ABSTRACT

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 in order to treat the effusion appropriately. (Tanaffos 2007; 6(2): 68-72)

Key words: Multiple myeloma, Pleural effusion, Plasmablas

شیردل, عباس

عطاران, داود

غیاثی, تقی

قبادی مراللو, حسن

English

ArUMS Digital Repository (سامانه اطلاعات زیست پزشکی و سلامت)

Tanaffos (2007) 6(2), 68-72 
2007 NRITLD, National Research Institute of Tuberculosis and Lung Disease, Iran  
 
 
Myelomatous Pleural Effusion 
 
 
Abbas Shirdel 
1
, Davood Attaran 
2
, Hassan Ghobadi 
2
, Taghi Ghiasi 
3
 
1 Department of Hematology, 2 Department of Pulmonary Medicine, 3 Department of Pathology, Ghaem Hospital, Mashhad University of 
Medical Sciences, MASHHAD-IRAN. 
 
ABSTRACT 
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 in order to treat the effusion appropriately. (Tanaffos 2007; 6(2): 68-72) 
Key words: Multiple myeloma, Pleural effusion, Plasmablast 
 
 
INTRODUCTION 
Multiple myeloma (MM) comprises about 1% of 
all malignancies as well as 10% of hematologic 
malignancies. The mean age of affliction is 65 years 
(1). 
Multiple myeloma is a malignant proliferation of 
β (type) plasma cells which results in production of 
monoclonal immunoglobulins. This disease causes 
clinical signs and symptoms due to the tumoral mass. 
Accumulation of protein in organs (kidneys and 
heart) causes the disease manifestation and reduces 
the secretion of natural immunoglobulins by            
the   normal   plasma cells which eventually results in  
 
Correspondence to: Attaran D 
Address: Ghaem Hospital, Mashhad University of Medical Sciences, 
MASHHAD-IRAN 
Email address: d-attaran@yahoo.com 
Received: 27 January 2007 
Accepted: 3 March 2007 
 
hypogammaglobulinemia. Hematogenesis is 
deranged and osteolytic lesions appear resulting in 
hypercalcemia and renal dysfunction (1). Clinical 
signs and symptoms are heterogenous and vary from 
a slowly progressive form to a very aggressive type 
with extramedullary symptoms (1). Prognosis is 
correlated with the level of β2 microglobulin (β2M), 
C-reactive protein (CRP), lactate dehydrognase 
(LDH), albumin, hemoglobin, platelets and the 
tumoral mass (5). Extramedullary symptoms and 
signs include involvement of liver, lymph nodes, 
spleen, kidneys, skin, meninges and the brain. 
Malignant pleural effusion is extremely rare in 
multiple myeloma patients and the first case was 
reported in 1994 (3). Almost 80 cases of 
myelomatous pleural effusion have been reported in 
the medical literature up to 2005 (7). 
CASE REPORT 
Shirdel A, et al.   69     
Tanaffos 2007; 6(2):68-72 
CASE REPORT 
A retired 64-year old man residing in Mashhad 
presented to our hospital complaining of diffuse pain 
of extremities and swelling of the left ankle joint. 
The patient was suffering from pain in his legs and 
his left ankle joint for eight months. Nocturnal pain 
was not relieved by ordinary analgesics. 
The patient developed skin lesions in the form of 
2-10 mm purple plaques two months prior to 
admission. He also gave a history of non-productive 
coughs and exertional dyspnea one month earlier. 
The patient did not recall any specific diseases in 
his past medical history. In review of systems, the 
patient mentioned a 3-5 kg weight loss during the last 
4 months which was associated with fatigue and 
malaise. He denied having fever or sweating. 
Diffuse skin lesions in the form of purple plaques 
were detected all over the body which were painless 
and asymptomatic. Jugular vein pressure (JVP) and 
thyroid examination were normal. Auscultation of 
the heart was unremarkable. Decreased breath sounds 
in the lower lobe of the left lung were found upon 
auscultation. The abdomen was soft without 
organomegaly. Peripheral pulses were normal in the 
extremities. Ankle swelling was not associated with 
any limitation of movement. 
The patient underwent evaluation and 
paracentesis of synovial fluid which was mostly 
lymphocytic and negative for TB bacilli, crystals or 
malignant cells. Thyroid, liver, and kidney function 
tests and serum electrolytes were all normal. Serum 
protein electrophoresis was performed for the patient 
which showed a monoclonal gammopathy. Urinalysis 
(U/A) was negative for Bence Jones protein, but 
contained lambda M component monoclonal IgG. In 
the bone scan osteolytic lesions were detected in the 
extremities. 
In the complete blood count (CBC) normocytic 
anemia with Hb=7 g/100cc, WBC= 2440/mm
3
 
(PMN: 54%, Lymph= 34%) and platelet= 
56,000/mm
3
 was noted. ESR was 146mm/h and PPD 
test was negative. Abdominal sonography was 
normal. In the serum immunoelectrophoresis, 
albumin and betaglobulin had decreased to 3.2 gr/100 
ml and 0.83 gr/100 ml and gammaglobulin had 
increased to 6.1 gr/100 ml (monoclonal 
gammopathy). On radiography of the skull, sclerotic 
lesions were detected in some areas. No lytic lesions 
were detected. 
Bone marrow biopsy was performed for the 
patient which contained: 
PMNs= 26%            bands=6%            blasts= 1-2% 
erythroblasts= 32%     plasma cells=15%     
lymphocytes= 19% 
Also, the bone marrow contained mononuclear 
cells and cells with side nuclei and obvious atypical 
changes. In the serum protein electrophoresis, the 
Kappa component was negative but the Lambda 
component was positive in 95% of the cells. The 
patient underwent systemic therapy for diagnosis of 
plasma cell myeloma. On further evaluation, CRP 
was positive, level of β2 microglobulin had increased 
to 11.8 (normal range 0-3), serum albumin 3.2 
gr/100, LDH=682 and alkaline phosphatase 86. 
According to the aforementioned rates, the patient 
was in stage 3 of IPI staging (considering the level of 
β2 microglobulin more than 5.5) (4-5). Also, the skin 
biopsy reported plasmocytoma. 
Chest radiography obtained 4 months prior to 
admission was normal. But considering the patient's 
symptoms including cough and dyspnea another 
chest x ray was obtained at the time of admission on 
which heart size was normal and left pleural effusion 
was observed. No lesion was detected in the 
parenchyma and soft tissue and thoracic cavity were 
normal (Figure 1). No parenchymal involvement was 
seen on the CT-Scan. 
Due to the presence of pleural fluid, diagnostic 
paracentesis of the pleural fluid was performed for 
the patient. The fluid was cloudy and bloody and 
contained the following elements: 
Protein=2.6 gr/dl       glucose= 62 mg/dl 
cholesterol= 52 mg/dl   WBC=420 (L=85%   P=16%) 
LDH= 754      hematocrit= 0.2% 
70   Myelomatous Pleural Effusion 
Tanaffos 2007; 6(2):68-72 
 
 
 
 
 
 
 
 
 
 
 
Figure 1. Chest-x-ray of patient. 
 
Cytologic examination of the pleural fluid 
revealed a large number of plasmocyte and 
plasmoblast type mononuclear cells with large 
atypical nuclei which was consistent with the 
diagnosis of pleural fluid plasmocytoma (Figures 2 
and 3).  
 
 
 
 
 
 
 
 
 
 
 
Figure 2. Cytologic examination of the pleural fluid. 
 
 
 
 
 
 
 
 
 
 
Figure 3. Cytologic examination of the pleural fluid. 
Pleural biopsy showed diffuse fibrosis, 
hypercellular calcified centers containing fibroblasts 
and fibrinous pleuritis. Pleural fluid was negative for 
TB (smear and culture). Pleural fluid adenosine 
deaminase (ADA) activity was normal and 
polymerase chain reaction (PCR) was negative for 
TB. Systemic chemotherapy was continued for the 
patient and due to the development of dyspnea, 
therapeutic paracentesis of pleural fluid was 
performed twice resulting in relative improvement of 
dyspnea. On both occasions, evaluation of pleural 
fluid was negative for TB. The patient underwent 
thoracoscopy and pleurodesis because of the 
recurrence of pleural effusion causing dyspnea. 
 
DISCUSSION AND CONCLUSION 
Pleural effusion is a rare manifestation of multiple 
myeloma. The first case of pleural effusion and 
involvement of serous cavities in multiple myeloma 
was reported in the Chest Journal in 1994 by 
Rodriguez and colleagues. 
It was the first case of multiple myeloma with 
raised IgA light chain Kappa (IgA-k). In the 
literature, 80 percent of myelomatous pleural 
effusions (MPE) were due to IgA MM (3-7). 
Pleural involvement in these cases carries a poor 
prognosis. Involvement of serous cavities (pleural 
effusion and ascites) in myeloma currently has no 
standard effective treatment regimen (2). 
Chemotherapy and pleurodesis are effective in 
resolving malignant pleural effusion but survival is 
short (7). Pleural effusions in MM occur in about 6 
percent of patients due to several etiologies requiring 
different types of therapy. Myelomatous pleural 
effusion is rare and occurs in less than 1% of cases. 
Pulmonary and pleural infiltrations are rare and 
might originate from the soft tissue plasmacytoma or  
present as a parenchymal mass, airway lesion or 
pleural effusion (8-11). 
Myelomatous pleural effusion develops within 12 
Shirdel A, et al.   71     
Tanaffos 2007; 6(2):68-72 
months of diagnosis of MM. All patients had high-
risk disease (2-7). The initial diagnosis of 
myelomatous pleural effusion is based on positive 
cytology. Pleural tissue infiltration is found on 
pleural biopsy (7). 
In every patient with malignancy who develops 
pleural effusion, diagnostic paracentesis of pleural 
fluid should be performed. Pleural effusion in 
myeloma may be due to plasma cell infiltration of the 
pleura, pulmonary embolism, nephrotic syndrome, 
secondary neoplasms, congestive heart failure 
concomitant with primary malignant disease, 
infections (parapneumonia), tuberculosis or other 
exudative or transudative causes (8, 9). 
In view of these multiple etiologies, diagnostic 
thoracentesis should be performed to treat the 
effusion appropriately. Pleural effusion can be a 
complication of myelomatous ascites (2). In our 
patient, abdominal sonography was normal without 
ascites. 
Extramedullary symptoms of multiple myeloma 
consist of involvement of organs such as liver, lymph 
nodes, spleen, kidneys, skin, brain, meninges and 
blood disorders (hemorrhage and thrombosis). 
Multiple myeloma is an uncommon cause of 
malignant pleural effusion. Electrophoresis and 
immunoelectrophoresis of the pleural fluid may be 
diagnostic. The most common thoracic disorders 
associated with myeloma are bone involvement or 
pulmonary infiltrates secondary to complicating 
infectious processes. Primary thoracic involvement 
by myeloma occurs in less than 1% of the cases. 
Reported patterns of extramedullary plasmacytoma 
of the thorax include lung mass, multiple pulmonary 
nodules, diffuse reticulonodular infiltration by 
myeloma cells, lymphadenopathy and mediastinal 
mass, pleural effusion and nodular pleural thickening 
and tracheobronchial infiltration (6). If the fluid is 
exudative (according to Mr. Light's criteria) the next 
step should be the cytologic examination of the 
pleural fluid, determining cellular differentiation and 
pleural biopsy if required. In this patient, cytologic 
examination was performed after aspiration of the 
pleural fluid which was positive for palsmacytoma. 
To our knowledge, myelomatous pleural effusion has 
never been reported in Iran and this is the first case 
of multiple myeloma presenting with malignant 
plasmacytoma effusion. Malignant pleural effusion 
(MPE) in patients with MM heralds a poor prognosis 
and short survival. These patients usually die about 
four months from onset of MPE (7). 
The first case of multiple myeloma was reportedly 
IgA-Kappa (3); whereas our patient had IgA-lambda 
multiple myeloma. Myelomatous pleural effusion in 
multiple myeloma is rare. Approximately 80 cases 
have been reported (till 2005) (7). Systemic 
chemotherapy in these patients is effective in 
resolving pleural effusion. However, pleurodesis may 
be required as well. 
Our patient improved clinically after two 
thoracentesis. After a few days he developed 
pulmonary symptoms again (dyspnea and cough) 
which was treated with pleurodesis. Pleural biopsy 
revealed fibrinous pleuritis. Determination of the 
etiology of pleural effusion is significantly important 
in order to treat the effusion appropriately. Rare 
etiologies of pleural effusion are also noteworthy. 
 
Acknowledgement 
We would like to thank Dr. Ghaffarzadegan for 
his sincere cooperation in preparing the photographs. 
 
REFERENCES 
1. Ernest Beutler, Marshall A, Lichtman. Barry S. Coller, 
Thomas J. Kipps. Williams Hematology, 6th Edition 2001; 
1279-1304. 
2. Inoue Y, Chua K, McClure RF, Jimenez MC, Gocke CD, 
Badros AZ, et al. Multiple myeloma presenting initially as a 
solitary pleural effusion later complicated by malignant 
plasmacytic ascites. Leuk Res 2005; 29 (6): 715- 8. 
72   Myelomatous Pleural Effusion 
Tanaffos 2007; 6(2):68-72 
3. Rodriguez JN, Pereira A, Martinez JC, Conde J, Pujol E. 
Pleural effusion in multiple myeloma. Chest 1994; 105 (2): 
622- 4.  
4. Durie BG, Kyle RA, Belch A, Bensinger W, Blade J, 
Boccadoro M, et al. Myeloma management guidelines: a 
consensus report from the Scientific Advisors of the 
International Myeloma Foundation. Hematol J 2003; 4 (6): 
379- 98. Erratum in: Hematol J 2004; 5 (3): 285.  
5. Robert A. Kyle, Vincent Rajkumar S. Multiple Myeloma.     
N Eng J Med 2004; 351: 1860-73. 
6. Cabrera A, Klein JS. Bilateral pleural masses and shortness 
of breath associated with multiple myeloma. Chest 1997; 111 
(6): 1750- 3.  
7. Kamble R, Wilson CS, Fassas A, Desikan R, Siegel DS, 
Tricot G, et al. Malignant pleural effusion of multiple 
myeloma: prognostic factors and outcome. Leuk Lymphoma 
2005; 46 (8): 1137- 42.  
8. Manley R, Monteath J, Patton WN. Co-incidental 
presentation of IgA lambda multiple myeloma and pleural 
involvement with IgM kappa non-Hodgkin's lymphoma. Clin 
Lab Haematol 1999; 21 (1): 61- 3.  
9. Hughes JC, Votaw ML. Pleural effusion in multiple 
myeloma. Cancer 1979; 44 (3): 1150- 4.  
10. Safa AM, Van Ordstrand HS. Pleural effusion due to 
multiple myeloma. Chest 1973; 64 (2): 246- 8.  
11. Kravis MM, Hutton LC. Solitary plasma cell tumor of the 
pleura manifested as massive hemothorax. AJR Am J 
Roentgenol 1993; 161 (3): 543- 4.  
 


Bilateral pleural masses and shortness of breath associated with multiple myeloma. Chest

Co-incidental presentation of IgA lambda multiple myeloma and pleural involvement with IgM kappa non-Hodgkin's lymphoma. Clin Lab Haematol

Malignant pleural effusion of multiple myeloma: prognostic factors and outcome. Leuk Lymphoma

Multiple myeloma presenting initially as a solitary pleural effusion later complicated by malignant plasmacytic ascites. Leuk Res 2005; 29 (6): 715- 8. 72 Myelomatous Pleural Effusion Tanaffos

Multiple Myeloma.

Myeloma management guidelines: a consensus report from the Scientific Advisors of the International Myeloma Foundation. Hematol J 2003; 4 (6): 379- 98. Erratum in: Hematol J

Myelomatous Pleural Effusion

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