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1.
American Journal of Gastroenterology ; 117(10 Supplement 2):S1760, 2022.
Article in English | EMBASE | ID: covidwho-2321507

ABSTRACT

Introduction: Dieulafoy lesion (DL) is a relatively rare and arguably under-recognized condition, accounting for 1-2% of acute GI bleeding. Most bleeding DLs occur in the stomach, followed by the small intestine, with less than 1% occurring in the jejunum. Bleeding DL on a jejunal diverticulum is even more rare, with a handful cases described in the literature. Here we present a rare case of a bleeding DL in a jejunal diverticulum with its endoscopic management. Case Description/Methods: A 65-year-old female with history of COVID-19 infection one month prior to presentation treated with steroids and therapeutic anticoagulation presented to the ED after having multiple episodes of coffee-ground emesis and two episodes of syncope at home. Last dose of Apixaban was 12 hours prior to admission. Physical exam revealed BP of 90/60 on Norepinephrine infusion, HR of 96, abdominal exam was soft and nontender, DRE revealed melena. Hemoglobin/hematocrit was significantly decreased at 3.6/12.8. Patient was appropriately resuscitated with blood products and fluids, and she was scheduled for an EGD. Initial EGD did not identify a clear source of her bleeding, and she was scheduled for colonoscopy. Colonoscopy with deep cannulation of the terminal ileum up to 40cm revealed significant amounts of fresh blood all throughout the colon and terminal ileum. Decision was made for push enteroscopy, which revealed a jejunal diverticulum containing a Dieulafoy lesion with an overlying clot (Image A). The lesion was first injected with epinephrine at 2 sites followed by a clot removal overlying the lesion using 13-0 circular snare. A clear stigma of recent bleeding was noticed from the lesion after clot removal (Image B), after which 2 metallic clips were placed over the lesion to achieve hemostasis (Image C). The patient had no further episodes of bleeding and was follow up in clinic eventually, recovering well. Discussion(s): Because of the life-threatening nature of Dieulafoy lesions, identification is of paramount importance for treatment purposes. Jejunal DLs are a rare entity but should be considered in cases with negative bidirectional endoscopies. In our case, push enteroscopy helped identify the bleeding lesion. DL in a diverticulum can pose a challenge to the endoscopist due to difficulty of access to the lesion. Epinephrine injection followed by mechanical clipping showed a positive outcome in our case which can be considered while approaching bleeding DLs in a diverticulum. (Figure Presented).

2.
Chest ; 162(4):A2177, 2022.
Article in English | EMBASE | ID: covidwho-2060907

ABSTRACT

SESSION TITLE: Systemic Diseases Causing Pulmonary Havoc SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Choriocarcinoma is the most common type of gestational trophoblastic neoplasm (GTN) and can occur in association with any pregnancy [1]. The main risk factors are advanced or very young maternal age, ethnicity, ectopic pregnancy, abortion, and prior molar pregnancy. The most common sites of choriocarcinoma metastasis are lungs, liver, and brain [2]. This case describes a patient with choriocarcinoma that presented with hemoptysis. CASE PRESENTATION: The patient is a 22 year-old G2P1 female presenting at 36 weeks-gestation with one week of hemoptysis. She denied any other symptoms. On presentation, she was tachycardic. Physical examination demonstrated bibasilar crackles. Admission chest x-ray revealed diffuse bilateral infiltrates (Fig 1). Hs-troponin was elevated to 144 ng/L;however, EKG did not show ischemic changes. Cultures were obtained prior to empirically initiating antibiotics. Despite antibiotic treatment, hemoptysis worsened over her course and oxygen requirements increased. Infectious workup was negative. CT obtained for pulmonary embolism revealed bilateral patchy airspace opacities in lungs, suspected due to multifocal pneumonia (Fig 2). AFB smear and quantiferon were negative. After an emergent C-section for increased oxygen requirements, bronchoscopy with BAL was obtained and demonstrated diffuse alveolar hemorrhage. BAL was only positive for mildly increased CD4:CD8 ratio. Transbronchial biopsy was aborted due to bleed risk. Subsequent right lobe wedge biopsy confirmed metastatic choriocarcinoma. Her serum human chorionic gonadotropin (ß-hCG) level was found to be 20,713 milli-international units/mL. DISCUSSION: The etiology of hemoptysis was initially thought to be secondary to pneumonia. Differential diagnoses also included an acute COVID infection, alveolar hemorrhage, tuberculosis in a recently-immigrated patient, myocarditis, autoimmune etiology, and malignancy. Patient's risk factors included a prior miscarriage. Rarely, bleeding can occur as a result of metastatic lesions and may result in abdominal pain, hemoptysis, melena, or evidence of increased intracranial pressure from intracerebral hemorrhage [2]. Patients, such as the one described in this case, can exhibit pulmonary symptoms of dyspnea, cough, and chest pain caused by lung metastases. Upon closer examination of the CT scans, several of the opacities are nodular and consistent with GTN. Patients treated with surgery, chemotherapy, or a combination of both demonstrated similar treatment outcomes;chemotherapy may still be the preferred option. The overall cure rate in treating these tumors is currently > 90% [2]. CONCLUSIONS: GTN, although rare, should be considered as a differential diagnosis in women with a pregnancy history and risk factors that present with the primary symptom of hemoptysis. High index of suspicion and awareness of these neoplasms are necessary for timely diagnosis. Reference #1: Savage P. Winter M. Parker V. et al. Demographics, natural history and treatment outcomes of non-molar gestational choriocarcinoma: a UK population study. BJOG. 2020;127: 1102-1107 Reference #2: Lurain, J., 2010. Gestational trophoblastic disease I: epidemiology, pathology, clinical presentation and diagnosis of gestational trophoblastic disease, and management of hydatidiform mole. American Journal of Obstetrics and Gynecology, 203(6), pp.531-539. DISCLOSURES: No relevant relationships by Crystal Ajja No relevant relationships by Heba Osman No relevant relationships by James Rowley

3.
Chest ; 162(4):A877, 2022.
Article in English | EMBASE | ID: covidwho-2060716

ABSTRACT

SESSION TITLE: Critical Care Infections SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 09:15 am - 10:15 am INTRODUCTION: Francisella tularensis is a zoonotic disease by an aerobic, gram negative coccobacillus. It is transmitted by exposure to infected animal or vectors in individuals who landscape or camp. Common symptoms are fever, chills, anorexia, and headache. Abdominal tularemia can present with abdominal pain, emesis, diarrhea, and rarely intestinal ulceration and hemorrhage. It is treated with aminoglycosides, fluoroquinolones and tetracycline. CASE PRESENTATION: 38-year-old male presented with fever, cough, anorexia, and black stool for 5 days. Patient worked as a landscaper. He has no pets, travel history or sick contacts. He does not take any medications at home. Physical exam was significant for sinus tachycardia and rhonchi of right upper lobe. Significant labs include WBC of 9.8 with 41% bands, hemoglobin 15.5, sodium 125, procalcitonin 27.3, and lactic acid 1.8. COVID-19, MRSA, Legionella and Pneumococcal urine antigen were negative. CTA chest revealed mass-like opacity in right upper lobe with multiple bilateral pulmonary nodules. Lower respiratory culture showed Candida albicans. Patient was empirically started on ceftriaxone and azithromycin. He was transferred to intensive care for worsening respiratory status and was placed on non-invasive ventilation on hospital day 1. Antibiotics were broadened to ceftaroline and levofloxacin due to suspicion of tularemia. Amphotericin B was added. Labs for Histoplasma, Blastomyces, TB, Leptospira, and HIV were negative. Patient then suffered a cardiac arrest on hospital day 2 after having large brown secretions pouring from his mouth. Cardiopulmonary resuscitation was initiated and patient was intubated and started on vasopressors with return of spontaneous circulation. Massive blood transfusion protocol was initiated. Emergent bedside upper endoscopy showed large blood clot adherent to duodenal ulcer. Interventional radiology planned on performing gastric duodenal artery embolization. However, patient suffered two more cardiac arrest with resuscitation efforts terminated per family request. Karius Digital Culture later was positive for Francisella tularensis. Autopsy revealed diffuse alveolar hemorrhage, hilar lymphadenopathy, and perforated duodenal ulceration with large adherent clot. DISCUSSION: Gastrointestinal tularemia is rare and usually from drinking contaminated water or oral inoculation of bacteria. Intestinal tract involvement can present with mesenteric lymphadenopathy and ulcerative lesions resulting in gastrointestinal bleeding with case fatality rate of 50%. Even though this is noted in the literature, to our knowledge no case reports have been published. CONCLUSIONS: Careful history taking and early identification of risk factors are important when severe tularemia infection is suspected such as in individuals with extensive outdoor activities. Treatment should be empirically initiated in high risk patients. Reference #1: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4585636/ Reference #2: https://casereports.bmj.com/content/2017/bcr-2017-22125. Reference #3: Altman GB, Wachs JE. Tularemia: A pathogen in nature and a biological weapon. Aaohn Journal. 2002 Aug;50(8):373-9. DISCLOSURES: No relevant relationships by Maria Haider Baig

4.
Journal of Clinical and Diagnostic Research ; 16(9):OD01-OD03, 2022.
Article in English | EMBASE | ID: covidwho-2044184

ABSTRACT

Mucormycosis or zygomycosis is a life threatening invasive fungal infection, usually seen in patients with alteration of their immune system. It is a lethal and an aggressive fungal infection caused by the fungi of the order Mucorales. The angioinvasive property of mucormycosis can lead to fatal complications such as intracranial bleed. Acute pancreatitis refers to inflammation of the pancreas which presents mainly as acute pain in the abdomen and is a potentially fatal condition. The association of mucormycosis with acute pancreatitis is rare but dangerous. This case report highlights a case of 32-year-old male patient, with no co-morbidities, who was admitted to an rural central Indian hospital with four days of abdominal pain and two days of headache. Patient appeared to be in good health prior to this event. He was ultimately diagnosed with mucormycosis of paranasal sinus with acute pancreatitis. The patient was treated with intravenous antifungals, antibiotics and fluid therapy along with other supportive measures. Patient later developed intracranial bleed five days after admission, and ultimately succumbed on day seven of admission. After an extensive review of literature it was found that this is the first article to report mucormycosis, acute pancreatitis and intracranial bleed all occurring at once in an immunocompetent male.

5.
Journal of General Internal Medicine ; 37:S457, 2022.
Article in English | EMBASE | ID: covidwho-1995812

ABSTRACT

CASE: A 73-year-old male with a history of prostate cancer, hypertension and hyperthyroidism presented with one week of worsening dyspnea, productive cough and pleurisy. He also endorsed new orthopnea and melena over the last three days. Home medications included abiraterone, prednisone, methimazole and amlodipine. On admission, vitals were notable for tachycardia, tachypnea and hypoxia (82% on room air and 90% on 3L by nasal canula (NC)). Initials labs showed WBC count 17.4, Hemoglobin 7.1, proBNP 256, two negative COVID-19 PCR tests, negative respiratory virus panel and normal TSH and PSA. CTPE was negative for pulmonary embolism but showed new diffuse ground glass opacities. The patient was started on broad spectrum antibiotics and IV diuretics for possible pneumonia and new heart failure. However, the patient's respiratory status continued to decline, now requiring 6L by NC. Hemoglobin also continued to drop precipitously. A broad rheumatologic and infectious workup was largely negative with findings notable for a positive ANA, CRP 74, LDH 359 and an undetectable haptoglobin. A urinalysis was positive for protein and blood. At this time, empiric treatment for pneumocystis pneumonia was initiated with a plan for bronchoscopy. The bronchoscopy with bronchoalveolar lavage (BAL) revealed diffuse alveolar hemorrhage (DAH) with studies negative for infection or malignancy. An upper endoscopy did not reveal any gastrointestinal source of bleeding but rather favored a pulmonary source due to some red blood in the esophagus and coffee ground material in the stomach. Given these findings, a diagnosis of “Methimazole induced vasculitis with DAH” was made, a diagnosis of exclusion. The patient was started on pulse steroids for three days and his methimazole was held. By day four, the patient reported improvement and his oxygen was decreased to 2L. He was subsequently discharged on a steroid taper. At his two-week follow-up, the patient had improving respiratory status and repeat labs showed an improved and stable hemoglobin, and normal haptoglobin. IMPACT/DISCUSSION: This case illustrates a rare but life-threatening complication of methimazole use. Common offenders of drug-induced DAH include propylthiouracil, carbimazole and hydralazine. This complication is reported in 15-37% of patients on propylthiouracil but only 0-3% of patients on methimazole. A third of patients with DAH do not present with hemoptysis making this diagnosis challenging. Lab findings can also be largely nonspecific making a thorough history, imaging and interdisciplinary collaboration key in identifying this adverse effect early on to prevent mortality. CONCLUSION: Include drug-induced DAH on the differential for patients presenting with respiratory failure in the setting of new anemia, melena or hemoptysis. Stopping the offending drug and initiating steroids is the treatment of choice. Consider empiric PCP treatment and BAL for patients with severe hypoxia, ground glass opacities and immunosuppression.

6.
Gastroenterology ; 162(7):S-490, 2022.
Article in English | EMBASE | ID: covidwho-1967326

ABSTRACT

Introduction: Since the advent of the COVID-19 pandemic, infected patients demonstrate severe coagulation disturbances leading to considerable mortality. COVID-19 vaccination has been shown to not only reduce infection risk but also to improve survival from breakthrough infections. It is not known if COVID vaccination improves outcomes from bleeding. Alabama has one of the lowest vaccination rates in the US. We, therefore, sought to examine the effect of vaccination on patient outcomes with GI bleeding in the setting of a COVID infection in this population. Methods: A retrospective review was conducted of adult patients admitted at a single institution with GI bleeding and COVID infection from May 2020 to October 2021. Inclusion Criteria included patients who had active COVID infection and evidence of GI bleeding (hematemesis, melena, hematochezia or anemia secondary to GI blood loss). Data collected included baseline demographics, vaccination status, mortality, and inpatient treatment including supplemental oxygen requirement, mechanical ventilation, and blood transfusions. The group was dichotomized by vaccination status and clinical outcomes were compared. Results: A total of 113 patients were included in the final analysis. The mean age was 57.3 years (range 19-93), 51.3% were female, and 68.1% identified as White. 44 patients (39.0%) and 63 (61.0%) were vaccinated and unvaccinated, respectively. Vaccinated patients were older than unvaccinated (mean age 63.3 vs. 53.1 years, p=0.003) and more likely to be White (72.7% vs. 50.7%, p=0.03) but had similar gender (%female, 45.5% vs. 54.4%, p=0.44). At presentation, the two groups had similar pulmonary status (vaccinated vs. unvaccinated, need for supplemental oxygen: 11.4% vs. 17.4%, p=0.43;need for mechanical ventilation, 0% vs. 5.8%, p=0.16). Vaccinated patients required significantly fewer blood transfusions (mean, 0.2 units vs. 1.4 units, p=0.03), and this translated to lower mortality (0% vs. 10.1%, p=0.04). In multivariable logistic analysis, the strongest predictor of mortality was lack of COVID-19 vaccination (OR=infinity, p=0.004). Conclusions: In this early analysis, COVID vaccination is associated with decreased mortality related to GI bleeding. This was true even when initial oxygen requirements were accounted for in either of the groups. Further work should be done to elucidate differences in the coagulation cascade in these patient cohorts.(Table Presented)

7.
IHJ Cardiovascular Case Reports (CVCR) ; 6(2):83-85, 2022.
Article in English | EMBASE | ID: covidwho-1956162
8.
Italian Journal of Medicine ; 16(SUPPL 1):30, 2022.
Article in English | EMBASE | ID: covidwho-1912886

ABSTRACT

Introduction: Haemophagocytic Lymphohistiocytosis (HLH) is a rare immune disorder with progressive systemic inflammatory desease. Diagnostic criteria includes: fever;hepato-splenomegaly;cytopenias affecting at least 2 of 3 lineage (HB, PLT, N);hypertriglycerideamia;haemophagocytosis in bone marrow, spleen, lymph nodes or liver;low NK cell activity;elevated ferritin;elevated sCD25. Description: A 67-years-old man was admitted in sept 2021 for melena and fever. Blood exams showed bicytopenia (hb: 8,3 g/dl, PLT: 22.000/mmc, PT, aPTT, fibrinogen normal, ALT 106 U/L, PCR 125.7 mg/L). Covid buffer was positive with a mild interstitial pneumonia. We started therapy with methylprednisolone 40 mg/die. A bone marrow aspiration showed haemophagocytosis however in few days hematological disorder resolved and patient was discharged. After 30 days he returned for headache. There was again bicytopenia, elevated PCR, increased transaminases, triglyceride: 302 mg/dl, ferritin: 10280 ug/L. A second bone marrow biopsy confirmed haemohphagocytosis. A PET and a CT were normal. This time there was hemolysis and we started methylprednisolone 1 mg/kg. After 10 days exams get worse and we increased steroid to 2 mg/kg/die without benefit. In suspected secondary HLH to covid infection we started intravenous immune globulin (IVIG) 1 gr/kg for 2 days. After 2 weeks emolytic signs and bycitopenia resolved. Conclusions: COVID-19 infection is correlated to a rare syndrome like HLH. In this case in strong suspect of HLH, after steroid failure, IGEV resolved the disorder.

9.
Hematology, Transfusion and Cell Therapy ; 43:S1-S2, 2021.
Article in English | EMBASE | ID: covidwho-1859582

ABSTRACT

Relato de caso: Homem de 73 anos;ex-etilista de consumo de destilados em grande quantidade diariamente e ex-tabagista. O histórico de comorbidades consiste em hipertensão arterial sistêmica com tratamento desconhecido, além de apresentar sintomas de hiporexia, hiporreflexia, fraqueza muscular, incontinência urinária/fecal e dificuldades em deambular. O paciente esteve há 60 dias passando grande parte do tempo acamado, devido aos sintomas terem se intensificado. Nos últimos 7 dias o paciente apresentou diarreia com coloração escura e presença de sangue (melena/hematoquezia). Foi encaminhado em maio à Unidade de Pronto Atendimento devido à falta de ar e tosse discretamente produtiva. Respectivo aos sintomas, iniciou-se o tratamento com Ceftriaxona 2 g de 12 em 12 horas, Oseltamivir, Enoxaparina 40 mg e Transamin 500 mg de 8 em 8 horas. A indicativa do tratamento foi decorrente da suspeita de Covid-19. Como havia suspeita de infecção por Covid-19, o paciente precisou ser transferido para para um Hospital Público do Oeste do Paraná. Os exames não-laboratoriais realizados no momento da admissão foram a ultrassom abdominal total, tomografia computadorizada e endoscopia digestiva alta que mostrou uma gastrite erosiva piana leve de antro, característica em casos de excesso de álcool. Já os exames laboratoriais, o hemograma mostrou-se descompensado com eritrócitos 0,68 milhões/mm3, hemoglobina 2,5 g/dL, hematócrito 6,4% VCM 94,1 pg/L e RDW 23,4%, indicando uma anisocitose++. Ainda na série vermelha, teve presença de poliquilocitose++ com macrócitos+ e pontilhado basófilo+;policromasia++ e 1 eritroblasto em 100 leucócitos contados. O leucograma com 2.909/mm3 apresentou 2% de linfócitos atípicos, e entre os 75% de segmentados, foram contados 48 hipersegmentados. Também foi relatado um quadro de plaquetopenia com 22.900/mm3. Outros exames laboratoriais realizados que tiveram valores aumentados e significativos, foram a Desidrogenase Láctica (5.227 U/L), Ferritina (584,4 ng/mL) e ProBNP N-Terminal (2.961,0 pg/mL), após o hemograma apontar possível anemia megaloblástica, foi realizada a dosagem de folato (1,2 ng/mL) e vitamina B12 (138 pg/mL). Devido à anemia severa apresentada através do hemograma, foi iniciado transfusão de 2 concentrados de hemácia ao paciente, mais 5 unidades de plaquetas e mais 600 mL de plasma. Novos hemogramas foram realizados posteriormente em dias alterados, porém não houve melhora significativa dos parâmetros hematimétricos, bem como leucograma, plaquetograma e laboratoriais bioquímicos, mantendo suspeita diagnóstica de anemia megaloblástica grave devido alcoolismo crônico;cirrose hepática alcoólica e gastrite erosiva plana leve de antro. Após confirmação do resultado negativo para Covid-19, o paciente recebeu alta e foi encaminhado para ser acompanhado pelo ambulatório do Hospital, recebeu prescrição de reposição de vitamina B12, Citoneurim, ácido fólico e omeprazol. Discussão e conclusão: A anemia megaloblástica faz parte do grupo das macrocíticas, capaz de afetar as linhagens sanguíneas, como a dissociação núcleo-citoplasma. São desencadeadas pela deficiência de vitamina B12 ou de ácido fólico, sendo os mesmos, indicados como reposição para tratamento. É sabido que um dos efeitos sistêmicos do alcoolismo é a alteração sanguínea, como anemia, leucopenia, trombocitopenia e macrocitose. Por isso, a necessidade de se investigar com tanta atenção pacientes etilistas ou ex-etilistas.

10.
Gastro Hep Adv ; 1(4): 487-499, 2022.
Article in English | MEDLINE | ID: covidwho-1814422

ABSTRACT

Background and Aims: Over 404 million people worldwide have been infected with coronavirus disease-2019 (COVID-19), 145 million in the United States (77 million) and Europe (151 million) alone (as of February 10, 2022). This paper aims to analyze data from studies reporting gastrointestinal bleeding (GIB) and/or endoscopic findings in COVID-19 patients in Western countries. Methods: We conducted a systematic review of articles on confirmed COVID-19 cases with GIB in Western countries published in PubMed and Google Scholar databases from June 20, 2020, to July 10, 2021. Results: A total of 12 studies reporting GIB and/or endoscopic findings in 808 COVID-19 patients in Western countries were collected and analyzed. Outcomes and comorbidities were compared with 18,179 non-GIB COVID-19 patients from Italy and the United States. As per our study findings, the overall incidence of GIB in COVID-19 patients was found to be 0.06%. When compared to the non-GIB cohort, the death rate was significantly high in COVID-19 patients with GIB (16.4% vs 25.4%, P < .001, respectively). Endoscopic treatment was rarely necessary, and blood transfusion was the most common GIB treatment. The most common presentation in GIB patients is melena (n = 117, 47.5%). Peptic, esophageal, and rectal ulcers were the most common endoscopic findings in upper (48.4%) and lower (36.4%) endoscopies. The GIB cohort had worse outcomes and higher incidence of hypertension (61.1%), liver disease (11.2%), and cancer (13.6%) than the non-GIB cohort. Death was strongly associated with hypertension (P < .001, r = 0.814), hematochezia (P < .001, r = 0.646), and esophagogastroduodenoscopy (P < .001, r = 0.591) in COVID-19 patients with GIB. Conclusions: Overall, the incidence of GIB in COVID-19 patients is similar to that estimated in the overall population, with melena being the most common presentation. The common endoscopic findings in GIB COVID-19 patients were ulcers, esophagitis, gastritis, and colitis. Patients with GIB were more prone to death than non-GIB COVID-19 patients.

11.
Blood ; 138:4220, 2021.
Article in English | EMBASE | ID: covidwho-1582199

ABSTRACT

BACKGROUND: Immune thrombocytopenia purpura (ITP) has a complex pathogenesis and may be a primary diagnosis or secondary to an underlying condition 1. Evaluation for underlying diagnoses in patients presenting with atypical features of classic ITP is key, as this can impact treatment decisions, therapy response, and prognosis. Genetic variants that predispose patients to ITP are especially important to investigate as patients may be at risk for additional autoimmune phenomenon or malignancy. The SARS CoV-2 pandemic has added further complexity as reports suggest the infection can lead to autoimmunity in those with genetic predispositions 2,3. Loss of the suppressor of cytokine signaling 1 (SOCS1) function has been described to manifest with autoinflammatory syndrome, with or without immunodeficiency 4,5. Reports of autoimmunity developing in patients with SOCS1 haploinsufficiency after SARS CoV-2 infection are documented, including multi-system inflammatory syndrome (MIS-C) 2. A proposed mechanism of this virus-triggered autoimmunity includes a transient innate and adaptive immunodeficiency 3. This raises the question whether patients harboring genetic variants with risk of autoimmunity are placed at an even higher risk for ITP in the wake of SARS-CoV2 infection. CASE PRESENTATION: We present a 6-year-old female with isolated thrombocytopenia of 4,000/uL identified during evaluation for severe arthralgias unresponsive to corticosteroid treatment (maximum dose 1mg/kg/day) over a 6-month period. Laboratory results at presentation were consistent with ITP, including presence of platelet autoantibodies. Evaluation revealed hypocellularity for age (~50%) on bone marrow evaluation as well as elevated IgE (2080 kU/L), with IgA, IgM, and IgG levels within reference range. She had a remote history of SARS CoV-2-like illness and SARS CoV-2 antibodies were found present in serologic assay, without a history of vaccination. Genetic testing, including chromosomal microarray from peripheral blood and marrow, was included in the diagnostic workup given concern for a history of developmental delays with macrocephaly and necessity to rule-out malignancy with the patient noted to have a 5 mega-base deletion at 16p13.2p13.11, which includes the SOCS1 gene. Comprehensive next generation sequencing for additional immune dysregulation/primary immunodeficiency associated variants was unremarkable. Functional studies of surface expression of interferon-inducible genes (CD169 (SIGLEC-1)) and STAT1 phosphorylation via analysis of CD14+ monocytes revealed excess interferon signaling previously described in patients with SOCS1 haploinsufficiency (Figure 1). Measurements of B-cell-activating factor were also found to be extremely elevated at 6432 pg/mL. The patient's ITP course was complicated by hematuria, melena and refractory platelet response to first line therapy consisting of intravenous immunoglobulin 1 g/kg x2 doses and 2 mg/kg/day prednisolone. She required escalation to high dose methylprednisolone (30mg/kg), rituximab 375 mg/m 2/weekly x4 doses, and concurrent romiplostim (2 doses) for control of thrombocytopenia and bleeding manifestations. Her rheumatologic symptoms subsided with initiation of corticosteroids, and she has subsequently completed a prolonged corticosteroid taper. She currently has a normal platelet count with non-steroidal anti-inflammatory therapy utilized for arthralgia management with plan to transition to JAK inhibition for maintenance therapy. CONCLUSION: This case highlights the potential impact of investigating for susceptibility genes for ITP with consideration for broader testing including targeted next generation sequencing panels or microarray analysis in patients with atypical ITP presentations or response to therapy, as knowledge of this patient's underlying genetics led to earlier treatment and use of alternative agents. Additionally, the case adds the novel finding of bone marrow hypocellularity to the clinical phenotype of SOCS1 haploinsufficiency, as this has not yet been reported and contributes to the literature on the relationship of autoimmunity and SARS CoV-2 infections in patients with predisposing genetic variants. [Formula presented] Disclosures: Walkovich: Horizon Pharmaceuticals: Honoraria, Membership on an entity's Board of Directors or advisory committees;Pharming: Honoraria, Membership on an entity's Board of Directors or advisory committees;Swedish Orphan Biovitrum AB (Sobi): Consultancy, Honoraria;X4 Pharmaceuticals: Other: Local PI for clinical trial involving mavorixafor and patients with neutropenia.

12.
J Intern Med ; 289(6): 887-894, 2021 06.
Article in English | MEDLINE | ID: covidwho-991596

ABSTRACT

BACKGROUND AND AIMS: Gastrointestinal (GI) bleeding has been observed amongst patients hospitalized with COVID-19. Recently, anticoagulation has shown to decrease mortality, but it is unclear whether this contributes to increased GI bleeding. The aims of this study are: (i) to examine whether there are risk factors for GI bleeding in COVID-19 patients and (ii) to study whether there is a mortality difference between hospitalized patients with COVID-19 with and without GI bleeding. METHODS: This is a propensity score matched case-control study from a large health system in the New York metropolitan area between March 1st and April 27th. COVID-19 patients with GI bleeding were matched 1:1 to COVID-19 patients without bleeding using a propensity score that took into account comorbidities, demographics, GI bleeding risk factors and length of stay. RESULTS: Of 11, 158 hospitalized with COVID-19, 314 patients were identified with GI bleeding. The point prevalence of GI bleeding was 3%. There were no identifiable risk factors for GI bleeding. Use of anticoagulation medication or antiplatelet agents was not associated with increased risk of GI bleeding in COVID-19 patients. For patients who developed a GI bleed during the hospitalization, there was an increased mortality risk in the GI bleeding group (OR 1.58, P = 0.02). CONCLUSION: Use of anticoagulation or antiplatelet agents was not risk factors for GI bleeding in a large cohort of hospitalized COVID-19 patients. Those with GI bleeding during the hospitalization had increased mortality.


Subject(s)
COVID-19/complications , Gastrointestinal Hemorrhage/etiology , Aged , Aged, 80 and over , COVID-19/mortality , Female , Gastrointestinal Hemorrhage/mortality , Hospital Mortality , Hospitalization/statistics & numerical data , Humans , Male , Middle Aged , New York City/epidemiology , Prevalence , Propensity Score , Risk Factors
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