ABSTRACT
Purpose of study A 32-years old male with known multi-system sarcoidosis in remission for 5 years off treatment presented to the emergency room with complaints of generalized weakness, hematemesis, epistaxis, and bruises. Physical examination was notable for petechiae, ecchymosis along with papules and plaques suggestive of active sarcoid skin lesions on his extremities. Laboratory workup was significant for thrombocytopenia 3000/uL, acute kidney injury with sub-nephrotic proteinuria. Peripheral blood smear did not show evidence of hemolysis and direct Coombs test was negative. Infectious workup including COVID-19, HIV, and hepatitis serologies were negative. Computed Tomography (CT) of chest, abdomen, and pelvis showed mild splenomegaly and an increased number of sub-centimeter hilar and mediastinal lymph nodes. The patient was treated with dexamethasone 40 mg daily for 4 days and intravenousimmunoglobulins (IVIG-2 gm/kg) for possible Immune Thrombocytopenic Purpura (ITP) with improvement in platelet count to 42000/uL by day 3. His workup for AKI and sub-nephrotic proteinuria was negative apart from a positive ANA (1: 160) with low complements. The anti-phospholipid antibody panel was negative. The ACE level was markedly elevated (>80U/L). The patient could not get a renal biopsy due to severe thrombocytopenia. He was discharged but was re-admitted in 15 days for severe thrombocytopenia of 1000/uL, epistaxis, and bruising. We continued high dose steroids along with IVIG 1 gm/kg for refractory ITP with minimal response and started anti-CD20 agent (Rituximab) 375 mg/m2 weekly with thrombopoietin-receptor agonist (Eltrombopag). His platelets count improved in response to treatment and subsequent renal biopsy showed focal and segmental glomerulosclerosis along with mild interstitial fibrosis, tubular atrophy thought to be from long standing sarcoidosis. There was also evidence of focal arteriosclerosis with no evidence of granulomas, immune complex, complement, or IgG4 deposition. Given skin lesions, thrombocytopenia, extensive lymphadenopathy, and renal involvement with markedly elevated ACE levels the overall picture was consistent with active multi-system sarcoidosis. His platelet count increased to 177,000/uL at the time of discharge. Currently, the patient is on slow steroid taper along with Eltrombopag 25 mg every other day without any recurrence of his symptoms so far. Methods used We described one case of sarcoidosis with hematologic and renal involvement. Summary of results Our patient developed hematologic and renal complications approximately 6 years after being diagnosed with sarcoidosis. Initially, he did not demonstrate sufficient clinical response to IVIG and high dose steroids. However, after a course of anti-CD20 agent (Rituximab) and with the addition of thrombopoietin-receptor agonist (Eltrombopag) he showed improvement of platelet count and stabilization of the renal function. Currently, the patient is receiving maintenance therapy with Prednisone 7.5 mg daily along with Eltrombopag 25 mg twice weekly with no recurrence of ITP and stable renal function. A further decision on whether the patient needs another cycle of Rituximab will be determined by the patient's clinical course. Conclusions Highly variable manifestations of Sarcoidosis can pose a significant diagnostic and therapeutic challenge as can be seen from our case. ITP is a rare hematological manifestation of sarcoidosis and addition of anti-CD20 agents should be considered in refractory cases.
ABSTRACT
Introduction: Lung cavitation is a rare finding in COVID-19 patients (1, 2). The aim of this presentation is to report a rare case of cavitary aseptic pulmonary infarct in post-COVID-19 period that is complicated with spontaneous pneumothorax. Case presentation: A 63-year old woman was presented with acute onset of chest pain and on 35th day of COVID19 infection. Chest X-Ray established right-sided total pneumothorax and tube thoracostomy was performed. Due to the persistent air leak computed tomography was performed on the 4th day of thoracostomy. Areas of lung consolidation with a cavitary mass with maximum diameter of 34 mm in the upper lobe were established (fig.1). The woman was scheduled for thoracoscopy and multiple petechiae on the lung surface with necrotic area (bronchopleural fistula) of the upper lobe were established. The procedure was converted to conventional operation with atypical resection of the upper lobe of the lung with removing the necrotic cavitary lesion. Histological examination of the resected lung specimen showed bland cavitary pulmonary infarct. Conclusion(s): This case presents one rare pathological condition in post COVID-19 period - blunt pulmonary cavitary infarct complicated with spontaneous pneumothorax.
ABSTRACT
Background: Antineutrophilic cytoplasmic antibody (ANCA) associated vasculitis presenting for the first time in pregnancy is very rare, but awareness is important as it can cause significant maternal and fetal morbidity and is potentially life-threatening if not recognised or under-treated. Method and Results:We describe a 19-year-old woman who developed ANCA-associated vasculitis in the second trimester of her first pregnancy. She initially presented with a petechial rash and cough at 25 weeks' gestation, and then developed breathlessness. Significant pulmonary haemorrhage was shown on Cross Sectional imaging of the chest, with a corresponding reduction in haemoglobin. She rapidly improved with prednisolone, cyclophosphamide and plasma exchange. SARS-CoV-2 infection identified on routine screening further complicated the management. At 34 weeks' gestation she experienced a flare, with the possibility of superimposed pre-eclampsia (increase in liver enzymes, creatinine and sFlt/PlGF ratio). After multidisciplinary team discussion she underwent a caesarean section. Postnatally she continued cyclophosphamide and started azathioprine. Conclusion(s): ANCA-associated vasculitis can result in life-threatening complications. The initial features can be non-specific, so a high index of suspicion is required, particularly in women with multisystem abnormalities. Close monitoring for potential complications is advised as urgent imaging may be needed. Aggressive immunosuppressive treatment is recommended as steroids alone are usually insufficient. Cyclophosphamide can be used in later pregnancy and can result in a dramatic improvement, as was seen here. If delivery needs to be expedited, mode of birth (i.e. caesarean delivery vs vaginal birth) is dictated by the obstetric picture, with caesarean delivery being indicated for the usual obstetric reasons.
ABSTRACT
Background: The coronavirus disease 2019 (COVID-19) has been sabotaging the world over the last two years and vaccine is one of the key solutions. However, the concerns over its side effects can cause vaccine refusal, subsequently affecting many countries' education system recovery plans. Objective(s): To actively evaluate adverse effects and their severity following COVID-19 immunization among schoolchildren aged 12 to 17 years, to support parents' decision-making. Material(s) and Method(s): The present study was an observational study whereby a Google-form survey on Pfizer COVID-19 vaccine adverse effects (CVAE) was responded between January and April 2022 by 537 participants. Descriptive statistics were used to analyze basic characteristics. Chi-square tests were performed for comparative analyses between junior (aged 12 to 15 years) versus senior (aged 16 to 17 years) high school students, and McNemar's test for the first dose versus second dose groups analysis with a significance level set at p-value less than 0.05. Result(s): At least one CVAE was reported in 93.85% of the included participants, albeit mostly mild. The most common symptom as a local event was tenderness at the puncture site (82.50%), whereas systemic events were predominated by myalgia (74.67%). The second dose was associated with increased frequency and severity of adverse effects compared to the first dose (p<0.001). The older age group had significantly more side effects compared to the younger group (p<0.05). Conclusion(s): The high incidence of CVAEs in schoolchildren was predominated by mild symptoms, with the second dose and older group associated with increased frequency of symptoms. The predominance of mild symptoms found in the present study may help reduce the concerns of parents over CVAEs, ultimately accelerating vaccine coverage in the children group, which is still a gap in vaccine administration.Copyright © 2023 JOURNAL OF THE MEDICAL ASSOCIATION OF THAILAND.
ABSTRACT
In the absence of respiratory system involvement, COVID-19 patients developing ARDS can clinically mimic other diseases including acute leukemia due to presence of atypical lymphocytes in peripheral blood smear and increased serum lactate dehydrogenase and serum uric acid levels. Herein, we report a case who was initially suspected to have acute leukemia based on his atypical symptoms without any respiratory system involvement and deranged laboratory parameters and finally, diagnosed with COVID-19. Our patient presented with fever, myalgia, gum bleed, and petechiae. On clinical and laboratory evaluation, he was suspected to have acute leukemia based on markedly deranged serum lactate dehydrogenase and serum uric acid and the presence of atypical cells in peripheral blood smear and bone marrow. On day 3 of hospitalization, he developed respiratory symptoms, breathing difficulty which progressed to ARDS, and subsequently, he succumbed to his illness. His real-time reverse transcriptase-polymerase chain reaction test for severe acute respiratory syndrome coronavirus-2 yielded positive results. Also, Flow cytometry and fluorescence in situ hybridization studies for leukemia workup did not show any abnormalities. Although we are reporting the findings of only a single case, we aim to enhance and contribute further to the understanding of this novel infection.Copyright © 2021 Necati Ozpinar. All right reserved.
ABSTRACT
Background: Rhino-orbital-cerebral and isolated cerebral involvement of basal ganglia by mucormycosis are two different manifestations of CNS mucormycosis. The former variant caused by inhaled fungal spores and is common with immunosuppressive conditions. The latter form is caused by intravascular inoculation of spores as seen in intravenous drug abusers. Case report: Here we describe a case of young, non-addict patient with a history of recent mild COVID-19 pneumonia who presented with isolated cerebral mucormycosis involving bilateral basal ganglia. Discussion(s): The pulmonary vasculitis associated with COVID-19 is probably the cause of direct intravascular entry of inhaled fungal spores leading to direct isolated cerebral involvement. Such condition may rapidly turn fatal. Conclusion(s): This is the first reported case of isolated cerebral mucormycosis following post-COVID-19 infection. Early tissue diagnosis and intravenous amphotericin B is the key management.Copyright © 2022
ABSTRACT
Case Report: Atypical Hemolytic Uremic Syndrome (atypical HUS) is a rare and severe form of thrombotic microangiopathy (TMA) characterized by thrombocytopenia, intravascular hemolysis, and acute kidney injury with an incidence of 1 per million.1 Dysregulation and overactivation of the complement alternative pathway due to genetic mutations have been detected in 40-60% of patients with sporadic or familial atypical HUS.2,4 Triggers include viral illness, pregnancy, malignancy, sepsis, or sporadically with no known inciting event.1 Atypical HUS is a severe disease with a 2-10% risk of mortality, 33% risk of end-stage renal failure, and 50% chance of relapse.5 A 24-year-old female with prior history of atypical HUS at the age of 16 (with response to plasmapheresis) presented to the ER with a 5-day history of fever, chills, sore throat, nausea, vomiting, and dark urine. She tested positive for COVID-19. The exam revealed scleral icterus and scattered petechiae. Labs demonstrated nadir hemoglobin (Hgb) of 9.2 g/dL, platelet count of 52 000k/uL, haptoglobin < 30 mg/dL, peak LDH 1128U/L and creatinine 4.62 mg/dL. Urinalysis is consistent with hemoglobinuria. Schistocytes were noted on the peripheral smear. Rapid streptococcal antigen test and C3, C4, and IgA levels were unremarkable. Chest X-Ray, X-ray KUB, and ultrasound abdomen were unremarkable. The pregnancy test was negative. ADAMTS13 was >100%. Genetic analysis after the initial episode at age 16 revealed autosomal recessive inheritance c.193A > c gene mutations in C3. The patient received IV fluids, ceftriaxone for cystitis, and two units of Fresh Frozen Plasma. She initiated treatment with eculizumab. She also received the MENVEO and meningitis B vaccine per protocol due to the risk of meningitis from terminal complement deficiencies. After 4 infusions of eculizumab, patient's labs improved to platelet count of 307 000 k/uL, Hgb 12.2 g/ dL (nadir 9.2 g/dL), haptoglobin 78 mg/dL normalization of LDH and improved creatinine. Atypical HUS is a rare form of TMAwith mutations in C3 noted in 5% of cases. Complement cascade dysfunction leads to endothelial deposits and microvasculature damage. The resulting prothrombotic state causes obstructive microvascular thrombi predominantly affecting the kidneys but can cause multiorgan dysfunction. The SARS-CoV-2 virus may precipitate atypical HUS relapse due to endothelial damage and complement activation further intensified in patients with existing complement aberrations. Plasma exchange remains a standard of care for atypical HUS, as it effectively removes the antibodies and other proteins. Eculizumab a humanized monoclonal IgG antibody binds to complement proteins, preventing cleavage into C5a and C5b blocking C5b-9(MAC) activation. In patients with CFH, CFI, C3, and CFB mutations, eculizumab is the preferred intervention. Copyright © 2023 Southern Society for Clinical Investigation.
ABSTRACT
SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Granulomatous polyangiitis (GPA), previously known as Wegener's granulomatosis, is a rare syndrome characterized by inflammation of small and medium sized vessels. The clinical presentation can be very heterogenous and differentiation from an infectious disease can be challenging initially. Here, we present a case of a young male presenting with respiratory symptoms during the pandemic, presumed to have coronavirus disease 2019 (COVID-19) though after extensive workup was later diagnosed with GPA. CASE PRESENTATION: A 19-year-old male presented to the emergency department (ED) with complaints of low-grade fever and dry cough for one week. He reported having abdominal pain, fatigue, loss of appetite and polyarthralgia. An outpatient upper gastrointestinal endoscopy revealed gastritis and duodenitis. In the ED, his vitals included a temperature of 101.8°F, blood pressure of 115/65mmHg, heart rate of 99/min, respiratory rate of 18/minute and oxygen saturation of 99% on room air. COVID-19 testing was negative. A computerized tomography of the chest revealed extensive ground glass opacities. He was presumptively diagnosed with COVID-19 and started on dexamethasone therapy along with azithromycin for atypical pneumonia. However multiple tests for SARS-CoV-2 were negative. Another consideration was COVID-19 induced multisystem inflammatory syndrome given the patients young age. Infectious workup included negative testing for human immunodeficiency virus, Legionella, tick borne diseases and mycoplasma. As febrile episodes continued, he developed microcytic anemia, microscopic hematuria, and petechial rash on his ankles. Antinuclear antigen screen was negative, but C-antineutrophil cytoplasmic and anti-proteinase-3 antibodies were positive. Renal biopsy revealed GPA. He was prescribed pulse dose steroids and transitioned to immunotherapy. DISCUSSION: GPA is a challenging diagnosis given multiple system involvement, though early identification and initiation of treatment are important to prevent long term sequalae. In our case, acute onset febrile illness and pulmonary ground glass opacities led to repeated COVID-19 testing potentially delaying the diagnosis. Ultimately, the correct diagnosis was made and confirmed on renal biopsy. We believe our case highlight the importance of keeping a broad differential and considering vasculitis in these situations for prompt diagnosis. CONCLUSIONS: GPA can often mimic respiratory infectious processes, a high index of suspicion is necessary for timely diagnosis. Reference #1: Selvaraj V, Moustafa A, Dapaah-Afriyie K, et alCOVID-19-induced granulomatosis with polyangiitis. BMJ Case Reports CP 2021;14:e242142 Reference #2: Qurratulain, Q., Ahmed, A., & Jones, Q. (2021). Lesson of the month: Severe granulomatosis with polyangiitis (GPA): a diagnostic challenge during the COVID-19 pandemic. Clinical Medicine, 21(1), 79. DISCLOSURES: No relevant relationships by Aamna Khan No relevant relationships by Usama Sadiq No relevant relationships by Rehan Saeed
ABSTRACT
SESSION TITLE: COVID-Related Critical Care Cases SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm INTRODUCTION: We present a case of diffuse alveolar hemorrhage (DAH) secondary to Immune Thrombocytopenia (ITP) temporally related to SARS-CoV-2 (CoV) vaccine. CASE PRESENTATION: An 80-year-old female presented with dyspnea, hemoptysis, diffuse petechiae, and ecchymosis;no focal neurological deficits or hepatosplenomegaly. She had no history of bleeding or autoimmune disorders;no recent respiratory or gastrointestinal infections;but received Moderna CoV vaccine 4 weeks prior to presentation. Chest X-ray (CXR) and CTA of chest demonstrated multifocal bilateral patchy airspace opacities. Initial platelet was 1 x 109/L with normal morphology of platelet and WBC, and no schistocytes. Coagulation panel, LDH, haptoglobin, and bilirubin were all normal. CoV NAAT was negative. Dexamethasone and IVIG for high suspicion of ITP was initiated. Supportive care including platelet transfusion and oxygen via nasal cannula was maintained. Platelets were severely consumed in spite of treatment with platelets undetectable at nadir and rapid decrease of hemoglobin, approximately 6 g/dL, within 24 hours of admission. IgM and IgG plasma platelet autoantibodies returned positive, confirming ITP diagnosis. Additional workup was unremarkable for infections, rheumatologic disorders, and malignancy. Respiratory state rapidly declined with worsening hemoptysis and significant increase of bilateral airspace opacities on repeat CXR, indicative of DAH. Lung protective mechanical ventilation protocol was initiated on day 2 with medically induced deep sedation and paralysis to minimize hemorrhage exacerbation. Rituximab, romiplostim, and nebulized tranexamic acid were added for severe and refractory ITP, which eventually slowed platelet consumption, reduced pulmonary hemorrhage, and stabilized hemoglobin. Platelets recovered above 30 x 109/L on day 9, and subsequent bronchoscopy showed persistent blood on bronchoalveolar lavage. She was successfully extubated after prolonged 14-day intubation. Platelet normalized before discharge. DISCUSSION: Incidence of ITP related to CoV vaccine is approximately 0.8-0.9 case per million vaccinated. Most cases present with superficial bleeding and respond to first-line agents with rapid recovery. GI bleeding and intracranial hemorrhage, but not DAH, have been reported in several cases, requiring third-line agents to promote platelets recovery and achieve hemostasis. We report a case of DAH secondary to ITP following CoV vaccine. Temporal relationship and severe presentation are consistent with other reports of ITP with life-threatening internal bleeding probably secondary to CoV vaccine. CONCLUSIONS: When DAH is suspected, rapid escalation of treatment to include third-line agents is desired. If intubated, lung protective ventilation with paralysis is preferred to minimize further lung injury due to DAH. Reference #1: Lee EJ, Cines DB, Gernsheimer T, et al. Thrombocytopenia following Pfizer and Moderna SARS-CoV-2 vaccination. Am J Hematol. 2021;96(5):534-537. doi:10.1002/ajh.26132 doi:10.1016/J.VACCINE.2021.04.054 Reference #2: Welsh KJ, Baumblatt J, Chege W, Goud R, Nair N. Thrombocytopenia including immune thrombocytopenia after receipt of mRNA COVID-19 vaccines reported to the Vaccine Adverse Event Reporting System (VAERS). Vaccine. 2021;39(25):3329-3332. Reference #3: Tarawneh O, Tarawneh H. Immune thrombocytopenia in a 22-year-old post Covid-19 vaccine. Am J Hematol. 2021;96(5):E133-E134. doi:10.1002/ajh.26106 DISCLOSURES: No relevant relationships by Timothy Barreiro No relevant relationships by Tiewei Cheng No relevant relationships by Zeina El Amil No relevant relationships by Jin Huang No relevant relationships by Sanaullah Khalid
ABSTRACT
SESSION TITLE: Management of COVID-19-Induced Complications SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Anti-neutrophil cytoplasmic antibody-associated vasculitis (ANCA-AV) is an autoimmune mediated inflammation of small and medium sized vessel walls. The occurrence of this autoimmune vasculitis is typically associated with underlying infection, medications, and genetic predisposition.(1) The objective of this case report is to describe a rare presentation of ANCA-AV in the setting of COVID-19 infection. CASE PRESENTATION: A 67-year-old male presented to the hospital with a three-week history of cough productive of brown sputum, epistaxis, fatigue, decreased appetite, and unintentional weight loss. During the previous week, he experienced worsening dyspnea and bilateral lower extremity swelling. On physical examination, he was hypoxic requiring 4L of supplemental oxygen to maintain saturations greater than 90%. Diffuse and bilateral wheezes were heard on auscultation of his lungs. A tender petechial rash was dispersed over his limbs, trunk, oropharynx, and nasopharynx. A basic metabolic panel revealed a mild, acute renal impairment. Urinalysis showed new onset proteinuria and hemoglobinuria. Nasopharyngeal swab was positive for SARS-COV-2. Contrast-enhanced computed tomography of the chest revealed diffuse, bilateral ground glass opacities and interstitial changes. Therapy with piperacillin-tazobactam was started for presumed superimposed bacterial community acquired pneumonia in the setting of COVID-19 infection. On day three of hospitalization, the petechial rash progressed to hemorrhagic blisters. His oral petechiae were now ulcerated. A punch biopsy of the affected skin showed leukocytoclastic vasculitis. Anti-Proteinase 3 (PR3) antibodies were positive. Subsequent renal biopsy showed pauci-immune focal necrotizing crescentic glomerulonephritis consistent with ANCA-AV. Therapy with intravenous pulse dose corticosteroids led to improvement in his rash and body aches, and he was discharged home on oral steroids ten days after admission. DISCUSSION: This report describes a rare case of ANCA-AV in the setting of recent COVID-19 infection. Differentiation of ANCA-AV, bacterial and COVID-19 pneumonia can be challenging on chest imaging alone.(1) New onset renal impairment, hematuria, proteinuria and the presence of the petechial rash were suspicious for co-existing ANCA-AV in this patient. COVID-19- associated cytokine storm and formation of neutrophil extracellular traps (NETs) is postulated to be the underlying cause.(1-3) NETs present myeloperoxidase (MPO) and PR3 antigens to the immune system. Formation of auto-antibodies to MPO and PR3 lead to the development of ANCA-AV. The findings of NETs on kidney biopsy specimens in patients with ANCA-AV supports this hypothesis.(1,2) CONCLUSIONS: To avoid the misdiagnosis of COVID-19-induced vasculitis, a low threshold to investigate co-existing vasculitis in patients with COVID-19 and associated clinical findings is highly recommended. Reference #1: Izci Duran T, Turkmen E, Dilek M, Sayarlioglu H, Arik N. ANCA-associated vasculitis after COVID-19. Rheumatol Int. 2021;41(8):1523-1529. Reference #2: Uppal NN, Kello N, Shah HH, et al. De Novo ANCA-Associated Vasculitis With Glomerulonephritis in COVID-19. Kidney Int Rep. 2020;5(11):2079-2083. Reference #3: Cobilinschi C, Cobilinschi C, Constantinescu A, Draniceanu I, Ionescu R. New-Onset ANCA-Associated Vasculitis in a Patient with SARS-COV2. Balkan Med J. 2021;38(5):318-320. DISCLOSURES: No relevant relationships by Andrei Hastings No relevant relationships by Jason Lane No relevant relationships by Tanya Marshall No relevant relationships by Palak Rath No relevant relationships by Sterling Shriber No relevant relationships by inderprit Singh No relevant relationships by Samuel Wiles
ABSTRACT
Background: Chronic lymphocytic leukemia (CLL) is the most common adult leukemia. Approximately 2% of patients with CLL develop immune thrombocytopenic purpura (ITP) during the course of the disease. When resistant to steroids, this constitutes as indication for treatment of the CLL. Here we report a patient with refractory ITP secondary to CLL successfully treated with venetoclax. Aims: To present an interesting case with CLL related refractory ITP treated successfully with novel agent venetoclax. Methods: Patient data was taken from the patient herself and Hospital records. Informed consent to publish the case is obtained from patient. Permission for off-label venetoclax and eltrombopag was obtained from Ministry of Health of Turkey. Results: 46-year-old female patient presented with lumps on her neck that were present for the last 9 months in November 2020. She has a history of frequent pneumonia and otitis but no constitutional symptoms. Her physical examination reveals multiple 2cm lymphadenopathies on her neck and no organomegaly or other pathological features. Blood work shows mild lymphocytosis (6800/mm3) with no serious cytopenias. Peripheral blood smear, flow cytometry and bone marrow biopsy were all compatible with CLL. She was classified as Binet A CLL and was followed up with no treatment after appropriate vaccinations against capsulated pathogens. In July and August 2021 she received two doses of mRNA vaccination against COVID-19. On 1st November 2021 she experienced excessive menstrual bleeding and blood work showed platelet count of 23000/mm3, she was started on steroids (1 mg/kg/day) and after 4 days platelet count has risen to 55000/mm3, she discontinued steroids on her own against medical advice. On 13th of November she presented with extensive petechiae and purpura and was again started on steroids and was given the courses of intravenous immunoglobulins (IVIG) without any sustained response. She was refractory to platelet transfusions too. She was transferred to our clinic. She was found to have del11q and del13q. She refused bone marrow examination. She was treated with rituximab, steroids, vincristine, IVIG and eltrombopag for ITP without success (Fig. 1). She had a minimal response to IVIG only. She received two courses of bendamustine (90 mg/kg for two days) also without success. Three courses of plasmapheresis yielded no response either. After mild success with immunadsorbtion apharesis she was started on venetoclax plus rituximab with ramp-up. Sustained response was achieved within the first week of venetoclax therapy. (Figure Presented ) Summary/Conclusion: Gordon et al. reported 2 CLL cases one with ITP and other with Evans syndrome successfully treated with venetoclax. We think, this treatment should be considered in patients with refractory immune cytopenias secondary to CLL and assessed with prospective clinical trials.
ABSTRACT
Multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 is a rare life-threatening immunopathological complication of COVID-19 that develops 1-6 weeks after the acute coronavirus infection. MIS-C is characterized by fever and multiorgan inflammation. We present a clinical case of a 10-year-old boy with skin lesions at the onset of MIS-C (erythematous malar rash, lacelike rash on the trunk and extremities and petechiae) with macrophage activation syndrome development and the early stage of primary Epstein-Barr virus infection (EBV infection) which required the exclusion of X-linked lymphoproliferative disease. This clinical case demonstrates the complexity of diagnosis in MIS-C with skin manifestations at the onset of the disease, especially with concurrent activation of other infections, particularly EBV infection.
ABSTRACT
CASE: A 25-year-old female with no medical history presented with progressive petechial rash at the chest, trunk, bilateral forearms, and thighs. Patient had the COVID-19 vaccine three weeks prior. However, denied recent travel or tick bites. No home medications nor recent hospitalizations, other than a tooth extraction a month earlier. On physical exam, the patient's vitals were unremarkable and had non-blanching petechial rash noted on her torso and bilateral extremities. Labs were significant of platelet count 1,000/mcL, hemoglobin 12.9 mg/dL, WBC 7.52 x103 /mcL, absolute lymphocytes 3.33x103 /mcL. Patient was administered two units of platelets followed by intravenous immunoglobulin (IVIG) and dexamethasone. No bleeding or hemodynamic instability was identified. Platelet count improved to 100,000/mcL over the next 24 hours. Further work-up revealed a positive HIV-1 antibody, absolute CD4 256 cells/mcL, viral load 27,300 copies/mcL. Once starting antiretroviral therapy (ART);bictegravir, emtricitabine, and tenofovir alafenamide, platelet count increased within a month to more than 200,000/mcL. IMPACT/DISCUSSION: Thrombocytopenia is defined as platelet count below 150,000/mcL. HIV-induced cytopenias are common, mainly neutropenia. However, sentinel events of thrombocytopenia are very rare in otherwise healthy individuals. A review of 5,290 HIV patients at the University of British Columbia from 1996 to 2012 revealed only 0.6% incidence of severe thrombocytopenia which they defined as platelet count <20,000/mcL. The exact pathophysiology is not clearly understood, but it is possible that antibodies against HIV cross-react with platelets or possible immune alteration. This is suggested by the prompt resolution of thrombocytopenia once ART is initiated. Immune thrombocytopenia (ITP) is a diagnosis of exclusion typically presenting with thrombocytopenia while other cell lines are normal. The greatest concern is when platelet counts drop less than 20,000/mcL due to fears of intracranial bleeding. Literature is not decisive in a correlation between platelet counts and risk of bleeding, yet it is suggested that circulating platelets are younger and more effective to maintain hematopoiesis in ITP when compared to other causes of thrombocytopenia. Treatment approach for ITP depends on the bleeding risk. In the presence of bleeding, urgent platelet transfusion, glucocorticoids, and IVIG are the mainstay of treatment. In absence of bleeding, individualized assessment of the condition is recommended to either monitor or treat. Platelet counts below 20,000-30,000/mcL require steroids or IVIG. In our case, she surprisingly presented only with minor petechial bleeding. Prompt initiation of ART, close monitoring of platelet response and CD4 count, as well as identifying resistant thrombocytopenia is indicated once patient is medically stable. CONCLUSION: Sole presentation of ITP due to HIV infection is rare. Risks of critical bleeding and further management are crucial to prevent fatal outcomes.
ABSTRACT
CASE: 54-year-old female presented with 1 week of generalized weakness, headache, congestion, cough with dark- colored phlegm, and several days of decreased smell and taste. She was unvaccinated and had positive sick contacts. Patient tested positive for Covid and found to have severe thrombocytopenia with platelets of 5K/uL, very rare schistocytes on smear, and no other notable abnormalities. She received platelet transfusion and was treated for presumed immune thrombocytopenia with IVIG and dexamethasone. The patient had no petechiae, bleeding, or other symptoms concerning for secondary TMA, notably TTP. The platelet count was 93 K/uL by day 5 and she was discharged home. Later that day her ADAMTS13 test resulted at <2% and the ADAMTS13 antibody was elevated. The patient was asked to return to the hospital for monitoring of TTP symptoms. She reported improvement in her weakness. Her thrombocytopenia and oxygen saturation remained normal. Bilateral lower extremity ultrasound showed no lower extremity VTE. On the day of discharge, 10 days after her original thrombocytopenia identified, she had a platelet count of 373 K/uL and repeated ADAMTS13 of 14.8%. IMPACT/DISCUSSION: ADAMTS13 is known as von Willebrand factor (VWF) protease as it cleaves prothrombotic and highly adhesive to platelets ultra-large multimers of VWF into smaller multimers, thus modulating VWF activity and regulating the adhesive function. A severe deficiency of ADAMTS13 characterizes TTP, a rare but potentially fatal disorder associated with thrombosis due to accumulation of prothrombotic ultra-large VWF multimers. There are literature reports of TTP and TTP-like syndromes in Covid-19. It is speculated that in COVID-19, the excess of VWF released in response to endothelial activation likely exhausts the available reserves of ADAMTS13, which may then propagate formation of microthrombi in different organs. We report an extreme thrombocytopenia, marked decrease of ADAMTS 13 and elevated ADAMTS13 antibodies, which would be confirmative evidence of TTP should our patient have clinical features of it. Our patient did not have fever, neurologic abnormalities, renal dysfunction, or active hemolysis. She was followed in outpatient clinic after the discharge. The platelet count recovered and ADAMTS 13 trended up without need for plasmapheresis. Our case is a good example of a fortunate outcome without any complications despite threatening presenting criteria. CONCLUSION: Covid-19 associated endothelial stimulation and damage could mimic a life-threatening disorder without expected fatal complications. On the other hand, it can ultimately lead to the most severe form of thrombotic microangiopathy, TTP, for which the mortality rate is close to 90%. It is hard to know which outcome to expect in different circumstances. Therefore, it is crucial for physicians to promptly recognize clinical picture of TTP as treatment is lifesaving.
ABSTRACT
Strongyloides stercoralis causes chronic, mostly asymptomatic infections but hyperinfection syndrome may occur in immunosup-pressed patients, especially in those receiving corticosteroids. We report a case of S. stercoralis hyperinfection syndrome in a solid organ transplant recipient that occurred approximately 2.5 months after heart transplantation. The patient presented to the inten-sive care unit with acute respiratory distress, bacteremia, and petechial rash on abdomen and toe. Microbiology testing of respiratory samples excluded infection with Pneumocystis jirovecii, respiratory viruses, pathogenic bacteria and fungi. No eosinophilia was found. Histopathological examination of the skin biopsy of the petechial rash provided the first indication of the diagnosis, revealing the presence of isolated filariform S. stercoralis larvae in the dermis. Subsequent microbiology testing confirmed the diagnosis. This case highlights the role of histopathological examination of a skin rash in diagnosing patients with atypical clinical presentation of Strongyloides hyperinfection syndrome.
ABSTRACT
COVID-19 has created a series of clinical conundrums since its emergence. We report a case of severe immune thrombocytopenic purpura (ITP) in a 67-year-old gentleman in April 2020. He presented to hospital with a rapidly evolving rash, 3 weeks following infection with COVID-19. Clinically he had a widespread non palpable petechial rash, haemorrhagic blisters across his oral mucosa and severe epistaxis. His platelet count was 2 × 109 L-1 (150-450 × 109 L-1). Full blood count and clotting studies were otherwise normal. With ITP not yet well reported as a complication of COVID-19, there was a treatment dilemma. ITP is an acquired autoimmune-mediated disorder (often with a viral or vaccine precipitant) and first-line treatment is immunosuppression. However, due to concurrent infection with the novel COVID-19 virus, a thrombopoietin receptor agonist (TPO-RA) (eltrombopag 50 mg once daily) was instead commenced. Persistent epistaxis, oral bleeding and a platelet count < × 109 L-1 required intravenous immunoglobulin (1 g kg-1) to be administered on day 7 of TPO-RA treatment. By day 12 of TPO-RA treatment the platelet count had successfully normalized. The patient remains in remission 18 months on. Since this case, ITP has become a recognized phenomenon of both COVID-19 infection and COVID-19 vaccination (Pishko AM, Bussel JB, Cines DB. COVID-19 vaccination and immune thrombocytopenia. Nat Med 2021;27: 1145-6). Moreover, corticosteroid therapy has become the first evidence-based therapy for severe COVID-19 infection (Horby P, Lim WS, Emberson J et al. Dexamethasone in hospitalized patients with Covid-19. N Engl J Med 2021;384: 693-704), although their use in COVID-19- related ITP remains unclear. This case demonstrates an important cutaneous manifestation of the COVID-19-provoked disrupted haemostasis pathways, which results in significant morbidity and mortality. Additionally, this case describes practical real-life multidisciplinary team decision-making to emerging complications of a uniquely studied virus.
ABSTRACT
Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 infection (PIMS-TS) is a novel condition which has recently emerged during the COVID-19 pandemic, consisting of persistent fever, inflammation, and evidence of single- or multiorgan dysfunction, with additional features where any other microbial causes has been excluded (https://www.rcpch.ac.uk/resources/paediatric-multisysteminflammatory- syndrome-temporally-associated-covid-19-pimsguidance). A 16-year-old healthy male presented with a 48-h history of headache, fever, diarrhoea, vomiting and a widespread rash. One month prior he had contracted SARS-CoV-2 with no complications. On examination there was a maculopapular rash across the trunk and buttocks with petechiae present on the lower limbs and flexural folds in addition to an erythematous rash across the nasal dorsum and cheeks. Blood tests revealed lymphopaenia, thrombocytopenia, hypertriglyceridaemia and raised C-reactive protein and ferritin. Autoimmune screen and SARS-CoV-2 polymerase chain reaction tests were negative. A skin biopsy revealed features of a SARS-CoV-2 related urticarial reaction pattern. The patient was treated for myocarditis, fulfilling the diagnosis of PIMS-TS. He was transferred to a specialist intensive care unit and treated with intravenous immunoglobulins and steroids, infliximab, aspirin and topical steroids. He is currently undergoing investigations for encephalitis post admission. PIMS-TS is a rare syndrome that shares features with Kawasaki disease, toxic shock syndrome, macrophage activation syndrome and bacterial sepsis. Haemophagocytic lymphohistiocytosis has also been linked to SARS-CoV-2 (Retamozo S, Brito-Zerón P, Sisó- Almirall A et al. Haemophagocytic syndrome and COVID-19. Clin Rheumatol 2021;40: 1233-44). Clinicians should consider PIMS-TS as a differential in any child presenting with a fever, rash and evidence of systemic inflammation. Early recognition, involvement of a multidisciplinary team and prompt referral to critical care is essential in managing this life-threatening condition.
ABSTRACT
Objective: NA Background: We present 3 clinically important, treatment-responsive COVID-19 related neurological cases. Diagnosis and treatment can be challenging but neurological sequelae may be treatable, and recovery can occur. Design/Methods: NA Case 1: An 81-year-old gentleman was admitted with a one-week history of incoherent speech, deteriorating mobility, and poor appetite. While admitted, he developed cyclical apnoeic spells during sleep. Neurological examination revealed profound spasticity, hyper-reflexia, myoclonus and startle response to loud noise. Clinically, he was treated as a case of progressive encephalopathy rigidity and myoclonus (PERM) and responded well to intravenous immunoglobulin with improvement in his cognition and mobility. To our knowledge, this is the first case report of post-Covid PERM. Case 2: A 76-year-old lady, recovered from COVID-19 infection, continued to deteriorate neurologically with cognitive slowing, aphasia, and dyskinesia. Cranial MRI was unremarkable and CSF was sterile. Encephalopathic changes were noted on scalp EEG. Autoimmune encephalitis was suspected, a 3-day course of methyl prednisolone yielded significant improvement and complete recovery ensued over 3 months. Case 3: A 44-year-old man admitted with COVID-19 pneumonitis developed sudden onset left arm weakness and sensory loss, treated as COVID-related ischemic stroke. Extensive white and grey matter hyper-intensity with punctate hemorrhage on MRI raised radiological suspicion of hemorrhagic encephalitis or vasculitis. There was no beading on a subsequent MR angiogram. He was treated with a course of intravenous methyl prednisolone and a weaning regime of oral prednisolone. At 4-month follow up, he had made a good functional recovery. Conclusions: We report three cases of neurological complications temporally associated with COVID-19 infection. Improvement occurred following immunosuppression, which supports hypotheses of virus-induced autoimmunity.
ABSTRACT
Introduction: In 2019, the SARS-CoV-2 coronavirus emerged, producing COVID-19, which caused a pandemic that spread rapidly around the world;in severe cases, it can trigger cytokine storm syndrome and lead to death. Cutaneous manifestations can be associated with the expression of the ACE2 receptor in keratinocytes, these have been described in the literature from different countries. This review seeks to document the skin presentations that have been described in patients with COVID-19 in Latin America. Methodology: We researched in nine databases for articles in Spanish, Portuguese and English, until March 10, 2021, using the terms: “Skin”, “Cutaneous manifestations”, “COVID-19”, “SARS-COV-2”, associating them with Latin American countries. Basic data was obtained from the patients, then a table was created in Microsoft Excel to analyze and compare this data. Results: 22 publications from nine countries were identified. We obtained information from 92 patients, predominantly female with an average age of 31.6 years. 99 dermatologic lesions were documented, in order of frequency: rash, wheals, petechiae-purpura and papules. In 70 patients the affected body segment was described, mainly the trunk, lower and upper limbs. We found 39 photographs and 5 histopathologies. The most indicated treatments were corticosteroids, antimalarials and recombinant interferon. Conclusions: Differences can be observed in the description of cutaneous manifestations caused by COVID-19, between Latin American publications and the ones found in other countries. There seems to be a specific pattern with a predominance of erythematous rash with wheals, while pseudo-perniosis was less representative.
ABSTRACT
Objective: To classify the haematological pattern, severity of anemia in children 5-12 years age admitted and to find its correlation with the clinical conditions. Methods Crossectional study of 160 patients in two years was done. Patients satisfying the inclusion criteria were selected for study. Relevant clinical data were recorded in a structured proforma including detailed history was recorded with particular symptoms suggestive of anemia such as weakness and easily fatigability, breathlessness on exertion and pica. A thorough clinical examination of every child was done followed by routine investigations for anemia Results Patients between 7-8 year were found to be the most affected. Anemia was found to be more common in female children as compared to male children (F:M=1.13). Anemia is more common in undernourished child. Most common presenting symptoms were gastrointestinal including vomiting, diarrhea and pain abdomen. Most common sign was Pallor followed by other common signs included signs of dehydration associated with diarrhea, hepatosplenomegaly. microcytic hypochromic anemia was the most common morphological type of anemia and macrocytic anemia was the least common.Thalassemia cases were most common among hemolytic anemias. Iron Deficiency Anemia (Nutritional Anemia) was the most common etiology of anemia. Conclusion Dietary deficits affect children aged 5 to 12, creating financial, emotional, and psychological burden for patients and their families, as well as depleting critical national resources. As a result, screening for these illnesses, as well as early detection of anemia and related problems, is essential.