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Bilateral Myelomatous Pleural Effusion in a
Patient with IgA Kappa Multiple Myeloma
Rebecca Asiamah , Shiva Kumar Mukkamalla , Tanmay Sahai , Xiao Ping Zhou , Eric Han
, Vincent Armenio
1. Internal Medicine, Roger Williams Medical Center 2. Internal Medicine/Hematology and Oncology,
Roger Williams Medical Center 3. Pathology, Roger Williams Medical Center
Corresponding author: Shiva Kumar Mukkamalla, shiva.mukkamalla@gmail.com
Disclosures can be found in Additional Information at the end of the article
Abstract
Multiple myelomas is a neoplastic plasma cell disorder that accounts for one percent of all
cancers and 13% of hematologic malignancies. Although primarily known to be a bone marrow
disorder, it can metastasize to extramedullary sites or it can present as a solitary
extramedullary plasmacytoma. Primary pleural effusion from myeloma is rare, occurring in less
than one percent of the patients. The following case report highlights a case of bilateral pleural
effusion, directly attributable to multiple myeloma after other causes were ruled out. The
diagnosis was made using cytology and immunohistochemical (IHC) staining of the pleural
fluid. Myelomatous pleural effusion (MPE) is a poor prognostic feature heralding an aggressive
underlying disease state, as represented in this case.
Categories: Pathology, Oncology, Pulmonology
Keywords: multiple myeloma, pleural effusion, bad prognosis
Introduction
Multiple myelomas is a neoplastic plasma cell disorder that is characterized by clonal
proliferation of malignant plasma cells [1]. It accounts for one percent of all cancers in the
United States with an incidence of about four to five cases per 100,000 population, as estimated
in 2016 [2]. Although primarily known to be a bone marrow disorder, it can metastasize to
extramedullary sites or can be present as a solitary extramedullary plasmacytoma [1, 3].
Extramedullary involvement usually occurs in advanced disease. Direct involvement of
myeloma causing pleural effusion is rare and occurs in less than one percent of patients [4]. We
report a case of myelomatous pleural effusion (MPE) in a patient with known immunoglobulin
A (IgA) kappa multiple myeloma that was confirmed by cytology and immunohistochemical
(IHC) staining. Informed consent statement was obtained for this study.
Case Presentation
A 68-year-old male with stage III IgA kappa multiple myeloma who had completed two cycles
of chemotherapy using bortezomib and dexamethasone presented to the hospital for shortness
of breath and respiratory distress. His diagnosis of multiple myeloma was made four months
prior after he was found to have lytic lesions in the right humerus, left tenth thoracic vertebra,
left first lumbar vertebra and right sacrum on imaging performed after a right humeral
pathological fracture. The imaging also revealed a large left chest wall mass, thought to be
likely from extraosseous involvement by myeloma. Staging workup was completed including
skeletal survey and bone marrow biopsy. Bone marrow biopsy showed hypercellularity with
90% involvement by atypical plasma cells which appear as large-sized, binucleated cells with
1 2 1 3 3
2
Open Access Case
Report DOI: 10.7759/cureus.1238
How to cite this article
Asiamah R, Mukkamalla S, Sahai T, et al. (May 10, 2017) Bilateral Myelomatous Pleural Effusion in a
Patient with IgA Kappa Multiple Myeloma. Cureus 9(5): e1238. DOI 10.7759/cureus.1238
dense chromatin and prominent nucleoli (Figure 1). Syndecan-1 (CD138) and kappa light chain
IHC staining showed extensive plasma cell involvement belonging to kappa subtype (Figures 2).
The cytogenetic analysis also revealed multiple chromosomal abnormalities compatible with a
complex cytogenetic profile of male karyotype with derivative chromosomes 17, 14 and 10.
Serum protein electrophoresis detected a monoclonal spike, with immunofixation detecting
IgA kappa type. Serum IgA levels were 2519 mg/dL (normal reference range: 66-436 mg/dL);
immunoglobulin G (IgG) and immunoglobulin M (IgM) levels were decreased. The patient was
determined to have stage III using the revised international staging system (R-ISS). The
treatment plan was to start the patient on nine to 12 weeks of bortezomib, lenalidomide and
dexamethasone velcade, revlimid, low dose (VRd) regimen followed by re-staging. However, the
patient did not receive lenalidomide due to poor performance status and underlying
comorbidities. He then completed two cycles of bortezomib and dexamethasone before
presenting to the hospital. At the time of presentation to the emergency room (ER), the patient
was in respiratory distress and required intubation. On computerized axial tomography (CAT),
on the scan of chest with contrast, the patient was noted to have large bilateral pleural
effusions with severe compressive atelectasis and in addition to previously seen chest wall
mass, there were new widespread pleural and paraspinal metastases (Figure 3). Thoracentesis
was performed and upon analysis, fluid was determined to be exudative containing 17,240
white blood cells with 84% being atypical plasmacytoid cells (Figure 4); Thoracentesis fluid
lactate dehydrogenase (LDH) was 665 U/L, total protein was 4.5 g/dL, albumin was 1.5 g/dL and
potential of hydrogen (pH) was 7.371. Patient’s serum LDH was 475 U/L and total protein was
7.6 g/dL. IHC staining of thoracentesis fluid further revealed CD138, CD31, and kappa light
chain positive plasma cells (Figure 5) which was consistent with MPE. Due to patient’s
respiratory status and progression of his disease, patient’s family decided to terminally
extubate the patient. After extubation patient was placed on comfort measures only (CMO) and
he passed away within the next twelve hours.
FIGURE 1: Hypercellular bone marrow with 90% involvement
by plasma cells
2017 Asiamah et al. Cureus 9(5): e1238. DOI 10.7759/cureus.1238 2 of 7
Discussion
Pleural effusion and diffuse pulmonary involvement from multiple myeloma are rare with a
frequency of only six percent, from all causes of pleural effusions in the setting of multiple
myeloma [5]. Most cases of pleural effusions in multiple myeloma are related to associated
pathologies such as pulmonary embolism, nephrotic syndrome and heart failure [3-6]. Pleural
effusions are directly attributable to multiple myeloma, i.e. occurring from direct plasma cell
infiltration and is seen in less than one percent of cases and less than 100 cases have been
reported worldwide [3]. The majority of cases have immunoglobulin A (IgA) disease,
approximating to 80% of all cases. A likely explanation for the preponderance of cases being
caused by the IgA subtype could be due to increased tendency for this subtype to involve
extraosseous structures including liver, spleen, and lymph nodes. Though the exact
pathogenesis of MPE remains unclear, it is thought to be likely related to direct extension from
thoracic skeletal lesions or chest wall plasmacytomas [3]. In the present case, the patient did
have direct bilateral chest wall and pleural metastatic involvement as seen on computed
tomography (CT) scan, which could explain the etiology of bilateral pleural effusions.
Rodriguez, et al. proposed three potential diagnostic criteria to confirm MPE: (i) demonstration
of monoclonal protein in pleural fluid electrophoresis, (ii) detection of atypical plasma cells in
pleural fluid and (iii) histological confirmation with a pleural biopsy made at autopsy [7]. This
was the first case reported in 1994. Since then, there have been other cases reported, that had
not always performed all the aforementioned procedures, including electrophoresis, cytology,
and biopsy, to confirm the diagnosis. In addition to aforementioned diagnostics, sometimes,
thoracoscopy and bone marrow biopsy have also been performed to diagnose MPE [5]. It is
paramount to exclude all other causes of pleural effusion using clinical judgment prior to
making a definitive diagnosis of MPE. Thus we postulate that although there have been
suggested criteria to attempt and define MPE, they are certainly helpful but not definitive in
reaching the diagnosis of MPE. The expertise and ability of the pathologist to make the
diagnosis from cytology and IHC is also essential and can prevent unnecessary and sometimes
invasive diagnostic testing. In this case, the cytology and IHC staining were sufficient to make
the diagnosis of MPE since the patient was already diagnosed with multiple myeloma. If the
diagnosis remains unclear after cytological and IHC analysis, additional testing including flow
cytometry, protein electrophoresis, and pleural biopsy may be employed to aid in the diagnosis
[6-7].
Conclusions
MPE confers a poor prognosis and is generally seen in advanced diseases. The current case
highlights the importance of appropriate diagnostic workup of patients with a history of
multiple myeloma presenting with pleural effusion. A similar approach can be employed in
identifying MPE without a known diagnosis of multiple myeloma, although a higher index of
suspicion, exclusion of other common etiologies and utilization of all available diagnostic
modalities might be needed. Since the exact pathogenesis of MPE remains unclear, there is a
need to explore predictive and prognostic factors which could further help in the development
of better treatment options for myelomas with MPE.
Additional Information
Disclosures
Human subjects: Consent was obtained by all participants in this study.
2017 Asiamah et al. Cureus 9(5): e1238. DOI 10.7759/cureus.1238 6 of 7
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