A rare presentation of myelomatous pleural effusion in immunoglobulin g subtype of multiple myeloma

Yawar Yaseen; Parvaiz Ahmad Shah; Bilal Ahmad Mir; Saika Amreen

doi:10.4103/oji.oji_32_20

CASE REPORT

Year : 2021 | Volume : 5 | Issue : 2 | Page : 82-84

A rare presentation of myelomatous pleural effusion in immunoglobulin g subtype of multiple myeloma

Yawar Yaseen¹, Parvaiz Ahmad Shah², Bilal Ahmad Mir², Saika Amreen³
¹ Department of Emergency Medicine, King Fahad Hospital, Medina, Saudi Arabia
² Department of Internal Medicine, GMC, Srinagar, Jammu and Kashmir, India
³ Department of Radiodiagnosis and Imaging, SKIMS, Srinagar, Jammu and Kashmir, India

Date of Submission	16-Jul-2020
Date of Decision	09-Oct-2020
Date of Acceptance	26-May-2021
Date of Web Publication	21-Aug-2021

Correspondence Address:
Yawar Yaseen
GMC, Karan Nagar, Srinagar - 190 008, Jammu and Kashmir
Saudi Arabia

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/oji.oji_32_20

Abstract

Myeloma is a disease of neoplastic plasma cells that synthesize an abnormal amount of immunoglobulins (Igs) or Ig fragments. Multiple myeloma is associated with many complications such as impaired hematopoiesis resulting in anemia and other cytopenias, osteolytic bone lesions, hypercalcemia, and renal dysfunction. However, myelomatous pleural effusion is a rare complication of multiple myeloma, particularly at initial presentation. Herein, we report such a rare complication of myelomatous pleural effusion as initial presentation in a 65-year-old female with IgG type of myeloma.

Keywords: Immunoglobulin G, multiple myeloma, myelomatous pleural effusion

How to cite this article:
Yaseen Y, Shah PA, Mir BA, Amreen S. A rare presentation of myelomatous pleural effusion in immunoglobulin g subtype of multiple myeloma. Oncol J India 2021;5:82-4

How to cite this URL:
Yaseen Y, Shah PA, Mir BA, Amreen S. A rare presentation of myelomatous pleural effusion in immunoglobulin g subtype of multiple myeloma. Oncol J India [serial online] 2021 [cited 2023 Jun 2];5:82-4. Available from: https://www.ojionline.org/text.asp?2021/5/2/82/324233

Introduction

Pleural effusion in multiple myeloma is relatively a rare complication accounting only in 6% of patients and of these myelomatous effusions occurs only in <1% of cases.^[1] Myelomatous effusion is commonly seen in later stages of the disease and presentation as initial finding is rarer. Moreover, among these myelomatous effusions, 80% are associated with IgA disease, whereas the presence of immunoglobulin (Ig) G subtype is further rarer.^[2] With only <100 cases reported in world literature till date, it makes our cases quite a rare one.^[3] We reported a case of myelomatous pleural effusion in a 65-year-old female as an initial finding at the time of diagnosis of multiple myeloma.

Case Report

A 65-year-old female presented to us with a 5-month history of easy fatigability with diffuse bony aches and pains which were progressively worsening with time. Over the last 15 days, she had a history of progressive breathlessness on exertion, chest discomfort, and cough; the breathlessness had increased so much in severity that she was having it even at rest. There was no history of fever or hemoptysis. Her general physical examination revealed pallor, and there was no jaundice or any organomegaly. The chest examination revealed the signs of bilateral pleural effusion. There was a swelling about 4 cm × 3 cm on the right side of the sternum which was hard and was immovable and was tender to palpate. At the time of admission, her hemoglobin was 8.5 g/dl, white blood cell count was 5220/mm³, and platelet count was 322,000/mm³. Serum creatinine and urea were within normal limits, serum calcium was 8.33 mg/dl, corrected calcium was 9.69 mg/dl (correction for albumin), her erythrocyte sedimentation rate was 70 mm, and serum total protein was 13.5 g/dl while serum albumin was 2.3 g/dl. Chest X-ray showed features of bilateral pleural effusion [Figure 1]. Her serum electrophoresis revealed an M spike in the gamma-globulin region. The immunochemistry using nephelometric assay revealed an IgG level of 23.0 g/dl (normal: 6.56–13.51 g/dl), thereby showing to be IgG-type myeloma. Bone marrow aspiration revealed severe plasmacytosis (90% of plasma cells in the marrow). Beta-2 microglobulin level was elevated to 16,737 ng/dl (normal: 609–2366 ng/dl) revealing a high disease burden. The pleural effusion analysis showed an exudative type of pleural effusion with sheets of plasma cells of abnormal morphology and occasional red blood cells [Figure 2]. Pleural fluid adenosine deaminase was within normal limits, and her pleural fluid polymerase chain reaction for Mycobacterium tuberculosis was negative. Pleural fluid lactate dehydrogenase was 1041 U/L. A pleural fluid protein electrophoresis revealed the presence of M spike in the gamma-globulin region same as that of the serum. Contrast-enhanced computed tomography scan of the chest [Figure 3] revealed an anterior chest wall soft-tissue lesion approximately 8.5 cm × 6 cm in size around the sternum with a necrotic center involving the right costochondral junction, infiltrating into pectoral muscle without any associated bone destruction, and the presence of bilateral pleural effusion. The lung parenchyma was normal. The fine-needle aspiration cytology of the chest wall soft-tissue lesion revealed features of plasmacytoma. A diagnosis of multiple myeloma was made based on the International Myeloma Working Group criteria. Moreover, a therapeutic pleural fluid paracentesis was done. The patient was started on combination chemotherapy with bortezomib and dexamethasone and received two cycles.

Figure 1: Chest X-ray of the patient showing bilateral pleural effusion

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Figure 2: Cytology of the pleural fluid showing sheets of plasma cells with occasional red blood cells

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Figure 3: Contrast-enhanced computed tomography coronal view (a and b) demonstrating enhancing solid necrotic midline anterior chest wall mass of size 8.5 cm × 6 cm

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Discussion

Myeloma is a disease of neoplastic plasma cells that synthesize an abnormal amount of Igs or Ig fragments. The diagnosis is based on the revised International Myeloma Working Group criteria,^[4] which require ≥10% of clonal bone marrow plasma cells or a biopsy-proven plasmacytoma plus evidence of one or more multiple myeloma-defining events, namely hypercalcemia, renal failure, anemia, or lytic bone lesion features felt related to the plasma cell disorder, bone marrow clonal plasmacytosis ≥60%, serum involved/uninvolved free light chain (FLC) ratio ≥100 (provided involved FLC is ≥100 mg/L), or >1 focal lesion on magnetic resonance imaging.

Pleural effusion in myeloma is a rare complication which occurs toward the later course of the disease.^[3] The first reported case of pleural effusion in multiple myeloma was published in 1994 by Rodríguez et al.^[5] Around 80% of myeloma patients with pleural effusion are associated with IgA disease, whereas IgG disease-associated myeloma is further rare.^[2]

There are varied mechanisms by which pleural effusion can occur in a myeloma patient such as (a) congestive heart failure secondary to amyloidosis, (b) chronic renal failure, (c) nephrotic syndrome secondary to renal tubular infiltration with paraprotein and development of glomerular damage, (d) direct infiltration of pleural fluid from adjacent tissues, (e) hypoalbuminemia, (f) pulmonary embolism, (g) secondary neoplasm, (h) lymphatic drainage obstruction by tumor infiltration, (i) infection, and (j) pleural myelomatous involvement.^[1],[6] In our case, pleural effusion is occurred due to myelomatous involvement of pleural tissues.

Myelomatous pleural effusion is an extremely rare phenomenon. The mechanism for malignant pleural effusion (MPE) is not clear. However, local invasion from nearby bone lesions or direct infiltration of a pleural plasmacytoma can be the possible mechanisms for the development of MPE which is usually a late finding of multiple myeloma.^[7] Whereas, our case of MPE is the initial presentation at the time of diagnosis of multiple myeloma which is an extremely rare presentation.

As far as the diagnosis of myelomatous pleural effusion is concerned, several methods have been described. The best means of diagnosis is the cytological identification of malignant plasma cells within the pleural fluid as seen in our case. Apart from this exfoliative cytology, ancillary techniques such as fluid electrophoresis and flow cytometric evaluation for plasma cell markers such as CD38, CD138 ,Ki- 67 and light chain restriction are used to confirm the diagnosis of myelomatous pleural effusion. Other methods include a pleural biopsy with immunohistochemical markers.^[8],[9] In our case, pleural fluid protein electrophoresis also revealed the presence of M spike in the gamma-globulin region same as that of the serum and add the diagnostic confirmation of myelomatous pleural efusion.s

Myelomatous pleural effusion is having aggressive behavior. The prognosis is poor, with a median survival time of 4 months.^[10] Therapy is two staged (a) to decrease the symptoms of mechanical complications of the effusion therapeutic paracentesis is undertaken and (b) to lower the tumor burden chemotherapy, especially with bortezomib and dexamethasone, has been found to be somewhat effective in this subgroup of patients though response is less than in patients without effusion.^[11]

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.

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