scholarly journals Acquired Aplastic Anemia in Immunocompetent Pediatric Patients with Asymptomatic COVID-19 Infection: A Case Series

Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 1103-1103
Author(s):  
Jordan E. Fritch ◽  
Taylor Losole ◽  
Lindsey Leyden ◽  
Jiri Bedrnicek ◽  
Deborah A. Perry ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Aplastic Anemia ◽  
Pediatric Patients ◽  
Bone Marrow Aspirate ◽  
Case Series ◽  
Exercise Intolerance ◽  
White Female ◽  
History Of ◽  
Work Up ◽  
Hispanic White

Abstract Background: In pediatrics, acquired aplastic anemia (AA) is most commonly due to infection, particularly viruses, when a cause can be identified. Coronavirus disease 2019 (COVID-19) has affected more than 197 million people worldwide, and children typically experience a less severe disease course. COVID-19 is known to cause transient hematologic abnormalities, including leukopenia, lymphopenia, anemia and thrombocytosis or thrombocytopenia in severe cases. Objectives: Describe three cases of COVID-19 associated acquired aplastic anemia in immunocompetent pediatric patients. Design/Methods: Case series established by retrospective review of the electronic medical record. Results: Case 1: An 8-year-old Hispanic male presented with a three-week history of increased bruising and a one-week history of progressive exercise intolerance, shortness of breath, pallor and fatigue. Labs showed pancytopenia. Bone marrow aspirate and biopsy was markedly hypocellular at 5-10% consistent with aplastic anemia (Figure 1). Work-up for the etiology of his aplastic anemia was only significant for positive SARS-COV-2 antibodies and a SEC23B variant of unknown significance on a comprehensive bone marrow failure (BMF)/myelodysplastic syndrome (MDS)/leukemia panel from the Children's Hospital of Philadelphia (CHOP). He was treated with eltrombopag olamine and then proceeded to immunotherapy with cyclosporine (CsA) and horse antithymocyte globulin (ATG) when a sibling match was not identified for hematopoietic stem cell transplant (HSCT). Three months later, his peripheral blood counts have improved, and he is no longer transfusion-dependent. Repeat bone marrow aspirate and biopsy continues to show markedly hypocellularity at <5%. Case 2: A 5-year-old non-Hispanic white female presented with a two-week history of easy bruising, petechial rash, fatigue and bone pain. Labs showed pancytopenia, and bone marrow aspirate and biopsy showed marked hypocellularity at 5-10% consistent with aplastic anemia (Figure 2). Her aplastic anemia work-up was significant for positive SARS-COV-2 antibodies and subclinical RBC and WBC paroxysmal nocturnal hemoglobinuria (PNH) clones. She was started on eltrombopag olamine and then proceeded to immunotherapy with CsA and ATG when a matched sibling donor was not identified. Three months later, she continues to be severely neutropenic, anemic and thrombocytopenic requiring multiple transfusions. Repeat bone marrow aspirate and biopsy showed variable cellularity with some areas 10-20% and others 70% with an overall cellularity of 50%. Case 3: An 8-year-old non-Hispanic white female presented with a 10-day history of fatigue, bilateral leg pain and pallor. Labs showed pancytopenia, elevated inflammatory markers and elevated hemoglobin F. Bone marrow aspirate and biopsy demonstrated mild-moderate hypocellularity at 40-50%, left-shifted myelopoiesis and dyspoiesis in the erythroid and megakaryocytic cell lines (Figure 3). MDS and acute lymphoblastic leukemia (ALL) fluorescence in situ hybridization (FISH) panels were negative. Additional work-up revealed positive SARS-COV-2 antibodies. Her pancytopenia resolved within two weeks of her initial hospitalization. Four months later, she presented with increased bruising and fatigue. Labs showed leukocytosis, thrombocytopenia, anemia and circulating peripheral blasts. Bone marrow aspirate and biopsy was consistent with B-cell ALL. She is receiving chemotherapy on study COG AALL1732. Conclusion: Severe aplastic anemia (SAA) has high morbidity and mortality, and timely diagnosis is needed for appropriate treatment. Multiple different viral infections have been known to cause acquired aplastic anemia. Data on all the sequelae of COVID-19 infection is still emerging, but it is plausible that COVID-19 infection may cause SAA. All three patients were found to have positive COVID-19 antibodies but did not have any evidence of previous COVID-19 infection. Further research and follow-up is needed to determine if previous COVID-19 infection is indeed a risk factor for development of SAA. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

scholarly journals Phases 1–3 Clinical Trials Using Adult Stem Cells in Osteonecrosis and Nonunion Fractures

2010 ◽  
Vol 2010 ◽  
pp. 1-4 ◽  
Author(s):  
Jean-Philippe Hauzeur ◽  
Valérie Gangji
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Adult Stem Cells ◽  
Bone Marrow Aspirate ◽  
Direct Injection ◽  
Case Series ◽  
Autologous Bone Marrow ◽  
Bone Necrosis ◽  
Concentrated Bone Marrow ◽  
Made In

Nonunion fractures and aseptic bone necrosis are two pathological conditions having some impairment of the cellular part of the repair: a reduction of MSC and of the osteoblastic activation. Both are good candidates for cell-based therapies using stem cells. We made a review of the published human trials. Only autologous bone marrow aspirate implantation was until now used. In Nonunion, a direct injection—15 to 150 ml—was made in 4 case series studies. In another, the bone marrow aspirate was concentrated before injection. The results were good. In bone necrosis, only one level 1 study was published. The results at 24 months were positive in terms of reduction of the necrosis and appearance of collapse. In 3 case series studies, a treatment with concentrated bone marrow aspirates was deemed useful with good results in 76 to 96%. These results are interesting but need confirmation by controlled studies.

The natural history of laryngo‐onycho‐cutaneous syndrome: A case series of six pediatric patients and literature review

2021 ◽  
Author(s):  
Christine Prodinger ◽  
Subhanitthaya Chottianchaiwat ◽  
Jemima E. Mellerio ◽  
John A. McGrath ◽  
Linda Ozoemena ◽  
...  
Keyword(s):  
Natural History ◽  
Literature Review ◽  
Pediatric Patients ◽  
Case Series ◽  
History Of

Immune-Mediated Neutropenia in a Miniature Poodle

2019 ◽  
Vol 55 (1) ◽  
Author(s):  
Benjamin Brunson
Keyword(s):  
Bone Marrow ◽  
Bone Marrow Aspirate ◽  
Clinical Signs ◽  
Response To Therapy ◽  
Tertiary Referral Center ◽  
Immune Mediated ◽  
Specialized Equipment ◽  
History Of ◽  
Light Sedation ◽  
The Right

ABSTRACT A 10 yr old spayed female toy poodle was presented to a tertiary referral center for a 10 day history of waxing and waning lethargy, vomiting, diarrhea, and anorexia. An immune-mediated neutropenia (IMN) was suspected to be the underlying cause of her clinical signs. A bone marrow aspirate was obtained from the chostochondral junction of the 11th and 12th ribs on the right side and provided a definitive diagnosis of IMN. A positive response to therapy and repeat blood work further confirmed the diagnosis. Obtaining bone marrow aspirates from the chostochondral junction is a safe, cheap, and reliable method of diagnosing IMN and can be performed in the private practice setting with light sedation and minimal need for specialized equipment.

Occipital Nerve Blocks for Relief of Headaches in Patients With Ventriculoperitoneal Shunts: A Case Series

2019 ◽  
Vol 34 (11) ◽  
pp. 674-678 ◽  
Author(s):  
Eugene Kim ◽  
Giovanni Cucchiaro
Keyword(s):  
Pediatric Patients ◽  
Case Series ◽  
Therapeutic Interventions ◽  
Shunt Revision ◽  
Occipital Neuralgia ◽  
Pulsed Radiofrequency ◽  
Nerve Blocks ◽  
Ventriculoperitoneal Shunts ◽  
Occipital Nerve ◽  
History Of

Pediatric patients with ventriculoperitoneal shunts commonly present with headaches. We report 7 children with ventriculoperitoneal shunts and occipital headaches who received occipital nerve blocks. Eighty-six percent of patients had a history of at least 1 ventriculoperitoneal shunt revision. Headaches improved in every patient after the block. Two patients (29%) were symptom free 11 and 12 months after the block. Four patients (57%) required repeat occipital nerve blocks. Two underwent pulsed radiofrequency ablation. No complications were noted. When patients with ventriculoperitoneal shunts present with headaches, a detailed physical examination is necessary. Persistent occipital headaches with tenderness and radiation in the path of the occipital nerves can be indicative of occipital neuralgia resulting from the shunt having crossed over the path of the greater or lesser occipital nerve. Occipital nerve blocks can help as both diagnostic and therapeutic interventions in these patients.

scholarly journals Autologous Stem Cells in Cervical Spine Fusion

2020 ◽  
pp. 219256822094847
Author(s):  
Patrick C. Hsieh ◽  
Andrew S. Chung ◽  
Darrel Brodke ◽  
Jong-Beom Park ◽  
Andrea C. Skelly ◽  
...  
Keyword(s):  
Stem Cells ◽  
Bone Marrow ◽  
Cervical Spine ◽  
Bone Marrow Aspirate ◽  
A Priori ◽  
Case Series ◽  
Fusion Rate ◽  
Spine Fusion ◽  
Retrospective Cohort Studies ◽  
Cervical Spine Fusion

Study Design: Systematic review. Objectives: To systematically review, critically appraise and synthesize evidence on use of stem cells from autologous stem cells from bone marrow aspirate, adipose, or any other autologous sources for fusion in the cervical spine compared with other graft materials. Methods: A systematic search of PubMed/MEDLINE was conducted for literature published through October 31, 2018 and through February 20, 2020 for EMBASE and ClinicalTrials.gov comparing autologous cell sources for cervical spine fusion to other graft options. Results: From 36 potentially relevant citations identified, 10 studies on cervical fusion met the inclusion criteria set a priori. Two retrospective cohort studies, one comparing cancellous bone marrow (CBM) versus hydroxyapatite (HA) and the other bone marrow aspirate (BMA) combined with autograft and HA versus autograft and HA alone, were identified. No statistical differences were seen between groups in either study for improvement in function, symptoms, or fusion; however, in the study evaluating BMA, the authors reported a statistically greater fusion rate and probability of fusion over time in the BMA versus the non-BMA group. Across case series evaluating BMA, authors reported improved function and pain and fusion ranged from 84% to 100% across the studies. In general, complications were poorly reported. Conclusions: The overall quality (strength) of evidence of effectiveness and safety of autologous BMA for cervical arthrodesis in the current available literature was very low. Based on currently available data, firm conclusions regarding the effectiveness or safety of BMA in cervical fusions cannot be made.

scholarly journals Vaping in today’s pandemic: E-cigarette, or vaping, product use–associated lung injury mimicking COVID-19 in teenagers presenting with respiratory distress

2020 ◽  
Vol 8 ◽  
pp. 2050313X2096959
Author(s):  
Daphne O Darmawan ◽  
Kriti Gwal ◽  
Brian D Goudy ◽  
Sanjay Jhawar ◽  
Kiran Nandalike
Keyword(s):  
Severe Acute Respiratory Syndrome ◽  
Lung Injury ◽  
Respiratory Distress ◽  
Pediatric Patients ◽  
Clinical Presentation ◽  
Case Series ◽  
Product Use ◽  
Abdominal Symptoms ◽  
History Of ◽  
High Index Of Suspicion

The clinical presentation of children and adolescents infected with severe acute respiratory syndrome coronavirus 2 can range from asymptomatic to mild or moderate manifestations. We present a case series of three adolescents who presented during the coronavirus disease 2019 (COVID-19) pandemic with symptoms concerning for COVID-19, including fever, abdominal symptoms, cough, respiratory distress, and hypoxemia. Their laboratory results showed elevated inflammatory markers that are also commonly seen in COVID-19. The chest imaging studies mimicked COVID-19 with non-specific ground glass opacities and interstitial prominence patterns. However, severe acute respiratory syndrome coronavirus 2 testing was negative and further questioning of these adolescents and their parents revealed a history of vaping marijuana-related products leading to the eventual diagnosis of e-cigarette, or vaping, product use–associated lung injury. Our patients were successfully treated with corticosteroids. The providers caring for pediatric patients, especially adolescents, should continue to have a high index of suspicion for e-cigarette, or vaping, product use–associated lung injury in patients presenting with unexplained respiratory failure, while ruling out COVID-19.

23 Years of Management: A Retrospective Review of Treatment for Aplastic Anemia.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 1091-1091
Author(s):  
Connie M Piccone ◽  
Marie Boorman Martin ◽  
Zung Vu Tran ◽  
Kim Smith-Whitley
Keyword(s):  
Bone Marrow ◽  
Aplastic Anemia ◽  
Retrospective Review ◽  
Pediatric Patients ◽  
Conflicts Of Interest ◽  
Bone Marrow Failure ◽  
Complete Response ◽  
Primary Objective ◽  
Hematopoietic Stem ◽  
Transfusion Independence

Abstract Abstract 1091 Poster Board I-113 Introduction Aplastic anemia (AA) is a syndrome of bone marrow failure characterized by peripheral pancytopenia and marrow hypoplasia. In the past, AA was considered to be a fatal disease; however, current therapies, including bone marrow transplantation or immunosuppressive therapy (IST) with antithymocyte globulin (ATG) and cyclosporine (CSA), are curative in the majority of patients. IST is effective at restoring hematopoietic stem cell production, but relapse and evolution to myelodysplastic syndromes remain clinical challenges. Additionally, there is no real consensus regarding optimal CSA levels, duration of CSA treatment, or the optimal use of growth factors and their relationship to the development of clonal disease. Objectives The primary objective was to review treatment management for severe AA in pediatric patients in order to elucidate treatment differences and review morbidity and mortality as they relate to treatment variation. Study Design/Methods A retrospective review of pediatric patients treated at the Children's Hospital of Philadelphia for AA (both severe and moderate) over a 23 year period was performed. Results A total of 70 patients with AA were treated at our institution from 1985 to July 2008. Exclusions included: 6 patients who received some type of initial treatment at outside institutions, 4 patients who had missing records, and 2 patients who had a diagnosis of moderate AA. Thus, a total of 58 patient records were included in the analysis. Of the total patients reviewed, 60% were male and 40% were female. 34.5% of patients were African-American, and 57% were diagnosed in 2000 or later. The mean age at diagnosis was 9.5±5.8 years. 52% fell into the category of very severe AA based on published diagnostic criteria, 45% had severe AA, and 2 patients (3%) had moderate AA. 15.5% of patients developed AA in the setting of acute hepatitis. More than half of the patients treated with IST had a complete response (CR). The average time to CR was 15±15 months. Average duration of CSA treatment was 15±13 months and 8.6±10.7 months for growth factor. Two patients (3.5%) died, one from complications unrelated to AA and one from infectious complications post-BMT after initial IST failure. Average time to transfusion independence for all patients was 8±11 months (with a range of 0-54 months). Not surprisingly, the time to transfusion independence was significantly associated with IST failure (p=0.010). Patients who failed treatment had an average time to transfusion independence of 17±16 months as compared to those who were complete responders who had an average time to transfusion independence of 3±3 months. Additionally, there was a significant association between IST failure and CSA levels (p=0.014). Patients who had nontherapeutic CSA levels overall had an increased rate of treatment failure. Of those patients who were nontherapeutic, 56% were noncompliant with CSA administration. There was no significant association between IST failure and bone marrow cellularity (p=0.251). PNH was diagnosed in 5% of patients; there were no patients with evidence of myelodysplastic syndrome (MDS). Two of the 3 patients with PNH failed initial IST. Another 2 patients had evidence of a cytogenetic abnormality (16q deletion), but never progressed to MDS. (Note: averages presented as mean±SD) Conclusions/Methods With current IST regimens, AA is curative in the majority of pediatric patients. IST failure was associated with nonadherence to CSA treatment. For patients with confirmed clonal disease, it is possible that IST failure and the ultimate development of clonal disease are related. Disclosures No relevant conflicts of interest to declare.

Diagnosis of Fanconi Anemia in An Asymptomatic Adult with Mosaicism and a Molecular Explanation.

Blood ◽  
2009 ◽  
Vol 114 (22) ◽  
pp. 4213-4213
Author(s):  
Blanche P. Alter ◽  
Neelam Giri ◽  
William Hogan ◽  
Monique Johnson ◽  
Susan Olson ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Dna Repair ◽  
Stem Cell ◽  
Aplastic Anemia ◽  
Hematopoietic Stem Cell ◽  
Nonsense Mutation ◽  
Chromosome Breakage ◽  
Splice Mutation ◽  
Hematopoietic Stem ◽  
History Of

Abstract Abstract 4213 INTRODUCTION Fanconi Anemia (FA) is a primarily autosomal recessive disorder with a defective DNA repair pathway associated with mutations in any of 13 genes. The majority of patients reported in the literature have one or multiple congenital anomalies, including low birth weight, short stature, café au lait spots, abnormal radii and/or thumbs, structural renal abnormalities, microcephaly, and deafness, among others. About 25% of reported patients had few or none of these findings. The hazard of severe aplastic anemia peaks at 10 years of age, and patients have very high risks of acute myeloid leukemia and specific solid tumors, such as head and neck and gynecologic squamous cell carcinomas. Only six patients have been reported who were diagnosed between 40 and 50 years of age. However, patients may present as adults with neoplasms, or remain asymptomatic and undiagnosed. METHODS We report the diagnosis of FA in the oldest known patient, an asymptomatic 55 year old woman (Case 2), identified only because she was a potential stem cell donor for her 42 year old sister (Case 1) with severe aplastic anemia. RESULTS Case 1 had a history of thrombocytopenia at 26, and anemia and thrombocytopenia during pregnancy; physical exam was normal except for a slightly deformed thumb; blood lymphocyte chromosome aberrations were increased with both diepoxybutane (DEB) and mitomycin C (MMC). The patient died following an HLA-matched bone marrow transplant from a non-FA brother. Case 2, the other of two siblings of Case 1 who were HLA matches, had higher than normal chromosome breakage in blood (3 cells with multiple radials with MMC) but not in the FA range; skin fibroblasts were diagnostic of FA, confirming hematopoietic somatic mosaicism. She had a normal appearance, a history of hypothyroidism and mitral valve prolapse, five pregnancies with five children (one miscarriage, one set of twins), a near normal blood count (Hb 13.3 g/dl, MCV 99 fl, WBC 3500/ul, and platelets 139,000/ul), and was a regular blood donor. Bone marrow cellularity and morphology were normal, but cytogenetics showed a small clone (46,XX,add(11)(q23)[6]/46,XX[14]). Complementation analysis of Case 1 indicated group A (FA-A), and molecular analysis identified two mutations in the FANCA gene. One mutation, p.S1208S (c.3624C>T) was a splice site mutation occurring in exon 36 and has been previously described. The second mutation was a novel nonsense mutation in exon 23, p.S674X (c.2021C>A). Five siblings had normal breakage results; four were heterozygous for the nonsense mutation and one was negative for both mutations. Case 2, with mosaicism for FA, had the familial splice mutation in both blood and fibroblasts, and the familial nonsense mutation in fibroblasts, but was skewed heavily toward wild-type in blood. cDNA studies confirmed that the predicted splice mutation created an alternate splice site resulting in multiple transcripts, including exon skipping, which varied in different tissues. The molecular mechanism for the loss of the nonsense mutation in the blood is most likely due to back mutation at a hot spot, which occurred in a hematopoietic stem cell which then had a selective growth advantage. CONCLUSIONS Reversion of one FANCA mutation probably occurred in a hematopoietic stem cell which was selected for and repopulated the peripheral blood. A plausible explanation for the lack of FA clinical features is the leaky splice mutation which may provide sufficient levels of protein for normal function in DNA repair. Family members should be tested for FA by chromosome breakage analysis in blood (and/or fibroblasts to identify those who may be mosaics). Those who have FA are at risk of syndrome-specific solid tumors, as well as aplastic anemia, myelodysplastic syndrome, and leukemia, if a non-gene corrected hematopoietic stem cell were to emerge. Even if asymptomatic, they should not be used as stem cell transplant donors for siblings with FA, because they may fail to repopulate the recipient marrow. FA is undoubtedly underdiagnosed in adults at this time. Disclosures: No relevant conflicts of interest to declare.

The Value of Contrast Administration in the MRI Evaluation of Scoliosis in the Pediatric Population

2008 ◽  
Vol 21 (6) ◽  
pp. 844-847
Author(s):  
K. Abul-Kasim ◽  
D. Gomez Hassan ◽  
L. McCormick ◽  
P. Maly ◽  
P.C. Sundgren
Keyword(s):  
Pediatric Patients ◽  
Pediatric Population ◽  
Benign Lesions ◽  
Contrast Administration ◽  
Spine Imaging ◽  
Contrast Enhanced ◽  
History Of ◽  
Work Up ◽  
Spine Mri ◽  
Mri Evaluation

The aim of this study was to analyze the added utility of contrast administration for spine MRI in children with scoliosis. A retrospective review of 663 consecutive contrast-enhanced spine MRI performed in 319 patients as part of the work up of scoliosis in children 2–18 years with clinically suspected or known scoliosis over a seven year period. Those patients with known tumors (13 patients) being evaluated for scoliosis were excluded from the study. In 306 patients with scoliosis and no history of tumor pathologic contrast enhancement was seen in seven (2%) patients. Lack of enhancement helped to characterize benign lesions in 31 (10%) of the patients. Although MRI is often recommended to exclude intraspinal pathology in pediatric patients with scoliosis, the need for contrast enhanced imaging is very limited and contrast medium should not be administered unless questionable pathology is detected on noncontrast MR spine imaging.

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