A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement
Aim. Systemic amyloidosis caused by the synthesis and deposition of immunoglobulin light chains (AL amyloidosis) is a relatively rare disease that involves heart, kidneys, peripheral nervous system, gastrointestinal tract, and has a large number of various clinical manifestations. We present a clini...
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Gastro LLC
2022-03-01
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| Series: | Российский журнал гастроэнтерологии, гепатологии, колопроктологии |
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| Online Access: | https://www.gastro-j.ru/jour/article/view/597 |
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| author | L. S. Kholopov N. B. Gubegrits O. A. Dyadyk Yu. E. Chirkov Yu. V. Tsohoyeva |
| author_facet | L. S. Kholopov N. B. Gubegrits O. A. Dyadyk Yu. E. Chirkov Yu. V. Tsohoyeva |
| author_sort | L. S. Kholopov |
| collection | DOAJ |
| description | Aim. Systemic amyloidosis caused by the synthesis and deposition of immunoglobulin light chains (AL amyloidosis) is a relatively rare disease that involves heart, kidneys, peripheral nervous system, gastrointestinal tract, and has a large number of various clinical manifestations. We present a clinical case of systemic AL amyloidosis with a predominant involvement of liver, stomach, intestines, and heart in a Caucasian female.Key points. A Caucasian woman presented to clinic with severe general weakness, abdominal pain, diarrhea, sudden weight loss, and palpitation. Initial examination revealed a duodenal bulb ulcer complicated by bleeding and polyps in the retrobulbar part of duodenum. Decreased hemoglobin levels, elevated levels of alkaline phosphatase, gamma-glutamyltransferase, and N-terminal prohormone of brain natriuretic peptide, signs of heart failure with preserved ejection fraction, and hepatomegaly became the basis for a clinical suspicion of AL amyloidosis and puncture liver biopsy. Histochemical and immunohistochemical studies of liver, stomach, and duodenum biopsy specimens confirmed AL amyloidosis. Timely diagnosis made it possible to conduct a specific therapy with melphalan plus dexamethasone, get a satisfactory response and improve the patient’s condition.Conclusion. A thorough examination of patients along with a pathomorphological and immunohistochemical study of the biopsy specimens is the basis for confirming the diagnosis of AL amyloidosis, selecting the proper therapy, improving the condition of patients and their survival. |
| format | Article |
| id | doaj-art-100611dd51474b29b2c98a25d0b6922a |
| institution | Kabale University |
| issn | 1382-4376 2658-6673 |
| language | Russian |
| publishDate | 2022-03-01 |
| publisher | Gastro LLC |
| record_format | Article |
| series | Российский журнал гастроэнтерологии, гепатологии, колопроктологии |
| spelling | doaj-art-100611dd51474b29b2c98a25d0b6922a2025-02-10T16:14:37ZrusGastro LLCРоссийский журнал гастроэнтерологии, гепатологии, колопроктологии1382-43762658-66732022-03-01316475510.22416/1382-4376-2021-31-6-47-55454A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney InvolvementL. S. Kholopov0N. B. Gubegrits1O. A. Dyadyk2Yu. E. Chirkov3Yu. V. Tsohoyeva4Odessa National Medical UniversityInto-Sana Multifield ClinicShupyk National Healthcare University of UkraineOberig Universal ClinicOdessa National Medical UniversityAim. Systemic amyloidosis caused by the synthesis and deposition of immunoglobulin light chains (AL amyloidosis) is a relatively rare disease that involves heart, kidneys, peripheral nervous system, gastrointestinal tract, and has a large number of various clinical manifestations. We present a clinical case of systemic AL amyloidosis with a predominant involvement of liver, stomach, intestines, and heart in a Caucasian female.Key points. A Caucasian woman presented to clinic with severe general weakness, abdominal pain, diarrhea, sudden weight loss, and palpitation. Initial examination revealed a duodenal bulb ulcer complicated by bleeding and polyps in the retrobulbar part of duodenum. Decreased hemoglobin levels, elevated levels of alkaline phosphatase, gamma-glutamyltransferase, and N-terminal prohormone of brain natriuretic peptide, signs of heart failure with preserved ejection fraction, and hepatomegaly became the basis for a clinical suspicion of AL amyloidosis and puncture liver biopsy. Histochemical and immunohistochemical studies of liver, stomach, and duodenum biopsy specimens confirmed AL amyloidosis. Timely diagnosis made it possible to conduct a specific therapy with melphalan plus dexamethasone, get a satisfactory response and improve the patient’s condition.Conclusion. A thorough examination of patients along with a pathomorphological and immunohistochemical study of the biopsy specimens is the basis for confirming the diagnosis of AL amyloidosis, selecting the proper therapy, improving the condition of patients and their survival.https://www.gastro-j.ru/jour/article/view/597primary al amyloidosisimmunoglobulin light chain amyloidosis |
| spellingShingle | L. S. Kholopov N. B. Gubegrits O. A. Dyadyk Yu. E. Chirkov Yu. V. Tsohoyeva A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement Российский журнал гастроэнтерологии, гепатологии, колопроктологии primary al amyloidosis immunoglobulin light chain amyloidosis |
| title | A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement |
| title_full | A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement |
| title_fullStr | A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement |
| title_full_unstemmed | A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement |
| title_short | A Case of Primary Amyloidosis Involving Liver, Stomach, Intestines, and Heart without Evident Kidney Involvement |
| title_sort | case of primary amyloidosis involving liver stomach intestines and heart without evident kidney involvement |
| topic | primary al amyloidosis immunoglobulin light chain amyloidosis |
| url | https://www.gastro-j.ru/jour/article/view/597 |
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