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- Title
Diagnosis of Bone Marrow Necrosis following Severe Vaso-Occlusive Crisis in Patient with Compound Heterozygous Sickle Cell Disease.
- Authors
Marco, Daniel N.; Cid, Joan; Garrote, Marta; Cortés-Bullich, Albert; Seguí, Ferran; Lozano, Miquel
- Abstract
Introduction: Bone marrow necrosis is a rare entity that can develop in context of a sickle cell disease vaso-occlusive crisis. Its physiopathology is related to an endothelial dysfunction taking place in bone marrow microvasculature. Case Presentation: A 30-year-old patient with history of compound heterozygous sickle cell disease was admitted following SARS-CoV-2 infection with fever and diarrhea. After initial favorable evolution, he developed a severe vaso-occlusive crisis with intense hemolysis and multi-organ ischemic complications. Patient then developed high fever and hypoxemia. With the suspicion of acute thoracic syndrome, a red blood cell exchange was performed. Respiratory symptoms ceased but patient persisted febrile with very high levels of acute phase reactants, persistent pancytopenia, and leucoerythroblastic reaction. An infectious cause was ruled out. Afterward, bone marrow aspiration and bone marrow biopsy showed a picture of bone marrow necrosis, which is an extremely rare complication of vaso-occlusive crisis but, paradoxically, more frequent in milder heterozygote cases of sickle cell disease. Ultimately, large deposits of complement membrane attack complex (particles C5b-9) were demonstrated after incubation of laboratory endothelial cells with activated plasma from the patient. Discussion: The clinical presentation and findings are consistent with a case of bone marrow necrosis. In this setting, the demonstration of complement as a potential cause of the endothelial dysfunction mimics the pattern of atypical hemolytic uremic syndrome and other microangiopathic anemias. This dysregulation may be a potential therapeutic target for new complement activation blockers.
- Subjects
ISCHEMIA; GLOMERULAR filtration rate; BIOMARKERS; COVID-19; FEVER; DIARRHEA; STAINS &; staining (Microscopy); HEMATOCRIT; RETICULOCYTES; BLOOD vessels; COMPLEMENT (Immunology); ENDOTHELIUM; HEMOLYSIS &; hemolysins; GENETIC carriers; DEHYDRATION; LACTATE dehydrogenase; BONE marrow diseases; BLOOD testing; BLOOD cell count; THROMBOCYTOPENIA; RED blood cell transfusion; COMPUTED tomography; NECROSIS; SICKLE cell anemia; RARE diseases; HYPOXEMIA; NEEDLE biopsy; ACUTE kidney failure; DISEASE complications
- Publication
Transfusion Medicine & Hemotherapy, 2023, Vol 50, Issue 4, p360
- ISSN
1660-3796
- Publication type
Article
- DOI
10.1159/000529500