439 Tüberküloz ve Toraks Dergisi 2008; 56(4): 439-442
Pleural effusion as the first sign of multiple myeloma
Bahadır Taha ÜSKÜL1, Hatice TÜRKER1, Fatma EMRE TURAN1, Özge ÜNAL BAYRAKTAR1, Alkın MELİKOĞLU1, Canan TAHAOĞLU2, Büge ÖZ3
1 SB Süreyyapaşa Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Hastalıkları,
2SB Süreyyapaşa Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, Patoloji,
3İstanbul Üniversitesi Cerrahpaşa Tıp Fakültesi, Patoloji Anabilim Dalı, İstanbul.
ÖZET
Multipl miyelomun ilk bulgusu olarak plevral efüzyon
Multipl miyelom seyrek görülen retiküloendotelyal dışı dokuları tutan bir hastalıktır ve çok nadir miyelomatöz plevral efüz- yona sebep olmaktadır. Elli altı yaşında erkek olgu son bir aydır olan nefes darlığı ve halsizlik şikayetleriyle kliniğimize başvurdu. PA akciğer grafisinde sol plevral efüzyon saptanan olgunun, plevral sıvısı eksüda karakterindeydi. Protein elekt- roforezinde hipergamaglobulinemi tespit edildi. Serum ve plevral sıvı immünfiksasyon elektroforezinde IgG ve kappa frak- siyonlarında artış saptandı. Yirmi dört saatlik idrarda Bence-Jones proteini yüksekti. Plevra biyopsisinde plazmosit infilt- rasyonu gösterildi. Yapılan kemik iliği biyopsisinde difüz paternde IgG/Kappa klonalitesi taşıyan bazıları immatür morfo- lojide atipik plazma hücre infiltrasyonu görüldü. Bu bulgularla IgG/κtipi multipl miyelom tanısı konan hastaya vinkristin, doksorubisin ve prednizolondan oluşan kemoterapi başlandı. İlk kür sonrası plevral efüzyonu tamamen gerileyen hasta 18 aydır takip altındadır.
Anahtar Kelimeler: Multipl miyelom, plevral efüzyon, miyelomatöz, kemoterapi, olgu sunumu.
SUMMARY
Pleural effusion as the first sign of multiple myeloma
Bahadır Taha ÜSKÜL1, Hatice TÜRKER1, Fatma EMRE TURAN1, Özge ÜNAL BAYRAKTAR1, Alkın MELİKOĞLU1, Canan TAHAOĞLU2, Büge ÖZ3
Yazışma Adresi (Address for Correspondence):
Dr. Bahadır Taha ÜSKÜL, S.B. Süreyyapaşa Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim ve Araştırma Hastanesi, Göğüs Hastalıkları, İSTANBUL - TURKEY
e-mail: tbuskul@yahoo.com
Multiple myeloma (MM) is a neoplastic disorder caused by the proliferation of transformed B lymphoid progenitor cells, which results in a ma- lignant clone of immunoglobulin-secreting plas- ma cells (1). MM constitutes 1% of all malignan- cies and 10% of all hematologic malignancies, with an incidence of 3 to 4 per 100.000. The mean age at the time of diagnosis is 65 years, while 3% of the cases are under 40 (2).
MM affects the bone marrow mainly, and other tissues to a lesser extent. The thorax can be in- vaded by myeloma, producing thoracic skeletal abnormalities, plasmocytoma, pulmonary infilt- rates, and pleural effusion, although a pleural ef- fusion in MM is relatively infrequent and a mye- lomatous pleural effusion is extremely rare (3).
We present a case of MM with a myelomatous pleural effusion that responded to systemic che- motherapy.
CASE REPORT
A 56-year-old male attended our clinic compla- ining of shortness of breath and fatigue for the past month. His complaints had begun slowly and increased. Nothing significant was observed in his personal or family history, except a 40 packs-a-year smoking history. His blood pressu- re was 130/90 mmHg, heart rate 94/min and re-
gular, respiratory rate 18/min, and temperature 37.5°C. The respiratory examination revealed decreased breath sounds in the middle and lo- wer left hemithorax, with dullness to percussion.
The remainder of the physical examination was normal.
Laboratory results revealed the following: gluco- se 90 mg/dL [normal value (NV) 70-110 mg/dL], blood urea nitrogen 15 mg/dL (NV 7-18 mg/dL), creatinine 1.1 mg/dL (NV 0.6-1.3 mg/dL), lactate dehydrogenase (LDH) 79 IU/L (NV 100-190 IU/L), total protein 11.3 g/dL (NV 6.4-8.2 g/dL), albumin 1.5 g/dL (NV 3.4-5.0 g/dL), globulin 9.8 g/dL (NV 3.8-5.2 g/dL), erythrocyte sedimentation rate 136 mm/h, he- moglobin 9.5 g/dL, white blood cell (WBC) co- unt 8.9 x 103/µL (54% neutrophils, 38%
lymphocytes, 7% monocytes, and 1% eosinop- hils), platelet count 333 x 103/µL, and the puri- fied protein derivative (PPD) was negative. Se- rum protein electrophoresis revealed a decrease in the β-globulin band and increase in the gam- ma globulin band. Serum immunofixation elect- rophoresis showed an increased IgG fraction (1980 mg/dL; NV 700-1600 mg/dL) and Ig light chain κ (1790 mg/dL; NV 620-1670 mg/dL), and decreases in the IgA, IgM, and Ig light cha- in λ fractions (15, 13, and 150 mg/dL; NV 70- 400, 40-240, and 336-899 mg/dL, respecti-
Pleural effusion as the first sign of multiple myeloma
Tüberküloz ve Toraks Dergisi 2008; 56(4): 439-442 440
1 Department of Chest Diseases, Süreyyapaşa Chest Diseases and Chest Surgery Teaching and Research Hospital, İstanbul, Turkey,
2Department of Pathology, Süreyyapaşa Chest Diseases and Chest Surgery Teaching and Research Hospital, İstanbul, Turkey,
3Department of Pathology, Cerrahpaşa Faculty of Medicine, İstanbul University, İstanbul, Turkey.
Multiple myeloma (MM) is a rare disorder that affects all tissues, except reticuloendothelial tissues, and seldom causes a myelomatous pleural effusion. A 56-year-old male patient attended our clinic complaining of shortness of breath and fati- gue for the past month. A posteroanterior chest radiograph revealed a left pleural effusion, which was subsequently asses- sed as being exudative in nature. Protein electrophoresis demonstrated hypergammaglobulinemia. Serum and pleural flu- id immunofixation electrophoresis showed an increase in the IgG and kappa fractions. The Bence-Jones protein level in the 24-hours urine was high. Pleural biopsy showed plasmocyte infiltration. Bone marrow biopsy revealed atypical plasma cell infiltration, some with immature morphology, carrying IgG/Kappa clonality in a diffuse pattern. The patient was diagno- sed with IgG/κtype MM and underwent chemotherapy with vincristine, doxorubicin, and prednisolone. Complete regres- sion of the pleural effusion was achieved after one round of chemotherapy, and the patient has been followed for 18 months.
Key Words: Multiple myeloma, pleural effusion, myelomatous, chemotherapy, case presentation.
vely). The 24-h urine sample contained Bence- Jones protein (63.2 mg/dL, NV < 50 mg/dL).
The posteroanterior chest radiograph showed a pleural effusion filling the lower left hemithorax.
Thoracic computerized tomography (CT) sho- wed a pleural effusion in the left hemithorax (Fi- gure 1). Serofibrinous fluid obtained by thora- centesis was exudative in nature. The pleural fluid contained glucose 142 mg/dL, LDH 118 IU/L, total protein 10.0 g/dL, and total adenosi- ne deaminase 24.56 IU/L (NV < 45 IU/L). Ple- ural fluid immunoelectrophoresis gave the same results as the serum immunoelectrophoresis.
Pleural fluid immunofixation electrophoresis in- dicated increases in the fractions of IgG (3680 mg/dL) and Ig light chain κ(2875 mg/dL), and decreases in the IgA, IgM, and Ig light chain λ fractions, as seen in the serum immunoelectrop- horesis (23, 17, and 202 mg/dL, respectively).
Cytologic examination of the pleural fluid reve- aled leukocytes of 2.3 x 103/µL (19% neutrop- hils, 81% lymphocytes) and a few atypical cells.
No acid-resistant bacilli were detected in the pleural fluid, which was sterile. Pleural biopsy showed plasmocyte infiltration (Figure 2).
On echocardiography, left ventricle systolic function was normal, the ejection fraction was 60%, and the anterolateral pericardium was hyperechogenic.
No lytic lesions were detected on radiography of the total skeletal system and on cranial CT. The
bone marrow biopsy revealed atypical plasma cell infiltration (76%) some of them were large plasma cells and had nucleolus, some with im- mature morphology, carrying IgG/kappa clona- lity in a diffuse pattern, and suppressed hemato- poiesis. The patient was diagnosed with IgG/κ type MM and underwent chemotherapy with vincristine, doxorubicin, and prednisolone.
Complete regression of pleural effusion was ac- hieved after one round of chemotherapy, and the patient has been followed for 18 months.
DISCUSSION
MM is a malignant proliferation of plasma cells that usually invades the bone marrow, but may involve other areas. One kind of thoracic invol- vement is pleural effusion. In 6% of patients with myeloma, a pleural effusion develops (myelo- matous or nonmyelomatous). A myelomatous pleural effusion is rare, with approximately 80 cases reported worldwide (4,5).
Several etiologic factors can lead to a pleural ef- fusion in MM: heart failure secondary to amylo- idosis, pulmonary embolism, chronic renal failu- re, a second neoplasm, and pleural myelomato- us involvement (4). It has been suggested that a myelomatous pleural effusion is a late manifes- tation of MM (6). Very seldom, a myelomatous pleural effusion occurs as the first sign of MM, as reported by Rodriquez et al. and Andre et al.
(4,7). In our case, a myelomatous effusion was the first sign of MM.
Üskül BT, Türker H, Emre Turan F, Ünal Bayraktar Ö, Melikoğlu A, Tahaoğlu C, Öz B.
441 Tüberküloz ve Toraks Dergisi 2008; 56(4): 439-442 Figure 1. Computed tomography of the thorax sho-
wing a moderate left-sided pleural effusion.
Figure 2. Histologic findings of the pleural biopsy showing CD38-positive plasmocytes (black arrows).
Renksiz!!!
Of the reported myelomatous pleural effusions, 80% occur in IgA myeloma, and the others are associated with IgG. Kim et al. reported a case with myelomatous pleural effusion related to IgD-λ myeloma (3). Our case was IgG-κ type myeloma.
Diagnostic criteria to confirm the myelomatous etiology are as follows:
1. Demonstration of a monoclonal protein in pleural fluid electrophoresis,
2. Detection of atypical plasma cells in pleural fluid, and
3. Histologic confirmation using pleural biopsy specimen or autopsy (4).
The detection of plasma cells in the pleural fluid is crucial, although reactive plasma cells can al- so appear in serous effusions secondary to car- diac surgery, tuberculosis, Hodgkin’s disease, and carcinomatosis. Therefore, the diagnosis of a myelomatous effusion requires high cellularity consisting mostly of immature and atypical plasma cells (8). In this case, monoclonal prote- ins were observed on pleural fluid electrophore- sis, and plasmocyte infiltration was confirmed histologically in pleural biopsy specimen.
It has been reported that a myelomatous pleural effusion indicates a poor prognosis in MM, altho- ugh other reports indicate that a myelomatous effusion can regress with systemic chemothe- rapy (7-9). The pleural effusion in our case reg-
ressed completely. The patient is still being fol- lowed clinically and radiologically.
In conclusion, a myelomatous pleural effusion is very rare and can be the first manifestation of MM. In our case, the patient responded well to chemotherapy.
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5. Kamble R, Wilson CS, Fassas A, et al. Malignant pleural effusion of multiple myeloma: Prognostic factors and out- come. Leuk Lymph 2005; 46:1137-42.
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Pleural effusion as the first sign of multiple myeloma
Tüberküloz ve Toraks Dergisi 2008; 56(4): 439-442 442
