Multiple Myeloma: A Rare Presentation As Unilateral Pleural Effusion.

Multiple myeloma (MM) is a hematologic malignancy characterized by the clonal proliferation of plasma cells in the bone marrow. It commonly presents with bone pain, anemia, renal failure, and hypercalcemia. Pleural effusion in MM usually has multiple causes, but it is rare for the effusion to be due to myelomatous deposition of the pleura. Here, we present a rare case in which the patient presented to the outpatient department with a dry cough, breathlessness, and generalized weakness. The patient was diagnosed with MM with myelomatous pleural effusion (MPE), highlighting the importance of considering MM as a differential diagnosis in patients with atypical presentations. MPE indicates a poor prognosis, and early consideration of MPE can lead to an earlier diagnosis and a more effective treatment of MM.

Myelomatous pleural effusion developing after autologous stem cell transplantation in a patient with multiple myeloma: A rare case.

Myelomatous pleural effusion (MPE) is a very rare condition with a poor prognosis. In our case of multiple myeloma (MM) with early recurrence presenting with a MPE, management of the treatment is discussed together with the case presentation. A 35 year old female patient with a diagnosis of lambda light chain MM presented with complaints of dyspnea and pain in the left shoulder 2 months after autologous transplantation. On physical examination, respiratory sounds were decreased in the lower lobe of the left lung and there was dullness. Pleural effusion and plasmacytoma, more prominent on the left, were detected on chest X ray and thorax computed tomography (CT). The pleural fluid collected during therapeutic thoracentesis was examined by flow cytometry, cytology, and peripheral smear examination and as a result, the patient was considered to have early recurrence after autologous transplantation, DRd chemotherapy was immediately started, and clinical and radiological improvement was observed. Pleural effusion developing in patients with MM should be evaluated in terms of MPE. In the presence of MPE, the duration of response to treatment is short, thus effective and dynamic treatment methods for bridging should be used before referral of the patients to clinical trials and hematopoietic stem cell transplantation.

Pleural plasmacytomas in a patient with multiple myeloma relapse.

Extramedullary plasmacytoma with pleural involvement in the setting of relapsed multiple myeloma (MM) is a rare yet serious condition, which is associated with an adverse prognosis. This report describes a patient with MM who was in complete remission but relapsed with multiple pleural plasmacytomas. The diagnosis was established in a timely manner and the patient was started on appropriate treatment.

Clinical Characteristics and Prognosis of Multiple Myeloma With Myelomatous Pleural Effusion: A Retrospective Single-Center Study.

Objectives: Myelomatous pleural effusion is a rare presentation of extramedullary disease in multiple myeloma, which has been reported with dismal prognosis. We aimed to explore whether it has distinctive clinical characteristics and outcomes compared to other anatomic locations of extramedullary involvements. Methods: Multiple myeloma patients diagnosed at our institution from 2010 to 2020 were retrieved retrospectively. In total, 42 pairs of patients with and without extramedullary disease were enrolled, including 13 with myelomatous pleural effusion. The clinical and laboratory parameters were collected and compared between different groups. Prognostic effect of myelomatous pleural effusion was assessed in cox regression model and Kaplan-Meier curves. Results: Myelomatous pleural effusion patients presented a higher level of ß2-microglobulin (P = .041), greater prevalence of multisites extramedullary lesions (69.2% vs 38.0%, P = .036) and International Staging System stage III (76.9% vs 44.8%, P = .016). Median overall survival was 60.6 months in patients without extramedullary disease versus 35.0 months in patients with extramedullary disease (P = .045). Notably, median overall survival was 13.0 months in myelomatous pleural effusion patients versus 37.0 months in other extramedullary disease patients with a significant difference (P = .029). Furtherly, multivariate analysis recognized myelomatous pleural effusion as an independent prognostic indicator (Hazard ratio: 2.669, 95% CI [1.132-6.293], P = .025). Conclusion: Myelomatous pleural effusion patients presented heavier tumor burden and worse outcomes than other extramedullary diseases.

Myelomatous Pleural Effusion: A Rare Occurrence in Multiple Myeloma.

Various factors can cause pleural effusion in multiple myeloma patients. Myelomatous pleural effusion (MPE) is an uncommon but potentially life-threatening complication of multiple myeloma with a poor prognosis. After ruling out all other probable causes, the present case reports MPE in a patient with IgG kappa multiple myeloma.

Myelomatous ascites and pleural effusion in relapsed multiple myeloma.

Extramedullary multiple myeloma is seen in advanced and aggressive disease and occurs due to plasma cell infiltration of sites other than the bone marrow. Myelomatous ascites or pleural effusion is seen in less than 1% of cases and can be differentiated from infectious etiologies based on fluid cytology.

Cytologic and flowcytometric findings in a case of myelomatous pleural effusion: A rare and ominous presentation of multiple myeloma.

Plasma cell myeloma is a hematologic malignancy characterized by multifocal clonal proliferation of plasma cells usually associated with M protein secretion in serum and/or urine and evidence of organ damage. Generally, the presenting features are related to anemia, bone lesions, and renal failure. Pleural effusion is rare in multiple myeloma and when present is often due to nonmalignant causes. Myelomatous pleural effusion that is, effusions directly due to pleural infiltration by plasma cells is even rarer. We present a case of a patient presenting with dyspnea due to myelomatous pleural effusion. The fluid in such a case may be subjected to cytology examination, protein electrophoresis, flowcytometric analysis, and cytogenetics. The case highlights the utility of cytomorphology and flowcytometry in the diagnosis of myelomatous pleural effusion and also highlights that this type of presentation portends a poor prognosis to the patient.

A Case of Plasmablastic Multiple Myeloma With Extramedullary Disease Manifesting as a Myelomatous Pleural Effusion.

Multiple myeloma (MM) is a neoplasm of plasma cell origin characterized by the proliferation of immunoglobulin-producing plasma cells in the bone marrow. Extramedullary disease (EMD) occurs in approximately 10% of patients with MM. Myelomatous pleural effusion (MPE) is a possible manifestation of EMD and has been associated with a poorer prognosis. A 66-year-old female was evaluated after an abnormal serum protein electrophoresis that showed a 2.1 g/dL M-spike in the gamma region, highly suggestive of plasma cell dyscrasia. Imaging subsequently confirmed the bony metastasis. A bone marrow biopsy confirmed plasmablastic MM, and she was started on chemotherapy. She presented three months later with bilateral pleural effusions, with cytology revealing neoplastic plasmacytoid cells. Despite transitioning to dexamethasone, cyclophosphamide, etoposide, and cisplatin (the V-DCEP regimen) due to disease progression, her myeloma remained refractory to treatment, and she expired one month later. MPE in MM is associated with a poor prognosis, with a median overall survival (OS) of 13 months in MPE compared to 37 months in other EMDs. A higher tumor burden and greater multisite extra-medullary lesions are also characteristics of MPE in MM. There is no standard of care for the management of EMD, and salvage regimens such as RVD and V-DCEP are commonly employed. The management of MM with EMD remains a challenge, and more investigation is required before effective treatment regimens may be employed in this setting.

Pleural effusion and multiple myeloma - more than meets the eye: A case report.

Multiple myeloma (MM) accounts for 1% of all cancers. It consists of malignant proliferation of plasma cells, which is often associated with hypersecretion of a monoclonal protein. Pleural effusion (PE) in MM is not an uncommon finding, comprising about 6-14% of patients with MM. The most common causes of MM-associated PE are congestive heart failure, renal failure, parapneumonic effusion and amyloidosis. In <1% of cases, the effusion is a direct result of MM, designated as myelomatous PE (MPE). MPE is usually a diagnosis of exclusion and carries a poor prognosis. Therefore, efforts should be made to optimally detect MPE. The present report describes an MPE associated with IgG/λ MM presenting as a septic shock and renal failure in which a rare diagnosis was made after excluding all other possible etiologies in a complex intensive care patient.

[Successful treatment of myelomatous pleural effusion with daratumumab administration before autologous peripheral stem cell transplantation].

A 50-year-old woman diagnosed with surgically resected plasmacytoma of the ovaries and uterus presented with another plasmacytoma in the pancreas with positive uptake on positron emission tomography (PET) and massive right pleural effusion with plasma cell infiltration (myelomatous pleural effusion). After four courses of the bortezomib, lenalidomide, and dexamethasone regimen as induction therapy, partial response was achieved with reduced myelomatous pleural effusion and negative uptake on PET in the pancreatic plasmacytoma. However, soon after she received bortezomib and high-dose cyclophosphamide for peripheral blood stem cell harvesting, right myelomatous pleural effusion increased without signs of heart failure or infection. Because of the progressive nature of the disease, daratumumab was introduced as 2 courses of daratumumab, lenalidomide, and dexamethasone (DLd) regimen, after which she achieved complete response with disappearance of the pleural effusion. After autologous peripheral blood stem cell transplantation, she received an additional four courses of the DLd regimen as consolidation therapy. She maintained relapse-free survival for two years with maintenance therapy containing daratumumab and dose-reduced lenalidomide. Our case may suggest the usefulness of daratumumab before autologous peripheral stem cell transplantation for relapsed/refractory myelomatous pleural effusion.

Transient Pleural Fluid Infiltration by Clonal Plasma Cells Associated with Pulmonary Tuberculosis.

Pleural effusion is a rare presentation of plasma cell myeloma, occurring in around 6% of patients during the course of their disease, most commonly as a consequence of a concurrent disease process like heart failure secondary to amyloid deposition. Direct infiltration of the pleural fluid by malignant cells leading to myelomatous pleural effusion is a rare mechanism occurring in less than 1% of patients with plasma cell myeloma, and it is associated with a worse prognosis. There are few case reports of myelomatous pleural effusion as an initial presentation of multiple myeloma. Pleural fluid infiltration by monoclonal plasma cells in the absence of an underlying plasma cell myeloma was not reported before in the literature. Tuberculosis is a known cause of polyclonal gammaglobulinemia, however few case reports described the coexistence of monoclonal gammopathy of undetermined significance and tuberculosis. Here we present an interesting case of pleural fluid infiltration by an abnormal looking clonal plasma cells associated with active pulmonary tuberculosis and parapneumonic effusion in a patient with a background of acute myeloid leukemia. Interestingly, the clonal plasma cell proliferation was confined to the pleural fluid without any evidence of an underlying plasma cell neoplasms (including monoclonal gammopathy of undetermined significance and plasmacytomas). Since our patient had an underlying meyloid neoplasm, we though about the possibility of secondary malignancy. However, in almost all patients with coexisting myeloid and plasma cell neoplasms, myeloid neoplasms developed following chemotherapeutic treatment of plasma cell neoplasms not the other way around. Given that, one must conclude localized extramedullary (pleural) plasma cell proliferation probably represents a transient reactive process to pulmonary tuberculosis which is an extremely rare phenomenon and not described before.

Myelomatous Pleural Effusion: A Rare Involvement in Myeloma.

Extramedullary disease (EMD) incidence is between 7% and 18% in multiple myeloma. Overall survival of patients who develop EMD is significantly shorter than that of patients without EMD. Malignant myelomatous pleural effusions (MPEs) are rarely observed, occurring in less than 1% of cases. The diagnosis of MPE was confirmed by the detection of myeloma cells in the pleural fluid using flow cytometric analyses. We present a case of a 67-year-old male patient with IgG-kappa myelom. After a few line treatment regimens, he was admitted to hospital with back pain and blurred consciousness, and pleural effusion was detected. Pleural fluid analysis showed malignant plasma cells. It is a rare presentation of multiple myeloma, but important in diagnosis.

Pleural effusion in 11:14 translocation q1 multiple myeloma in the setting of proteasome inhibitor presents therapeutic complexity.

BACKGROUND: Primary malignant pleural effusion has been reported in about 134 cases of multiple myeloma (MM). Associated pleural effusions in cases of MM portend a poor prognosis and identifying them is highly relevant. Reported is the case of a man diagnosed with MM who developed primary myelomatous pleural effusion in the setting of multiple relapses and subsequent mortality within 2 months of the pleural effusion diagnosis. PRESENTATION: A 61-year-old African American man was diagnosed with MM in 2011. He received induction therapy of lenalidomide and dexamethasone and an autologous stem cell transplant in 2012. Over the next 5 years, the patient went through alternating periods of remission and relapse that were treated with two rounds of thoracic spine radiation therapy and chemotherapeutic agents. In September 2017, the patient presented with worsening dyspnea and was found to have pleural effusion. Fluid analysis showed plasma cell dyscrasia. Fluid drainage was performed, then the patient was discharged after 1 week which was followed by rapid re-accumulation of fluid and rehospitalization about 10 days after discharge. The patient passed away a few weeks after the second admission. CONCLUSION: Pleural effusion carries a differential diagnosis which may include malignancy but is commonly thought to be less specific to multiple myeloma but should still remain in the differential diagnosis. To our knowledge, this is the first case of myelomatous pleural effusion (MPE) that was reported after multiple relapses of MM. MPE is a very rare complication of MM, and its presence is a strong indicator of imminent mortality and need for comfort care in case of multiple relapses. End-stage pleural effusion in MM in the setting of proteasome inhibitor adds more therapeutic and diagnostic challenges.

Characteristics of patients with myelomatous pleural effusion. A systematic review. / Características de los pacientes con derrame pleural mielomatoso. Revisión sistemática.

BACKGROUND: Myelomatous pleural effusion (MPE) is rare in multiple myeloma, and therefore its characteristics are not well defined. METHODS: A systematic review (4 online databases) was conducted of articles describing the clinical characteristics of patients with MPE, pleural effusion's biochemical characteristics and treatment efficacy. We analysed isolated cases and small retrospective series. RESULTS: We included 98 articles with a total of 153 patients with MPE. The median age was 62years, and the ratio of males to females was 1.7:1. The most common symptoms were dyspnoea (98.8%), bone pain (100%) and chest pain (95.3%), and the most relevant abnormal laboratory test results were anaemia (90.1%) and renal failure (53.8%). MPE was predominantly unilateral (63.9%) and covered more than two-thirds of the hemithorax (54.5%). The pleural fluid (PF) had a haematologic/serohaematologic appearance (87%) and met the criteria for lymphocytic (78.6%) exudate (94.7%). The most cost-effective diagnostic procedures were pleural cytology (95.9%) and the observation of a monoclonal peak in the PF (94.7%). In a significant proportion of patients (54.7%), the MPE did not respond to treatment, and the best response was achieved when chemotherapy (with/without corticosteroids) was combined with therapeutic thoracentesis, chest drainage or pleurodesis. CONCLUSIONS: MPE predominates in middle to older age men, is symptomatic and is usually unilateral. PF is an exudate with a haemorrhagic appearance, and the most cost-effective diagnostic procedure is pleural cytology. Treatment response is unfavourable in more than half of patients.

Catch me if you can-How to make the best use of pleural effusion.

Etiology diagnosis of pleural effusion is a commonly encountered yet challenging problem. How to make the best use of pleural effusion evokes thinking. Myelomatous pleural effusion (MPE) is a rare condition and has been reported sporadically. We report an extremely rare case of multiple myeloma (MM) [IgD-lambda (λ) type, stage III B] with MPE as initial presentation and complicated with massive pericardial effusions during the progression of the disease. Cytological identification of malignant plasma cells in pleural effusion is the most direct method to diagnose MPE. What we learn from this case is that cell block can increase the sensitivity of detecting malignancies compared with conventional smear. Doctors should make best use of pleural effusion before further invasive procedure.

Myelomatous pleural effusion: A rare case entity reported from a tertiary care cancer center in South India.

Multiple myeloma (MM) is a plasma cell neoplasm and constitutes 10% of hematologic malignancies. Malignant myelomatous pleural effusions are very rare and occur in <1% of cases of MM. In this article, we report a rare case of a patient who initially presented with pleural effusion and was subsequently found to be secondary to MM with an underlying raised IgG paraprotein. The patient symptomatically improved and was in partial remission with palliative radiotherapy, VTD chemotherapy, and bisphosphonates.

Flow Cytometry in Diagnosis of Myelomatous Pleural Effusion: A Case Report.

Plasma cell myeloma is a multifocal plasma cell neoplasm associated with increased monoclonal protein in serum and/or urine. Pleural effusions in patients with myeloma are uncommon (6 %). However, effusions due to direct infiltration of the pleura by plasma cells (myelomatous pleural effusion) are extremely rare (<1 %) and usually seen with IgA myeloma. The diagnosis of such cases requires pleural fluid cytology, electrophoresis or pleural biopsy. We present a case of myelomatous pleural effusion diagnosed using flow cytometry immunophenotyping in addition to the pleural fluid cytology. A 45 year old female was diagnosed as plasma cell myeloma (IgG kappa) in 2007. She received multiple lines of therapy during the course of her treatment including thalidomide, dexamethasone, lenalidomide, bortezomib, and doxorubicin based regimens. However, the patient had progressive extramedullary disease and developed pleural effusion in 2014. Cytological examination of the pleural fluid showed degenerative changes. Few preserved areas showed mononuclear cells including morphologically abnormal plasma cells. Immunophenotyping of these cells by flow cytometry revealed a pattern indicating neoplastic plasma cells. There was expression of CD38, CD138, and CD56, with absence of CD19, CD10 and CD45. This confirmed the diagnosis of myelomatous pleural effusion. Subsequently, the patient was offered a dexamethasone, cyclophosphamide, etoposide and cisplatin based regimen but, she declined further treatment and succumbed to her disease 3 months later. Myelomatous pleural effusion is a rare complication of plasma cell myeloma. Flow cytometry can be used as an adjunctive technique in its diagnosis particularly in cases with equivocal cytology and electrophoresis findings.