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Wang XF, Li T, Yang M, Huang Y. Periorbital purpura can be the only initial symptom of primary light chain amyloidosis: A case report. World J Clin Cases 2024; 12:5946-5951. [PMID: 39286381 PMCID: PMC11287502 DOI: 10.12998/wjcc.v12.i26.5946] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/06/2024] [Revised: 06/20/2024] [Accepted: 06/27/2024] [Indexed: 07/19/2024] Open
Abstract
BACKGROUND Primary light chain amyloidosis is a rare and complex disease with complex clinical features and is highly susceptible to misdiagnosis and underdiagnosis in the early stages. CASE SUMMARY We report a case of a 47-year-old female patient whose only initial symptom was periorbital purpura, which was not taken seriously enough. As the disease progressed, pleural effusion gradually appeared, and after systematic diagnosis and treatment, she was diagnosed with "primary light chain amyloidosis". She achieved rapid hematological remission after treatment with a daratumumab + bortezomib + cyclophosphamide + dexamethasone regimen. CONCLUSION Periorbital purpura can be the only initial symptom of primary light chain amyloidosis; we should pay attention to the cases where the initial clinical symptoms are only periorbital purpura.
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Affiliation(s)
- Xiu-Feng Wang
- Department of Hematology, The First Affiliated Hospital of Xinxiang Medical University, Xinxiang 453100, Henan Province, China
| | - Ting Li
- Department of Blood Purification, The First Affiliated Hospital of Xinxiang Medical University, Xinxiang 453100, Henan Province, China
| | - Man Yang
- Department of Hematology, The First Affiliated Hospital of Xinxiang Medical University, Xinxiang 453100, Henan Province, China
| | - Yan Huang
- Department of Hematology, The First Affiliated Hospital of Xinxiang Medical University, Xinxiang 453100, Henan Province, China
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Miao HL, Shen KN, Su W, Zhang L, Cao XX, Zhou DB, Li J. [Clinical presentation and prognosis in patients with light-chain amyloidosis with an ultra-high level of serum free light-chain]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2021; 42:199-204. [PMID: 33910304 PMCID: PMC8081949 DOI: 10.3760/cma.j.issn.0253-2727.2021.03.004] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 09/02/2020] [Indexed: 11/05/2022]
Abstract
Objective: To investigate the clinical features and outcomes of patients with light-chain (AL) amyloidosis with an ultra-high level of serum free light-chain (FLC) . Methods: Five hundred and ninety-five patients with AL amyloidosis were retrospectively reviewed between January 2009 and January 2020 at Peking Union Medical College Hospital. We analyzed the clinical features and prognosis of patients with ultra-high FLC levels [difference between involved and uninvolved light chains (dFLC) >500 mg/L; n=124] and those without ultra-high FLC levels (dFLC≤500 mg/L; n=471) . Results: Patients with ultra-high FLC presented with more frequent cardiac involvement (82.3% vs 70.1%, P=0.007) , and a higher percentage of patients with 2004 Mayo Ⅲ stage (41.8% vs 33.8%, P=0.029) , but less frequent renal involvement than patients without an ultra-high FLC (59.7% vs 71.8%, P=0.009) . Patients with an ultra-high FLC achieved a lower proportion of hematologic (72.4% vs 82.3%, P=0.048) and cardiac response (37.3% vs 54.7%, P=0.016) and had shorter overall survival (13.0 months vs not reached, P<0.001) and a higher early death rate within 3 months (28.2% vs 11.3%, P<0.001) than those without an ultra-high FLC. Ultra-high FLC independently predicted worse prognosis in patients with AL amyloidosis (HR=2.279, 95%CI 1.685-3.083, P<0.001) . Conclusions: Patients with an initially ultra-high FLC represented a subgroup with more common cardiac involvement, more advanced cardiac stages, and extremely poor prognosis.
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Affiliation(s)
- H L Miao
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - K N Shen
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - W Su
- Department of Clinical Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - L Zhang
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - X X Cao
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - D B Zhou
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
| | - J Li
- Department of Hematology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China
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Ren C, Ren J, Tian Z, Du Y, Hao Z, Zhang Z, Fang W, Li F, Zhang S, Hsu B, Huo L. Assessment of cardiac amyloidosis with 99mTc-pyrophosphate (PYP) quantitative SPECT. EJNMMI Phys 2021; 8:3. [PMID: 33411102 PMCID: PMC7790978 DOI: 10.1186/s40658-020-00342-7] [Citation(s) in RCA: 26] [Impact Index Per Article: 6.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2020] [Accepted: 11/24/2020] [Indexed: 02/07/2023] Open
Abstract
Background 99mTc-PYP scintigraphy provides differential diagnosis of ATTR cardiomyopathy (ATTR-CM) from light chain cardiac amyloidosis and other myocardial disorders without biopsy. This study was aimed to assess the diagnostic feasibility and the operator reproducibility of 99mTc-PYP quantitative SPECT. Method Thirty-seven consecutive patients who underwent a 99mTc-PYP thorax planar scan followed by SPECT and CT scans to diagnose suspected ATTR-CM were enrolled. For the quantitative SPECT, phantom studies were initially performed to determine the image conversion factor (ICF) and partial volume correction (PVC) factor to recover 99mTc-PYP activity concentration in the myocardium for calculating the standardized uptake value (SUV) (unit: g/ml). SUVmax was compared among groups of ATTR-CM, AL cardiac amyloidosis, and other pathogens (others) and among categories of Perugini visual scores (grades 0–3). The intra- and inter-operator reproducibility of quantitative SPECT was verified, and the corresponded repeatability coefficient (RPC) was calculated. Results The ICF was 79,327 Bq/ml to convert count rate in pixel to 99mTc activity concentration. PVC factor as a function of the measured activity concentration ratio in the myocardium and blood-pool was [y = 1.424 × (1 − exp(− 0.759 × x)) + 0.104]. SUVmax of ATTR-CM (7.50 ± 2.68) was significantly higher than those of AL (1.96 ± 0.35) and others (2.00 ± 0.74) (all p < 0.05). SUVmax of grade 3 (8.95 ± 1.89) and grade 2 (4.71 ± 0.23) were also significantly higher than those of grade 1 (1.92 ± 0.31) and grade 0 (1.59 ± 0.39) (all p < 0.05). Correlation coefficient (R2) of SUVmax reached 0.966 to 0.978 with only small systematic difference (intra = − 0.14; inter = − 0.23) between two repeated measurements. Intra- and inter-operator RPCs were 0.688 and 0.877. Conclusions 99mTc-PYP quantitative SPECT integrated with adjustable PVC factors is feasible to quantitatively and objectively assess the burden of cardiac amyloidosis for diagnosis of ATTR-CM.
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Affiliation(s)
- Chao Ren
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Shuaifuyuan, Dongcheng District, Beijing, 100730, People's Republic of China
| | - Jingyun Ren
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Shuaifuyuan, Dongcheng District, Beijing, 100730, People's Republic of China
| | - Zhuang Tian
- Department of Cardiology, Peking Union Medical College Hospital, Shuaifuyuan, Dongcheng District, Beijing, People's Republic of China
| | - Yanrong Du
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Shuaifuyuan, Dongcheng District, Beijing, 100730, People's Republic of China
| | - Zhixin Hao
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Shuaifuyuan, Dongcheng District, Beijing, 100730, People's Republic of China
| | - Zongyao Zhang
- Department of Nuclear Medicine, Fuwai Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, People's Republic of China
| | - Wei Fang
- Department of Nuclear Medicine, Fuwai Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, People's Republic of China
| | - Fang Li
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Shuaifuyuan, Dongcheng District, Beijing, 100730, People's Republic of China
| | - Shuyang Zhang
- Department of Cardiology, Peking Union Medical College Hospital, Shuaifuyuan, Dongcheng District, Beijing, People's Republic of China
| | - Bailing Hsu
- Nuclear Science and Engineering Institute, University of Missouri-Columbia, E2433 Lafferre Hall, Columbia, MO, 65211, USA.
| | - Li Huo
- Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing Key Laboratory of Molecular Targeted Diagnosis and Therapy in Nuclear Medicine, Shuaifuyuan, Dongcheng District, Beijing, 100730, People's Republic of China.
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Du J, Hou J. [How I treat immunoglobulin light chain amyloido-sis]. ZHONGHUA XUE YE XUE ZA ZHI = ZHONGHUA XUEYEXUE ZAZHI 2017; 38:469-474. [PMID: 28655088 PMCID: PMC7342979 DOI: 10.3760/cma.j.issn.0253-2727.2017.06.002] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [MESH Headings] [Download PDF] [Subscribe] [Scholar Register] [Received: 01/03/2017] [Indexed: 11/29/2022]
Affiliation(s)
| | - J Hou
- Department of Hematology, The Myeloma & Lymphoma Center, Changzheng Hospital, The Second Military Medical University, Shanghai 200003, China
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