Respiratory Medicine CME 4 (2011) 12e14
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Case Report
Lambda light chain multiple myeloma presenting as pleural mass Mohammad Shameem*, Jamal Akhtar, Rakesh Bhargava, Zuber Ahmad, Ummul Baneen, Nafees Ahmad Khan Department of Tuberculosis and Chest Diseases, Jawaharlal Nehru Medical College, Aligarh Muslim University, Aligarh, UP 202002, India
a r t i c l e i n f o
a b s t r a c t
Article history: Received 8 January 2010 Accepted 18 January 2010
Light chain multiple myeloma occurs in 20% of multiple myeloma cases. Extramedullary dissemination of multiple myeloma is rare and involvement of pleura by it is even more rare. A 40 years old female patient presented with complains of recurrent vomiting for last 2 months and cough with expectoration for last 1 week. Chest X-Ray (PA view) showed a rounded homogenous opacity in mid zone of left lung field. CECT (thorax) showed pleura based soft tissue density mass lesions overlying apicoposterior segment of left upper lobe and lingular lobe with lytic destruction of underlying 2nd rib. Ultrasound scan of abdomen was normal. CT guided FNAC from soft tissue mass lesion of left side revealed atypical plasma cells, some of them were binucleate and few appear pleomorphic. Bone marrow aspiration showed 20% atypical plasma cells. Serum protein electrophoresis revealed a thin discrete band in gamma globulin region, while serum immunofixation electrophoresis showed presence of lambda light chain band only, with absence of kappa band, IgG band, IgM band and IgA band. Patient was diagnosed as a case of lambda light chain multiple myeloma with extramedullary dissemination to pleura presenting as pleural mass. Ó 2010 Elsevier Ltd. All rights reserved.
Keywords: Lambda light chain multiple myeloma Extramedullary dissemination Pleural mass
1. Introduction Multiple myeloma is characterized by malignant proliferation of plasma cells with an overproduction of monoclonal proteins usually producing IgG and IgA, but in 20% of cases only light chains are produced, which is termed as light chain myeloma.1 Multiple myeloma is usually restricted to the bone and bone marrow and extramedullary involvement in multiple myeloma is rare, usually involving the nasopharynx, upper respiratory tract, or gastrointestinal tract.2e5 The pleural involvement is even more rare in multiple myeloma.6,7 Here, we are presenting a case of lambda light chain multiple myeloma, in which the malignant plasma cells had disseminated to the pleura and presented as pleural mass. 2. Case report A 40 years old female patient presented with complains of recurrent vomiting for last 2 months and cough with expectoration for last 1 week. There was no history of fever, chest pain, dyspnoea, haemoptysis, abdominal pain or abdominal distension. On physical examination, BP-110/80,PR-78/min, RR-20/min and patient was a febrile. There was no lymadenopathy. Examination of respiratory
* Corresponding author. E-mail address:
[email protected] (M. Shameem). 1755-0017/$36.00 Ó 2010 Elsevier Ltd. All rights reserved. doi:10.1016/j.rmedc.2010.01.007
system, cardiovascular system, central nervous system and GI system revealed no abnormal finding. Laboratory investigations revealed, Hb 8.0 g/dl, TLC 10,200 per cu mm, platelets count 1.2 lakh per cu mm and ESR 60 mm in Ist h (Westergren). The blood sugar-90 mg/dl, blood urea-52.4 mg/dl and serum creatinine-1.46 mg/dl at presentation. The serum uric acid, liver function test, serum calcium, and phosphorus were within normal limit. Chest X-Ray-PA view (Fig. 1) showed a rounded homogenous opacity in mid zone of left lung field. CECT-thorax showed pleura based soft tissue density mass lesions overlying apicoposterior segment of left upper lobe and lingular lobe with lytic destruction of underlying 2nd rib (Fig. 2). Ultrasound scan of abdomen was normal. CT guided FNAC from soft tissue mass lesion of left side revealed atypical plasma cells, some of them were binucleate and few appear pleomorphic. Bone marrow aspiration showed 20% atypical plasma cells. X-ray of skull and dorsolumbar spine was done which revealed absence of any lytic lesion. Serum protein electrophoresis revealed a thin discrete band in gamma globulin region. The total protein was 4.4 g/dl [6.4e8.2], albumin-2.74 g/dl [3.20e4.60], alpha1 globulin-0.16 g/dl [0.15e0.33], alpha2 globulin-0.49 g/dl [0.72e1.06], beta globulin-0.43 g/dl [0.74e1.06] and gamma globulin-0.58 g/dl [0.91e1.71]. Serum immunofixation electrophoresis revealed presence of lambda light chain band only .The kappa band, IgG band, IgM band and IgA band
M. Shameem et al. / Respiratory Medicine CME 4 (2011) 12e14
Fig. 1. CXR PA view showing rounded homogenous opacity in mid zone of left lung field.
were not detected on immunofixation. Urine for Bence Jones protein was negative. Patient was diagnosed as a case of lambda light chain multiple myeloma with extramedullary dissemination to pleura presenting as pleural mass. Patient was referred to department of radiotherapy for further management, where lenalidomideedexamethasone chemotherapy regimen was started. 3. Discussion Multiple myeloma is a neoplastic disorder of plasma cells. Plasma cells are a type of B cell, responsible for the production of immunoglobulins. It is the second most common hematological malignancy after non Hodgkins lymphoma.8 It occurs primarily in the 65e75 years of age group. The classic triad of myeloma is marrow plasmacytosis (>10%), lytic bone lesions and a serum or urine monoclonal (M) component. The presenting features are bone pains, renal dysfunction, infections (pneumonia, pyelonephritis), and neurological manifestations. Hypercalcaemia, hyperuricemia and anaemia are common lab findings.
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In multiple myeloma, there is increased concentration of monoclonal protein, which is an abnormal immunoglobulin produced by malignant plasma cells, along with normal or reduced concentration of other normal immunoglobulins. Generally one heavy type of immunoglobulin is increased, most commonly IgG followed by IgA. Monoclonal protein is seen as a spike in gamma region of serum electrophoretic pattern. However, in 20% of multiple myeloma cases, there is production of only light chain as monoclonal protein and this condition is known as light chain myeloma.1 The heat test for detecting Bence Jones protein is falsely negative in 50% of patients with light chain myeloma.1 The light chain isotype may have an impact on survival. Patients secreting lambda light chains have a significantly shorter overall survival than those secreting kappa light chains. In IgD myeloma, the light chain is lambda and the patients often present with a protein electrophoretic pattern showing a normal or a hypogammaglobulinemic pattern. All these features make IgD myeloma to be similar to lambda light chain myeloma.9 Multiple myeloma is usually restricted to bone and bone marrow. Extramedullary involvement in multiple myeloma is rare and if present usually involves the nasopharynx, upper respiratory tract, or gastrointestinal tract.2e5 Extramedullary involvement may be primary or secondary to dissemination of multiple myeloma.5Sites such as lymph node, thyroid, intestinal tract, ovary, kidney, and skin were reported to be involved by myeloma cells by Hellwig.10,11 Intrathoracic involvement in multiple myeloma is very rare.12,13 Myeloma cells may involve lung parenchyma in form of lung mass14,15 or pulmonary nodules.16 Pleural involvement in multiple myeloma may manifest as pleural effusion,17 pleural mass or nodule.16 Herskovic found intrathoracic involvement in 21 patients out of 303 patients of multiple myeloma reviewed at Mayo clinic and out of these 21 cases some presented as intrathoracic mass.18 A case of pleural mass associated with myeloma was reported by Galgano,19 as well as by Arnold et al.5 Here, we are presenting a case of lambda light chain multiple myeloma that presented to us as pleural mass due to extramedullary dissemination of myeloma cells to pleura, in order to highlight atypical presentation of multiple myeloma. Treatment of multiple myeloma depends on the patient's age and comorbidities. Under 65 years of age, thalidomideedexamethasone, lenalidomideedexamethasone or bortezomib based high dose chemotherapy regimen is given, along with hematopoietic stem-cell transplantation.20 Patients over age 65 and patients with significant concurrent illness, who cannot tolerate stem-cell transplantation, are treated with melphalan and prednisone chemotherapy regimen. However, new chemotherapy regimens such as bortezomib, melphalan and prednisolone,21 lenalidomide plus low dose dexamethasone or melphalan, prednisolone and lenalidomide has shown improved outcome in this age group. Conflict of interest No conflict of interest of interest to disclose.
References
Fig. 2. CECT(thorax)showing pleura based soft tissue density mass lesion with lytic destruction of 2nd rib on left side.
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