scholarly journals SARS-CoV-2 infection in a child with Fanconi anemia and determined immunosuppressed status

2021 ◽  
Vol 24 (3) ◽  
pp. 146-148
Author(s):  
Claudia Chirilă ◽  
◽  
Geta Vancea ◽  
Dana Ispas ◽  
Nicoleta Voicu-Pârvu ◽  
...  

Introduction. SARS-CoV-2 virus infection affects all age groups. In children, the infection mainly causes asymptomatic or mildly symptomatic forms of the disease, regardless of their immune status. Case presentation. We describe the case of a 7-year-old male child, known to have Fanconi anemia, scheduled for bone marrow transplantation. The patient comes from a family outbreak of COVID-19, which is why he was tested for SARS-CoV-2 infection. He is asymptomatic at the time of admission to our clinic. The clinical examination performed at the time of admission shows a patient in good general condition, afebrile, with pale skin and mucous membranes, without respiratory changes. Paraclinically, severe neutropenia, severe normochromic normocytic anemia and severe thrombocytopenia are detected, for which transfusions of erythrocyte mass and platelet mass are performed. Due to the immunocompromised status, antibiotic therapy is instituted. If necessary, symptomatic treatment is administered. The evolution is favorable, and the SARS-CoV-2 RT-PCR control test is negative on the eighth day of hospitalization. Conclusions. Immunocompromised status is not a major risk factor for severe COVID-19 in children.

Blood ◽  
1998 ◽  
Vol 91 (1) ◽  
pp. 181-186 ◽  
Author(s):  
Juergen Bux ◽  
Georg Behrens ◽  
Gudrun Jaeger ◽  
Karl Welte

Abstract Primary autoimmune neutropenia (AIN) is caused by granulocyte-specific autoantibodies and occurs predominantly in infancy. Clinical presentation and diagnosis have not been well established, resulting in burdening diagnostic investigations and unnecessary treatment with granulocyte colony-stimulating factor (G-CSF). In the present study, clinical, laboratory, and immunologic data of 240 infants with primary AIN were evaluated. Suspected association with parvovirus B19 infection was investigated using serologic and DNA-based methods. Primary AIN was mainly diagnosed at the age of 5 to 15 months but was observed as early as day 33 of life. In 90% of the cases, AIN was associated with benign infections despite severe neutropenia. Spontaneous remission, shown by 95% of the patients, usually occurred within 7 to 24 months. Autoantibodies in the patient's sera were not always present, and screening had to be repeated several times until antibody detection succeeded. About 35% of the autoantibodies showed preferential binding to granulocytes from NA1 and NA2 homozygous donors. Bone marrow was typically normocellular or hypercellular, with a variably diminished number of segmented granulocytes. A significant association with parvovirus B19 infection was not found. Symptomatic treatment with antibiotics was sufficient in most patients. Eighty-nine percent of the patients received antibiotics (cotrimoxazole) for prophylaxis of infections. For severe infections or for surgical preparation, G-CSF, corticosteroids, and intravenous IgG were administered, resulting in increased neutrophil counts in 100%, 75%, and 50% of the patients treated, respectively. In combination with the detection of granulocyte-specific antibodies, the typical clinical picture allowed diagnosis of AIN without burdening investigations. Treatment with G-CSF was found to be a reliable alternative to temporarily increase the neutrophil count.


Author(s):  
Kanwal Zahra ◽  
Maryam Shahid ◽  
Waqas Aslam ◽  
Usman Shahid Butt ◽  
Nida Zahra

Objective: To determine the audit of wheat pill cases at medicolegal clinic of Mayo Hospital Lahore. Materials and Methods: This descriptive study was conducted at Department of Forensic Medicine and Toxicology (medicolegal clinic), King Edward Medical University Lahore. This audit was of 9 months from September 2020 to May 2021. Patients presented at emergency department with attempt of wheat pills poisoning of all age groups and both genders were included. Cases were diagnosis via history of wheat pills consumption by the relatives. All the cases were given primary recovery and were shifted to the Intensive Care Unit (ICU). Data was collected on monthly basis form September 2020 to May 2021. All the data was collected via study proforma. Results: Total 49 wheat pill cases were observed during 9 months, their mean age was 29.26±12.68 years. Out of all 51.0% were males and 49.0% were females. Labourer males and housewives were most common 34.7% and 40.8% respectively. Frequency of wheat pill cases were high in months of September, October and May. At the time of admission 59.2% cases were seen conscious, 36.7% were semiconscious and 4.1% were unconscious. Out of all 59.2% were died. Conclusion: This study observed that the wheat pills are highly toxic with the quick and high mortality rate. Strict legislations are recommended to the sale control of these pills all over the country.


Rheumatology ◽  
2019 ◽  
Vol 59 (4) ◽  
pp. 709-717 ◽  
Author(s):  
Kathleen M M Vanni ◽  
Houchen Lyu ◽  
Daniel H Solomon

Abstract Objective To conduct a systematic literature review and meta-analysis to estimate the incidence of anaemia, leucopoenia, neutropenia and thrombocytopenia associated with MTX plus folic acid among patients with rheumatic diseases. Methods We searched MEDLINE, PubMed and EMBASE through August 2016 for all randomized controlled clinical trials with a MTX monotherapy arm. We excluded randomized controlled clinical trials for cancer and included only double-blind studies that reported on haematologic adverse events. Studies were excluded if patients did not receive folic acid or leucovorin supplementation. Full text articles were assessed by two independent reviewers. Incidence estimates were calculated using random-effects models. Results Of 1601 studies identified, 30 (1.87%) were included, representing 3858 patients; all had RA. Seventeen trials reported on anaemia (n = 2032), 17 reported on leucopoenia (n = 2220), 16 reported on neutropenia (n = 2202) and 12 reported on thrombocytopenia (n = 1507). The incidence for any anaemia was 2.55% (95% CI 0.60–5.47%), any leucopoenia 1.17% (95% CI 0.16–2.80%), any neutropenia 1.77% (95% CI 0.33–4.00%), and any thrombocytopenia 0.19% (95% CI 0.00–0.86%). Four cases of severe anaemia were reported, as defined by authors, along with three cases of severe neutropenia. No cases of severe leucopoenia, severe thrombocytopenia or pancytopenia were reported. Conclusion Cytopenias are an uncommon side effect of low-dose MTX with folic acid supplementation among RA patients. Further research is needed to reach a more precise estimate.


PEDIATRICS ◽  
1956 ◽  
Vol 17 (3) ◽  
pp. 368-382
Author(s):  
Morris Green ◽  
William L. Nyhan ◽  
Mildred D. Fousek

The clinical records of 99 infants and children admitted to the Grace-New Haven Community Hospital with acute hematogenous osteomyelitis are reviewed. The presenting symptomatology and physical findings are discussed. Detection of localized tenderness is the most significant early clinical sign. This was not, however, a common finding at the time of admission. The diagnoses entertained in these patients at the time of admission are reviewed and the differential diagnosis is discussed. The distribution of the bony lesions was similar to that noted by other authors. However, attention is called to the frequent involvement of the bony pelvis. A specific bacteriologic diagnosis was made in 87 per cent of the patients. In 63 per cent staphylococcus was the etiologic agent. Beta-hemolytic streptococcus was considered the etiologic agent in 18 per cent. Staphylococcus was the most frequent offender in all age groups. In infants less than 2 years of age osteomyelitis was streptococcal in 27 per cent. The average interval from the onset of symptoms to the development of positive roentgenographic signs was 10 days. The range was 3 to 17 days. In treatment of patients suspected of having osteomyelitis, vigorous antibiotic therapy should be continued until at least 3 weeks have elapsed since the onset of symptoms. If at the end of that period roentgenograms remain normal and clinical signs and symptoms have been absent for at least 1 week, it would seem safe to discontinue treatment. A detailed program for medical therapy is Presented and the indications for surgery are considered. Emphasis is placed on vigorous search for the infecting organism. The necessity for intensive treatment given over an extended period of time is stressed.


Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1387-1387
Author(s):  
Philipp J Rauch ◽  
Corinne Widmer ◽  
Kristin Fritsch ◽  
Jana M Ellegast ◽  
Jeroen S Goede ◽  
...  

Abstract Acute myeloid leukemia (AML) induces profound impairment of healthy hematopoiesis. The production deficit in the bone marrow (BM) leads to development of peripheral anemia, thrombocytopenia and neutropenia, which is a major cause of AML-associated morbidity and mortality. Despite much progress in understanding of AML biology, the mechanisms by which AML blasts interact with elements of normal hematopoiesis to cause cytopenia are unclear. Conventional wisdom has it that blasts infiltrate the marrow and displace normal hematopoiesis. If this concept were to be true, there should be a strong correlation between BM blast count and peripheral cytopenia. Surprisingly, analysis of 223 patients with newly diagnosed AML at a tertiary referral center revealed lack of correlation between initial BM blast count [% of cellularity] and hemoglobin level (ρ=-0.11, P=0.12), platelet count (ρ=-0.00, P=0.53) and absolute neutrophil count (ρ=0.13, P=0.06). This indicates that mechanisms other than displacement of normal hematopoiesis dictate the severity of cytopenia in AML patients. Hematopoiesis is tightly regulated by cytokines. Among them, thrombopoietin (TPO) acts through its receptor c-Mpl as the master regulator of megakaryopoiesis, but also exerts upstream effects on hematopoietic stem and progenitor cells (HSPC). TPO levels are controlled by receptor-mediated scavenging by cells carrying c-Mpl on the surface, with platelets representing the lion's share in a healthy organism. This negative feedback loop results in strong negative correlation between serum TPO concentration and platelet count in the steady state. When we examined this relationship in our AML cohort, TPO levels did not follow the expected negative correlation with platelet counts (ρ=-0.10, P=0.59). Comparison with historic controls with thrombocytopenia induced by chemotherapy for non-hematopoietic malignancy revealed that the lack of correlation was driven by AML cases with severe thrombocytopenia that had lower than expected levels of TPO in the serum. As HSPC are known to express c-Mpl, we hypothesized that HSPC-derived AML blasts may also express the receptor and cause insufficiency of hematopoiesis by means of receptor-mediated TPO scavenging. To test this hypothesis, we compared c-Mpl expression on blasts in AML cases with severe thrombocytopenia and low TPO concentration (potential scavenger cases) to cases with TPO levels adequate for the degree of cytopenia. Both surface flow cytometry and qPCR demonstrated higher c-Mpl expression in potential scavenger cases (3.1-fold, P=0.02). To determine whether this difference in expression translates into increased serum TPO clearance, we incubated AML blasts with high (c-Mpl+) and low (c-Mpl-) receptor expression in serum containing recombinant human TPO at a concentration of 100 pg/mL. After 2h, TPO clearance reached 45 pg per 106 cells in wells with c-Mpl+ blasts, compared to only 4 pg per 106 cells in wells with c-Mpl- blasts (P=0.02). This confirms the hypothesis that AML blasts can lower TPO levels by virtue of their c-Mpl expression. Validation studies in an independent, multi-center Dutch-Belgian-Swiss cohort of 437 AML cases confirmed lack of correlation between initial BM blast count and cytopenia. Ranked gene list correlation analysis of whole genome microarray data proved significant enrichment of the MPL transcript in patients with severe thrombocytopenia when compared to patients with average platelet counts (rank 27/20'589, FDR<10-6). MPL enrichment could also be observed in patients with severe neutropenia (P<0.01), but there was no correlation between MPL transcript level and degree of anemia. Lastly, we asked if MPL expression was related to cytogenetic or molecular AML subtype: indeed, microarray analysis showed higher MPL expression in cases of AML with t(8;21) than in any other subtype (P<10-4). Concurrently, these patients displayed significantly lower platelet count (40 vs 83 x 109/L, P=0.02) when compared to all other AML cases. In summary, our study demonstrates that cytopenia in AML is independent of BM blast count, but strongly correlated with c-Mpl expression on blasts. We show that c-Mpl+ blasts clear TPO, causing insufficient TPO levels and contributing to development of thrombocytopenia and neutropenia. The work may have important ramifications for treatment of AML-induced cytopenia, especially in the relapsed or refractory setting. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
1997 ◽  
Vol 90 (7) ◽  
pp. 2565-2573 ◽  
Author(s):  
Karen J. Neelis ◽  
Simone C.C. Hartong ◽  
Torstein Egeland ◽  
G. Roger Thomas ◽  
Dan L. Eaton ◽  
...  

Abstract Thrombopoietin (TPO) was evaluated for efficacy in a placebo-controlled study in rhesus monkeys with concurrent administration of either granulocyte/macrophage colony-stimulating factor (GM-CSF) or granulocyte CSF, (G-CSF). Rhesus monkeys were subjected to 5 Gy total-body irradiation (TBI), resulting in 3 weeks of profound pancytopenia, and received either TPO 5 μg/kg intravenously (IV) at day 1 (n = 4), GM-CSF 25 μg/kg subcutaneously (SC) for 14 days (n = 4), TPO and GM-CSF (n = 4), G-CSF 10 μg/kg/d SC for 14 days (n = 3), TPO and G-CSF (n = 4), or placebo (carrier, n = 4; historical controls, n = 8). Single-dose IV treatment with TPO 1 day after TBI effectively counteracted the need for thrombocyte transfusions (provided whenever thrombocyte levels were <40 × 109/L) and accelerated platelet reconstitution to normal levels 2 weeks earlier than placebo controls. TPO/GM-CSF was more effective than single-dose TPO alone in stimulating thrombocyte regeneration, with a less profound nadir and a further accelerated recovery to normal thrombocyte counts, as well as a slight overshoot to supranormal levels of thrombocytes. Monkeys treated with TPO/GM-CSF uniformly did not require thrombocyte transfusions, whereas those treated with GM-CSF alone needed two to three transfusions, similar to the placebo-treated monkeys, which required, on average, three transfusions. Also, reticulocyte production was stimulated by TPO and further augmented in monkeys treated with TPO/GM-CSF. TPO alone did not stimulate neutrophil regeneration, whereas GM-CSF shortened the period of neutrophil counts less than 0.5 × 109/L by approximately 1 week; TPO/GM-CSF treatment elevated the neutrophil nadir, but did not further accelerate recovery to normal values. TPO also augemented the neturophil response to G-CSF, resulting in similar patterns of reconstitution following TPO/G-CSF and TPO/GM-CSF treatment. TPO/GM-CSF resulted in significantly increased reconstitution of CD34+ bone marrow cells and progenitor cells such as GM-CFU and BFU-E. Adverse effects of combining TPO with the CSFs were not observed. It is concluded that (1) a single IV administration of TPO is sufficient to prevent severe thrombocytopenia following myelosuppression, (2) TPO/G-CSF and TPO/GM-CSF treatment result in distinct response patterns, with TPO/GM-CSF being superior to TPO/G-CSF in stimulating thrombocyte and erythrocyte recovery while being equivalent in stimulating neutrophil recovery; and (3) TPO significantly improves the performance of CSFs in alleviating severe neutropenia.


2021 ◽  
Vol 162 (8) ◽  
pp. 306-313
Author(s):  
Ágnes Bakos ◽  
Anna Bátyi

Összefoglaló. A methaemoglobinaemia az oxigén szállítására képtelen methemoglobin szintjének kóros emelkedését jelenti a vérben, ami jelentős szöveti oxigénhiányt okozhat, súlyos, akár életveszélyes tünetekhez vezethet. Methaemoglobinaemiát számos, oxidáló hatású exogén anyag idézhet elő, ezek közé tartoznak a partidrogként használt alkil-nitritek, az ún. „popperek” is. A „poppereket” korábban „alacsony rizikójú” drogként tartották számon, azonban számos esetet közöltek, amikor súlyos, időnként fatális kimenetelű methaemoglobinaemiát okoztak. A folyadékok gőzének belélegzése euforizáló, szexuálisvágy-fokozó és simaizom-lazító hatású, ezért a „popperek” igen népszerűek a homo- és biszexuális férfiak körében, de fiatal felnőttek és tinédzserek is használják. A folyadékok szájon át való fogyasztása különösen veszélyes. A szerzők két esetet ismertetnek, amelyekben a „popperek” használatát követően methaemoglobinaemia alakult ki. Mindkét betegnél, a jó általános állapot mellett, centrális és perifériás cyanosis tüneteit észlelték. Az alkalmazás módja (inhaláció/lenyelés), a methaemoglobinaemia súlyossága (16,4% és 57%) és a terápia eltérő volt a két betegnél. Az első beteg oxigén adása és tüneti kezelés mellett gyógyult, a másodiknál antidotum (metilénkék) adására is szükség volt. Mindketten panaszmentesen távoztak a kórházból. A szerzők célja az volt, hogy felhívják a figyelmet az illékony alkil-nitrit-származékok által okozott methaemoglobinaemiára, annak felismerésére, kezelésére, és bemutassák azok kevésbé ismert szövődményeit is. Orv Hetil. 2021; 162(8): 306–313. Summary. Methemoglobinemia means the abnormally elevated level of methemoglobin in the blood, which is incapable of oxygen transport, accordingly it can cause significant tissue hypoxia, leading to severe or even life-threatening clinical symptoms. Several exogen oxidative agents can induce methemoglobinemia, including alkyl-nitrites which are also used as party drugs, the so-called ‘poppers’. The ‘poppers’ were previously considered ‘low-risk’ drugs, however, several cases have been published when they caused severe, sometimes fatal methemoglobinemia. Inhaling vapours from liquids has euphoric, smooth-muscle relaxing and aphrodisiac effects, therefore ‘poppers’ are extremely popular among gay and bisexual men but also used by young adults and teenagers. Oral consumption of the fluids is particularly dangerous. The authors present two cases when methemoglobinemia developed after ‘poppers’ usage. Both patients were in good general condition and symptoms of central and peripheral cyanosis were detected. The method of application (inhalation/ ingestion), the severity of methemoglobinemia (16,4% and 57%) and the treatment were different in the two patients. The first patient recovered with inhalation of oxygen and symptomatic treatment; the second patient required administration of antidote (methylene blue). Both patients left the hospital without complaints. The authors’ aim was to attract attention to methemoglobinemia caused by volatile alkyl-nitrites, its recognition, treatment and to present their lesser-known complications. Orv Hetil. 2021; 12(8): 306–313.


2021 ◽  
Vol 14 (12) ◽  
pp. e245695
Author(s):  
Shafini Beryl ◽  
Benjamin Jeyanth Ross ◽  
Mintoo Tergestina ◽  
Manish Kumar

Evans syndrome is a rare and chronic autoimmune disease seen in both paediatric and adult age groups. We present a case of severe thrombocytopenia in a neonate born to a mother with Evans syndrome who showed no response to intravenous immunoglobulin therapy initially and improved after treatment with methylprednisolone.


2015 ◽  
Vol 6 (4) ◽  
pp. 19-22
Author(s):  
Nina Olegovna Postanogova ◽  
Lyudmila Vasil’evna Sofronova ◽  
Ferdausa Ravkhatovna Milyakova

The article contains the results of observations in 54 children aged 1 to 6 years with infectious mononucleosis. The evaluated of clinical and laboratory data with consideration of the etiological factor of the disease. Depending on the etiology there are 3 groups of patients: 18 with EBV-associated IM, 18 with CMV IM and 18 with mixed etiology mononucleosis (EBV + CMV). All children received antibiotic therapy (cephalosporin of third generation), immunostimulatory drugs, in a dosage of age (recombinant interferon Alfa-2b) and symptomatic treatment. Verification of pathogens was carried out by enzyme-linked immunosorbent assay, were determined separately specific immunoglobulins M and G antibodies to EBV antigens and CMV. Disease in all children was accompanied by fever, tonsillitis, lymphadenopathy, enlarged liver and spleen. A laboratory study of characteristics was carried out at the time of admission and on day 11 of hospitalization. Intoxication with EBV IM were significantly more common than with CMV IM (p = 0,018). The pasty face was noted when mixed IM significantly more frequently than when monoethylamine versions (in comparison with EBV-IM p = 0,03, with CMV IM - p = 0,001). Splenomegaly in EBV + CMV IM more often met in comparison with EBV-IM (p = 0,01) and CMV- IM (p = 0,02). More significant levels of neutropenia and limfomonotsitozis occurred during the EBV-IM. Leukocytosis and increased ESR were more pronounced with CMV IM. Indicators of aminotransferases increased more significantly if EBV IM + CMV IM (in comparison with EBV IM p = 0,04, with CMV IM p = 0,05). On 11th day of hospitalization when the EBV IM in comparison with CMV IM remained more pronounced neutropenia and monocytosis (p = 0,04 and p = 0,05 respectively), nonspecific changes in laboratory indicators (leucocytosis, increased erythrocyte sedimentation rate) - CMV IM (p = 0,05 and p = 0,04 respectively).


Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4520-4520
Author(s):  
Annamaria Nosari ◽  
Guido Nador ◽  
Andrea de Gasperi ◽  
Nichelatti Michele ◽  
Anghilieri Michela ◽  
...  

Abstract CVC-related complications were retrospectively analysed for 373 CVCs inserted in 261 hematologic pts consecutively admitted to our Hematology Department between January 2002 and March 2004. Pts diagnosis comprised AML (96 pts, 36.8%), ALL (19 pts, 7.3%), lymphoproliferative disorders (74 pts, 28.3%), MM (51 pts, 19.5%), CML (13 pts, 5%), others (8 pts, 3.1%). The CVCs were polyurethane Plastimed three lumen 7 Fr (193 cases) for chemotherapy and polyurethane Arrow three lumen 12 Fr (173 cases) for chemotherapy and peripheral blood stem cell (PBSC) apheresis. CVCs were inserted, according to physician’s judgement, either at bedside (369 cases) or in the operating room (4 cases) and were used for drug infusion in 309 cases (82.8%), for total parenteral nutrition in 19 cases (5.1%), for blood transfusions in 226 cases (60.6%), for PBSC apheresis in 152 cases (40.7%). Fifty five CVCs were inserted in neutropenic pts ( N < 1 x 109/L); severe thrombocytopenia (Plts < 30 x 109/L) was present in 33/373 cases. Antithrombotic prophylaxis with low molecular weight heparin because of previous thrombosis was instituted in 33 cases (8.8%). At univariate and multivariate analysis the following risk factors for catheter-related bloodstream infections were considered: pt age, number of days/catheter, haematological disease, catheter lumen, administration of chemotherapy (standard- vs high-dose), presence of neutropenia. The median duration of CVCs after placement was 22.7 days (range 2–70) for the 7 Fr lumen, and 9 (range 1–39) for the 12 Fr. Major hemorrhagic complications related to the insertion procedure were observed in 4 cases of whom 2 were severely thrombocytopenic. CVC occlusion were observed in 24 cases (6.4%). Thrombotic complications developed in 7 cases (1.87% of inserted CVCs) and in 2/7 were complicated by pulmonary embolism; thrombophilia tests were negative in all 7 pts who developed thrombosis. Among febrile pts the frequency of bacteriemias was 19.6% (73/373 cases) of which 52/373 were CVC-related (13.9%). Approximately 6% of cases (23/373 CVCs) had CVC exit point infection with or without associated bacteriemia. Gram positive bacteria were isolated in 49 cases (67.1%), 38 of which were CVC-related (19 S. epidermidis, 4 S. pneumoniae, 3 S. aureus, 3 Enterococci species, 9 others). Gram negative bacteria were isolated in 23 cases, of which 13 were CVC-related (5 E.coli, 4 E. cloacae, 2 P. aeruginosa, 2 K pneumoniae). Candida was isolated in one case. Overall mortality in our patient population was 4.2% (11/261 pts); one patient only died of infection (P. aeruginosa sepsis) not CVC-related. At univariate and multivariate analysis significant risk factors for infection (p<0.0001) were only the number of days/catheters and duration of neutropenia. Among severely thrombocytopenic pts, haemorrhagic complications at CVC insertion were infrequent (6%). Although pts in our study population frequently displayed prolonged and severe neutropenia, frequency of CVC-related bacteremias was low (13.9%) compared to a general population reported frequency of 5–25%. Moreover, none of the pts died of CVC-related infection.


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