Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature
Bone marrow necrosis (BMN) is a rare clinical entity that was first described in an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined necrotic cells in an amorphous eosinophilic background with preservation of cortical bone. The pathophysiology of BMN is not well known; ho...
Saved in:
| Main Authors: | , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wiley
2017-01-01
|
| Series: | Case Reports in Hematology |
| Online Access: | http://dx.doi.org/10.1155/2017/7185604 |
| Tags: |
Add Tag
No Tags, Be the first to tag this record!
|
| _version_ | 1832553439786172416 |
|---|---|
| author | Sameera A. Alsafwani Abdulwahed Al-Saeed Rehab Bukhamsin |
| author_facet | Sameera A. Alsafwani Abdulwahed Al-Saeed Rehab Bukhamsin |
| author_sort | Sameera A. Alsafwani |
| collection | DOAJ |
| description | Bone marrow necrosis (BMN) is a rare clinical entity that was first described in an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined necrotic cells in an amorphous eosinophilic background with preservation of cortical bone. The pathophysiology of BMN is not well known; however, occlusion of the bone marrow microcirculation with subsequent hypoxia and cell injury has been thought to be common underlying features. Malignancy has been identified to be the primary cause in 90% of the cases whereas SCD was found in only 2%. In this report we present an unusual case of SCD with late onset of the disease whose initial presentation was extensive BMN. The patient was not known previously to have SCD, when suddenly she presented with severe cytopenias and marked elevation in serum lactate dehydrogenase (LDH). Bone marrow examination was done to exclude bone marrow infiltration, and BMN with dilated marrow sinuses full of irreversibly sickled cells were the unexpected findings. Patients with a mild SCD phenotype are at high risk of BMN. Thus, a high index of suspicion must be borne in mind, particularly in an area of high SCD prevalence, to recognize and prevent this catastrophic complication. |
| format | Article |
| id | doaj-art-f9a4924e03ad47259a1958829559dc93 |
| institution | Kabale University |
| issn | 2090-6560 2090-6579 |
| language | English |
| publishDate | 2017-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Hematology |
| spelling | doaj-art-f9a4924e03ad47259a1958829559dc932025-02-03T05:54:01ZengWileyCase Reports in Hematology2090-65602090-65792017-01-01201710.1155/2017/71856047185604Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the LiteratureSameera A. Alsafwani0Abdulwahed Al-Saeed1Rehab Bukhamsin2Qatif Central Hospital (QCH), Qatif, Saudi ArabiaDammam Medical Complex (DMC), Dammam, Saudi ArabiaDammam Regional Laboratory and Blood Bank (DRL), Dammam, Saudi ArabiaBone marrow necrosis (BMN) is a rare clinical entity that was first described in an autopsy of a sickle cell disease (SCD) patient and is defined as ill-defined necrotic cells in an amorphous eosinophilic background with preservation of cortical bone. The pathophysiology of BMN is not well known; however, occlusion of the bone marrow microcirculation with subsequent hypoxia and cell injury has been thought to be common underlying features. Malignancy has been identified to be the primary cause in 90% of the cases whereas SCD was found in only 2%. In this report we present an unusual case of SCD with late onset of the disease whose initial presentation was extensive BMN. The patient was not known previously to have SCD, when suddenly she presented with severe cytopenias and marked elevation in serum lactate dehydrogenase (LDH). Bone marrow examination was done to exclude bone marrow infiltration, and BMN with dilated marrow sinuses full of irreversibly sickled cells were the unexpected findings. Patients with a mild SCD phenotype are at high risk of BMN. Thus, a high index of suspicion must be borne in mind, particularly in an area of high SCD prevalence, to recognize and prevent this catastrophic complication.http://dx.doi.org/10.1155/2017/7185604 |
| spellingShingle | Sameera A. Alsafwani Abdulwahed Al-Saeed Rehab Bukhamsin Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature Case Reports in Hematology |
| title | Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature |
| title_full | Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature |
| title_fullStr | Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature |
| title_full_unstemmed | Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature |
| title_short | Extensive Bone Marrow Necrosis: Initial Presentation in Sickle Cell Anemia—A Case Report and Review of the Literature |
| title_sort | extensive bone marrow necrosis initial presentation in sickle cell anemia a case report and review of the literature |
| url | http://dx.doi.org/10.1155/2017/7185604 |
| work_keys_str_mv | AT sameeraaalsafwani extensivebonemarrownecrosisinitialpresentationinsicklecellanemiaacasereportandreviewoftheliterature AT abdulwahedalsaeed extensivebonemarrownecrosisinitialpresentationinsicklecellanemiaacasereportandreviewoftheliterature AT rehabbukhamsin extensivebonemarrownecrosisinitialpresentationinsicklecellanemiaacasereportandreviewoftheliterature |