Ultrastructural Characteristics of the Plasma Cells of a Patient with Nonsecretory Myeloma and Plasma Cell Leukemia

1981 ◽  
Vol 66 (2) ◽  
pp. 129-133 ◽  
Author(s):  
H. Bassan ◽  
H. Gutmann ◽  
M.. Djaldetti
Keyword(s):  
Plasma Cell ◽  
Plasma Cells ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Ultrastructural Characteristics

Plasma Cell Leukemia: A Report on Three Patients

1983 ◽  
Vol 69 (6) ◽  
pp. 589-591 ◽  
Author(s):  
Leonardo Pacilli ◽  
Paolo Ferraro ◽  
Silvia Cochi ◽  
Antonio De Laurenzi
Keyword(s):  
Plasma Cell ◽  
Peripheral Blood ◽  
Plasma Cells ◽  
Periodic Acid ◽  
Previous History ◽  
Bone Marrow Aspiration ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Periodic Acid Schiff ◽  
The Third

Three patients with plasma cell leukemia are reported. Two of them had a previous history of myeloma; the third one started with a plasma cell leukemia. Diagnosis was made from the required presence of 20% plasma cells in the peripheral blood. In all 3 cases, bone marrow aspiration and peripheral blood showed plasma cells strongly positive for acid phosphatase and alpha-naphthyl acetate esterase, and negative for periodic acid-Schiff. The first patient was treated with a polychemotherapy regimen that included vincristine, cyclophosphamide, chlorambucil and prednisone, and the second patient with melphalan and prednisone; the third one, who started with plasma cell leukemia, received total body irradiation at the dose of 600 rad. The results of the therapy and survival time, which was never more than 3 months, are in accord with other reports in the literature.

A Pilot Study of Lenalidomide and Dexamethasone as First Line Therapy in Patients with Primary Plasma Cell Leukemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4951-4951
Author(s):  
Pellegrino Musto ◽  
Maria Teresa Petrucci ◽  
Fortunato Morabito ◽  
Francesco Nobile ◽  
Fiorella D'Auria ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Early Response ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Primary Plasma Cell Leukemia

Abstract Abstract 4951 Background Primary Plasma Cell Leukemia (PPCL) is an aggressive, rare variant of multiple myeloma, with clinical, molecular and phenotypic peculiarities, which accounts approximately for 2% to 4% of all myeloma diagnoses. The prognosis of PPCL patients is usually poor, with less than half of patients responding to conventional chemotherapy and a median survival of 7 months. Even by using autologous or allogeneic transplant procedures, survival generally does not exceed three years. Bortezomib has recently provided some promising results in this setting, but, given all the above, new treatments for PPCL are greatly awaited. Lenalidomide is a new immunomodulating agent with great efficacy in multiple myeloma, especially when associated with dexamethasone or other drugs. There are, indeed, some sporadic case reports of PPCL patients treated with lenalidomide as salvage therapy, but no data are currently available on the use of this drug as first line therapy in this disease. Patients and Methods On March, 2009, we started an open label, prospective, multicenter, exploratory, single arm, two-stage study aiming to evaluate safety and antitumor activity of the lenalidomide/low dose dexamethasone combination (Rd), as first line therapy in patients with PPCL. The primary endpoint was early response rate according to International Uniform Criteria. The secondary endpoints were TTP, PFS, OS, percentage of eligible PPCL patients able to collect peripheral blood stem cells and to undergo autologous or allogeneic stem cells transplantation after Rd, serious and severe adverse event rate. According to this study protocol, all eligible, newly diagnosed adult patients with PPCL receive Lenalidomide at a dose of 25 mg daily for 21 days every 28 days. Oral dexamethasone is administered at a dose of 40 mg daily on days 1, 8, 15, and 22 for each 28-day cycle. After 4 cycles, patients who achieve at least PR and not eligible for autologous or allogeneic stem cell transplantation, continue with Rd until clinically appropriate (disease progression, unacceptable toxicity, patient's decision to leave the protocol). In these patients, a maintenance dose of lenalidomide alone equal to 10 mg/die days 1-21 every month is considered after at least 8 full dose Rd cycles. Patients responding after 4 Rd cycles and eligible for transplant procedures, proceed according to single Centre transplant policy. Patients not responding after 4 cycles or progressing under Rd treatment are considered off-study. Appropriate contraception methods and anti-thrombotic prophylaxis are planned. Results Four enrolled patients (1 male, 3 female, mean age 65 years, range 58-69) are currently evaluable for early response. All had unfavourable cytogenetics, including del13, t(4;14), t (14;16), or a complex karyotype. Circulating plasma cells ranged from 4.4 to 9.2 ×10e9/l. One patient had at baseline a moderate degree of renal failure (serum creatinine levels 2 mg/dl). After at least 2 Rd cycles (range 2-4), two PR and two VGPR were achieved (overall response rate 100%), with disappearance or near complete reduction of circulating plasma cells in all cases. The most relevant toxicities were grade 3 neutropenia and pneumonia, occurring in one patient and resolved by appropriate lenalidomide dose reduction, introduction of G-CSF and antibiotic therapy. One patient died in PR, due to causes unrelated to PPCL or treatment. As, according to the Simon, two-stage design adopted, more than two responses occurred within the first ten patients enrolled (stage 1), a total of 22 PPCL subjects will be accrued to complete the stage 2 of the trial. Conclusions These findings, though very preliminary, suggest that the combination of lenalidomide and dexamethasone may be a safe and promising initial therapy for PPCL patients, which can rapidly control the disease and could permit to perform following single patient-adapted therapeutic strategies. An update of this study, including molecular data, a larger number of patients and a longer follow-up, will be presented at the Meeting. Disclosures Musto: Janssen-Cilag: Honoraria; Celgene: Honoraria, Research Funding. Off Label Use: Lenalidomide is approved in Italy for advanced multiple myeloma, not for plasma cell leukemia. This is a clinical trial registered at AIFA (Italian regulatory Agency for Drugs), EudraCT No. 2008-003246 28. Petrucci:Janssen-Cilag: Honoraria; Celgene: Honoraria. Morabito:Celgene: Honoraria; Janssen-Cilag: Honoraria. Cavo:Celgene: Honoraria; Janssen-Cilag: Honoraria. Boccadoro:Celgene: Honoraria; Janssen-Cilag: Honoraria. Palumbo:Celgene: Honoraria; Janssen-Cilag: Honoraria.

scholarly journals Metastatic plasma cell leukemia to the skin: a case report with review of the literature

2021 ◽  
Author(s):  
Elena Pezzolo ◽  
Deborah Saraggi ◽  
Luigi Naldi
Keyword(s):  
Plasma Cell ◽  
Plasma Cells ◽  
Clinical Presentation ◽  
Clinical Course ◽  
Skin Lesions ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Leukemia Cutis ◽  
Aggressive Clinical Course ◽  
New Skin

Plasma cell leukemia (PCL) is a rare variant of leukemia with an aggressive clinical course and a poor prognosis. The cutaneous involvement in PCL is very rare either at clinical presentation of leukemia, namely “leukemia cutis”, or in the metastatic PCL to the skin. We present a case of eruptive multiple cutaneous nodules in a 56-year-old man with metastatic PCL. Histologically, a diffuse dermal and subcutaneous infiltration of ovoid cells with amphophilic cytoplasm and eccentrically located nucleus consistent with plasmacytoid morphology was observed. Neoplastic cells showed strong immunoexpression for CD138 and CD38 consistent with plasma cells phenotype, and loss of expression of CD56. Kappa light chain restriction similar to the phenotype of his PCL was demonstrated. We suggest that the evaluation of new skin lesions in leukemic patients should include a histopathologic examination to establish the diagnosis as soon as possible and a correct management of the disease.

A RARE CASE PRESENTATION OF PRIMARY PLASMA CELL LEUKEMIA WITH REVIEW OF LITERATURE, A CASE REPORT

2021 ◽  
pp. 21-22
Author(s):  
Gaurav Sharma ◽  
Smita Sharma
Keyword(s):  
Case Report ◽  
Plasma Cell ◽  
Peripheral Blood ◽  
Plasma Cells ◽  
Plasma Cell Leukemia ◽  
Rare Entity ◽  
Cell Leukemia ◽  
Case Presentation ◽  
Blood Examination ◽  
Primary Plasma Cell Leukemia

Introduction: Primary Plasma Cell Leukemia (pPCL) is Plasma cell dyscrasia subtype which is rare and aggressive. It carries very poor prognosis. It has unique clinical and laboratory prole. Its rst clinical presentation is leukemia. Peripheral blood examination shows circulating mature looking yet clonal, plasma cells. On molecular and cytogenetic examinations, many aberrations are seen which are unique and make it a distinct entity different from traditional Multiple Myeloma (MM). Case presentation: 37 yr old Indian female presented with difculty in breathing for last 3 months and was initially evaluated for cardiac function & COVID-19 screening. Peripheral blood examination revealed circulating plasma cells. Bone marrow apirate conrmed the initial diagnosis of pPCL. She received BIODRONATE + Inj. BORTEZOMIB + Inj. CYCLOPHOSPHAMIDE + Tab Dexa and was advised for PETscan and skeletal survey. But due to nancial constraints, family decided to go for complete systemic workup in next phase of chemotherapy cycle. She was discharged with advise to be in close follow up and to complete her treatment cycles. Discussion: pPCL needs to be diagnosed promptly to formulate optimal intensive therapy. This atypical presentation with shortness of breath of rare entity of pPCLin such young age emphasizes the need for quick and thorough initial workup. Conclusion: Because of rarity of this disease, there is paucity of literature from India and especially the impact of the standard therapies in resource poor countries. Our case report highlights these challenges for conclusive management of this rare entity

scholarly journals A life-threatening presentation of primary plasma cell leukemia

2021 ◽  
Vol 2 (2) ◽  
Author(s):  
Roma S Fourmanov ◽  
◽  
Annemiek Joosen ◽  
Lidwine Tick ◽  
Heleen S de Lil ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Hemoglobin Concentration ◽  
Plasma Cell Leukemia ◽  
Plasma Cell Dyscrasia ◽  
Bone Lesions ◽  
High Dose ◽  
Cell Leukemia ◽  
Primary Plasma Cell Leukemia

Background: Multiple myeloma is a relatively common type of plasma cell dyscrasia, in which monoclonal plasma cells proliferate. This frequently leads to anemia, renal failure, hypercalcemia and bone lesions. Primary plasma cell leukemia is a much rarer type of plasma cell dyscrasia, with measurable plasma cells in the blood circulation and usually more acute presenting signs. Case: A 57-year-old woman presented to the emergency department with dyspnea. Because of hypoxemia due to a hemoglobin concentration of 3.1 g/dL (1,9 mmol/L), asystole occurred, and cardiopulmonary resuscitation had to be started. The severe anemia turned out to be due to a primary plasma cell leukemia. Palliative treatment was started with combination chemotherapy with VTD (bortezomib, thalidomide and dexamethasone) with a very good partial response, after which she proceeded to an autologous stem cell transplantation with high dose melphalan conditioning. Conclusion: Primary plasma cell leukemia is a plasma cell dyscrasia with both resemblances and differences from the better-known multiple myeloma. It is less common, but presenting signs often are more acute and more severe. Currently there is no curative treatment. Keywords: Plasma cell leukemia; Hematological emergency; Multiple myeloma; VTD.

scholarly journals BRAF V600E Positive Hairy Plasma Cell Leukemia; Are the Cytoplasmic Projections in Plasma Cells Predictive of a Particular Molecular Characterization?

2021 ◽  
Vol In Press (In Press) ◽  
Author(s):  
Elham Jamali ◽  
Ehsan Sarraf Kazerooni ◽  
Akbar Hashemi Tayer ◽  
Reza Ranjbaran
Keyword(s):  
Plasma Cell ◽  
Braf Mutation ◽  
Plasma Cells ◽  
Lymphoid Cells ◽  
Plasma Cell Leukemia ◽  
Braf V600e ◽  
Plasma Cell Dyscrasia ◽  
Cell Leukemia ◽  
Braf Mutations ◽  
Plasma Cell Neoplasms

Introduction: Plasma cell leukemia (PCL) is a rare and clinically aggressive form of plasma cell dyscrasia. Despite the significant role of BRAF mutation in plasma cell neoplasms, this mutation has been rarely considered in these cases. Finding evidence guiding us toward assessing the BRAF mutation in patients with plasma cell neoplasms could help make the suitable decision for targeted therapy. Case Presentation: A 79-year-old man presented with leukocytosis. Peripheral blood smear exhibited marked lymphocytosis and infiltration of about 50% abnormal lymphoid cells with slender cell-surface projections and oval shape nucleus. These findings raised the provisional diagnosis of hairy cell leukemia (HCL) or HCL variants (HCL-v). Molecular analysis confirmed the presence of BRAFV600E mutation, which was in agreement with HCL diagnosis, albeit the flow cytometric assessment of abnormal lymphocytes corroborated PCL. Conclusions: Together with the previous comprehensive analysis regarding the association of cytoplasmic projections and BRAF mutations, our findings could suggest this morphological characteristic in plasma cells (PCs) as an indication for the assessment of BRAF V600E mutation in PC dyscrasias.

Dengue Fever Mimicking Plasma Cell Leukemia

2003 ◽  
Vol 127 (8) ◽  
pp. 1026-1027 ◽  
Author(s):  
John M. Gawoski ◽  
Winnie W. Ooi
Keyword(s):  
Dengue Fever ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Secondary Infection ◽  
Liver Function Tests ◽  
Immunoglobulin M ◽  
Plasma Cell Leukemia ◽  
Cell Leukemia ◽  
Extensive Evaluation ◽  
Elevated Liver Function Tests

Abstract Extreme plasmacytosis in peripheral blood is a rare finding most often associated with plasma cell leukemia but rarely with other malignancies, infectious diseases, or drug reactions. We report the case of a 40-year-old man who was a US expatriate working and traveling in East Asia. He presented with complaints of fever, myalgia, headache, vomiting, and diarrhea of 3 days' duration. An initial evaluation revealed elevated liver function tests, thrombocytopenia (68 × 103/μL), and a white blood cell count of 5.8 × 103/μL with 19% plasma cells (1100/μL), 9% abnormal plasmacytoid lymphocytes (520/μL), 37% polymorphonuclear leukocytes, 3% band forms, 27% lymphocytes, 4% monocytes, and 1% eosinophils. An extensive evaluation was performed, including infectious disease serologies, a bone marrow biopsy, and flow cytometry. During the course of 3 days, his symptoms and hematologic findings improved dramatically. Serologic results were reactive for dengue (immunoglobulin M [IgM] positive, reciprocal IgG titer, 655 360), consistent with a secondary infection of unknown serotype. He remains well 4 years later. To our knowledge, plasmacytosis to this degree has not been described in dengue fever, but atypical lymphocytosis is common. In patients from dengue-endemic areas, even extreme plasmacytosis should be assessed to determine whether it is transient and related to an acute illness before proceeding to an extensive evaluation.

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