Two Serum Free Light Chain Detection Systems in the Diagnosis of Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To investigate the comparability of the Freelite, Binding Site, Beckman and N Latex FLC, Siemens in the detection of serum free light chain (sFLC) .@*METHODS@#Fifty newly diagnosed multiple myeloma (MM) patients in Tianjin Institute of Blood Research from November 2019 to February 2020 were enrolled. The two systems (Freelite, Binding Site, Beckman and N Latex FLC, Siemens) were used to detect the sFLC of the samples. Outlier detection was performed by ESD method, methodological comparison and deviation assessment were performed by Passing-Bablok regression and Bland-Altman regression.@*RESULTS@#Both the systems could quantitatively analyze free kappa light chain serum samples and free lambda light chain samples. Freelite, Binding Site, Beckman and N Latex FLC, Siemens free light chain test showed FLC-κ：36.5 (6.5, 194), 40.5 (6.94, 288), FLC-λ: 30.1 (4.3, 170.5), 35.1 (2.28, 526), rFLC (FLC-κ/ FLC-λ) : 0.82 (0.05, 43.25), 1.03 (0.03, 32.04), dFLC (｜FLC-κ- FLC-λ｜) : -5.8 (-161.97, 183.7), 1.1 (-505.1, 279.01), which existed no outliers. There were systematic differences, and the deviation level was not within the clinically acceptable range.@*CONCLUSION@#Both the systems can meet the needs of clinical diagnosis and treatment, but there is a significant deviation between the two systems, the results are not comparable, and should be analyzed separately. In particular, the same system should be selected for monitoring the prognosis of MM.

Value of Serum Light Chain in Diagnosis and Evaluation of Efficacy for Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To explore the clinical application value of serum light chain (sLC) in the diagnosis and therapeutic efficacy evaluation for multiple myeloma.@*METHODS@#46 patients with newly diagnosed multiple myeloma were selected as MM group and 50 healthy persons as control group. Rate scattering immunoturbidimetry was used to detect serum light chain and immunoglobulin (Ig) in two groups, serum protein electrophoresis was used to detect M protein by agarose gel. Then, the sensitivity and specificity of the two methods in MM diagnosis were analyzed and compared, and the significance of sLC detection in MM diagnosis were discussed. In addition, 15 MM patients after received conventional therapy were tracked, sLC levels in five different therapentic times were recorded, and the effect of sLC in efficacy evaluation of MM was analyzed.@*RESULTS@#There were 11 cases of IgA type, 15 cases of IgG type, 8 cases of light chain κ type, 8 cases of light chain λ type, 2 cases of IgD type, and 2 cases of non-secretion type. The sLC-κ, sLC-λ and their ratio (including light chain type and double clone type), IgA and IgG (except IgD type), as well as albumin, beta-globulin and gamma-globulin levels showed statistically significant differences (P＜0.05) compared with the control group. The sensitivity of serum protein electrophoresis, Ig quantification, sLC and its ratio in the diagnosis of multiple myeloma were 57%, 76% and 65%, and their specificity were 83%, 61% and 90%, respectively. After the second or third chemotherapy, the sLC-κ/λ ratio gradually approached the normal range as the disease reliefes, and the sLC-κ/λ ratio continued to be on or off the line at outliers or further away from the reference value as the disease progresses in MM patients with κ type or λ type.@*CONCLUSION@#sLC detection shows positive significance in early diagnosis of multiple myeloma, SLC monitoring can be used for the efficacy evaluation for treatment of MM patients.

AL amyloidosis in the Chilean public health system: a pending debt: multicenter study of the Chilean Monoclonal Gammopathies Cooperative Group / Amiloidosis AL en el sistema público de Chile: una deuda pendiente: estudio multicéntrico

ABSTRACT Background: Immunoglobulin light chain (AL) amyloidosis is a rare and underdiagnosed entity. Aim: To characterize patients with AL amyloidosis in Chilean public health centers. Material and Methods: We conducted a retrospective, multicenter study. Public centers of the Chilean Monoclonal Gammopathies Cooperative Group were asked to search for patients with AL amyloidosis in their databases. Epidemiological, clinical and laboratory characteristics were evaluated. Results: Forty-two patients aged 22 to 84 years were found. Twenty four percent had localized AL amyloidosis; 64% had a lambda light chain clone; 47% were associated with multiple myeloma and 9% with non-Hodgkin lymphoma. The most commonly involved organ was the kidney (76%). Serum free light chains were measured in 31% and an echocardiogram was performed in 74% of patients. Seventeen percent of patients received only palliative care, 17% were treated with bortezomib, 21% with thalidomide, and 40% with melphalan. No patient was transplanted. The mean overall survival (OS) of the group was 19 months. The 5-year OS was 28%. Conclusions: It is important to obtain these realistic, national data to initiate strategies to improve early diagnosis and proper management of this disease.

La amiloidosis AL es una entidad poco frecuente y subdiagnosticada. Mientras todo el mundo discute sobre las nuevas herramientas diagnósticas y terapéuticas, en Chile y en América Latina en general, estamos lejos de esa realidad. El objetivo del presente estudio fue caracterizar a los pacientes con amiloidosis AL en centros del sistema público de nuestro país. Se realizó un estudio retrospectivo, multicéntrico, descriptivo. Los centros públicos del grupo cooperativo hematológico chileno buscaron en sus bases de datos pacientes diagnosticados con amiloidosis AL. Se evaluaron las características epidemiológicas, clínicas y de laboratorio. La edad media fue de 65 años. A 24% de los pacientes se les diagnosticó amiloidosis AL localizada; 64% tuvo paraproteína con cadena ligera lambda; 47% se asoció con mieloma múltiple y 9% con linfoma no Hodgkin. El órgano afectado con mayor frecuencia fue el riñón (76%). Las cadenas ligeras libres de suero se realizaron en 31% y ecocardiograma en 74%. El 17% recibió solo cuidados paliativos, 17% recibió tratamiento con bortezomib, 21% con talidomida y 40% con melfalán. Ningún paciente fue trasplantado. La media de sobrevida global (SG) del grupo fue de 19 meses. La SG a 5 años fue de 28%. Es importante reportar estos resultados nacionales para iniciar estrategias que mejoren tanto el diagnóstico temprano como el tratamiento de esta patología. Por lo tanto, mejorar la sospecha diagnóstica es crucial.

Clinical Significance of Serum Free Light Chain and Its κ/λ Ratio in Patients with Multiple Myeloma / 中国实验血液学杂志

OBJECTIVE@#To analyze the clinical significance of serum free light chain (sFLC) and sFLC-κ/λ ratio in patients with newly diagnosed multiple myeloma (MM), so as to provide the theoretical basis for the diagnosis, treatment and prognosis of MM patients.@*METHODS@#A total of 43 patients newly diagnosed as MM in our hospital and 40 cases of other diseases that could cause sFLC increase were selcted. The clinical features of newly diagnosed MM patients were analyzed. On the basis of the type of sFLC, MM patients were divided into sFLC-κ and sFLC-λ group. The correlation of sFLC with clinical index in two groups was analyzed. The difference of sFLC-κ/λ ratio between MM and non-MM patients was analyzed. According to whether the involved/uninvolved sFLC ratio was greater than 100, the MM patients were divided into the low ratio and the high ratio group. The clinical index of these 2 groups were analyzed.@*RESULTS@#Among MM patients, IgG type was the most. In sFLC-κ and -λ groups, the hemoglobin level negatively correlated with sFLC (r=-0.307, r=-0.494, P＜0.05), and the serum β2 microglobulin level positively correlated with sFLC (r=0.453, r=0.689, P＜0.05), and myeloma cells ratio also positively correlated with sFLC (r=0.307, r=0.309, P＜0.05). The concentration of albumin, lactate dehydrogenase and serum calcium did not correlate with sFLC (P＞0.05). The serum creatinine level also did not correlate with sFLC in the sFLC-κ group (P＞0.05), but in the sFLC-λ group they were obviously positive (r=0.697, P＜0.01). The ratio of sFLC-κ/λ ＜0.26 or ＞1.65 in MM patients was higher than that of non-MM patients, and the ratio of involved/uninvolved sFLC≥100 in MM patients was also higher than that of non-MM patients with statistically significant differences (P＜0.01). The levels of serum albumin, creatinine and β2 microglobulin, as well as myeloma cell ratio and ISS international staging of the patients between 2 groups were significantly different (P＜0.05), while the differences in sex, age and levels of hemoglobin, lactate dehydrogenase and blood calcium were not significant between the 2 groups (P＞0.05).@*CONCLUSION@#The light chain type λ in MM patients correlates with renal damage. The abnormalities of sFLC-κ/λ ratio occur mostly in MM patients. The MM patients with involved/uninvolosed sFLC ratio ≥100 show the severe symptoms and poor prognosis.

Papel del médico de familia en el diagnóstico concomitante de mieloma y amiloidosis primaria en una misma paciente: caso clínico / Multiple myeloma associated with a secondary amyloidosis: report of one case

Multiple Myeloma is a myeloproliferative disorder of plasma cells, which may be complicated with secondary amyloidosis. We report a 48 year old woman consulting to primary care for weight loss and malaise. An initial laboratory study revealed a hypogammaglobulinemia with a monoclonal component and lambda light chains. These results motivated her derivation to hematology: her serum calcium was 11.8 mg/dl, immunofluorescence showed a monoclonal component of lambda chains and urine Bence-Jones protein was positive. A bone marrow biopsy confirmed plasma cell infiltration. A Congo-red stain of a rectal biopsy was positive. The patient was treated with thalidomide, bortezomid and dexamethasone.

Kappa and lambda determination by single colored immunohistochemistry and chromogenic in situ hybridization techniques in B-cell malignancies

Background: kappa and lambda light chains detection in bone marrow trephine sections help in the determination of B-cell clonality through evaluation of light chain restriction

Aim of the Work: was to compare the efficacy of single color detection-based immunohistochemistry [IHC] and chromogenic in situ hybridization [CISH] in evaluating kappa/lambda expression in tissues harboring B-lymphoid lesions

Patients and Methods: Forty patients were enrolled in this study. They were divided into three groups chronic lymphocytic leukemia [CLL/SLL] group I [n=13], non-Hodgkin lymphoma [NHL] group II [n=24] and hairy cell leukemia [HCL] group III [n=3]. The 24 NHL cases comprised of [11 diffuse large B-cell lymphoma, 6 mantle cell lymphomas, 3 marginal zone lymphoma, 2 lymphoplasmacytic lymphoma, 1 follicular lymphomas and 1 Burkitt's lymphoma]. Kappa and lambda light chains were detected in their bone marrow trephine sections using single colored immunohistochemistry, chromogenic in situ hybridization and the results were compared to the flowcytometry as reference method

Results: Light chain restriction [LCR] was detected by FCM in 100% of the cases followed by CISH [52.1%; 12/23] of the cases and finally IHC [43%; 18/40]

Conclusion: Both conventional CISH and IHC are effective in determining monoclonality in cases of mature B- cell neoplasm that has plasmacytic differentiation and with high amount of cytoplasmic Ig light chains such as MZL and LP. However, they are not effective in determining monoclonality in cases with low amount of Ig light chain such as cases of pregerminal and germinal center lymphoma. Yet, CISH is more informative than IHC due to the lack of background staining which allowed for greater discrimination between absence and presence of monoclonality

Plasma Cell Cheilitis: A Clinicopathological and Immunohistochemical Study of 13 Cases

BACKGROUND: Plasma cell cheilitis is an unusual benign plasma cell proliferative disease of an unknown etiology that typically presents on the lip. OBJECTIVE: The aim of this study was to investigate the clinicopathological characteristics of 13 cases of plasma cell cheilitis. METHODS: The present study investigated the clinical manifestations, treatment modalities, and outcome of 13 patients diagnosed with plasma cell cheilitis from 2011 to 2016 at Kangdong Sacred Heart Hospital and Hallym University Sacred Heart Hospital. Biopsy specimens of the all cases were evaluated using conventional hematoxylin and eosin staining with kappa and lambda immunoglobulin light chain immunohistochemistry. RESULTS: The age of the patients ranged from 39 to 86 years (mean, 64.7 years), with male predominance. Histopathologically, 61.5% and 38.5% of patients showed band-like and pan dermal plasmacytic infiltrates, respectively. Eosinophilic infiltration was noted in 69.2% of patients. All cases showed both kappa and lambda immunoglobulin light chain reactivities, and kappa predominance was confirmed in 9 patients (69.2%). A majority of the patients was treated with local therapy, such as intralesional steroid injection with topical tacrolimus. Among the 13 patients, plasma cell cheilitis completely resolved, partially resolved, and recurred in 3 (23.1%), 5 (38.5%), and 5 patients (38.5%), respectively. CONCLUSION: Plasma cell cheilitis presented as erosive edematous circumscribed patches or plaques affecting mainly the lower lip of elderly male patients. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for immunoglobulin light chain, with kappa predominance.

Test of Serum Free Light Chain and Its Clinical Significance in Light Chain Multiple Myeloma / 中国实验血液学杂志

<p><b>OBJECTIVE</b>To investigate the clinical significance of serum free light chain (sFLC) detection in light chain multiple myeloma (LCMM).</p><p><b>METHODS</b>A total of 37 newly diagnosed LCMM patients were enrolled in this study, including 17 patients with k light chain type and 20 patients with λ light chain type, the sFLC and 24 hours urine light chain (ULC) were measured before and after chemotherapy. The correlation of sFLC level with ULC and renal impairment was analyzed.</p><p><b>RESULTS</b>All the patients displayed an abnormally increased level of sFLC at diagnosis wtih median value of 105.44 mg/L and 146.39 mg/L for k and λ light chain types, respectively. The sFLC did not correlate with ULC before and after chemotherapy. Among the 12 patients with very good partial remission and normal ULC level, the sFLC still was abnormally increased in 8 patients. Renal impairment was associated with the urine λ-type light chain, and the area under the ROC curve of urine λ light chain at diagnosis is 0.792 (P = 0.031).</p><p><b>CONCLUSION</b>All patients with LCMM show an abnormally increased level of sFLC at diagnosis. sFLC can be used to monitor the response to chemotherapy because it is more sensitive for analysis of therapeutic effect than urine λ light chain.</p>

A Case of Primary Gastric Amyloidosis with Fulminant Heart Failure / 대한소화기학회지

A 53-year-old woman was admitted with epigastric discomfort and weakness. Laboratory examination at admission showed mild anemia and proteinuria. Esophagogastroduodenoscopy revealed marked mucosal atrophy, diffuse nodularity and granular appearance with mucosal friability. Biopsy was performed on the antrum and body of the stomach. On the next day, the patient began to complain of severe dyspnea, and hypoxia was present on pulse oximetry. Therefore, emergency echocardiography was conducted and it showed restrictive cardiomyopathy along with thrombus in the left atrium. With time, heart failure was aggravated despite intensive management. The result of gastric biopsy revealed amyloid deposits which stained positively with Congo red. On immunohistochemistry study, kappa and lambda chain were present. In addition, kappa chain was significantly elevated in urine and serum on electrophoresis. Although the patient was finally diagnosed as having primary gastric amyloidosis with restrictive cardiomyopathy, her general condition rapidly deteriorated and died at 12th hospital day. When obscure gastric lesion is encountered, performing gastric biopsy is strongly recommended since it be primary gastric amyloidosis. Herein, we present an unusual case of primary gastric amyloidosis.

Serum levels of immunoglobulin free light chains in patients with chronic hepatitis C presenting cryoglobulinemia

Hepatitis C virus (HCV) infects B-lymphocytes, provokes cellular dysfunction and causes lymphoproliferative diseases such as cryoglobulinemia and non-Hodgkin's B-cell lymphoma. In the present study, we investigated the serum levels of kappa and lambda free light chains (FLC) of immunoglobulins and the kappa/lambda FLC ratio in Brazilian patients with chronic HCV infection and cryoglobulinemia. We also analyzed the immunochemical composition of the cryoglobulins in these patients. Twenty-eight cryoglobulinemic HCV patients composed the target group, while 37 HCV patients without cryoglobulinemia were included as controls. The median levels of kappa and lambda FLC were higher in patients with cryoglobulinemia compared to controls (p = 0.001 and p = 0.003, respectively), but the kappa/lambda FLC ratio was similar in patients with and without cryoglobulinemia (p > 0.05). The median FLC ratio was higher in HCV patients presenting with advanced fibrosis of the liver compared to HCV patients without fibrosis (p = 0.004). Kappa and lambda FLC levels were strongly correlated with the IgA, IgG and IgM levels in the patients with cryoglobulinemia. In patients without cryoglobulinemia, the kappa FLC level was only correlated with the IgG level, whereas the lambda FLC were weakly correlated with the IgA, IgG and IgM levels. An immunochemical pattern of mixed cryoglobulins (MC), predominantly IgM, IgG, IgA and kappa light chain, was verified in these immune complexes. We concluded that HCV-infected patients presenting cryoglobulinemia have vigorous polyclonal B-lymphocyte activation due to chronic HCV infection and persistent immune stimulation.

Immunophenotypic analysis of abnormal plasma cell clones in bone marrow of primary systemic light chain amyloidosis patients / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Primary systemic light chain amyloidosis (AL) is a rare plasma cell disease, our purpose was to analyze the immunophenotypic characteristics of the plasma cells in bone marrow in AL patients, and explore whether the detection of abnormal plasma cell clones in bone marrow by flow cytometry (FCM) could be used as an important indicator of AL diagnosis.</p><p><b>METHODS</b>Fresh bone marrow samples were collected from 51 AL, 21 multiple myeloma (MM), and 5 Waldenström's macroglobulinemia (WM) patients. The immunophenotype of bone marrow cells were analyzed and compared by FCM using a panel of antibodies including CD45, CD38, CD138, CD117, CD56, and CD19.</p><p><b>RESULTS</b>In AL, light chain restriction could be identified in 31 cases (60.9%), in which the λ light chain restriction was found in 24 cases (77.4%). In MM, κ light chain restriction was found in 13 cases (61.9%), and λ light chain restriction in eight cases. CD45 on abnormal plasma cells was negative to weakly positive in both AL and MM, but was positive to strongly positive in WM. In the bone marrow plasma cells of the 51 AL, 78.4% were CD56+, 68.6% were CD117+, and 88.2% were CD19-. While in the 21 MM cases, 66.7% were CD56+, 38.1% were CD117+, and 90.4% were CD19-. The plasmacytoid lymphocytes in the five WM patients were CD19+ and CD56-, CD117-.</p><p><b>CONCLUSION</b>Detection of abnormal plasma cell clones in bone marrow by FCM is valuable for the diagnosis of AL.</p>

Localized Thymic Amyloidosis Presenting with Myasthenia Gravis: Case Report

A mediastinal mass was incidentally found on chest radiography in a 46-yr-old woman who had had myasthenia gravis (MG) for 2 months. Computed tomography revealed a 4-cm in size, well-defined, and lobulating mass with nodular calcification that was located in the thymus. Microscopically, the mass consisted of diffuse amorphous eosinophilic materials. These deposits exhibited apple-green birefringence under polarized light microscopy after Congo red staining. Immunohistochemical analysis revealed that they were positive for both kappa and lambda light chains and negative for amyloid A. A diagnosis of localized primary thymic amyloidosis was finally made. After thymectomy, the symptoms of MG were controlled with reduced corticosteroid requirements. Localized thymic amyloidosis associated with MG has not been reported to date.

Multiple Myeloma with Biclonal Gammopathy Accompanied by Prostate Cancer / 대한진단검사의학회지

We report a rare case of multiple myeloma with biclonal gammopathy (IgG kappa and IgA lambda type) in a 58-year-old man with prostate cancer who presented with lower back pain. Through computed tomography (CT) imaging, an osteolytic lesion at the L3 vertebra and an enhancing lesion of the prostate gland with multiple lymphadenopathies were found. In the whole body positron emission tomography-computed tomography (PET-CT), an additional osteoblastic bone lesion was found in the left ischial bone. A prostate biopsy was performed, and adenocarcinoma was confirmed. Decompression surgery of the L3 vertebra was conducted, and the pathologic result indicated that the lesion was a plasma cell neoplasm. Immunofixation electrophoresis showed the presence of biclonal gammopathy (IgG kappa and IgA lambda). Bone marrow plasma cells (CD138 positive cells) comprised 7.2% of nucleated cells and showed kappa positivity. We started radiation therapy for the L3 vertebra lesion, with a total dose of 3,940 cGy, and androgen deprivation therapy as treatment for the prostate cancer.

Gingival plasma cell granuloma.

Plasma cell granuloma is a rare reactive lesion composed of polyclonal plasma cells. It manifests primarily in the lungs, but may occur in various other anatomic locations like the oral cavity. Intraoral plasma cell granulomas involving the tongue, lip, oral mucosa and gingiva have been reported in the past. This case presents a 54-year-old female with chronic periodontitis and mandibular anterior gingival overgrowth treated by Phase I therapy (scaling and root planing) and excisional biopsy. Histological examination revealed inflammatory cell infiltrate containing sheets of plasma cells. Immunohistochemistry for kappa and lambda light chains showed a polyclonal staining pattern confirming a diagnosis of plasma cell granuloma. This case highlights the need to biopsy for unusual lesions to rule out potential neoplasms.

Morphologic diversity of plasma cell neoplasms / 中华病理学杂志

<p><b>OBJECTIVE</b>To investigate the architectural and cytological variations of plasma cell neoplasms, and discuss the diagnosis and differential diagnosis.</p><p><b>METHODS</b>Histological and immunohistochemical examinations were used to study the morphologic and immunophenotypic features of 46 cases of plasma cell neoplasms.</p><p><b>RESULTS</b>40 out of 46 cases were diffuse growth pattern. 3 cases had a nestlike architecture that can mimic neuroendocrine tumors and 3 cases had a prominent fibrous sclerosis background. Amyloid deposition, calcification or ossification and angiomatoid areas can be prominent and may obscure the neoplastic plasma cells. Cytologically, 30 cases were composed of relatively mature plasma cells and can be recognized without too much difficulty. Tumor cells resembled immunoblasts in 6 cases and small lymphocytes in 4 cases. In another 2 cases tumor cells were easily confused with Signet-ring cells or clear cells. Tumor cells were composed of anaplastic cells, histocytoid cells and spindle cells in each one case, respectively. Lastly, tumor cells can be polymorphous which composed of multilobated, monocytoid or multinucleated cells in one case. 93.1% (27/29) cases expressed CD79a while only 5.1% (2/39) cases expressed CD20.87.1% (27/31) cases expressed CD38 and 83.3% (25/30) cases expressed CD138, 96.8% (30/31) cases expressed MUM-1. Light chain restrictions were detected in 38 cases, that 27 cases expressed lambda and 11 for kappa.</p><p><b>CONCLUSIONS</b>Except for the common architecture and cytology in plasma cell tumor, unusual morphology may appear. Thus, pay attention to distinguish from lymphoma such as small lymphocytic lymphoma and anaplastic large cell lymphoma, pooly differentiated carcinoma, clear cell carcinoma or Signet-ring cell carcinoma, sarcoma, etc. And immunohistochemistry is essential in the diagonosis.</p>

Development of metaplastic bone formation of intracranial blood vessels after isolated deposition of amyloid in a case of monoclonal gammopathy of undetermined significance

Systemic amyloidosis manifests in several organs and causes different clinical scenarios, To date, an isolated affection of intracranial blood vessels is only known to occur in cerebral amyloid angiopathy [CAA] but not is occult systemic amyloidosis or in monoclonal gammopathy of undetermined significance [MGUS]. This report describes a very rare disease because the amyloid protein was deposited for a period of nine years only in corticoleptomeningeal blood vessels as it occurs in CAA. In contrast to CAA, the reported case is coincident with a monoclonal gammopathy of undetermined significance that was not clinically and chemically detectable during about nine years. Systemic amyloid deposition was positive for the first time nine years after initial manifestation caused by a plasma cell-secreted monoclonal lambda-light chain protein representing a progression of MGUS. A pathological plasma cell clone or any signs of a proliferative plasma cell disease were not detectable until today except for the monoclonal protein. A case like has never been published before

A Case of Lambda-expressing Pulmonary MALT Lymphoma with Dual Clonal Rearrangements of Kappa and Lambda Immunoglobulin Light Chain Gene / 대한진단검사의학회지

A 70-yr-old woman was hospitalized with a history of dry cough. Bronchial endoscopy and transbronchial lung biopsy were performed. However, the findings of histopathology and immunohistochemistry were not sufficient to decide whether the lesion was benign or malignant, because of the presence of crush artifacts in the biopsy specimens. We performed B-cell clonality studies using BIOMED-2 multiplex PCR (InVivoScribe Technologies, USA) to detect clonal rearrangements in the immunoglobulin gene. The results of multiplex PCR showed clonal rearrangements of both kappa and lambda immunoglobulin light chain genes. The findings of immunochemistry revealed that the lesion expressed lambda light chain, but not kappa light chain. Based on the clinical, pathologic, and molecular findings, this case was diagnosed as pulmonary MALT lymphoma. We report the first case in Korea of lambda-expressing MALT lymphoma that is shown to have dual clonal rearrangements of kappa and lambda immunoglobulin light chain gene by multiplex PCR.

[Waldenstrom's Macroglobulinemia in Cryptogenic Cirrhosis]

The coexistence of Waldenstrom's macrglobulinemia and cryptogenic cirrhosis has been rarely reported.We describe a 72-year-old man with compensated cryptogenic cirrhosis whose major clinical presentation was hyperviscosity syndrome. Serum protein electrophoresis revealed the presence of an IgM-kappa spike. Lambda light chain was found in urine. Bone marrow biopsy was performed, which showed at least 22%infiltration with atypical plasma cells. No bone lesion was found. ByWaldenstrom'smacroglobulinemia diagnosis, patient treated with melphalan, thalidomide and dexamethasone. After three weeks of treatment, his symptoms disappeared and serum immunoglobuline M decreased below 1000 mg/dl

Measurement of serum free light chains and its clinical significance in 20 newly diagnosed patients of multiple myeloma / 中国实验血液学杂志

The objective of this study was to explore the clinical significance of measuring serum free light chains (sFLC) and to compare with serum total light chains (free and binded) in multiple myeloma (MM). sFLC in 20 newly diagnosed MM patients and 20 cases of healthy people as control were measured by immuno-nephelometric assays; the serum light chains and kappa/lambda ratio were measured in all patients, while immunofixation electrophoresis (IFE) tests were carried out at the same time in 18 out of 20 patients. The results showed that the abnormality of serum free light chains and kappa/lambda ratio were found in all of the 20 newly diagnosed MM patients (p < 0.01). The measurement of sFLC showed higher sensitivity than the total serum chains (p < 0.01). It is concluded that the method testing sFLC by immuno-nephelometric assay combined with kappa/lambda ratio is valuable for MM diagnosis, and the measurement of sFLC can be used as one of indicators for MM diagnosis.