Bone marrow embolism in sickle cell disease: A review

Dang, Nghia C.; Johnson, Cage S.; Eslami-Farsani, Mahmoud; Haywood, L. Julian

doi:10.1002/ajh.20348

Cited by 84 publications

(109 citation statements)

References 77 publications

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“…In the presence of polyarteritis nodosa and vasculitis, microinfarcts develop in the intestine and skin, and resident SCs do not repair the damaged organs (21). In nonsolid organs, infarcts of the bone marrow are seen with sickle cell anemia (21). Thus, the SC compartment appears to be properly equipped to modulate growth during postnatal development and regulate homeostasis in adulthood.…”

Section: Discussionmentioning

confidence: 99%

“…In all cases, occlusion of a supplying artery leads to scar formation mimicking cardiac pathology (17)(18)(19)(20). In the presence of polyarteritis nodosa and vasculitis, microinfarcts develop in the intestine and skin, and resident SCs do not repair the damaged organs (21). In nonsolid organs, infarcts of the bone marrow are seen with sickle cell anemia (21).…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Human cardiac stem cells

Bearzi

Rota

Hosoda

et al. 2007

Proc. Natl. Acad. Sci. U.S.A.

917

896

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The identification of cardiac progenitor cells in mammals raises the possibility that the human heart contains a population of stem cells capable of generating cardiomyocytes and coronary vessels. The characterization of human cardiac stem cells (hCSCs) would have important clinical implications for the management of the failing heart. We have established the conditions for the isolation and expansion of c-kit-positive hCSCs from small samples of myocardium. Additionally, we have tested whether these cells have the ability to form functionally competent human myocardium after infarction in immunocompromised animals. Here, we report the identification in vitro of a class of human c-kit-positive cardiac cells that possess the fundamental properties of stem cells: they are self-renewing, clonogenic, and multipotent. hCSCs differentiate predominantly into cardiomyocytes and, to a lesser extent, into smooth muscle cells and endothelial cells. When locally injected in the infarcted myocardium of immunodeficient mice and immunosuppressed rats, hCSCs generate a chimeric heart, which contains human myocardium composed of myocytes, coronary resistance arterioles, and capillaries. The human myocardium is structurally and functionally integrated with the rodent myocardium and contributes to the performance of the infarcted heart. Differentiated human cardiac cells possess only one set of human sex chromosomes excluding cell fusion. The lack of cell fusion was confirmed by the Cre-lox strategy. Thus, hCSCs can be isolated and expanded in vitro for subsequent autologous regeneration of dead myocardium in patients affected by heart failure of ischemic and nonischemic origin.generation of human myocardium ͉ progenitor cells ͉ stem cell niches

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Human cardiac stem cells

Bearzi

Rota

Hosoda

et al. 2007

Proc. Natl. Acad. Sci. U.S.A.

917

896

View full text Add to dashboard Cite

show abstract

“…One-fourth of FES patients are comatose on admission [8] and 43% of patients with an underlying SCD and FES have HbSC disease [3]. Laboratory investigation of patients with FES will typically show thrombocytopenia, worsening anemia, and leukocytosis secondary to bone marrow necrosis noted upon biopsy [8]. A review of the peripheral blood smear will often demonstrate a leukoerythroblastic picture, while the patient will also have high levels of serum LDH [9].…”

Section: Discussionmentioning

confidence: 99%

“…A continuum of vaso-occlusive pain crises, fat embolism syndrome (FES), and ACS has been proposed [3], as the occlusion of pulmonary vessels by fat emboli has been identified as a cause of ACS [2]. One-fourth of FES patients are comatose on admission [8] and 43% of patients with an underlying SCD and FES have HbSC disease [3]. Laboratory investigation of patients with FES will typically show thrombocytopenia, worsening anemia, and leukocytosis secondary to bone marrow necrosis noted upon biopsy [8].…”

Section: Discussionmentioning

confidence: 99%

Cerebral fat embolism syndrome mimicking thrombotic thrombocytopenic purpura in a patient with hemoglobin SC disease

2016

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A 54-year-old man with hemoglobin SC disease and a history of substance abuse presented to the Emergency Department from a nursing home with 2 days of progressive weakness, shortness of breath, and lower back pain. He developed a fever, hypoxia, and tachycardia on the day of admission. He did not have any recent changes in medications, and his family as well as the nursing home staff denied any access to illicit drugs.The patient's clinical presentation raises concern for a vaso-occlusive crisis, specifically acute chest syndrome (ACS.) ACS should be suspected when a patient with a sickle cell disorder (SCD) experiences fever, chest pain, and dyspnea, with laboratory testing demonstrating the presence of a leukocytosis and an associated decrease in hemoglobin and platelets [1]. Supportive care with hydration, oxygen, pain control, and broad-spectrum antibiotics should be initiated while awaiting hematologic, metabolic, infectious, and radiographic workups.On arrival to the hospital, his vital signs demonstrated a temperature of 39.48C, a heart rate of 140 beats per minute, and a blood pressure of 159/120 mmHg. He was hypoxic and required supplemental oxygen. Physical exam was remarkable for altered mental status (AMS) and a rapidly decreasing level of consciousness, dry mucous membranes, dilated, slightly irregular, and nonreactive pupils, and a torsional nystagmus. A later review of records from his ophthalmologist documented the presence of iris atrophy as a complication of his sickle cell disease. Upon evaluation, the patient was intubated for airway protection.ACS and the systemic inflammatory response syndrome (SIRS) remain atop the differential diagnosis. Other important considerations include severe sepsis secondary to pneumonia or meningitis, illicit drug use, metabolic disturbances, heart failure, hypertensive emergency, and embolic diseases. In general, neurologic complications are seen in 11% of patients presenting with ACS, with 31% of those patients also developing a relative thrombocytopenia [2]. While there are many etiologies that might contribute to this clinical picture, one finding that stands out is his torsional nystagmus, indicating a dysfunction in his cerebellum or brainstem. With such a finding, a metabolic or ischemic cause for his acute encephalopathy becomes more likely.Initial complete blood count showed a decrease in hemoglobin from a baseline of 13-14 to 12.4 g/dl, a decrease in platelets from a baseline of 222 to 92 k/mm 3 , and serum WBCs of 11.8 k/ml. Peripheral blood smears showed moderate amount of target cells and sickle cells, a markedly elevated number of nucleated red blood cells (65 nucleated RBCs/100 WBCs), and a slight number of schistocytes.There also was evidence of neutrophil precursors with 3% neutrophil bands, 2% metamyelocytes, and 1% myelocytes. A comprehensive metabolic panel showed an elevation of serum creatinine from a baseline of 1.2 to 1.6 mg/dl and a total bilirubin of 3.4 mg/dl. His troponin I level was 0.96 ng/ml. Further hematological work-up demonstrat...

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“…Acute thoracic syndrome (STA), a severe complication characterized by pulmonary infiltrates associated with at least one clinical sign or symptom such as chest pain, cough, wheezing, tachypnea and fever [8,[14][15][16][17]. The clinical characteristics of STA vary with age and can overlap with infectious causes and other pulmonary symptoms, preventing adequate diagnosis [1].…”

Section: Acute Thoracic Syndromementioning

confidence: 99%

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

Ac¹,

Costa²,

Tec³

et al. 2017

J Blood Lymph

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Sickle cell disease (SCD) is an autosomal recessive inheritance disorder that affects the beta-globin gene and results in the replacement of the amino acid glutamic acid by valine in the β chain of the hemoglobin molecule, producing erythrocytes with defective forms and functions. A range of pathological conditions is associated with SCD, however, one in particular stands out by gravity. Acute thoracic syndrome (STA), characterized by the presence of pulmonary infiltrates associated with a clinical symptom such as chest pain, cough, wheezing, tachypnea and fever, is considered a leading cause of death in patients with sickle cell disease. Early diagnosis and introduction of an effective approach to complication are needed to improve outcomes and minimize associated morbidity and mortality. Risk factors associated with STA in the SCDAbdominal surgery for splenectomy and cholecystectomy [19] Hypersensitivity [20,21] Smoking [20,21] Asthma [22] Infections [9,[23][24][25] Post-Surgical Hypoventilation [9,[23][24][25] Fatty Embolism [9,[23][24][25]

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Bone marrow embolism in sickle cell disease: A review

Cited by 84 publications

References 77 publications

Human cardiac stem cells

Human cardiac stem cells

Cerebral fat embolism syndrome mimicking thrombotic thrombocytopenic purpura in a patient with hemoglobin SC disease

Therapeutic Approaches of Acute Thoracic Syndrome in Patients with Falciform Disease

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