged home. The patient was being considered for a bone marrow transplant evaluation within two months of discharge.CONCLUSION PCL is a rare and aggressive form of leukemia with a poor prognosis. Multicenter studies and clinical trials should be conducted to develop accurate criteria for the initial diagnosis and prompt treatment of this disease.
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.
