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Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease
Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
São Paulo, SP: Universidade de São Paulo, Hospital Universitário
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448297/ https://www.ncbi.nlm.nih.gov/pubmed/28580322 http://dx.doi.org/10.4322/acr.2014.012 |
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author | de Campos, Fernando Peixoto Ferraz Ferreira, Cristiane Rúbia Felipe-Silva, Aloísio |
author_facet | de Campos, Fernando Peixoto Ferraz Ferreira, Cristiane Rúbia Felipe-Silva, Aloísio |
author_sort | de Campos, Fernando Peixoto Ferraz |
collection | PubMed |
description | Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC), which apparently shows a less severe clinical course, may exhibit the same complications of HbSS. These complications are usually manifested late in the course of life, when compared with the HbSS patients. It is noteworthy that HbSC may present a normal hematocrit, and therefore stays unknown until the first complication, that may be disastrous. The authors report a case of an African-Descendant woman, aging 65 years, with no previous diagnosis of anemia who sought medical attention because of a thoracic back pain followed by fever and altered mental status. The clinical picture deteriorated very fast with multiple organ failure and death. The autopsy findings concluded by generalized vaso-occlusive crisis, bone marrow necrosis and bone marrow and fat embolism, mainly to the lungs and kidney. The authors call attention for the knowledge of this severe life threatening complication, mainly in a country with a high Afro-Descendant population. |
format | Online Article Text |
id | pubmed-5448297 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | São Paulo, SP: Universidade de São Paulo, Hospital Universitário |
record_format | MEDLINE/PubMed |
spelling | pubmed-54482972017-06-02 Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease de Campos, Fernando Peixoto Ferraz Ferreira, Cristiane Rúbia Felipe-Silva, Aloísio Autops Case Rep Article / Autopsy Case Report Sickle Cell Disease encompasses a group of disorders related with the hemoglobin S and other hemoglobin genotypes. The clinical manifestation and the severity of symptoms are dependent on the specific genotype. In this setting, homozygous genotype (HbSS) presents an early onset of symptoms and a low expectancy of lifetime. However, the SC genotype (HbSC), which apparently shows a less severe clinical course, may exhibit the same complications of HbSS. These complications are usually manifested late in the course of life, when compared with the HbSS patients. It is noteworthy that HbSC may present a normal hematocrit, and therefore stays unknown until the first complication, that may be disastrous. The authors report a case of an African-Descendant woman, aging 65 years, with no previous diagnosis of anemia who sought medical attention because of a thoracic back pain followed by fever and altered mental status. The clinical picture deteriorated very fast with multiple organ failure and death. The autopsy findings concluded by generalized vaso-occlusive crisis, bone marrow necrosis and bone marrow and fat embolism, mainly to the lungs and kidney. The authors call attention for the knowledge of this severe life threatening complication, mainly in a country with a high Afro-Descendant population. São Paulo, SP: Universidade de São Paulo, Hospital Universitário 2014-06-30 /pmc/articles/PMC5448297/ /pubmed/28580322 http://dx.doi.org/10.4322/acr.2014.012 Text en Autopsy and Case Reports. ISSN 2236-1960. Copyright © 2014. http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed of terms of the Creative Commons Attribution Non-Commercial License which permits unrestricted non-commercial use, distribution, and reproduction in any medium provided article is properly cited. |
spellingShingle | Article / Autopsy Case Report de Campos, Fernando Peixoto Ferraz Ferreira, Cristiane Rúbia Felipe-Silva, Aloísio Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease |
title | Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease |
title_full | Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease |
title_fullStr | Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease |
title_full_unstemmed | Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease |
title_short | Bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin SC disease |
title_sort | bone marrow necrosis and fat embolism: an autopsy report of a severe complication of hemoglobin sc disease |
topic | Article / Autopsy Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5448297/ https://www.ncbi.nlm.nih.gov/pubmed/28580322 http://dx.doi.org/10.4322/acr.2014.012 |
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