Secondary Plasma Cell Leukaemia (original) (raw)
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Primary plasma cell leukaemia: a report of 18 cases
Leukemia Research, 2001
Primary plasma cell leukaemia (P-PCL) is a variant of multiple myeloma (MM) first diagnosed in the leukemic phase, with \2000/mm 3 circulating plasma cells (PCs) and plasmacytosis \ 20% of the white cell count. We investigated the clinical characteristics, therapy, immunophenotype and prognosis factors of 18 patients. Common features at diagnosis were asthenia (seven patients), renal insufficiency (ten patients), bone pain (seven patients), splenomegaly or hepatomegaly (five patients). Hypercalcemia was present at diagnosis in seven patients and was the most potent poor prognosis factor (PB 0.05). Most patients (16 out of 18) were treated with an anthracyclin containing regiment; complete remission was attained in one patient and partial remission in 11 patients while six patients had no response. The median survival time from diagnosis was 7 months (2 -12, 95% confidence interval), but response to treatment had favorable predictive value (PB 0.05). The PCs were usually positive for mature B-cell markers (PCA-1, CD38). They expressed integrins which may increase their binding to endothelial cells and thus participate in PCL physiopathology by favoring plasmocyte extramedullary spread.
Primary plasma cell leukaemia in a 22-year-old woman: A case report
African Journal of Laboratory Medicine, 2015
Introduction: Primary plasma cell leukaemia is a rare and highly aggressive disease that is commonly diagnosed a decade earlier than multiple myeloma, at a median age of 55 years. However, it has also been described in younger patients, as documented in this case report. It often presents with hepatosplenomegaly and lymphadenopathy, whilst the presence of bony lesions are less-commonly seen when compared to multiple myeloma.Case presentation: This report describes the case of a young woman who presented with symptoms of anaemia and a history of menorrhagia. On further careful examination, she was found to have additional signs and symptoms and was later diagnosed with primary plasma cell leukaemia.Management and outcome: On admission, the patient received supportive care measures, including blood products. At diagnosis, a specific chemotherapy regimen was commenced; however, this failed to induce remission. The decision to continue with supportive care only was made and the patient ...
Porto Biomedical Journal, 2016
Plasma cell leukaemia (PCL) is a rare and aggressive disease. Diagnosis is made when there are >2000/#mL circulating plasma cells in peripheral blood or plasmacytosis >20% of total leukocyte count. We report a case of a 51-year old man with generalized bone pain and constitutional symptoms. Blood peripheral smear revealed leukocytosis with 39% plasma cells. Bone marrow biopsy showed plasma cell invasion, which confirmed the diagnosis of PCL. Additionally, the patient had markers of advanced disease. Chemotherapy with vincristine, adriamycin and dexamethasone was started. Despite an initial favourable response, the patient died 2 months later due to an infectious complication. PCL has no established treatment and has a dismal prognosis, requiring the achievement of better data to improve the disease course.
Indian Journal of Hematology and Blood Transfusion, 2012
Plasma cell leukemia (PCL) represents a rare and aggressive form of plasma cell dyscrasia which can be primary (pPCL) or secondary (sPCL). It is diagnosed based on absolute plasma cell count of more than 2.0 9 10 9 /l or a relative proportion of greater than 20% of the peripheral blood leukocyte count. Although pPCL and sPCL share several clinical features, important differences exist. Patients with pPCL are younger; often have extra osseous organ involvement (liver, spleen and other extramedullary sites), increased frequency of renal failure, fast declining performance status and rapid progression to the terminal stage. Patients with sPCL have advanced bone disease. Presented in this article is India data of a short series of five cases of PCL diagnosed at a tertiary care centre from south India over last 5 years. All cases were de novo and had varied spectrum of presentation and so were not suspected to be plasma cell dyscrasia clinically. Detailed hemato-pathological evaluation clinched the diagnosis in all the cases.
Primary Plasma Cell Leukemia: A Case Report and Literature Review
Saudi journal of medicine, 2023
Primary plasma cell leukemia (pPCL) is a rare lymphoproliferative disease characterized by a malignant proliferation of plasma cells in the bone marrow and peripheral blood. It is either primary (in 60% of cases) or a secondary complication of multiple myeloma [1]. In this context, we report the case of a 37-year-old patient with respiratory distress, whose bone marrow smear showed the presence of 64% of dystrophic plasma cells. A serum protein electrophoresis with immunofixation was performed, revealing results in favor of pPCL. A cytogenetic study was not performed due to lack of resources. The patient was put on multidrug therapy with a favorable evolution.
Primary plasma cell leukemia in 32 years old male-A case report
2015
Plasma cell leukemia (PCL) is a rare disease and is the least common variant of multiple myeloma accounting for 2-3% of all plasma cell dyscrasias. Histogenetically, plasma cell leukemia is derived from terminally differentiated B cells. It is diagnosed by presence of absolute plasma cell count >2000 per cm or >20% circulating plasma cells. Here we report a case of plasma cell leukemia, who presented with easy fatigability, weakness and high grade fever since 1 month. Hematological investigation revealed leukocytosis with plasmacytosis (7420/ mm 3). On bone marrow examination, >45% plasma blasts were seen. Biochemical analysis showed high LDH level (4236 U/L) and serum calcium level was also raised (12.3 mg/dl). Final diagnosis of plasma cell leukemia was made. As PCL is rare disease and it is even rarer to find them in a 32 years old. Here we are able to find and document the typical features of PCL.
Journal of Ayub Medical College, Abbottabad : JAMC, 2019
Plasma cell leukaemia (PCL) is a very rare plasma cell dyscrasia with a significant number of monoclonal plasma cells in the peripheral blood. It is diagnosed by the presence of ≥ 2x109 /L plasma cells in the blood or by plasma cells making up ≥20% of the leukocyte count. It can arise from a leukemic transformation of multiple myeloma, or more commonly it can be primary. Regardless of its origin, it carries a very dire prognosis. It responds very poorly to the traditional chemotherapy regimens used for multiple myeloma. We present the case of a 50 years old female who presented to our hospital with a complicated UTI and severe generalized body aches. She was diagnosed as a case of plasma cell leukaemia and was treated with cyclophosphamide and dexamethasone, however she failed to go into remission. Her condition deteriorated and she ultimately passed away 1.5 months after diagnosis. The recommended treatment for PCL is aggressive combination chemotherapy followed by stem cell transp...
Secondary plasma cell leukemia: a multicenter retrospective study of 101 patients
Leukemia & lymphoma, 2018
This multicenter retrospective study included 101 patients (median age 62 years) with secondary plasma cell leukemia (sPCL). The median time from initial multiple myeloma diagnosis to sPCL was 31 months. Fifty-five out of 72 patients (75%) who received any therapy were treated with immunomodulators (IMiDs) and/or proteasome inhibitors (PIs), and 14/72 (19%) underwent salvage autologous stem cell transplantation (ASCT). The overall response rate in patients who received ASCT or PI (either alone or in combination) was higher than in those who did not (93% vs. 36% and 60% vs. 30%, respectively). The median overall survival (OS) in patients who received therapy was 4.2 months (95% CI: 1.3; 8.0) with a 1-year OS of 19%. Platelet count ≤100 × 10/L at sPCL diagnosis was the only independent predictor of a poorer OS in treated patients (HR = 3.98, p = .0001). These findings suggest that patients with sPCL may benefit from salvage ASCT- and PI-based regimens.
Plasma cell leukemia about four cases
2017
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell neoplasm that may either originate de novo (primary PCL) or by leukemic transformation of multiple myeloma (MM) to secondary PCL (sPCL). It is defined by the presence of >2 G/L plasma cells or >20% plasmacytosis of the differential white cell count in the peripheral blood. In this case series, we describe the clinicopathologic, biologic and immunophenotypic, of four patients diagnosed with PCL within a four years period (2013-2016) at CHU Hassan II, Fès, Morocco.
2014
Plasma cell leukemia (PCL) is a rare disease and the least common variant of multiple myeloma. It accounts for 2-3 % of all plasma cell dyscrasias with poor prognosis. We report a 65 year old Sudanese lady presented to oncology clinic with complaint of inability to walk for 2 weeks. Laboratory findings showed anemia, thrombocytopenia and white blood cell count of 49.4x109/L, 62 % of which were plasma cells. Bone marrow aspirate showed infiltration by 75 % plasma cells some with abnormal forms, Immunophenotyping revealed plasma cell population which were CD38 and CD138 positive, surface, cytoplasmic kappa and lambda were negative. Plasma protein electrophoresis was normal denoting it as non-secretory plasma cell leukemia. Unfortunately the patient passed away before doing further investigations or receiving any treatment.