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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 7  |  Issue : 2  |  Page : 108-112

Multiple myeloma with different thoracic manifestations: Case series


1 Department of Pulmonary Medicine, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
2 Department of Gynaecolgy, Javitri Hospital, Lucknow, Uttar Pradesh, India

Date of Web Publication28-Jun-2018

Correspondence Address:
Dr. Kumar Abhishek
MRA A 48, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Rae Bareli Road, Lucknow - 226 014, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijrc.ijrc_11_18

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  Abstract 


Multiple myeloma is a malignant proliferation of the plasma cells mainly affecting bone marrow, but other organs may also be involved. Multiple myeloma is rarely associated with lung plasmacytoma. Patients with extramedullary plasmacytomas have coexistent multiple myeloma in only 5% of cases. The incidence of malignant pleural effusion in multiple myeloma is very low and is approximately 1%. In this case series, we report three patients with multiple myeloma, who presented with different thoracic manifestations such as left-sided myelomatous pleural effusion, posterior mediastinal mass with central airway obstruction, and pulmonary consolidation.

Keywords: Central airway obstruction, mediastinal mass, multiple myeloma, myelomatous pleural effusion, pleural-based mass


How to cite this article:
Abhishek K, Ejazi MA, Hashim Z, Chaudhary R, Niharika K. Multiple myeloma with different thoracic manifestations: Case series. Indian J Respir Care 2018;7:108-12

How to cite this URL:
Abhishek K, Ejazi MA, Hashim Z, Chaudhary R, Niharika K. Multiple myeloma with different thoracic manifestations: Case series. Indian J Respir Care [serial online] 2018 [cited 2018 Dec 14];7:108-12. Available from: http://www.ijrconline.org/text.asp?2018/7/2/108/235511




  Introduction Top


Multiple myeloma is characterized by malignant proliferation of plasma cells derived from a single clone. Several organ dysfunctions occur due to the tumor, its products and the host response to it. This leads to several symptoms including bone pain or fracture, renal failure, infections, anemia, hypercalcemia, some clotting abnormalities, and neurological symptoms.[1] Multiple myeloma is rarely associated with lung plasmacytoma.[2],[3] Only 5% of patients with extramedullary plasmacytomas have coexistent multiple myeloma.[4] Pleural effusion due to myeloma is extremely rare (1%–2% cases).[5] Most of the effusions in multiple myeloma are not directly attributed to plasma cell infiltration but due to other causes such as pulmonary thromboembolism, congestive heart failure, and nephrotic syndrome. In this case series of multiple myeloma, patients presented with different thoracic manifestations.


  Case Reports Top


Case 1

A 50-year-old male was diagnosed with multiple myeloma in 2013. At diagnosis, he had complaints of persistent low back pain for which he was evaluated, and serum electrophoresis revealed M band positive in gamma region. Subsequent investigations showed raised beta-2 microglobulin (5.48 mcg/ml), serum kappa-free light chain 35.6 mg/L and lambda-free light chain 12.4 mg/dl, and kappa-to-lambda ratio 2.87. On MDP bone scan, there was an increased uptake in D10– D 12 and L1–L 2 vertebra with collapse of D11, focal uptake in multiple ribs bilaterally, and multiple osteoblastic lesions at the above-mentioned sites. However, bone marrow smear and biopsy were inconclusive. Accordingly, he was advised oral thalidomide and dexamethasone. The patient was doing well at follow-up. In November 2015, he developed shortness of breath, left-sided pleuritic chest pain, and dry cough for 1 month.

There was no history of fever, hemoptysis, and weight loss. On physical examination, he was tachypneic and was using accessory muscles of respiration. He was afebrile with a pulse rate of 98/min, blood pressure of 120/80 mmHg, respiratory rate of 26 breaths/min, and oxygen saturation of 98% on room air. Examination revealed dullness and decreased breath sounds over the whole of the left hemithorax. Chest X-ray showed complete opacity of left-sided hemithorax with mediastinal shift to the right side [Figure 1]a. Laboratory blood investigations showed hemoglobin was 10.0 g/dl, total leukocyte count was 8100 cells/mm 3, platelets were 364 × 103 cells/mm 3, serum albumin was 2.4 g/dl, and total proteins were 11.6 mg/dl, AST 66 IU/L, ALT 304 IU/L, and ALP 72 IU/L. Rest of the investigations were within normal limits. Computerized tomography (CT) of the thorax revealed large (12.1 cm × 8.9 cm × 5 cm) heterogeneous mass involving left lateral chest wall with focal erosion of lateral aspect of 4th rib. Radiating strands of bony spicules were noted arising from outer cortex of lateral aspect of 4th rib. Diffuse rind-like heterogeneously enhancing irregular pleural thickening with moderate loculated left pleural effusion was also seen [Figure 1]b. Pleural fluid was hemorrhagic, exudative, lymphocyte predominant with ADA-49 U/L, and smear and culture for  Mycobacterium tuberculosis Scientific Name Search re negative. Pleural fluid cytology revealed proliferation of plasma cells with eccentric nuclei, perinuclear halo, and abundant amount of basophilic cytoplasm in a hemorrhagic background. A few binucleate forms were also seen (suggestive of plasmacytosis) [Figure 2].
Figure 1: (a): Chest X-ray posteroanterior view: Left-sided homogenous opacity occupying whole hemithorax with shift of mediastinum to opposite side. (b) Contrast-enhanced computerized tomography THORAX: A large (12.1 cm × 8.9 cm × 5.0 cm) heterogeneously involving mass involving left lateral chest wall with focal erosion of lateral aspect of 4th rib. Diffuse rind-like heterogeneously enhancing irregular pleural thickening with moderate loculated left pleural effusion

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Figure 2: Pleural fluid cytology revealed the proliferation of plasma cells with eccentric nuclei, perinuclear halo and abundant amount of basophilic cytoplasm

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On bronchoscopic evaluation, there was extra-luminal compression in the left side with no endobronchial growth. An intercostal drainage tube was inserted in view of increasing dyspnea and rapid refilling of pleural fluid. Thoracoscopic-guided pleural biopsy was done for confirmation of diagnosis. During thoracoscopy, thickened nonsmooth parietal pleura studded with multiple reddish nodules were found. There were dense pleura-parenchymal adhesions with red membranous glistening surface of the pleura. Pleural biopsies were taken from pleural nodules [Figure 3].
Figure 3: Nodular aggregates and sheets of atypical plasma cells with high N: C ratio, eccentric nuclei, and abundant amount of eosinophilic cytoplasm

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Histopathology report revealed nodular aggregates and sheets of atypical plasma cells with high nuclear-cytoplasmic ratio, eccentric nuclei, and abundant amount of eosinophilic cytoplasm, suggestive of plasmacytoma [Figure 4]. In view of malignant myelomatous pleural effusion, pleurodesis was done, and since it was a recurrence of multiple myeloma, second-line drugs were started.
Figure 4: Thoracoscopic appearance of the pleural cavity. Nonsmooth parietal pleura studded with multiple reddish nodules

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Case 2

A 58-year-old male was diagnosed with multiple myeloma in October 2016. The chemotherapy regimen given was bortezomib, thalidomide, and dexamethasone. He was doing well in the follow-up till November 2017, when he developed shortness of breath for 2 weeks, orthopnea for 3 days, cough, and hemoptysis for 2 weeks and stridor for 2 days. There was no history of fever, chest pain, or weight loss. On examination, the patient was found to be tachypneic and was using accessory muscles of respiration. He was afebrile with a pulse rate of 118/min, blood pressure 120/78 mmHg, respiratory rate 36/min, and oxygen saturation of 97% on 4 L/min of oxygen. On examination of the chest, breath sounds were decreased on the right side.

Laboratory investigation showed hemoglobin 8.0 g/dl, total leukocyte count 7000 cells/mm 3, platelets 300 × 103 cells/mm 3, serum albumin 2.6 g/dl, and total proteins 10.6 mg/dl. Liver and kidney function tests were within normal limits. Contrast-enhanced CT (CECT) thorax revealed a large, well-defined homogenous soft-tissue density in azygous region in paravertebral location displacing right main bronchus and bronchus intermedius anteriorly with multiple mediastinal lymphadenopathies [Figure 5]. On videobronchoscopic examination, a mass was seen arising from the right main bronchus and obstructing the distal trachea [Figure 6]. In view of central airway obstruction and stridor, urgent rigid bronchoscopy was done under general anesthesia. Coring of mass was performed, and a silicone Y-stent of size 16 was placed and distal segments identified. Post procedure, symptoms of breathlessness and stridor decreased. Histopathological examination (HPE) showed a tumor comprising of sheets of atypical plasma cells having eccentric-placed nuclei, coarse chromatin, occasional prominent nucleoli, and abundant amount of eosinophilic cytoplasm [Figure 7]. Few binucleate and multinucleate forms were also seen. Features were suggestive of plasmacytoma. The patient was started on second-line drugs for multiple myeloma.
Figure 5: Contrast computerized tomography thorax revealed a large well-defined homogenous soft-tissue density in azygous region in paravertebral location displacing right main bronchus and bronchus intermedius anteriorly with multiple mediastinal lymphadenopathies

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Figure 6: Endobronchial mass was seen arising from the right main bronchus and obstructing the distal trachea

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Figure 7: Section from the right main bronchus show a tumor comprises sheets of atypical plasma cells having eccentric placed nuclei, coarse chromatin, occasional prominent nucleoli, and abundant amount of eosinophilic cytoplasm. Few binucleate and multinucleate forms were also seen. Feature suggestive of plasmacytoma

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Case 3

A 65-year-old male was diagnosed multiple myeloma in January 2017. He presented to us with weight loss, decreased appetite, and dry cough for 2 months. The patient had no previous history of pulmonary tuberculosis, diabetes, and hypertension. On examination, the patient was afebrile with pulse rate of 98 beats/min, blood pressure of 110/80 mmHg, respiratory rate 20 beats/min, and oxygen saturation 98% on room air. On auscultation of the chest, crepitations were present on the left side. Laboratory investigations showed hemoglobin of 10.3 g/dl, total leukocyte count 9000 cells/mm 3, platelets 154 × 103 cells/mm 3, serum albumin 2.5 g/dl, and total proteins 11.0 mg/dl. Liver and kidney function tests were normal. Chest radiograph showed left-sided irregular opacities in lower zone. CECT thorax showed left-sided pleural-based heterogeneous mass with consolidation and rib erosion [Figure 8]. Videobronchoscopy was done and no endobronchial abnormalities were detected. Bronchoalveolar lavage (BAL) sample was taken from left lower lobe. BAL Gene Xpert was positive for mycobacterium tuberculosis and rifampin sensitive. Cytopathology for malignant cells was negative. To confirm the diagnosis, CT-guided biopsy was done. HPE showed largely necrotic tissue with multiple granulomas composed of epitheloid histiocytes, lymphocytes, and multinucleated giant cell. Stain for acid-fast staining was negative [Figure 9]. Antitubercular treatment (HRZE) was initiated, and he was doing well at follow-up.
Figure 8: Contrast-enhanced computerized tomography thorax–left-sided heterogeneous pleural-based mass with consolidation and rib erosion

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Figure 9: Lung biopsy specimen showed largely of necrotic tissue with multiple granulomas composed of epithelioid histiocytes, lymphocytes, and multinucleated giant cell. Stain for acid-fast staining is negative

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  Discussion Top


Multiple myeloma is a neoplastic disorder caused by the proliferation of monoclonal plasma cells and associated with production of large amount of monoclonal immunoglobulins. The classical thoracic manifestation of the disease is involvement of bone of the thoracic cage. Other pulmonary manifestations include consolidation, lung mass, mediastinal lymphadenopathy, interstitial involvement such as reticulonodular pattern and intrapulmonary calcification. In lower airways, plasmacytoma is mainly found in the tracheobronchial tree, hilar structures, and rarely in the lung parenchyma.[6],[7] In a study of 13 patients of multiple myeloma with different thoracic manifestations, six had consolidation, two had mass lesions, two had multiple nodular lesions, and three had interstitial infiltrates.[8]

Pleural effusion is very rare, occurs in <1% and <100 cases have been reported.[9] The pleural effusion develops at an average of 12 months after the diagnosis.[2],[3],[10] [Table 1] lists different thoracic radiology of multiple myeloma seen in different studies.[2],[3],[11],[12],[13],[14]
Table 1: Review of studies describing different thoracic radiologies of multiple myeloma

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Myelomatous pleural effusion is diagnosed by (a) pleural fluid electrophoresis demonstrating monoclonal protein, (b) demonstration of plasma cell in pleural fluid and (c) histological evidence in pleural biopsy.[15] In our case, we found plasma cells in pleural fluid and histopathology of parietal pleura was suggestive of myelomatous origin.

Treatment for myelomatous pleural effusion is not well established. Various antimyeloma agents in combination with local treatment are targeted at pleural effusion, such as pleurodesis, high-dose chemotherapy with peripheral blood stem cell support, intrapleural administration of alpha-interferon, doxorubicin and bortezomib.[16] Thalidomide remains the first-line treatment in multiple myeloma. In various studies, extramedullary plasmacytoma has been found to be less responsive to thalidomide.[17],[18] Our patient had a relapse despite treatment with thalidomide and dexamethasone. Pleural involvement in myeloma is associated with an aggressive course, with poor response to first- or second-line therapies used for myeloma treatment.


  Conclusion Top


The incidence of myelomatous pleural effusion and endobronchial plasmacytoma is rare. Involvement of these structures often signifies a poor prognosis and follows an aggressive natural course. In these situations, rapid diagnosis and treatment with second-line drugs should be considered.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

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Koss MN, Hochholzer L, Moran CA, Frizzera G. Pulmonary plasmacytomas: A clinicopathologic and immunohistochemical study of five cases. Ann Diagn Pathol 1998;2:1-11.  Back to cited text no. 12
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Weber CK, Friedrich JM, Merkle E, Prümmer O, Hoffmeister A, Mattfeldt T, et al. Reversible metastatic pulmonary calcification in a patient with multiple myeloma. Ann Hematol 1996;72:329-32.  Back to cited text no. 13
    
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Saha K, Sit NK, Maji A, Jash D. A case of multiple myeloma with lung involvement. Middle East J Cancer 2012;3:75-8.  Back to cited text no. 14
    
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Rodríguez JN, Pereira A, Martínez JC, Conde J, Pujol E. Pleural effusion in multiple myeloma. Chest 1994;105:622-4.  Back to cited text no. 15
    
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Iannitto E, Minardi V, Tripodo C. Use of intrapleural bortezomib in myelomatous pleural effusion. Br J Haematol 2007;139:621-2.  Back to cited text no. 16
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Nakazato T, Suzuki K, Mihara A, Sanada Y, Kakimoto T. Refractory plasmablastic type myeloma with multiple extramedullary plasmacytomas and massive myelomatous effusion: Remarkable response with a combination of thalidomide and dexamethasone. Intern Med 2009;48:1827-32.  Back to cited text no. 17
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Rosiñol L, Cibeira MT, Bladé J, Esteve J, Aymerich M, Rozman M, et al. Extramedullary multiple myeloma escapes the effect of thalidomide. Haematologica 2004;89:832-6.  Back to cited text no. 18
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9]
 
 
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