scholarly journals Primary Plasma Cell Leukaemia: Case report and review of the literature

2018 ◽  
Vol 18 (3) ◽  
pp. 397
Author(s):  
Sarika Singh ◽  
Ashutosh Rath ◽  
Surekha Yadav
Keyword(s):  
Case Report ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Renal Involvement ◽  
Specific Treatment ◽  
Peripheral Blood Smear ◽  
Cell Leukaemia ◽  
Plasma Cell Dyscrasia ◽  
Diagnostic Features ◽  
Plasma Cell Leukaemia

Plasma cell leukaemia (PCL) is one of the most aggressive and rarest forms of plasma cell dyscrasia. However, the diagnostic criteria for this condition have not yet been revised and there is no specific treatment to significantly improve the course of the disease. We report a 69-year-old male who presented to the Lok Nayak Hospital, New Delhi, India, in 2017 with dyspnoea and chest pain. A peripheral blood smear showed an absolute plasma cell count of 2.16 × 109/L. A bone marrow examination showed 61% atypical plasma cells exhibiting kappa light chain restriction. Biochemical investigations were consistent with a diagnosis of primary PCL with renal involvement. Bortezomib-based chemotherapy was initiated, which resulted in an improvement in the patient’s haematological and biochemical parameters. This case report includes a comprehensive review of the clinical and diagnostic features, pathobiology and treatment of PCL.Keywords: Plasma Cell Leukemia; Multiple Myeloma; Plasma Cells; Case Report; India.

2 Translocations, t(11;14) and t(l;6), in a Patient with Plasma Cell Leukaemia and 2 Populations of Plasma Cells

2009 ◽  
Vol 24 (1) ◽  
pp. 42-46 ◽  
Author(s):  
Gösta Gahrton ◽  
Lore Zech ◽  
Kenneth Nillsson ◽  
Berit Lönnqvist ◽  
Anders Carlström
Keyword(s):  
Plasma Cell ◽  
Plasma Cells ◽  
Cell Leukaemia ◽  
Plasma Cell Leukaemia

Rapid and Excellent Response to Hyper CVAD, Particularly with Thalidomide, in Plasma Cell Leukaemia and Long Term Remissions Following Allogeneic Stem Cell Transplantation.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4963-4963 ◽  
Author(s):  
Vidhya Murthy ◽  
Anne Mwirigi ◽  
Susan Ward ◽  
Saad M.B. Rassam
Keyword(s):  
Multiple Myeloma ◽  
Renal Function ◽  
Stem Cell ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Cell Leukaemia ◽  
First Line ◽  
Plasma Cell Disorders ◽  
Plasma Cell Leukaemia

Abstract Abstract 4963 Plasma cell leukemia [PCL] is the most aggressive form of plasma cell neoplasms constituting 2% to 4% of all cases of plasma cell disorders. The presentation may be primary or secondary from an existing multiple myeloma. Approximately 60 to 70% of cases are primary. Immunophenotype of PCL cells differs from the most of other myelomas with more frequent expression of CD20 antigen (50% vs. 17%), and lack CD56 antigen present on the majority of multiple myeloma cells. PCL patients have a higher presenting tumor burden with higher frequencies of anaemia, organomegaly, renal impairment, increased levels of serum lactate dehydrogenase (LDH), β-2 microglobulin and plasma cell proliferative activity. Prognosis of PCL is exceptionally poor with median survival of 6.8 months for patients with primary PCL and 1.3 months for patients with secondary PCL. It responds poorly to conventional myeloma treatment and polychemotherapy approach has yielded some short lived success. Recently, bortezomib has been reported first line in combination with other agents with good initial response. In the UK, bortezomib is not approved as a first line treatment for plasma cell disorders. The Hyper CVAD regimen (fractionated high dose cyclophosphamide and dexamethasone with infusional vincristine and adriamycin) has been developed for acute lymphoblastic leukaemia by the M D Anderson group and has also been shown to be effective in other aggressive B-cell disorders such as mantle cell and Burkitt's lymphoma. There are few single patient anecdotal reports of its efficacy in plasma cell leukaemia. We report three cases of plasma cell leukaemia successfully induced with limited courses of Hyper CVAD chemotherapy and long term remissions achieved with stem cell transplantation. Two men aged 53 and 56 and one woman aged 40 presented with PCL. All were anaemic (median Hb 8.5 g/dl), had impaired renal function, raised beta-2 microglobulin, creatinine, circulating plasma cells and plasmablasts and almost total marrow replacement by plasma cells. Two had IgG kappa paraprotein and one had light chains only. All had weak CD56 expression and two, where tested, were CD38 positive. Cytogenetic analysis was positive in one patient with t(4,14). All received hydration, bisphosphonate and allopurinol preparation before induction with the Hyper CVAD regimen. Two, given Thalidomide as well, achieved morphological complete remission (CR) after one course of therapy with marked reduction of paraprotein and normalisation of renal function. They received one further course of Hyper CVAD before receiving a Melphalan conditioned autlogous stem cell (ASCT) followed by a reduced intensity conditioning (RIC) sibling allogeneiec transplant in one patient. She remains in CR and full donor chimerism 11 months post SCT. The other is being prepared for ASCT to be followed by a RIC voluntary unrelated transplant. The patient with light chain disease achieved partial response (20% plasma cells in the bone marrow) after one course of Hyper CVAD without Thalidomide but a complete response after the second. He was consolidated with a third cycle, followed by a course of mini BEAM and then a RIC sibling allogeneic SCT. He had an early relapse 12 months following his SCT but responded to a course of donor lymphocyte infusion (DLI) and remains in CR 8.5 years from his SCT. Discussion To our knowledge, this is the largest series using this approach in PCL reported in the literature. PCL is a rare but aggressive disease with poor response to conventional therapy and short survival. Hyper CVAD appears to be highly effective in inducing a good response after 1-2 cycles of therapy particularly when combined with thalidomide. It appears that PCL is sensitive to the graft-versus-myeloma effect with long lasting remissions after allogeneic SCT and DLI therapy. Disclosures Rassam: Johnson and Johnson: Research Funding.

scholarly journals Lenalidomide, Melphalan, and Prednisone Association Is an Effective Salvage Therapy in Relapsed Plasma Cell Leukaemia

2009 ◽  
Vol 2009 ◽  
pp. 1-2 ◽  
Author(s):  
Tommasina Guglielmelli ◽  
Roberta Merlini ◽  
Emilia Giugliano ◽  
Giuseppe Saglio
Keyword(s):  
Plasma Cell ◽  
Salvage Therapy ◽  
Plasma Cells ◽  
Single Case ◽  
Case Reports ◽  
Cell Leukaemia ◽  
First Line Therapy ◽  
Plasma Cell Leukaemia ◽  
Plasma Cell Disorder ◽  
Cell Disorder

Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder, characterized by the presence of a peripheral blood absolute plasma cell count of at least2×109/l and more than 20% circulating plasma cells. The prognosis of PCL patients remains poor. Even by using autologous or allogenic transplant procedures, median survival does not exceed 3 years (Saccaro et al., 2005). Thalidomide, bortezomib and lenalidomide (Revlimid) have emerged as high active agents in the treatment of PCL (Johnston and abdalla, 2002; Musto et al., 2007; Finnegan et al., 2006). In particular, Lenalidomide is a structural analogue of thalidomide with similar but more potent biological activity; it is used as first line therapy in MM (Palumbo et al., 2007; Niesvizky et al., 2007), although information regarding its associated use with dexamethasone use as salvage therapy in PCL derives from anecdotal single case reports (Musto et al., 2008). We would like to describe a case of primary PCL with adverse cytogenetic in which excellent response was achieved with the combination of lenalidomide, melphalan, and prednisone as salvage therapy.

scholarly journals Primary plasma cell leukaemia in a 22-year-old woman: A case report

2015 ◽  
Vol 4 (1) ◽  
Author(s):  
Robyn Marshall ◽  
Jenifer Vaughan ◽  
Ria David ◽  
Elise Schapkaitz ◽  
Sergio Carmona ◽  
...  
Keyword(s):  
Case Report ◽  
Supportive Care ◽  
Plasma Cell ◽  
Signs And Symptoms ◽  
Careful Examination ◽  
Cell Leukaemia ◽  
Laboratory Findings ◽  
Plasma Cell Leukaemia ◽  
Bony Lesions ◽  
History Of

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 died seven months later.Discussion: This case study is presented because of its rarity, the young age of the patient at presentation and the unusual clinical and laboratory findings. Persistent anaemia unresponsive to standard treatment should raise the index of suspicion and further investigations directed to exclude malignancies should be considered. (Full article to follow)

Double Syngeneic Transplantation in Plasma Cell Leukaemia.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 5347-5347
Author(s):  
Cristina Barrenetxea ◽  
Manuel Callis ◽  
Javier Bueno ◽  
Antonio Julia ◽  
Jose L. Diaz
Keyword(s):  
Multiple Myeloma ◽  
Complete Remission ◽  
Plasma Cell ◽  
Cell Leukaemia ◽  
Plasma Cell Dyscrasia ◽  
Differential Count ◽  
Neoplastic Disease ◽  
First Case ◽  
Plasma Cell Leukaemia ◽  
Total Body

Abstract INTRODUCTION Plasma cell leukemia (PCL) is a rare disorder, characterized by circulating clonal plasma cell. It accounts for less than 1% of all plasma cell dyscrasias and has a fatal prognosis. It can be primary or secondary, when there was a previously diagnosed plasma cell dyscrasia. The median survival is 7–12 moths in the first case and 2 moths in the second. CASE We present a 54 years old man, diagnosed in November 2003, with multiple myeloma IgA kappa, Bence Jones +, that presented weight loss, retinal hemorrhages, respiratory distress, hepatomegaly, splenomegaly, osteolytic lesions, the cariotype showed hyperploid, chromosome 13 monosomy, translocations t (1,12) and t (4,14). He was treated upfront with tree cycles of a drug’s combination with melphalan, carmustine, vincristine and dexametasone with no response, therefore, was changed to cyclophosphamide, adriamycin, vincristine, methotrexate and citarabine. After two cycles, the patient got complete remission. The patient had a twin brother, and we decided, to do a double transplantation to consolidate the response. The first transplantation was condicionated with carmustine, etoposide, citarabine and melphalan (BEAM), and the second with cyclophosphamide and total body irradiation; the patient remained in complete remission. Eight months after second transplant was admitted to the hospital with disorientation, bradypsiquia, headache, and sensoriomotor loss of lower extremity. Laboratory examination showed differential count of leukocytes, haemoglobin and platelets were normal, LDH increase, absence of monoclonal gammophaty in blood and urine by immunofixation, and the brain’s computerized tomography showed multiple intraparenchymatous lesions, with peripheral edema, in both cerebral hemispheres, confirmed by magnetic nuclear resonance, all of that suggested a neoplastic disease. These lesions were biopsied with the result of multiple myeloma lambda. The patient died one day after biopsy because intracranial hypertension. CONCLUSION Plasma cell disease has poor prognosis, and transplantation could be a good option for some patients, but in our case we only achieve to extend life a few months.

14q32 Abnormalities and 13q Deletions Are Common in Primary Systemic Amyloidosis Using Cytoplasmic Immunoglobulin Fluorescence In Situ Hybridization (cIg-FISH).

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2477-2477 ◽  
Author(s):  
Alan H. Bryce ◽  
Rhett Ketterling ◽  
Morie Gertz ◽  
Steve Zeldenrust ◽  
Martha Lacy ◽  
...  
Keyword(s):  
Plasma Cell ◽  
Prognostic Value ◽  
Statistical Power ◽  
Plasma Cells ◽  
Chromosomal Abnormalities ◽  
Renal Involvement ◽  
Systemic Amyloidosis ◽  
Plasma Cell Dyscrasia ◽  
Female Ratio ◽  
Conventional Cytogenetics

Abstract Background: Primary systemic amyloidosis is an uncommon plasma cell dyscrasia characterized by organ deposition of immunoglobulin light or heavy chain fragments. It is related to the other plasma cell dyscrasias such as multiple myeloma and monoclonal gammopathy of undetermined significance. cIg-FISH on bone marrow plasma cells has become well established in the initial evaluation of myeloma as the t(4;14), t(14;16),13q-, and the 17p- abnormality are of major prognostic value. The corresponding data does not exist in amyloidosis, as there are only three small studies published to date. Methods: Using a prospectively held dysproteinemia database, we reviewed all cases of amyloidosis seen at our institution who had cIg-FISH or cytogenetics performed as part of their routine clinical testing. Three hundred thirty nine patients were identified with amyloidosis and cytogenetic testing. Of these 69 had cIg-FISH testing results. cIg-FISH was performed with fluorescent-tagged antibodies to kappa/lambda immunoglobulin and FISH probes corresponding to the following anomalies: trisomies 3, 7, 9 and 15; del17p/-17; -13/del13q; 14q32 split; and t(11;14). CCND1/IGH. If an IGH rearrangement was detected that did not involve CCNDI, we also evaluated t(4;14) or IGH/FGFR3 and t(14;16) or IGH/c-MAF. These probes constitute the standard screening panel used in MM at our institution. Results: The study population had a median age of 61 years with a male to female ratio of 1.6:1. Median follow up time was 13.5 months. By conventional cytogenetics, 88% of patients had no detectable abnormality. In contrast, only 33% were normal by cIg-FISH. The abnormalities demonstrated were 28% 13-/del13q, 42% IgH translocation, 33% t(11;14), and 3% t(14;16). No patients had a -17/del 17p- or t(4;14). Nine patients had del13q/-13 with an IgH translocation, with 7 of these having t(11;14). Both t(14;16) patients had del 13q. A trend towards increased mortality was seen in patient with any abnormality (p=0.11), but this could not be demonstrated for any single abnormality, likely due to lack of statistical power. There was no correlation between renal involvement or cardiac involvement and cIg-FISH results. Conclusion: The majority of chromosomal abnormalities in amyloidosis are undetected by conventional cytogenetics. With further study, chromosomal abnormalities may carry the same prognostic value in amyloidosis that they currently enjoy in myeloma. This effect may be independent of currently recognized risk factors. Chromosomal abnormalities in Amyloidosis as detected by cIg-FISH Abnormality Amyloidosis % (n=69) Normal 33(23) −13/del13q 28(19) IgH Abnormality 42(29) t(4;14) 0(0) t(11;14) 33(23) t(14;16) 3(2) del 17 0(0) Other 3(2)

Plasma cell leukaemia with t(11;14) not responsive to venetoclax

2021 ◽  
Vol 14 (1) ◽  
pp. e238641
Author(s):  
Mohammed Isaac Abu Zaanona ◽  
Priyank Patel
Keyword(s):  
Multiple Myeloma ◽  
Plasma Cell ◽  
Plasma Cells ◽  
In Situ Hybridisation ◽  
Haematopoietic Stem Cell ◽  
Successful Outcome ◽  
Cell Leukaemia ◽  
Plasma Cell Leukaemia ◽  
History Of ◽  
Early Phase Clinical Trials

A 70-year-old man with medical history of IgG kappa multiple myeloma, initially diagnosed in 2017, underwent induction therapy with carfilzomib, lenalidomide and dexamethasone followed by autologous haematopoietic stem cell transplantation. Nine months following transplant, disease relapsed in the form of plasma cell leukaemia. Fluorescent in situ hybridisation of malignant plasma cells revealed t(11;14). A combination therapy including venetoclax was used based on efficacy data for Bcl-2 inhibitor venetoclax from available early-phase clinical trials in patients with relapsed multiple myeloma with t(11;14) and other published case studies. Unfortunately, the disease was primary refractory, and after further ineffective therapies, the patient did not have a successful outcome.

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