Hyperhomocysteinemia-related lung disease and hemolytic anemia with bone marrow features masquerading as myelodysplasia

xlomafota13 shared this article with you from Inoreader Hyperhomocysteinemia-related lung disease and hemolytic anemia with bone marrow features masquerading as myelodysplasia Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):266-270. eCollection 2021. ABSTRACT Hyperhomocysteinemia is linked to TMA-related clinical symptoms such as apparent thromboembolism, microangiopathic hemolytic anemia (MAHA), and various types of end-organ damage due to microvascular thrombi; this is because high plasma levels of homocysteine impair the vascular endothelium. However, the association between hyperhomocysteinemia and pulmonary involvement is unclear. Here, we describe a 63-year-old male who was hospitalized with respiratory failure and MAHA with MDS-like features in the bone marrow. Plasma homocysteine levels were elevated significantly with 199.4 µmol/L (reference: 6.3-18.9) due to a homozygous (T/T) polymorphism for the 677C>T mutation within the MTHFR gene associated with chronic alcoholism-induced folate deficiency. Pulmonary lesions showed ground-glass opacity and there was pleural effusion. The patient was managed succe ssfully with a combination of folate/mecobalamin supplementation, plasma exchange, and a methylprednisolone pulse, followed by oral prednisolone. Clinical symptoms, lung disease, MAHA, and bone marrow abnormalities improved as plasma homocysteine levels normalized. PMID:34322290 | PMC:PMC8303007 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

SARS-CoV-2 infection in a pediatric acute leukemia patient on chemotherapy and concurrent sofosbuvir/velpatasvir for HCV

xlomafota13 shared this article with you from Inoreader SARS-CoV-2 infection in a pediatric acute leukemia patient on chemotherapy and concurrent sofosbuvir/velpatasvir for HCV Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):286-289. eCollection 2021. ABSTRACT There are new targets identified by experimental and animal research for treatment of SARS-COV-2 (Severe acute respiratory syndrome-Corona Virus-2) infection. Out of many clinical trials registered, there are ongoing human studies highlighting Sofosbuvir's possible role in the treatment of Covid-19 (Coronavirus Disease 2019). Here we present a case of acute leukemia on directly acting antiviral therapy (DAAs) for HCV infection mitigating SARS-COV-2 infection in a patient undergoing chemotherapy. The child was undergoing chemotherapy, along with directly acting antiviral for acute hepatitis C infection. He initially had features of hypoxia and radiological evidence of covid-19. He had an uneventful course and tested negative ten days after onset of illness. With ongoing trials on Sofosbuvir in covid 19 treatment, our finding, albeit coincidental, points to the possibl e role even in immune-compromised children. PMID:34322293 | PMC:PMC8303012 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Haemoglobin cut-off values for the diagnosis of anaemia in preschool-age children

xlomafota13 shared this article with you from Inoreader Haemoglobin cut-off values for the diagnosis of anaemia in preschool-age children Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):248-254. eCollection 2021. ABSTRACT BACKGROUND: The World Health Organisation (WHO) suggests haemoglobin that (Hgb) cut-off levels below 2SD from the population mean to initiate anaemia investigations. In the absence of epidemiological data, Hgb less than 11 g/dL is considered abnormal in children up to the age of 59 months (4 years and eleven months). OBJECTIVES: This study reports on the Hgb cut-off levels among children at 1 and 4 years of age. The study compared the prevalence based on the WHO generic cut-off levels and population-specific cut-off-based value defined as below 2SD from the population mean. DESIGN, SETTINGS, AND PARTICIPANTS: A cross-sectional record-based study of healthy children below the age of 59 months attending primary care settings in Qatar. 3 years of Hgb data were collected and analysed using descriptive analyses. We excluded children with any pre-existing disea se or who have altered biological parameters indicating a non-healthy child. RESULTS: 39407 Participants were stratified into different sub-groups according to age, gender, and ethnicity. Hgb levels were expressed as the mean ± 2SD for children of one and four years of age. Most children were from Western Asia (45.6%), followed by Northern Africa (23.7%), and Southern Asia (21.7%). Our findings for one-year-old children cut-off levels for anaemia might be as low as 9.9 g/dL and 10.6 g/dL for 4-years old. CONCLUSION: Hgb cut-off values may be set at higher levels for one-year and four-year age groups and many different ethnicities. Higher cut-off points may overestimate the problem as a public health issue. Children may be unnecessarily treated with iron or have needless investigations. PMID:34322287 | PMC:PMC8303014 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Hypomethylating agents+venetoclax induction therapy in acute myeloid leukemia unfit for intensive chemotherapy - novel avenues for lesser venetoclax duration and patients with baseline infections from a developing country

xlomafota13 shared this article with you from Inoreader Hypomethylating agents+venetoclax induction therapy in acute myeloid leukemia unfit for intensive chemotherapy - novel avenues for lesser venetoclax duration and patients with baseline infections from a developing country Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):290-302. eCollection 2021. ABSTRACT Both elderly acute myeloid leukemia (AML) patients and those with baseline infections, when treated with intensive chemotherapy, are associated with high induction mortality. We report 24 patients (16-newly-diagnosed, 8-relapsed/refractory) with AML deemed unfit for intensive chemotherapy (by virtue of age >60 years, ECOG-PS 3-4, or those with non-resolving infections at baseline), treated with azacytidine-venetoclax combination as induction chemotherapy. Median follow-up of the study group was 8 months. The overall complete remission (CR)+CR with incomplete count recovery (CRi) rate was 58.3%. 1-year progression-free survival and overall survival of the whole cohort was 44.4% and 55.8%, respectively. On subgroup analysis, newly-diagnosed AML (p=0.05), intermediate-risk cytogenetics (p=0.007), and HMA-naïve (p=0.05) patients had a significantly better outcome. AM L patients with baseline infections (versus without infections) treated with azacytidine-venetoclax induction, have lesser induction mortality (compared with historic intensive chemotherapy) with equivalent response rates. A detailed analysis amongst cohorts with different venetoclax durations revealed that, shorter duration (<21 days) venetoclax (versus 21-28 days duration) in induction therapy leads to similar response rates and similar severity of myelosuppression, however, with early count recovery and lesser duration of intravenous antibiotics. PMID:34322294 | PMC:PMC8303019 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

A new FLT3 inhibitor with two cases: the gilteritinib experience

xlomafota13 shared this article with you from Inoreader A new FLT3 inhibitor with two cases: the gilteritinib experience Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):271-278. eCollection 2021. ABSTRACT INTRODUCTION: In acute myeloid leukemia (AML), a heterogeneous group of leukemias, there are various factors to determine prognosis. Among these prognostic factors, cytogenetic results are increasing in importance day by day. FLT3 mutations are among the most common molecular abnormalities in AML, patients with recurrent or refractory (R/R) AML with this mutation have a low response rate to salvage therapy. Gilteritinib has activity against FLT3, ALK and AXL. This article shall present two cases, for which Gilteritinib was used, a new FLT3 inhibitor, and the results of the treatment. Case 1: A 52-year-old female patient presented to the emergency clinic with weakness and fever. In initial biochemical analysis, leukocyte was 104000/mm3. Peripheral smear contained diffuse myeloid blastoid cells, peripheral blood flow cytometry also supported the AML M0-1 phe notype. The bone marrow biopsy aspiration performed on the 14th day of induction "3+7" treatment, contained diffuse blastic infiltrate and supported refractory disease. In addition to the FLAG-IDA salvage regimen, 120 mg/day Gilteritinib was also started. Bone marrow aspiration performed on the 28th day of salvage therapy was compatible with remission. Case 2: 53 years old male patient with also no comorbidity other than known hypertension. In the initial biochemical analysis of the patient, leukocyte was 156000/mm3, platelet 58000/mm3 and hemoglobin 7.6 g/dl. Peripheral blood flow cytometry supported the AML M5 phenotype, whose peripheral smear showed diffuse monoblastoid cells. On the 14th day of the patient's 3+7 induction treatment, the control bone marrow aspiration showed diffuse blast infiltration and was considered refractory, FLAG-IDA salvage therapy with again 120 mg/day Gilteritinib per oral were started. On the 28th day, control bone marrow aspiration was evaluated as remission. DISCUSSION AND CONCLUSION: Unlike other FLT 3 inhibitors, Gilteritinib has been shown to be a highly effective agent in R/R AML with FLT3 mutations. Being the first data to be reported from Turkey, we think it would be quite guiding the titular. PMID:34322291 | PMC:PMC8303015 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Therapeutic lessons from transfusion in pregnancy-effect on hematological parameters and coagulation profile

xlomafota13 shared this article with you from Inoreader Therapeutic lessons from transfusion in pregnancy-effect on hematological parameters and coagulation profile Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):303-316. eCollection 2021. ABSTRACT INTRODUCTION: Transfusion is commonly done in clinical indications and complications arising due to Anemia, shock, blood loss, thrombocytopenia due to any cause, ineffective erythropoiesis. Pregnancy is a physiological condition characterized by Anemia, fluid overload, hypercoagulable state, and antifibrinolytic condition, which can cause various reactions that could be anticipated during a blood transfusion. With an aim to understand the effects of transfusions on hematological parameters in pregnancy. The results of whole blood and component transfusion were studied to understand increments and their effects so that rationalized transfusion decisions during pregnancy can be undertaken, considering the physiological changes in pregnancy on hemodynamics are present. METHODOLOGY: A prospective study with 80 pregnant females undergoing blood transfusion was studi ed. Their coagulation and hematological profile were correlated to derive a conclusion for the effect of transfusion of blood and its products. RESULTS: A mean increment of 0.55+0.07 g/dL hemoglobin (Hb) was noted along with a slight increase in RBC count (0.25+0.07 millions/mm3), hematocrit (HCT) (1.9+0.42%), TLC (400+565 cells/mm3). This statistically significant mean increase in hemoglobin, RBC count, and hematocrit was significantly lower than that compared to studies in the west and non-anemic patients. A mean increment of 7.79+1.51 µg/dL (statistically significant) in serum iron was seen. A significant improvement in their coagulation profile was achieved by plasma transfusion (FFP). Clotting time (CT) decreased by a mean value of 196.43+56.69 secs and prothrombin time (PT) by 2.64+0.63 secs (P<0.05). All transfusion reactions in our study were associated with PRBC transfusion, non-hemolytic immunological type, urticarial transfusion reactions ( UTR) more common in multiparous women-0.2% in primigravida to 21.7% and 37.5% in 3rd and 4th parity similar to that observed in other studies. CONCLUSION: Although different researchers have done numerous studies, the physiological profile of pregnant females in India is markedly different in nutritional profile, ethnicity, environmental factors, and background. The availability of tertiary care medical facilities during ANCs is also known to affect pregnancy outcomes and the presentation of patients at term or in labor. The variety of factors affect the baseline hematological status of pregnant females and, hence, post-transfusion hematological factors. These are therefore markedly different from prior published studies. It is concluded that PRBC transfusion in pregnant women causes a lower increase in mean Hb and HCT values than in the west, and ferritin and serum iron are not reliable indicators of Anemia in transfusion. Due to lower increments in all values except platelet s could be the reason for this could be contributed by confounding factors like Anemia, hyperfibrinogenemia, volume overload, and ethnicity. PMID:34322295 | PMC:PMC8303008 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Nivolumab in relapsed/refractory Hodgkin lymphoma: towards a new treatment strategy?

xlomafota13 shared this article with you from Inoreader Nivolumab in relapsed/refractory Hodgkin lymphoma: towards a new treatment strategy? Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):261-265. eCollection 2021. ABSTRACT Chemo-refractory Hodgkin lymphoma (HL), especially after failure of high-dose therapy and autologous stem cell transplantation (ASCT), has a very poor prognosis. Nivolumab, an anti-PD-1 monoclonal antibody, demonstrated durable responses and manageable toxicity in a significant proportion of HL patients who fail both ASCT and brentuximab vedotin. Although anti-PD-1 treatment is often well tolerated, immune-related adverse events (iAE) were frequently observed. New perspectives could be represented by treatment discontinuation in patients with prolonged response or toxicity with the possibility of a re-treatment at relapse, subsequent chemotherapy or a modification of the dose-intensity or treatment duration. The efficacy of anti-PD-1 re-treatment was demonstrated in several cases and we have successfully managed 1 case with this strategy. With the main aim of avoidin g the relapse-related psychophysical stress for the patient with manageable toxicity, we have successfully administered nivolumab every 4 weeks to 3 patients in prolonged complete remission, who presented with iAE during treatment. We believe that nivolumab should not only represent a bridge to allogeneic SCT, but it may play an important role also beyond the approved indication and current standard clinical care. PMID:34322289 | PMC:PMC8303013 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Personalization of Visual Scene Displays: Preliminary Investigations of Adults With Aphasia, Typical Females Across the Age Span, and Young Adult Males and Females

xlomafota13 shared this article with you from Inoreader Personalization of Visual Scene Displays: Preliminary Investigations of Adults With Aphasia, Typical Females Across the Age Span, and Young Adult Males and Females Via Topics in Language Disorders - Current Issue Visual scene displays (VSDs) are becoming an increasingly popular method of message representation within augmentative and alternative communication (AAC) supports; however, design factors can influence the effectiveness of these images as communication supports. One issue that has come to light in recent years is the fact that selecting personalized VSDs, which depict the person with complex communication needs or an individual with whom they are familiar, is preferred over generic VSDs, which depict unfamiliar individuals. Although personalization is likely an important factor in the usability of VSDs, th ese images may be difficult for clinicians to obtain. As such, compromises must be identified. The purpose of this study was to explore the effects of controlling personal relevance factors (i.e., age and gender of the people depicted in generic VSDs) on the image preference patterns of adults with and without aphasia. Results from three very preliminary study summaries indicate that gender and age are both mitigating factors in image preference, as males tended to indicate preference for VSDs containing males over those containing females. In addition, females tended to indicate preference for females of a similar age depicted in VSDs. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Personalized AAC Intervention to Increase Participation and Communication for a Young Adult With Down Syndrome

xlomafota13 shared this article with you from Inoreader Personalized AAC Intervention to Increase Participation and Communication for a Young Adult With Down Syndrome Via Topics in Language Disorders - Current Issue Many adults with Down syndrome (DS) experience difficulty with speech production, and intelligibility challenges and communication breakdowns are common. Augmentative and alternative communication (AAC) intervention can provide important supports for persons with these complex communication needs but must be customized to address the goals, strengths, and needs of the individual. This article provides a description of a personalized AAC intervention for a young adult with DS whose speech was frequently unintelligible. The AAC intervention made use of a video visual scene display (VSD) approach and was inves tigated in 2 separate studies in 2 key community settings: An inclusive post–secondary education program (a single-case reversal ABAB design), and a community shopping activity (a nonexperimental AB case study design). The participant demonstrated sharp increases in successful communication and participation in both settings following the introduction of the video VSD, and both the participant and the key stakeholders viewed the intervention positively. The results provide preliminary evidence that personalized AAC intervention, including the use of a video VSD approach, can provide important supports for communication and participation in community settings for adults with DS and complex communication needs. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Personalization of Restorative and Compensatory Treatments for People With Aphasia: A Review of the Evidence

xlomafota13 shared this article with you from Inoreader Personalization of Restorative and Compensatory Treatments for People With Aphasia: A Review of the Evidence Via Topics in Language Disorders - Current Issue Speech–language pathologists serving individuals with aphasia must make many decisions regarding assessment and intervention practices to support comprehension and expression of language. Each of these decisions can influence the effectiveness of therapy and long-term functional outcomes for individuals with aphasia. One factor that must be considered is the impact of personalizing therapy techniques to match the unique strengths and challenges of a client as well as incorporating personally relevant stimuli and methodologies into therapy. Research indicates that treatment customization can have a positive impact on service provision quality and can potentially lead to positive outcomes in therapy. The purpose of this article is to provide a broad overview of how personalization ca n be achieved across a variety of interventions. Furthermore, we explore factors (e.g., client motivation, cognitive resources) that influence therapeutic outcomes and discuss the benefits, challenges, and rationale for therapy customization. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Personalized Early AAC Intervention to Build Language and Literacy Skills: A Case Study of a 3-Year-Old With Complex Communication Needs

xlomafota13 shared this article with you from Inoreader Personalized Early AAC Intervention to Build Language and Literacy Skills: A Case Study of a 3-Year-Old With Complex Communication Needs Via Topics in Language Disorders - Current Issue Personalized augmentative and alternative communication (AAC) intervention refers to an approach in which intervention is tailored to the individual's needs and skills, the needs and priorities of the individual's family and other social environments, the evidence base, and the individual's response to intervention. This approach is especially relevant to AAC intervention for young children with complex communication needs given their unique constellations of strengths and challenges, and the qualitative and quantitative changes that they experience over time as they develop, as well as the diversity of their families, schools, and communities. This article provides detailed documentation of personalized AAC intervention over a 6-month period for a 3-year-old girl with developmental delay and complex communication needs. The article describes (1) personalization of multimodal AAC supports to provide this child with the tools to communicate; (2) personalized intervention to build semantic and morphosyntactic skills; and (3) personalized instruction in literacy skills (i.e., letter sound correspondences, sound blending, decoding, sight word recognition, reading simple stories, reading comprehension, and encoding skills). Specific goals, instructional materials, and procedures are described; data on speech, language, and literacy outcomes are presented. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.