|Year : 2014 | Volume
| Issue : 1 | Page : 191-193
Lambda light chain myeloma presenting as nodular hepatic lesion: A clinical rarity
Santanu Pal1, Bitoti Chattopadhyay1, Atri Chatterjee2, Biswamit Bhattacharya1
1 Department of Radiotherapy, Medical College and Hospitals, Kolkata, India
2 Department of General Medicine, Nilratan Sircar Medical College and Hospital, Kolkata, India
|Date of Web Publication||23-Apr-2014|
Flat 2G, Nature's Nest, 140, Prince Gulam Hussain Shah Road, Jadavpur, Kolkata - 700 032
Source of Support: None, Conflict of Interest: None
We report a case of a 63-year-old lady presenting with pain in the right hypochondrium, jaundice, anorexia, and firm tender hepatomegaly with remarkably high serum alkaline phosphatase. Abdominal ultrasonography revealed a hypoechoic solid space-occupying lesion in right lobe of liver which was cytologically diagnosed as hepatic plasmacytoma. Serum and urine immunofixation electrophoresis, serum free light chain ratio, and bone marrow examination further confirmed the presence of lambda light chain multiple myeloma in the background. The patient achieved complete remission after four cycles of induction therapy with thalidomide and dexamethasone protocol and consolidated with further four cycles of the same regimen.
Keywords: Hepatic plasmacytoma, lambda light chain, multiple myeloma
|How to cite this article:|
Pal S, Chattopadhyay B, Chatterjee A, Bhattacharya B. Lambda light chain myeloma presenting as nodular hepatic lesion: A clinical rarity. J Can Res Ther 2014;10:191-3
|How to cite this URL:|
Pal S, Chattopadhyay B, Chatterjee A, Bhattacharya B. Lambda light chain myeloma presenting as nodular hepatic lesion: A clinical rarity. J Can Res Ther [serial online] 2014 [cited 2020 Feb 26];10:191-3. Available from: http://www.cancerjournal.net/text.asp?2014/10/1/191/131409
| > Introduction|| |
Multiple myeloma (MM) is a malignant plasma cell disorder characterized by plasma cell infiltration of the bone marrow and overproduction of immunoglobulin or light chains. Although plasma cell infiltration of the liver occurs in 40% cases of MM,  most of them are diffuse infiltration and usually reported as autopsy findings. Nodular liver lesion in MM as liver plasmacytoma is a clinical rarity and associated with aggressive disease and poor outcome even with upfront aggressive treatment approaches.  We report a 63-year-old lady who presented with jaundice and pain in the right hypochondrium and later discovered to harbor a hepatic plasmacytoma in the background of light chain myeloma with excellent response to thalidomide and dexamethasone. There is a real dearth of such cases being reported from India.
| > Case History|| |
A 63-year-old previously healthy nondiabetic, nonhypertensive lady presented with progressively worsening dull aching pain in the right hypochondrium along with anorexia, fatigue, and weight loss for 3 months without any history of preceding fever, backache, or oliguria. Clinical examination revealed presence of moderate pallor, mild jaundice, bilateral pitting pedal edema with normal neck veins, and firm tender hepatomegaly. Other systemic examinations were noncontributory.
Initial laboratory investigations showed normocytic normochromic anemia (hemoglobin (Hb): 7.6 g/dL, mean corpuscular volume (MCV): 94.9 fL), high erythrocyte sedimentation rate (ESR) (128 mm/h), normal white blood cells (WBC) and platelet count (total leukocyte count (TLC): 8,500/mm 3 , platelet count: 165,000/mm 3 ), deranged renal function test (urea: 95 mg/dL, creatinine: 2.5 mg/dL), deranged liver function test (serum bilirubin total: 3.1 mg/dL, conjugated: 2.6 mg/dL, unconjugated: 0.5 mg/dL, total protein: 5.2 g/dL, albumin: 2.4 g/dL, globulin: 2.8 g/dL, alkaline phosphatase: 1,294 U/L, aspartate aminotransferase (AST): 55 U/L, alanine transferase (ALT): 83 U/L), normal coagulation profile. Urine routine and microscopic examination showed proteinuria. Ultrasonography of the abdomen revealed an enlarged liver with a hypoechoic solid space occupying lesion (SOL) measuring 2.7 cm × 2.6 cm in the right lobe of liver superiorly with normal intrahepatic biliary radicles, common bile duct of normal caliber, normal gall bladder, pancreas and spleen with no ascites or retroperitoneal lymphadenopathy, and normal sized both kidneys with increased cortical echogenicity but maintained corticomedullary differentiation. The findings corroborated with contrast enhanced computed tomography (CECT) scan of abdomen [Figure 1]. Ultrasound-guided fine needle aspiration cytology (FNAC) from the hepatic SOL showed clusters of reactive hepatocytes, plenty of plasma cells, both mature and immature form, in clusters as well as individual cell dispositions consistent with solitary plasmacytoma of liver [Figure 2]. Serum protein electrophoresis showed presence of M-band in gamma globulin region (0.31 g/dL). Serum and urine immunofixation electrophoresis (IFE) showed presence of monoclonal λ-light chain band [Figure 3]. Kappa: lambda free light chain ratio was 0.03 (reference range: 0.26-1.65). Quantitative assays of other immunoglobulin subtypes were essentially normal. Bone marrow aspirate showed 70% plasma cells with atypical forms and frequent clustering [Figure 4]. Bone marrow biopsy with immunohistochemistry revealed the plasma cells to be CD38+, CD138+, CD56+, and CD19- which is typical of neoplastic plasma cells. Serum β2 -microglobulin was elevated (46,400 μg/L; reference range: 800-2200 μg/L) and serum calcium was 9.9 mg/dL. Serological markers for viral hepatitis and HIV were negative. Skeletal survey showed multiple punched-out bony defects in the skull [Figure 5]. Congo red staining of abdominal fat pad aspirate was negative for amyloid deposit.
|Figure 1: Contrast enhanced computed tomography (CECT) scan of abdomen showing a solitary (space occupying lesion) SOL in right lobe of liver|
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|Figure 2: Photomicrograph (May-Grunwald-Giemsa (MGG) stain, 100) of ultrasound guided fine needle aspiration cytology (FNAC) of the lesion showing plenty of plasma cells, both mature and immature form with reactive hepatocytes in the background, consistent with hepatic plasmacytoma|
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|Figure 3: Urine (left) and serum (right) IFE showing lambda light chain monoclonal band|
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|Figure 4: Photomicrograph (Leishman stain, 400) of bone marrow aspirate showing clusters of atypical plasma cells|
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|Figure 5: Lateral radiograph of skull showing multiple punched out bony defects|
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So the patient was finally diagnosed to have hepatic plasmacytoma in the background of λ-light chain myeloma, International Staging System (ISS) stage III. She was put on thalidomide-dexamethasone protocol (tab. thalidomide p/o (200 mg/day, D 1 -D 28 ) and tab. dexamethasone 40 mg p/o D 1 -D 4 , D 9 -D 12 , D 17 -D 20 , q28d] for four cycles. She achieved complete remission following four cycles of induction therapy with excellent symptom relief and normalization of serum free light chain ratio. Thereafter, she received another four cycles of tab. thalidomide p/o (200 mg/day, D 1 -D 28 ) and tab. dexamethasone 40 mg p/o D 1 -D 4 . Her renal function also returned to normal by this time. She was not put on further maintenance therapy with thalidomide as she developed Common Terminology Criteria for Adverse Events (CTCAE) grade 3 peripheral neuropathy.
| > Discussion|| |
Myeloma may affect the liver through direct diffuse neoplastic plasma cell infiltration, or it may present as a single or multiple space occupying lesions as plasmacytomas with extrahepatic biliary obstruction, ascites, jaundice, and hepatomegaly.  Diffuse hepatic infiltration in multiple myeloma has been described essentially as an autopsy finding and they are silent clinically. However, the nodular form as solitary extramedullary plasmacytoma of liver causing pain and jaundice, as in the case reported here, is a clinical rarity. When MM affects the liver, diffuse (sinusoidal, portal, and mixed), and nodular patterns of microscopic plasma cell infiltration have been described.  Diffuse rather than nodular hepatic infiltration is the predominant pattern of liver involvement. Perez-Soler et al.,  reported 128 patients with MM, histologic findings were available in the liver of 21 patients, and diffuse plasma cell infiltration of the liver was observed in ten patients, while no case of nodular liver infiltration was observed. In a database of 2,584 patients with MM,  liver involvement could be found as masses or macroscopic nodules only in nine cases. However, in a retrospective study on 52 autopsied cases with MM,  hepatic invasion could be observed in 15 patients (28.8%) with circumscribed tumor nodules in seven cases (13.4%) and diffuse tumor involvement in eight cases (15.4%). In fact, most nodular liver lesions involving MM have been reported in single cases. ,,, Thalidomide-dexamethasone has been used as a first-line therapy for patients with multiple myeloma, especially for those who are candidates for autologous stem cell transplantation. 
Although there has been concern about myeloma patients suffering from extramedullary relapses following thalidomide based regimens, there are evidences to the contrary as well. Katodritou et al.,  reported successful treatment of extramedullary plasmacytoma of the cavernous sinus using a combination of thalidomide and dexamethasone. Similarly, Nakazato et al.,  reported successful management of a patient with multiple extramedullary plasmacytomas and myelomatous pleural effusion with the combination of thalidomide and dexamethasone.
| > Conclusion|| |
In conclusion, we report a patient of extramedullary plasmacytoma of liver in the background of λ-light chain myeloma who responded very well to upfront thalidomide and high dose dexamethasone. Extramedullary plasmacytoma of liver, though rare, is a possibility in differential diagnosis of hepatic space-occupying lesions.
| > Acknowledgement|| |
Dr. Anup Kr. Boler, Associate Professor, Department of Pathology, Nilratan Sircar Medical College and Hospital, Kolkata.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]