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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...

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Autores principales: de Campos, Fernando Peixoto Ferraz, Ferreira, Cristiane Rúbia, Felipe-Silva, Aloísio
Formato: Online Artículo Texto
Lenguaje:English
Publicado: São Paulo, SP: Universidade de São Paulo, Hospital Universitário 2014
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.
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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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