We present the case of a 45-year-old woman who was hospitalized due to severe macrocytic anemia and renal failure. The patient presented a morbid obesity. The immunological study showed anti-ENA anti-SSA (Ro52) positive, with negative antinuclear antibodies. Also in the proteinogram (serum immunofixation) the presence of monoclonal bands IgG lambda and IgG kappa, monoclonal component 7.2% (4.68 g/L), with elevation of free light chains (kappa 95.94 mg/L (3.3-19.4), evidenced, lambda 145.17 mg/L (5.71-26.3)). The bone marrow study showed an infiltration of 5% of plasma cells and positive for AA amyloid. Finally, a percutaneous renal biopsy was performed, which again showed amyloid infiltration. In the genetic study, 2 mutations of the family Mediterranean fever gene (MEFV) have been identified. Secondary AA amyloidosis has been described associated with obesity, in addition to a percentage of cases of unknown etiology. (C) 2018 Elsevier Espana, S.L.U. and Sociedad Espanola de Reumatologia y Colegio Mexicano de Reumatologia. All rights reserved.
机构:
Jichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
Tatara, Raine
Nagai, Tadashi
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Jichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
Nagai, Tadashi
Kobayashi, Hiroyuki
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Jichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
Kobayashi, Hiroyuki
Hatano, Kaoru
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Jichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
Hatano, Kaoru
Suzuki, Takahiro
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Jichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
Suzuki, Takahiro
Muroi, Kazuo
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Jichi Med Univ, Div Cell Transplantat & Transfus, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
Muroi, Kazuo
Ozawa, Keiya
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Jichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, JapanJichi Med Univ, Div Hematol, Shimotsuke, Tochigi 3290498, Japan
机构:
Post Grad Inst Med Educ & Res, Dept Nephrol, Chandigarh, India
Donald & Barbara Zucker Sch Med Hofstra Northwell, Glomerular Ctr Northwell Hlth, Dept Med, Div Kidney Dis & Hypertens, Great Neck, NY 11549 USAPost Grad Inst Med Educ & Res, Dept Nephrol, Chandigarh, India
Bharati, Joyita
Lahoud, Oscar B.
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Mem Sloan Kettering Canc Ctr, Dept Med, Adult Bone Marrow Transplant Serv, 1275 York Ave, New York, NY 10021 USAPost Grad Inst Med Educ & Res, Dept Nephrol, Chandigarh, India
Lahoud, Oscar B.
Jhaveri, Kenar D.
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Donald & Barbara Zucker Sch Med Hofstra Northwell, Glomerular Ctr Northwell Hlth, Dept Med, Div Kidney Dis & Hypertens, Great Neck, NY 11549 USAPost Grad Inst Med Educ & Res, Dept Nephrol, Chandigarh, India
Jhaveri, Kenar D.
Izzedine, Hassan
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Ramsay Gen Sante, Peupliers Private Hosp, Dept Nephrol, Paris, FrancePost Grad Inst Med Educ & Res, Dept Nephrol, Chandigarh, India
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Univ Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
Bernabei, Luca
Waxman, Adam
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Univ Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
Waxman, Adam
Caponetti, Gabriel
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Univ Penn, Dept Pathol & Lab Med, Philadelphia, PA USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
Caponetti, Gabriel
Fajgenbaum, David C.
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Univ Penn, Div Translat Med & Human Genet, Perelman Sch Med, Philadelphia, PA 19104 USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
Fajgenbaum, David C.
Weiss, Brendan M.
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Univ Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USAUniv Penn, Abramson Canc Ctr, Penn Amyloidosis Program, Philadelphia, PA 19104 USA
机构:
St Thomas Hosp, St Johns Histopathol Dept, Synnovis Analyt, London, EnglandSt Thomas Hosp, St Johns Histopathol Dept, Synnovis Analyt, London, England