Gelatinous transformation of bone marrow associated with ring sideroblasts: A diagnostic pitfall
Gelatinous bone marrow transformation (GBMT) is a rare condition characterized by adipocyte atrophy, deposition of extracellular gelatinous substance in the bone marrow and associated hypoplastic hematopoiesis. The underlying pathogenic mechanisms of GBMT remain poorly understood. Here we describe 3...
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| Veröffentlicht in: | Annals of diagnostic pathology 2024-12, Vol.73, p.152385, Article 152385 |
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| creator | Hussain, Mahreen Rawas, Faisal Gosnell, Joseph Medeiros, Leonard J. Lyapichev, Kirill A. |
| description | Gelatinous bone marrow transformation (GBMT) is a rare condition characterized by adipocyte atrophy, deposition of extracellular gelatinous substance in the bone marrow and associated hypoplastic hematopoiesis. The underlying pathogenic mechanisms of GBMT remain poorly understood. Here we describe 3 cases of GBMT associated with ring sideroblasts. An electronic search of institutional archives was conducted via the laboratory information system to identify patients with a body mass index (BMI) of |
| doi_str_mv | 10.1016/j.anndiagpath.2024.152385 |
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•Gelatinous bone marrow transformation is characterized by adipocyte atrophy and deposition of extracellular gelatinous substance.•The pathogenic mechanisms are poorly understood, highlighting the need for further research.•The gelatinous bone marrow transformation has never been reported to associate with ring sideroblasts.•Recognizing ring sideroblasts association with gelatinous transformation could prevent misdiagnosis with myelodysplastic syndrome.•We report three cases of gelatinous bone marrow transformation associated with ring sideroblasts.</description><identifier>ISSN: 1092-9134</identifier><identifier>ISSN: 1532-8198</identifier><identifier>EISSN: 1532-8198</identifier><identifier>DOI: 10.1016/j.anndiagpath.2024.152385</identifier><identifier>PMID: 39427374</identifier><language>eng</language><publisher>United States: Elsevier Inc</publisher><subject>Adult ; Aged ; Anemia, Sideroblastic - diagnosis ; Anemia, Sideroblastic - pathology ; Bone Marrow - pathology ; Bone Marrow Diseases - diagnosis ; Bone Marrow Diseases - pathology ; Diagnosis, Differential ; Female ; GBMT ; Gelatinous ; Humans ; Male ; Middle Aged ; Myelodysplastic Syndromes - diagnosis ; Myelodysplastic Syndromes - pathology ; Ring-sideroblasts</subject><ispartof>Annals of diagnostic pathology, 2024-12, Vol.73, p.152385, Article 152385</ispartof><rights>2024 Elsevier Inc.</rights><rights>Copyright © 2024 Elsevier Inc. All rights reserved.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><cites>FETCH-LOGICAL-c251t-25440a36bfcea943571141ed206a6cb2ae9ef05b1bbadb6d2edecaaf784ae96f3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://www.sciencedirect.com/science/article/pii/S1092913424001229$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,776,780,3537,27901,27902,65534</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/39427374$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Hussain, Mahreen</creatorcontrib><creatorcontrib>Rawas, Faisal</creatorcontrib><creatorcontrib>Gosnell, Joseph</creatorcontrib><creatorcontrib>Medeiros, Leonard J.</creatorcontrib><creatorcontrib>Lyapichev, Kirill A.</creatorcontrib><title>Gelatinous transformation of bone marrow associated with ring sideroblasts: A diagnostic pitfall</title><title>Annals of diagnostic pathology</title><addtitle>Ann Diagn Pathol</addtitle><description>Gelatinous bone marrow transformation (GBMT) is a rare condition characterized by adipocyte atrophy, deposition of extracellular gelatinous substance in the bone marrow and associated hypoplastic hematopoiesis. The underlying pathogenic mechanisms of GBMT remain poorly understood. Here we describe 3 cases of GBMT associated with ring sideroblasts. An electronic search of institutional archives was conducted via the laboratory information system to identify patients with a body mass index (BMI) of <18.5 who underwent bone marrow evaluation. The slides and reports for these bone marrow specimens were reviewed. Bone marrow specimens of 10 patients were identified and reviewed. Three (30 %) were found to have GBMT and ring sideroblasts, ranging from 2 to 20 %. Blasts were not increased and there was no other morphologic evidence of dysplasia. Every patient had one or more peripheral blood cytopenias. In one patient, copper deficiency was proven providing an explanation for the ring sideroblasts. To the best of our knowledge, ring sideroblasts have not been well documented in GBMT and aims to contribute to a better understanding of disease recognition and pathogenesis and also to prevent potential misdiagnosis as a myelodysplastic syndrome.
•Gelatinous bone marrow transformation is characterized by adipocyte atrophy and deposition of extracellular gelatinous substance.•The pathogenic mechanisms are poorly understood, highlighting the need for further research.•The gelatinous bone marrow transformation has never been reported to associate with ring sideroblasts.•Recognizing ring sideroblasts association with gelatinous transformation could prevent misdiagnosis with myelodysplastic syndrome.•We report three cases of gelatinous bone marrow transformation associated with ring sideroblasts.</description><subject>Adult</subject><subject>Aged</subject><subject>Anemia, Sideroblastic - diagnosis</subject><subject>Anemia, Sideroblastic - pathology</subject><subject>Bone Marrow - pathology</subject><subject>Bone Marrow Diseases - diagnosis</subject><subject>Bone Marrow Diseases - pathology</subject><subject>Diagnosis, Differential</subject><subject>Female</subject><subject>GBMT</subject><subject>Gelatinous</subject><subject>Humans</subject><subject>Male</subject><subject>Middle Aged</subject><subject>Myelodysplastic Syndromes - diagnosis</subject><subject>Myelodysplastic Syndromes - pathology</subject><subject>Ring-sideroblasts</subject><issn>1092-9134</issn><issn>1532-8198</issn><issn>1532-8198</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2024</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqNkMtOWzEQhq2qqFDoK1Rm180Jvp1bdyiitBISG1ibsT0ncXRip7ZTxNvjKBR12dWMZv65_B8hl5wtOOPd1WYBITgPqx2U9UIwoRa8FXJoP5Az3krRDHwcPtacjaIZuVSn5HPOG8Y4V23_iZzKUYle9uqMPN3iDMWHuM-0JAh5imlbCzHQOFETA9ItpBSfKeQcrYeCjj77sqbJhxXN3mGKZoZc8nd6TQ9PhZiLt3TnywTzfEFOasj45S2ek8cfNw_Ln83d_e2v5fVdY0XLSyNapRjIzkwWYVSy7euvHJ1gHXTWCMARJ9Yabgw40zmBDi3A1A-qtrpJnpNvx727FH_vMRe99dniPEPAak5LzodBKtV1VToepTbFnBNOepd8dfmiOdMHwHqj_wGsD4D1EXCd_fp2Zm-26N4n_xKtguVRgNXsH49JZ-sxWHQ-oS3aRf8fZ14BYDiVPA</recordid><startdate>202412</startdate><enddate>202412</enddate><creator>Hussain, Mahreen</creator><creator>Rawas, Faisal</creator><creator>Gosnell, Joseph</creator><creator>Medeiros, Leonard J.</creator><creator>Lyapichev, Kirill A.</creator><general>Elsevier Inc</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>202412</creationdate><title>Gelatinous transformation of bone marrow associated with ring sideroblasts: A diagnostic pitfall</title><author>Hussain, Mahreen ; Rawas, Faisal ; Gosnell, Joseph ; Medeiros, Leonard J. ; Lyapichev, Kirill A.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c251t-25440a36bfcea943571141ed206a6cb2ae9ef05b1bbadb6d2edecaaf784ae96f3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2024</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Anemia, Sideroblastic - diagnosis</topic><topic>Anemia, Sideroblastic - pathology</topic><topic>Bone Marrow - pathology</topic><topic>Bone Marrow Diseases - diagnosis</topic><topic>Bone Marrow Diseases - pathology</topic><topic>Diagnosis, Differential</topic><topic>Female</topic><topic>GBMT</topic><topic>Gelatinous</topic><topic>Humans</topic><topic>Male</topic><topic>Middle Aged</topic><topic>Myelodysplastic Syndromes - diagnosis</topic><topic>Myelodysplastic Syndromes - pathology</topic><topic>Ring-sideroblasts</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Hussain, Mahreen</creatorcontrib><creatorcontrib>Rawas, Faisal</creatorcontrib><creatorcontrib>Gosnell, Joseph</creatorcontrib><creatorcontrib>Medeiros, Leonard J.</creatorcontrib><creatorcontrib>Lyapichev, Kirill A.</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Annals of diagnostic pathology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Hussain, Mahreen</au><au>Rawas, Faisal</au><au>Gosnell, Joseph</au><au>Medeiros, Leonard J.</au><au>Lyapichev, Kirill A.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Gelatinous transformation of bone marrow associated with ring sideroblasts: A diagnostic pitfall</atitle><jtitle>Annals of diagnostic pathology</jtitle><addtitle>Ann Diagn Pathol</addtitle><date>2024-12</date><risdate>2024</risdate><volume>73</volume><spage>152385</spage><pages>152385-</pages><artnum>152385</artnum><issn>1092-9134</issn><issn>1532-8198</issn><eissn>1532-8198</eissn><abstract>Gelatinous bone marrow transformation (GBMT) is a rare condition characterized by adipocyte atrophy, deposition of extracellular gelatinous substance in the bone marrow and associated hypoplastic hematopoiesis. The underlying pathogenic mechanisms of GBMT remain poorly understood. Here we describe 3 cases of GBMT associated with ring sideroblasts. An electronic search of institutional archives was conducted via the laboratory information system to identify patients with a body mass index (BMI) of <18.5 who underwent bone marrow evaluation. The slides and reports for these bone marrow specimens were reviewed. Bone marrow specimens of 10 patients were identified and reviewed. Three (30 %) were found to have GBMT and ring sideroblasts, ranging from 2 to 20 %. Blasts were not increased and there was no other morphologic evidence of dysplasia. Every patient had one or more peripheral blood cytopenias. In one patient, copper deficiency was proven providing an explanation for the ring sideroblasts. To the best of our knowledge, ring sideroblasts have not been well documented in GBMT and aims to contribute to a better understanding of disease recognition and pathogenesis and also to prevent potential misdiagnosis as a myelodysplastic syndrome.
•Gelatinous bone marrow transformation is characterized by adipocyte atrophy and deposition of extracellular gelatinous substance.•The pathogenic mechanisms are poorly understood, highlighting the need for further research.•The gelatinous bone marrow transformation has never been reported to associate with ring sideroblasts.•Recognizing ring sideroblasts association with gelatinous transformation could prevent misdiagnosis with myelodysplastic syndrome.•We report three cases of gelatinous bone marrow transformation associated with ring sideroblasts.</abstract><cop>United States</cop><pub>Elsevier Inc</pub><pmid>39427374</pmid><doi>10.1016/j.anndiagpath.2024.152385</doi></addata></record> |
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| subjects | Adult Aged Anemia, Sideroblastic - diagnosis Anemia, Sideroblastic - pathology Bone Marrow - pathology Bone Marrow Diseases - diagnosis Bone Marrow Diseases - pathology Diagnosis, Differential Female GBMT Gelatinous Humans Male Middle Aged Myelodysplastic Syndromes - diagnosis Myelodysplastic Syndromes - pathology Ring-sideroblasts |
| title | Gelatinous transformation of bone marrow associated with ring sideroblasts: A diagnostic pitfall |
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