Cargando…
Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor
Extramedullary hematopoiesis is widely known to occur in patients with primary myelofibrosis (PMF). Autopsy studies on individuals with PMF revealed that extramedullary hematopoiesis occurred in the kidneys in 35% of the cases, but there is little awareness regarding such lesions. A 63-year-old man...
Autores principales: | , , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2021
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7879319/ https://www.ncbi.nlm.nih.gov/pubmed/33614736 http://dx.doi.org/10.1159/000510142 |
_version_ | 1783650502661111808 |
---|---|
author | Asou, Mea Asakawa, Tomohiko Araki, Makoto Ehara, Takashi Hishima, Tsunekazu Sakamaki, Hisashi |
author_facet | Asou, Mea Asakawa, Tomohiko Araki, Makoto Ehara, Takashi Hishima, Tsunekazu Sakamaki, Hisashi |
author_sort | Asou, Mea |
collection | PubMed |
description | Extramedullary hematopoiesis is widely known to occur in patients with primary myelofibrosis (PMF). Autopsy studies on individuals with PMF revealed that extramedullary hematopoiesis occurred in the kidneys in 35% of the cases, but there is little awareness regarding such lesions. A 63-year-old man was diagnosed with PMF based on a detailed examination of persistent high white blood cells. An examination of the patient's medical records revealed an increased white blood cell count, deterioration of kidney function, and urinary protein excretion developed simultaneously. Thus, a kidney biopsy was performed. Advanced lymphocyte invasion was recognized in the interstitial tissue, and the tubular structure was highly disrupted. Based on these findings, he was diagnosed with interstitial nephritis. However, because of the large number of cells with nuclear atypia in the stroma, additional immunohistochemical staining was also performed, such as glycophorin A, naphthol AS-D, myeloperoxidase, and CD42b. As a result, invasion of three lineages of immature cells, erythroblasts, megakaryocytes, and granulocytes, was identified. Renal dysfunction resulting from interstitial cellular infiltration due to extramedullary hematopoiesis was therefore diagnosed. Treatment with ruxolitinib was initiated after a renal biopsy and the rate of decline in renal function was slightly reduced. Although, in myeloproliferative disorders, proliferative glomerular lesions are widely considered to be renal disorders, there is little awareness regarding interstitial lesions. Extramedullary hematopoiesis of the kidney in PMF is not uncommon, but 40% of cases are reportedly misdiagnosed as interstitial nephritis. Because extramedullary hematopoiesis can be controlled by ruxolitinib, early detection is important. |
format | Online Article Text |
id | pubmed-7879319 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2021 |
publisher | S. Karger AG |
record_format | MEDLINE/PubMed |
spelling | pubmed-78793192021-02-18 Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor Asou, Mea Asakawa, Tomohiko Araki, Makoto Ehara, Takashi Hishima, Tsunekazu Sakamaki, Hisashi Case Rep Nephrol Dial Case Report Extramedullary hematopoiesis is widely known to occur in patients with primary myelofibrosis (PMF). Autopsy studies on individuals with PMF revealed that extramedullary hematopoiesis occurred in the kidneys in 35% of the cases, but there is little awareness regarding such lesions. A 63-year-old man was diagnosed with PMF based on a detailed examination of persistent high white blood cells. An examination of the patient's medical records revealed an increased white blood cell count, deterioration of kidney function, and urinary protein excretion developed simultaneously. Thus, a kidney biopsy was performed. Advanced lymphocyte invasion was recognized in the interstitial tissue, and the tubular structure was highly disrupted. Based on these findings, he was diagnosed with interstitial nephritis. However, because of the large number of cells with nuclear atypia in the stroma, additional immunohistochemical staining was also performed, such as glycophorin A, naphthol AS-D, myeloperoxidase, and CD42b. As a result, invasion of three lineages of immature cells, erythroblasts, megakaryocytes, and granulocytes, was identified. Renal dysfunction resulting from interstitial cellular infiltration due to extramedullary hematopoiesis was therefore diagnosed. Treatment with ruxolitinib was initiated after a renal biopsy and the rate of decline in renal function was slightly reduced. Although, in myeloproliferative disorders, proliferative glomerular lesions are widely considered to be renal disorders, there is little awareness regarding interstitial lesions. Extramedullary hematopoiesis of the kidney in PMF is not uncommon, but 40% of cases are reportedly misdiagnosed as interstitial nephritis. Because extramedullary hematopoiesis can be controlled by ruxolitinib, early detection is important. S. Karger AG 2021-01-25 /pmc/articles/PMC7879319/ /pubmed/33614736 http://dx.doi.org/10.1159/000510142 Text en Copyright © 2021 by S. Karger AG, Basel http://creativecommons.org/licenses/by-nc/4.0/ This article is licensed under the Creative Commons Attribution-NonCommercial-4.0 International License (CC BY-NC) (http://www.karger.com/Services/OpenAccessLicense). Usage and distribution for commercial purposes requires written permission. |
spellingShingle | Case Report Asou, Mea Asakawa, Tomohiko Araki, Makoto Ehara, Takashi Hishima, Tsunekazu Sakamaki, Hisashi Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor |
title | Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor |
title_full | Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor |
title_fullStr | Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor |
title_full_unstemmed | Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor |
title_short | Primary Myelofibrosis-Related Renal Disorders Treated with a Janus Kinase Inhibitor |
title_sort | primary myelofibrosis-related renal disorders treated with a janus kinase inhibitor |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7879319/ https://www.ncbi.nlm.nih.gov/pubmed/33614736 http://dx.doi.org/10.1159/000510142 |
work_keys_str_mv | AT asoumea primarymyelofibrosisrelatedrenaldisorderstreatedwithajanuskinaseinhibitor AT asakawatomohiko primarymyelofibrosisrelatedrenaldisorderstreatedwithajanuskinaseinhibitor AT arakimakoto primarymyelofibrosisrelatedrenaldisorderstreatedwithajanuskinaseinhibitor AT eharatakashi primarymyelofibrosisrelatedrenaldisorderstreatedwithajanuskinaseinhibitor AT hishimatsunekazu primarymyelofibrosisrelatedrenaldisorderstreatedwithajanuskinaseinhibitor AT sakamakihisashi primarymyelofibrosisrelatedrenaldisorderstreatedwithajanuskinaseinhibitor |