Monoclonal Gammopathy of Undetermined Significance가 동반된 강직성 척추염 환자에서 발생한 AA형 및 AL형 심아밀로이드증

김우현 ( Woohyeon Kim ),김선아 ( Seon A Kim ),윤경진 ( Kyung Jin Yun ),나수진 ( Soo Jin Na ),현지인 ( Ji In Hyun ),정정임 ( Jung Im Jung ),곽승기 ( Seung Ki Kwok ),박성환 ( Sung Hwan Park ) 대한류마티스학회 2014 대한류마티스학회지 Vol.21 No.3

심아밀로이드증의 궁극적 치료는 아밀로이드 침착의 병인에 달려 있으므로 증상치료와 함께 초기에 아밀로이드증의 원인을 밝히는 것이 무엇보다 중요하다. 강직성 척추염에서 AA형 아밀로이드증이 심장을 침범하는 경우는 흔치 않으며, 특히 본 증례는 동반질환인 MGUS와 연관된 AL형 심아밀로이드증이 심장 조직검사를 통해 함께 진단되어 치료를 시행한 경우로, 이와 같은 예는 국내 보고된 사례가 없어 저자들이 경험한 1예를 문헌고찰과 함께 보고한다. Amyloidosis is a clinical disorder caused by extracellular deposition of proteinaceous insoluble fibrils in various tissues, resulting in organ compromise. Amyloid L (AL) amyloidosis is the most common type of systemic amyloidosis, which occurs in association with multiple myeloma or monoclonal gammopathy of undetermined significance (MGUS). Secondary amyloid A (AA) amyloidosis is a complication of chronic inflammatory conditions, such as rheumatoid arthritis or ankylosing spondylitis. We report a case of a 49-year-old manwith a 11-year history of ankylosing spondylitis, who was recently diagnosed with MGUS presented with cardiac amyloidosis of both the AA and AL types. We report this case along with a review of relevant literature.

Five-Year Follow-up Study of Monoclonal Gammopathy of Undetermined Significance in a Korean Elderly Urban Cohort

Lee, Yun-Gyoo,Bang, Soo-Mee,Lee, Jeong-Ok,Kim, Jin Won,Lee, Keun-Wook,Kim, Jee Hyun,Song, Jung Han,Kim, Tae-Hee,Kim, Ki Woong,Lee, Jong-Seok Korean Cancer Association 2015 Cancer Research and Treatment Vol.47 No.2

<P><B>Purpose</B></P><P>We previously reported the prevalence of monoclonal gammopathy of undetermined significance (MGUS) to be 3.3% among an elderly Korean urban cohort recruited during 2005-2006. Here, we report a 5-year follow-up study of the previously identified MGUS cohort.</P><P><B>Materials and Methods</B></P><P>The 680 participants from the initial cohort were followed-up for a median of 5 years. Sera were collected between 2010 and 2011. Two-step screening was performed with standard serum electrophoresis followed by immunofixation and determination of the serum concentration of monoclonal-protein (M-protein).</P><P><B>Results</B></P><P>Of the 680 participants (21 with MGUS), 348 (51%) agreed to participate in the follow-up study and 10 were found to have MGUS. Among the 21 MGUS patients initially identified, nine were followed-up, six had persistent M-protein, and one patient had progressed to multiple myeloma (progression rate, 1.0%/yr). The M-protein disappeared in the remaining two individuals. Among the 339 participants without MGUS who were followed-up, four developed an M-protein. There was no significant difference in survival with respect to the presence of MGUS (p=0.66).</P><P><B>Conclusion</B></P><P>The 5-year follow-up data show that the natural clinical course of MGUS in Korea is similar to that in Western countries. MGUS was not associated with an increased risk of death over the 5-year study period.</P>

Electrophysiological features and prognosis of peripheral neuropathy associated with IgM monoclonal gammopathy: a single-center analysis in South Korea

김수영,이빛나,김승우,신하영 대한임상신경생리학회 2023 Annals of Clinical Neurophysiology Vol.25 No.2

Background: Clinical spectrum of immunoglobulin M (IgM) monoclonal gammopathy varies from IgM monoclonal gammopathy of unknown significance (IgM-MGUS) to hematological malignancies. We evaluated the clinical features, electrophysiological characteristics, and prognosis of patients with peripheral neuropathy associated with IgM monoclonal gammopathy (PN-IgM MG). Methods: We retrospectively evaluated 25 patients with PN-IgM MG. Peripheral neuropathy was classified as axonal, demyelinating, or undetermined, based on electrophysiological studies. We classified the enrolled patients into the IgM-MGUS and malignancy groups, and compared the clinical and electrophysiological features between the groups. Results: Fifteen patients had IgM-MGUS and 10 had hematologic malignancies (Waldenström’s macroglobulinemia: two and B-cell non-Hodgkin’s lymphoma: eight). In the electrophysiological evaluation, the nerve conduction study (NCS) criteria for demyelination were met in 86.7% of the IgM-MGUS group and 10.0% of the malignancy group. In particular, the distal latencies of the motor NCS in the IgM-MGUS group were significantly prolonged compared to those in the malignancy group (median, 9.1 ± 5.1 [IgM-MGUS], 4.2 ± 1.3 [malignancy], p = 0.003; ulnar, 5.4 ± 1.9 [IgM-MGUS], 2.9 ± 0.9 [malignancy], p = 0.001; fibular, 9.3 ± 5.1 [IgM-MGUS], 3.8 ± 0.3 [malignancy], p = 0.01; P-posterior tibial, 8.3 ± 5.4 [IgM-MGUS], 4.4 ± 1.0 [malignancy], p = 0.04). Overall treatment responses were significantly worse in the malignancy group than in the IgM-MGUS group (p = 0.004), and the modified Rankin Scale score at the last visit was higher in the malignancy group than in the IgM-MGUS group (2.0 ± 1.1 [IgM-MGUS], 4.2 ± 1.7 [malignancy], p = 0.001), although there was no significant difference at the initial assessment. Conclusions: The risk of hematological malignancy should be carefully assessed in patients with PN-IgM MG without electrophysiological demyelination features.

Dysphagia as a Clinical Manifestation of Monoclonal Gammopathy of Undetermined Significance: A Case Report

Hyunjung Koo,임선,박근영,Yeonjae Han,Sangah Jeong 대한연하장애학회 2019 대한연하장애학회지 Vol.9 No.2

Swallowing can be affected by a variety of systemic diseases. The etiology of dysphagia in the geriatric population isusually overlooked due mainly to a presumed diagnosis of presbyphagia or difficulty in revealing the direct cause. On the other hand, dysphagia can be a meaningful clinical sign of premalignant systemic disease. A 78-year-oldman, without any prior medical or family history, was admitted with the chief complaint of dysphagia with recentaspiration pneumonia. Instrumental swallowing tests revealed a severe degree of dysphagia due to decreased laryngopharyngeal sensation and weakness of the pharyngeal constrictor muscles. Extensive workup, including electromyography and laboratory tests, revealed severe sensorimotor peripheral polyneuropathy related to monoclonal gammopathy. Monoclonal gammopathy of undetermined significance (MGUS) is a premalignant precursor of multiple myeloma, which is characterized by the proliferation of monoclonal proteins. These conditions are often associated withperipheral polyneuropathy, ataxia, and sometimes even muscle weakness. Although dysphagia can occur in othersystemic disorders, such as vasculitis or paraneoplastic syndrome-related malignancies, there are few reports of dysphagia related to MGUS. The patient was followed up for three years. The MGUS showed no further progression,but the patient showed no improvement, indicating a protracted clinical course and poor prognosis when dysphagiais related to MGUS.

Case Reports : Aspergillus fumigatus Scleritis Associated with Monoclonal Gammopathy of Undetermined Significance

( Dong Hyun Jo ),( Joo Youn Oh ),( Mee Kum Kim ),( Jang Won Heo ),( Jin Hak Lee ),( Won Ryang Wee ) 대한안과학회 2010 Korean Journal of Ophthalmology Vol.24 No.3

A 68-year-old woman presented with pain in her left eye. Necrosis with calcium plaques was observed on the medial part of the sclera. Aspergillus fumigatus was isolated from the culture of the necrotic area. On systemic work-up including serum and urine electrophoresis studies, the serum monoclonal protein of immunoglobulin G was detected. The patient was diagnosed with monoclonal gammopathy of undetermined significance and fungal scleritis. Despite intensive treatment with topical and oral antifungal agents, scleral inflammation and ulceration progressed, and scleral perforation and endophthalmitis developed. Debridement, antifungal irrigation, and tectonic scleral grafting were performed. The patient underwent a combined pars plana vitrectomy with an intravitreal injection of an antifungal agent. However, scleral and intraocular inflammation progressed, and the eye was enucleated. Aspergillus fumigatus was isolated from the cultures of the eviscerated materials. Giemsa staining of the excised sclera showed numerous fungal hyphae.

Monoclonal Gammopathy in Acute Myeloblastic Leukemia Transformed from Refractory Anemia with Exess of Blasts -A Case of Report-

Shin, Bo-Moon,Paik, In Ki 인제대학교 1994 仁濟醫學 Vol.15 No.1

다발성 골수종 등에서 발생하는 악성 단클론성 감마글로불린증과는 감별을 요하는 양성 단클론성 감마글로불린증이 급성 골수성 백혈병에서 관찰되는 것은 아주 드문 예로, 골수이형성증후군에서 급성 골수성 백혈병으로 전환되었던 77세의 남자 환자에서 발생한 사례를 보고하고자 한다. Monoclonal gammopathy of undetermined significance(MGUS) associated with acute leukemia is very rare. The authors experienced a case of MGUS in acute myeloblastic leukemia transformed from refractory anemia with excess of blasts in 77 year-old male.

Case Report : Concurrence of Fibrillary Glomerulonephritis and AL Amyloidosis Associated with Monoclonal Gammopathy

( Dong Suk Chang ),( Won Ik Jang ),( Dae Eun Choi ),( Ki Ryang Na ),( Kwang Sun Suh ),( Yong Tai Shin ),( Kang Wook Lee ) 대한신장학회 2011 Kidney Research and Clinical Practice Vol.30 No.2

Renal diseases with organized deposits include amyloid, fibrillary, immunotactoid, and cryoglobulinemic glomerulopathies. AL amyloidosis and fibrillary glomerulonephritis are different in the composition of their immunoglobulin deposits. Fibrils of fibrillary glomerulonephritis are usually composed of polyclonal, occasionally oligoclonal or monoclonal, immunoglobin G, but amyloidosis consists of monoclonal light chains. Simultaneous occurrence of fibrillary glomerulonephritis and AL amyloidosis is very rare. We report a case of fibrillary glomerulonephritis combined with AL amyloidosis in a 71-yr-old man.

The Association between Multiple Myeloma and Ankylosing Spondylitis: A Report of Two Cases

Hyung-Il Moon,Hyoung-Jin Chang,Ji-Eun Kim,Hoon-Young Ko,Soe-Hee Ann,민창기 대한혈액학회 2009 Blood Research Vol.44 No.3

Multiple myeloma is a malignant disease of plasma cells, whereas ankylosing spondylitis is a chronic inflammatory disease of axial joints. The relationship between the two diseases is uncertain, but chronic inflammation could trigger multiple myeloma. The authors report the cases of two ankylosing spondylitis patients with a disease duration of more than 20 years, that subsequently developed IgA kappa and IgG lambda chain myeloma, respectively, and discuss the possible pathogenetic relationship between these diseases.