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malignant myelomatous pleural effusions ( mpes ) are very rare and occur in < 1% of cases of mm .
since this condition is associated with poor prognosis , an accurate diagnosis of this condition is necessary . in this article ,
we report a rare case of a patient who initially presented with pleural effusion , which was subsequently found to be secondary to mm .
a 63-year - old female presented to the outpatient department of our hospital in december 2014 , with a complaint of feeling increasingly breathless and fatigued over a period of 2 months .
she also complained of right sided chest pain and nonradiating pain in the lower back since a month . on evaluation ,
the chest x - ray revealed a homogenous opacity in the right mid and lower zones [ figure 1 ] .
biochemical analysis of the pleural fluid confirmed that it was exudative in nature ( protein 9.8 g / dl , glucose 4.9 mg / dl , lactate
dehydrogenase 1020 iu / l , adenosine deaminase 69.6 u / l [ ref : 33 u / l ] ) .
the pleural fluid sent for culture , and cytology showed no acid - fast bacilli .
a computed tomography ( ct ) scan thorax was done and showed an ill - defined nodular heterogeneously enhancing lesion in right posterior hemithorax with right sided gross pleural effusion , collapse of right lung , and involvement of the right 4 rib [ figure 2 ] .
fine - needle aspiration cytology ( fnac ) done from the right pleural lesion showed numerous plasma cells , binucleate forms , and few atypical plasma cells [ figure 3 ] .
a cell block was prepared after centrifuging the pleural fluid and immunohistochemistry done on the cell block showed tumor cells positive for cd20 , cd138 , and lambda and negative for leukocyte common antigen , cd3 , cytokeratin , and kappa [ figure 4 ] .
serum electrophoresis showed an m - spike in beta region with elevated serum igg levels ( 3586 mg / dl , ref range 7001600 mg / dl ) .
beta 2 microglobulin level was elevated ( 3.23 mg / l ref : 0.82.2 mg / l ) .
magnetic resonance imaging whole spine was done , and confirmed the presence of a lobulated enhancing soft tissue lesion in the right paravertebral region , measuring 6 cm  8.6 cm , with involvement of the adjacent rib and also intraspinal extension .
ct - guided fnac done from this paravertebral mass showed multiple plasma cells , contributing to the diagnosis of mm . bone marrow aspiration and biopsy showed scattered and < 2% infiltration with plasma cells .
she received palliative radiotherapy of 30 gray in 10 fractions to the t12l2 spine and paravertebral mass .
she was started on vtd regimen with bortezomib , thalidomide , and dexamethasone chemotherapy along with monthly bisphosphonates .
after 6 cycles of chemotherapy , she was symptomatically better , and her chest x - ray showed resolution of the pleural effusion .
computed tomography image plasma cells on cytology low- and high - power view cd20 ( top  low- and high - power ) , cd138 ( middle- low- and high - power ) , lambda ( bottom  low- and high - power )
in history , mm was first described in egyptian mummies . the word mm was first coined by rustizky in 1873 .
the median age of presentation is 69 years and is more common in men , and increases with advancing age .
patients with mm may be asymptomatic or can usually present with hematologic manifestations such as anemia , bleeding disorders , or bone related problems , infections and other end organ damage like renal failure . in mm , the development of pleural effusion
pleural effusion occurs in around 6% of patients with mm during the course of the disease , while mpe is even rarer presenting in < 1% of patients as reported by kintzer et al .
it is associated with poor prognosis , and there are previous studies with the survival of < 4 months .
mpes may arise from either extension of plasmacytomas of the chest wall , invasion from adjacent skeletal lesions , direct pleural involvement by myeloma ( pleural plasmacytoma ) or also following lymphatic obstruction secondary to lymph node infiltration .
pleural effusions are rarely a direct consequence of the myeloma itself and usually occur due to the concurrent disease process or coexisting illness such as cardiac failure , amyloidosis , pulmonary embolism , pneumonia , or a second malignancy .
reported that about 50% of mm patients accompanied with pleural effusion were due to congestive heart failure .
the other possible mechanisms are a pulmonary embolism , chronic renal failure accompanying mm , and second malignancy .
mpes are most commonly associated with the presence of an iga paraprotein ( in up to 80% of cases ) .
sasser et al . reported 56 cases of mm with the involvement of the serous cavities .
the sites of involvement were mostly the pleural cavity ( thirty cases ) and among them , 50% of the cases with cavitary involvement were of the iga type . in this case ,
this is an important contributory factor for the development of mpes and explains the apparent aggressive nature of myelomatous disease .
this case is distinctive in regards to the direct involvement of the pleural cavity by malignant plasma cells , which was confirmed with the fnac and ihc .
only 11 cases have been described previously , including the present case ( to the best of our knowledge ) .
chemotherapy with various newer drugs such as bortezomib , thalidomide , lenalidomide along with dexamethasone or 2 generation proteasome inhibitors like carfilzomib remains the first - line treatment for mm . in practice , pleural involvement with myeloma cells is associated with an aggressive course which is poorly responsive to first or second - line therapies used in conventional myeloma treatment . in this case , the patient was doing better with vtd chemotherapy and palliative radiotherapy .
this patient was started on treatment early and had igg paraprotein mpe probably indicating better response in these cases .
the occurrence of mpes in mm is rare . it indicates a poor prognosis because of an aggressive natural course .
early consideration of mpes helps in rapid diagnosis and early initiation of treatment which may help in improving prognosis as seen in the present case .
multiple myeloma ( mm ) is a plasma cell neoplasm and constitutes 10% of hematologic malignancies . 
 malignant myelomatous pleural effusions are very rare and occur in < 1% of cases of mm . in this article , we report a rare case of a patient who initially presented with pleural effusion and was subsequently found to be secondary to mm with an underlying raised igg paraprotein . the patient symptomatically improved and was in partial remission with palliative radiotherapy , vtd chemotherapy , and bisphosphonates .