Myelomatous pleural effusion with plasmablastic morphology and high genetic complexity as isolated extramedullary relapse (original) (raw)

Myelomatous Pleural Effusion

Tanaffos

Multiple myeloma (MM) is a common hematologic malignancy. Pleural effusion is a rare presenting feature of multiple myeloma which carries a poor prognosis. Few cases of multiple myeloma with pleural involvement have been reported in the medical literature. We report a patient with MM diagnosed by cytologic examination of pleural fluid. Our patient was a 64-year old man with multiple myeloma who was receiving chemotherapy. He had developed dry coughs and exertional dyspnea about a month prior to the admission. Radiographic examination showed left pleural effusion with mediastinal shift to the opposite side. Diagnostic thoracentesis of pleural fluid was performed for the patient. Pathologic examination of pleural fluid showed plasmocytes and plasmablast type mononuclear cells with atypical nuclei, consistent with the diagnosis of pleural effusion due to multiple myeloma. In view of multiple etiologies of pleural effusion in malignant diseases, rare etiologies should also be considered...

Pleural Effusion in Multiple Myeloma

Objective Pleural effusion is rarely observed in patients with multiple myeloma (MM). Myeloma cell infiltration or invasion to the pleura is very rare. This study aimed to investigate the clinical characteristics of pleural effusion in patients with MM. Methods We retrospectively reviewed the medical records of patients diagnosed with pleural effusion, MM, and pleural effusion with MM between 2004 and 2014 at Beijing Jishuitan Hospital. The present study included patients with pleural effusion who underwent cytological, bacteriological, biochemical and other testing. The cytopathology of abnormal pleural effusion cells was not diagnostic, thus flow cytometry was performed. MM was defined using the diagnosis standard of NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) 2014 for MM. Results This study included 3,480 pleural effusion patients and 319 MM patients. There were 34 patients with both MM and pleural effusion (17 men and 17 women). The average age was 63 years (range, 48-84 years). Pleural effusion with MM was caused by congestive heart disease, chronic renal failure, hypoalbuminemia, pulmonary infarctions, cirrhosis, pulmonary arterial hypertension, parapneumonic effusion, tuberculous pleural effusion, and myelomatous pleural effusion (MPE). The diagnosis of MPE was confirmed by the detection of myeloma cells in the pleural fluid using flow cytometric analyses. There were only 2 MPE cases in our study. The first MPE case was a woman. The first clinical manifestation was pleural effusion, and the diagnosis was non-secretory MM, DSS stage IIIA (Durie-Salmon staging system); ISS stage I (the International Staging System). The second MPE case was a man who was diagnosed with MM IgA-κ, DSS stage IIIA; ISS stage II. Conclusion The detection rate of MPE was very low. MPE tended to present with yellow exudates and the lack of physical and chemical characteristics. Furthermore, patients with MPE exhibited many yellow nodules on the pleura. These nodules were lobulated and had abundant blood supply. The routine pleural effusion pathological examination had low sensitivity. Flow cytometry may be more useful for improving the detection rate of MPE.

Clinicopathological Profile of Myelomatous Pleural Effusion: Single-center Real-world Experience and Review of Literature

Clinical Lymphoma Myeloma and Leukemia, 2019

Myelomatous pleural effusion (MPE) portends a poorer prognosis in patients with multiple myeloma. It is a rare extramedullary manifestation. There are no reported data from the real-world setting where the patients present late and often have severe/extensive disease process. The incidence of MPE in our setting was 2.65% (11/415) over a study period of 5 years. The median survival after MPE diagnosis was 2.47 months (range, 0.1-5 months). Background: Multiple myeloma (MM) is a hematologic malignancy of plasma cell origin. MM primarily affects bone marrow, but extramedullary sites can also be involved. Myelomatous pleural effusion (MPE) is an atypical and rare complication of MM. We aimed to systematically study the incidence and clinicopathologic profile of patients with MPE in a real-world setting. Patients and Methods: In this retrospective study, 415 consecutive patients with MM managed at a tertiary care center in North India during a study period of January 1, 2010 to December 31, 2015 were evaluated for MPE. The patients with MPE were analyzed for their clinical profile, diagnosis, treatment, and outcomes. Results: Of these 415 patients, 11 (2.65%) patients had MPE. The median age of the study population was 50 years with male preponderance. The majority of these patients had immunoglobin (Ig)G Kappa disease. All patients had higher than International Staging System stage I disease. MPE was a presenting feature at MM diagnosis in 45.45% (n ¼ 5) of the patients, whereas the rest developed MPE during follow-up. MPE presented predominantly (81.8%) as a unilateral effusion. Concurrent extramedullary involvement at other site was seen in 45.45% (n ¼ 5), with 3 (27%) patients having concurrent myelomatous ascites. Six of these were managed aggressively, whereas 5 patients opted for palliation. The outcomes were dismal (90.9% mortality), with a median survival of 2.47 months. Conclusion: MPE is a rare entity, and positive outcomes of therapy remain low with dismal prognosis.

Myelomatous pleural effusion as an initial presenting sign in a case of multiple myeloma

International Journal of Research in Medical Sciences, 2020

Multiple Myeloma (MM) is a neoplastic disease which mainly affects bone marrow but rarely may infiltrate extramedullary tissues as well. Myelomatous pleural effusions (MPE) develop due to extension of plasmacytoid cell lesions of thoracic bones into pleural tissue and directly presenting as an initial sign in a case of MM is exceedingly rare. It indicates poor prognosis, resistance to treatment and more chance of relapse in spite of aggressive chemo-radiotherapy. The effusions of serous cavities in MM generally develop as a late complication of the disease like heart failure, renal failure, pneumonia and amyloidosis. We are reporting a rare case of IgG subtype myelomatous pleural effusion demonstrating abundance of plasmacytoid cells in pleural fluid. Bone marrow smear examination favoured the diagnosis of multiple myeloma with the presence of predominant population of plasma cells with high cellularity. There were also presence of a heterogenous myelomatous mass lesion in the right...

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

Molecular and Clinical Oncology, 2021

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.

Myelomatous Pleural Effusion: Case report and review of the literature

Sultan Qaboos University Medical Journal, 2011

Plasma cell myeloma is an uncommon disease which, besides primarily involving the bone marrow, has a tendency to involve other organs thus presenting with different clinical manifestations. While pleural effusions are infrequent in this disease, true myelomatous pleural effusions are extremely rare. We report the case of a middle-aged Omani man with relapsed plasma cell myeloma who developed bilateral pleural effusions. The diagnosis of myelomatous pleural effusion was made by finding many abnormal plasma cells as well as a high level of a monoclonal protein (IgG κ) in the pleural fluid. In spite of a good initial response to therapy, the patient had progressive disease and died 6 months later with bacterial sepsis. We present a review of the literature that indicates the rarity of such a manifestation and its association with poor prognosis and short survival.

Pleural effusion as the first sign of multiple myeloma

Tüberküloz ve toraks, 2008

Multiple myeloma (MM) is a rare disorder that affects all tissues, except reticuloendothelial tissues, and seldom causes a myelomatous pleural effusion. A 56-year-old male patient attended our clinic complaining of shortness of breath and fatigue for the past month. A posteroanterior chest radiograph revealed a left pleural effusion, which was subsequently assessed as being exudative in nature. Protein electrophoresis demonstrated hypergammaglobulinemia. Serum and pleural fluid immunofixation electrophoresis showed an increase in the IgG and kappa fractions. The Bence-Jones protein level in the 24-hours urine was high. Pleural biopsy showed plasmocyte infiltration. Bone marrow biopsy revealed atypical plasma cell infiltration, some with immature morphology, carrying IgG/Kappa clonality in a diffuse pattern. The patient was diagnosed with IgG/k type MM and underwent chemotherapy with vincristine, doxorubicin, and prednisolone. Complete regression of the pleural effusion was achieved ...

Unilateral Massive Pleural Effusion: A Presentation of Unsuspected Multiple Myeloma

Indian Journal of Hematology and Blood Transfusion, 2010

Pleural effusion in case of multiple myeloma is usually multifactorial but effusion due to myelomatous deposition of pleura is extremely uncommon. We are reporting a unique case of unsuspected multiple myeloma presenting as left sided massive pleural effusion due to myelomatous deposit in pleura and a rare M-band in the alpha-2 region in serum protein electrophoresis. A 61 year lady presented with cough, weakness and progressive shortness of breath. Examination revealed mild pallor and left sided massive pleural effusion that re-accumulated despite treatment. PAP stained smear of pleural fluid showed a large number of plasma cells and pleural biopsy revealed infiltration of plasma cells. Fiber-optic bronchoscopy was inconclusive. Blood examination revealed high value of alpha-2 globulin. Serum electrophoresis showed M band in alpha-2 region and urine electrophoresis showed faint monoclonal light chain pattern. X-ray skull showed multiple punched out osteolytic lesions. Bone marrow examination revealed hypercellular marrow with atypical plasma cells including binucleate forms in large number (above 55% of nucleated cell population).