We herein report an extremely rare case of a patient with IgD-lambda positive multiple myeloma presenting with myelomatous pleural effusion and ascites. A 58-year-old man visited our hospital with dyspnea as his initial symptom. His chest radiograph findings on admission revealed a left pleural effusion, and later, bilateral involvement. Computed tomography (CT) of the chest showed a paraspinal tumor with extension from the upper mediastinum to the abdomen. The cytological examination demonstrated myeloma cells in the pleural effusion and ascites, and histologically, in the pleura, an abdominal subcutaneous tumor and bone was observed. The pleural effusion was an exudate and slightly bloody. The ADA was 70 IU/L. Pleural effusion accompanying myeloma or primary pleural myeloma is very rare and, furthermore, the extremely rare findings of both myeloma cells in the ascites (although the ascites was mainly caused by liver cirrhosis) and a high ADA activity in the pleural fluid were also observed in this case.
"Nevertheless, an elevated ADA activity in pleural fluid has also been reported in some cases of benign or malignant diseases, such non-Hodgkin’s lymphoma, breast cancer
[7,8]. Yokoyama et al. also reported a myelomatous pleural effusion case with high ADA activity in pleural effusion
, but the ADA activity in our patient was much higher. "
[Show abstract][Hide abstract] ABSTRACT: Multiple myeloma (MM) is a type of hematological malignancy that can affect all types of tissues in human. However, it is extremely rare that pleural effusion presents as the first sign in MM patients. A 54-year-old male patient attended our department of respiratory medicine complaining of shortness of breath for the past 3 months. A chest computer tomography (CT) radiograph revealed a bilateral pleural effusion, which was further assessed as exudative type. Sinus spiral CT scan demonstrated diffuse bone destruction of craniofacial bone. A broad reduction of the lumbar bone signal was confirmed by MRI. Furthermore, pleural biopsy showed abnormal proliferation of plasmocytes whereas bone marrow biopsy showed active hyperplasia of plasmacytoid cells. Interestingly, Bence-Jones protein in urine and serum protein electrophoresis was negative. The patient was diagnosed as non-secretory MM. He then underwent chemotherapy with vincristine, adriamycin and dexamethasone. Partial regression of the pleural effusion was achieved after two rounds of chemotherapy, and the patient has been followed for more than one year.
"In previous studies investigating the levels of ADA in hematologic malignancies, abnormal activities were found in some myeloma cases [24, 25]. High ADA levels in the pleural fluid have been previously reported in 2 patients with MPE: 61 U/L in a patient with IgA myeloma, and 50.2 U/L in a patient with IgD myeloma [3, 21]. Thus, we could assume that a small fraction of myeloma cells express ADA on their surfaces, and this may partly explain the high ADA levels in some MPE patients. "
[Show abstract][Hide abstract] ABSTRACT: Myelomatous pleural effusion (MPE) is rare in myeloma patients. We present a consecutive series of patients with MPE in a single institution.
We retrospectively reviewed the medical records of 19 patients diagnosed with MPE between 1989 and 2008 at the Asan Medical Center. Diagnoses were confirmed by cytologic identification of malignant plasma cells in the pleural fluid.
Our patients showed dominance of IgA (36.8%) and IgD (31.6%) subtypes. Of 734 myeloma patients, the incidence of MPE was remarkably high for the IgD myeloma subtype (16.7%), compared to the other subtypes (1.4% for IgG and 4.6% for IgA). At the time of diagnosis of MPE, elevated serum β2-microglobulin, anemia, elevated serum lactate dehydrogenase, and elevated creatinine levels were found in 100%, 89.5%, 83.3%, and 57.9% of the patients, respectively. Approximately one-third (31.3%) of the patients had adenosine deaminase (ADA) activities in their pleural fluid exceeding the upper limit of the reported cutoff values for tuberculous pleural effusion (55.8 U/L). Chromosome 13 abnormality was seen in 77.8% of the tested patients. The median survival period from the development of MPE was 2.8 months.
Patients with MPE have aggressive clinical and laboratory characteristics. The preponderance of IgD myeloma in MPE patients is a noteworthy finding because IgD myeloma is a rare subtype. Elevated ADA activity in the pleural fluid is also noteworthy, and may be helpful for detecting MPE. Physicians treating myeloma patients should monitor the development of MPE and consider the possibility of a worse clinical course.
The Korean Journal of Laboratory Medicine 10/2011; 31(4):225-30. DOI:10.3343/kjlm.2011.31.4.225 · 1.31 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Malignant pleural effusion caused by multiple myeloma (MM) is rare, occurring as a late complication with a poor prognosis. Myelomatous pleural effusion (MPE) as an initial manifestation of the disease is extremely uncommon.
A 54-year-old, previously healthy man presented with right-sided pleural effusion. Cytologic examination of the aspirated fluid revealed large, slightly pleomorphic atypical cells, some having eccentric nuclei. Immunocytochemistry (ICC) performed on cytospin smears and cell block sections gave a positive reaction for CD138, EMA and vimentin and a negative reaction for CK7, CK20, calretinin, S-100 and E-cadherin.
Cytopathologists may be caught unawares by atypical presentations of hematologic malignancies. The diagnosis of MPE requires a high level of suspicion, and routine incorporation of ICC in the cytologic evaluation will ensure accurate diagnosis and proper patient management.
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