scholarly journals Myelomatous Pleural Effusion in a Relapse Plasmablastic Myeloma

2021 ◽  
Author(s):  
Prachi Tripathi ◽  
Amit Tripathi ◽  
Nidhi Shukla ◽  
Raj Narain Srivastava
Keyword(s):  
Multiple Myeloma ◽  
Pleural Effusion ◽  
Poor Prognosis ◽  
Groin Pain ◽  
Plasma Cells ◽  
Cell Leukaemia ◽  
Rare Presentation ◽  
Present Case Report ◽  
Plasma Cell Leukaemia ◽  
Peripheral Blood Plasma

Multiple myeloma is characterised by malignant proliferation of plasma cells in bone marrow with rare involvement of peripheral blood (plasma cell leukaemia). Sometimes tissue involvement (plasmacytoma) is also seen. However, pleural effusion is very rare in myeloma patients. Pleural effusion in myeloma is usually secondary and reactive in nature. Malignant Myelomatous Pleural Effusion (MPE) is usually associated with poor prognosis. Hereby, the author report a case of a 46-year-old male who presented with groin pain with ureteric calculus. The patient was diagnosed with multiple myeloma with 42% plasma cells in the marrow. On treatment, patient went into remission, however he relapsed twice. On second relapse, the marrow examination showed plasma cells with plasmablastic morphology (24%). Plasmablastic morphology is associated with poor prognosis. The patient also developed pleural effusion. The cytospin smears of the pleural fluid showed clusters of atypical plasma cells (positive for CD38, CD138 and kappa light chain restriction). Thus, the present case report an extremely rare presentation of multiple myeloma with plasmablastic morphology and MPE.

scholarly journals Myelomatous pleural effusion and extensive extraskeletal soft tissue involvement: a rare presentation of clonal plasma cell disorders

2019 ◽  
Vol 12 (9) ◽  
pp. e230560
Author(s):  
Vijay Alexander ◽  
Aditya John Binu ◽  
Sowmya Sathyendra
Keyword(s):  
Multiple Myeloma ◽  
Pleural Effusion ◽  
Soft Tissue ◽  
Plasma Cells ◽  
Previous History ◽  
Rare Presentation ◽  
Left Hemithorax ◽  
Tissue Mass ◽  
Fluid Analysis ◽  
Breath Sounds

A 65-year-old woman presented with a history of progressive dyspnoea, left pleuritic pain, loss of weight and appetite. Previous history was significant for pulmonary tuberculosis diagnosed 10 years before. Physical examination revealed a left supraclavicular soft tissue mass with absent breath sounds over the left hemithorax. Investigations revealed hypercalcemia with albumin:globulin reversal. The bone marrow biopsy was consistent with the diagnosis of multiple myeloma (IgG). Pleural fluid analysis revealed an exudative effusion; cytology showed mature plasma cells and plasmablasts. Serum electrophoresis revealed an M band in the gamma region. Biopsy of the supraclavicular mass revealed plasma cells which were CD 138+ with Kappa light chain restriction. She was initiated on chemotherapy and is currently doing well. Myelomatous pleural effusion is a rare presentation of multiple myeloma.

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.

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.

scholarly journals Myelomatous pleural effusion: A rare presentation of multiple myeloma

2011 ◽  
Vol 2 (3) ◽  
pp. 128-130 ◽  
Author(s):  
Mohammad Shameem ◽  
Jamal Akhtar ◽  
Nafees Ahmad Khan ◽  
Ummul Baneen ◽  
Rakesh Bhargava ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Pleural Effusion ◽  
Rare Presentation

Loss of Spleen Visualization on Whole-Body Diffusion-Weighted Imaging in Patients with Multiple Myeloma Is Associated with Poor Prognosis and High Tumor Burden

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3797-3797
Author(s):  
Toshiki Terao ◽  
Youichi Machida ◽  
Ukihide Tateishi ◽  
Takafumi Tsushima ◽  
Kentaro Narita ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Diffusion Weighted Imaging ◽  
Poor Prognosis ◽  
Tumor Volume ◽  
Plasma Cells ◽  
Myeloma Cell ◽  
Stage Iii ◽  
Whole Body ◽  
Cell Infiltration ◽  
Group A

Abstract Introduction Multiple myeloma (MM) is caused by the proliferation of monoclonal malignant plasma cells in the bone marrow (BM). Imaging has played a major role in visualizing myeloma lesions, assessing tumor volume, and predicting the prognosis. Recently, we reported that the total diffusion volume (tDV), assessed using a pretreatment whole-body diffusion-weighted imaging (WB-DWI), was associated with a high BM plasma cells (BMPCs) and a poor prognosis in patients with MM (Terao. et al., Eur Radiol 2021). During that study, we unexpectedly found the frequent absence of a spleen signal in patients with MM and its reappearance after treatment. Therefore, this study aimed to investigate the association between spleen visualization changes on WB-DWI and myeloma tumor load and prognosis in patients with MM. Methods The data of 96 consecutive patients with symptomatic newly-diagnosed MM (NDMM) at Kameda Medical Center from January 2016 to December 2020, 15 consecutive patients with smoldering MM (sMM), and two autopsied spleens of patients with PC dysplasia were retrospectively reviewed. All patients underwent at least one WB-DWI prior to treatment. The detail of WB-DWI was previously reported (Terao. et al., Eur Radiol 2021). "Loss of spleen visualization" (LSV) was defined as a visual loss of the spleen in maximum intensity projection on the WB-DWI (Fig1). The spleen-to-spinal cord (SC) ratio (SSR) was used in each regions-of-interest (ROI) to compare the signal intensity. The spleen ROIs were defined as non-overlapping ROIs of 30-50 pixels. The SC ROI was the largest ROI without overhanging in the image depicting the maximum size of the spleen. This study was approved by the institutional review board and conducted in accordance with the Declaration of Helsinki. All participants provided informed consent. Results The median patient age was 75.5 years and 81 patients (84.4%) were 65 years or older. Almost all patients (n=91) received proteasome inhibitors (PIs) as remission induction therapy and 33 patients received autologous stem-cell transplantation (ASCT). LSV was observed on the WB-DWI of 56/96 (58.3%) patients with NDMM and in one patient with sMM (1/15, 6.7%). Patients with NDMM and LSV had a higher median BMPC infiltration as assessed by CD138-immunohistochemistry (80.0% vs. 50.0%, p<0.001), a higher median tDV (540.2 mL vs. 137.0 mL, p=0.003), higher rate of ISS stage III (p<0.01), a lower SSR (0.36 vs. 0.96; p<0.001), and lower tDV (540.2 mL vs. 137.0 mL; p=0.003) than those without LSV. The three-year PFS (p=0.27) and three-year OS (p=0.021) were lower in patients with NDMM with LSV (PFS: 51.2% and OS: 72.5%) than in patients without LSV (PFS: 63.4% and OS: 100%). Next, we investigated the spleen signal change of patients who underwent WB-DWI twice or more during treatment (n=74). Of 42 out of the 74 patients with LSV at diagnosis, the spleen during treatment became visible on 31/42 (73.8%) patients. Representative patients with various spleen signal changes during treatment are shown in Figure 3 as group A (n=32; patients without LSV at diagnosis and during treatment), group B (n=31; patients who had LSV at diagnosis but the spleen reappeared after treatment), and group C (n=11; patients who had LSV at diagnosis, and despite treatment response, did not regain the spleen signal). Patients in group C showed significantly worse three-year PFS and OS (not available due to early events) than those in group A and B, even after excluding patients who did not achieve partial response or worse (n=11) (Fig1). In the multivariate analysis, the group C retained its prognostic significance for both PFS (hazard ratio [HR], 1.98, 95% confidence interval [CI] 1.00-3.90, p = 0.049) and OS (HR, 5.16, 95% CI 1.27-21.0, p = 0.022) even after adjustment for age over 70 years and the revised-ISS stage III. At last, to investigate the pathological cause of LSV, we reviewed two patients who underwent autopsies, who had both received WB-DWI within 3 months before their deaths (Fig1). One patient showed diffuse myeloma cell infiltration in the spleen and the other showed the amyloid deposition without myeloma cell infiltration. Conclusion This study showed that LSV and a low SSR on pretreatment WB-DWI are correlated with a high tumor volume and poor prognosis. As patients with LSV during treatment had very poor prognosis, the relationships between LSV and other variables should be investigated. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals A hematologic condition expressed as a lung disease

F1000Research ◽  
2012 ◽  
Vol 1 ◽  
pp. 28 ◽  
Author(s):  
Mónica Egozcue-Dionisi ◽  
José Nieves-Nieves ◽  
Ricardo Fernández-Gonzalez ◽  
Rosángela Fernández-Medero ◽  
Raúl Reyes-Sosa ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Pleural Effusion ◽  
Plasma Cells ◽  
Rare Complication ◽  
Disease Stage ◽  
Fluid Analysis ◽  
Progressive Dyspnea ◽  
Breath Sounds ◽  
The Right ◽  
Pleural Involvement

Pleural involvement secondary to Multiple Myeloma is considered a very rare complication. According to the literature only 1% of these patients develop a myelomatous pleural effusion. We present a case of a 39 year old man with multiple myeloma diagnosed six years prior to our evaluation, which developed progressive dyspnea, dry cough and right pleuritic chest pain two weeks prior to admission. On physical examination the patient had decreased breath sounds over the right posterior hemithorax accompanied by dullness to percussion. The chest radiogram was consistent with a right sided pleural effusion. Pleural fluid analysis revealed the presence of abundant abnormal plasma cells. The patient died four weeks after hospitalization. The presence of myelomatous pleural effusion is considered to be a poor prognostic finding, no matter at what disease stage it develops. So far no definite treatment has been shown to improve survival.

scholarly journals High incidence of chromosome 13 deletion in multiple myeloma detected by multiprobe interphase FISH

Blood ◽  
2000 ◽  
Vol 96 (4) ◽  
pp. 1505-1511 ◽  
Author(s):  
John Shaughnessy ◽  
Erming Tian ◽  
Jeffrey Sawyer ◽  
Klaus Bumm ◽  
Reid Landes ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Poor Prognosis ◽  
Plasma Cells ◽  
Suppressor Gene ◽  
Homozygous Deletion ◽  
Cell Labeling ◽  
Fish Analysis ◽  
Chromosome 13 ◽  
High Incidence ◽  
Homozygous Deletions

Abstract Multiple myeloma (MM) is a hypoproliferative malignancy yielding informative karyotypes in no more than 30% of newly diagnosed cases. Although cytogenetic and molecular deletion of chromosome 13 is associated with poor prognosis, a MM tumor suppressor gene (TSG) has not been identified. To localize a minimal deleted region of chromosome 13, clonotypic plasma cells from 50 consecutive patients with MM were subjected to interphase fluorescence in situ hybridization (FISH) analysis using a panel of 11 probes spanning the entire long arm of chromosome 13. Whereas chromosome 13 abnormalities were absent in plasma cells from 25 normal donors, 86% of patients with MM demonstrated such aberrations. Heterogeneity, both in deletion frequency and extent, was confirmed by simultaneous FISH with 2 chromosome 13 probes. Deletion hot spots were noted at D13S272 (70%) and D13S31 (64%), 2 unlinked loci at 13q14. Homozygous deletions at these loci occurred in 12% (simultaneously in 8%) of the cases. Molecular deletions were found in all 14 patients with morphologic deletions, in 21 of 24 with uninformative karyotypes, and 8 of 12 patients with karyotype abnormalities lacking chromosome 13 deletion. Homozygous deletion of any marker was noted in 4% with low and in 36% with higher plasma cell labeling index greater than 0.4% (P = .01). The absence of increasing deletion incidence and extent with therapy duration suggests that the observed lesions are not induced by treatment. The high incidence and extent of chromosome 13 deletions require the correlation of specific deletion(s) with poor prognosis. These analyses will provide valuable guidance toward cloning of an MM-TSG.

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

Multiple Myeloma Presenting as a Clivus and Brain Stem Plasmacytoma.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5061-5061
Author(s):  
Arash Rezazadeh ◽  
Goetz H. Kloecker ◽  
Damian A. Laber
Keyword(s):  
Multiple Myeloma ◽  
Brain Stem ◽  
Plasma Cell ◽  
Plasma Cells ◽  
Complete Blood Count ◽  
Thoracic Vertebrae ◽  
Rare Presentation ◽  
Organ Systems ◽  
Brain Involvement ◽  
The Brain

Abstract Multiple myeloma is the most common hematological malignancy. MM can affect many organ systems; therefore, it may mimic different clinical syndromes at presentation. We report an extremely rare presentation of MM. CASE REPORT: A 62-year-old man presented with progressive cervical pain for 2 months and double vision for 2 weeks. Physical exam demonstrated a cranial nerve 6 palsy, nuchal rigidity and no other abnormalities. A magnetic resonance image (MRI) of the brain revealed a 5 cm enhancing mass in the clivus which extended into the brain stem, left internal auditory and carotid canal, nasopharynx, sphenoid and cavernous sinuses, without midline shift or hydrocephalus. MRI of the spine showed 2 small enhancing lesions in thoracic vertebrae 4 and 11. Computer tomography of the chest abdomen and pelvis demonstrated a lesion in the sternum and no other abnormalities. Technetium-99 bone scan showed uptake in the skull base mass only. Complete blood count and metabolic profile were normal except for an albumin of 3.2 g/dL. Serum protein electrophoresis with immunofixation revealed hypogammaglobulinemia without a monoclonal protein. 24 hour urine collection demonstrated monoclonal free kappa light chains. Transsphenoidal stereotactic biopsy of the mass showed an atypical plasma cell proliferation with multi-nucleation and mitotic activity, positive for kappa light chain. Bone marrow aspirate revealed 17% of the nucleated cell to be abnormal plasma cells, some with plasmablastic morphology. He improved rapidly with dexamethasone and was referred for radiotherapy. DISCUSSION: The most common neurologic complications of MM are spinal cord compression due to epidural plasmacytomas or vertebral fractures, and peripheral polyneuropathy due to the presence of antibodies directed against myelin structures or due to amyloid deposits. Brain involvement in MM is uncommon. Furthermore, neurologic symptoms due to plasmacytomas located either in the clivus and brain stem are extremely rare. A MEDLINE search from 1950 until July 2005 revealed only 9 cases of MM or plasmacytoma involving the brain stem. Since there is no published autopsy series on MM patients describing the percentage of patients with brain involvement at death, the denominator is difficult to assess. Clinically, extramedullary involvement from MM bears a poor prognosis, and has been described in cases of plasma cell leukemia. We speculate that the aggressive behavior of the MM in our patient might be consistent with the atypical phenotype of the plasma cells. CONCLUSION: The unusual presentation of this patient adds to the medical knowledge about the clinical spectrum of multiple myeloma.

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