cell disorder
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2022 ◽  
Vol 29 (1) ◽  
pp. 1
Author(s):  
Eglė Žilėnaitė ◽  
Laura Malinauskienė ◽  
Kęstutis Černiauskas ◽  
Linas Griguola ◽  
Kotryna Linauskienė ◽  
...  

Background: Allergic reactions after messenger RNA (mRNA)-based COVID-19 vaccines have been reported but detailed descriptions and further actions are not well characterized. Objective: To describe the symptoms of possible allergic reactions after the mRNA COVID-19 vaccine and outcomes of further vaccination. Methods: We descriptively analyzed data of adult (≥18 years of age) patients, who were sent for vaccination to our outpatient center for the Diagnostics and Treatment of Allergic and Immune diseases. All patients were vaccinated with the Pfizer–BioNTech Comirnaty® vaccine.Results: From January 2021 to July 2021 twenty-two patients were vaccinated in our center. Six patients experienced a reaction after the first Comirnaty® dose in different vaccination centers. The majority of them complained of various types of rashes after the first dose, one case was consistent with anaphylaxis. The latter patient was tested with the skin prick using Pfizer–BioNTech Comirnaty® vaccine and the test was negative. Other sixteen patients were vaccinated in our center from the first dose because of past allergic reactions to other medication or due to concomitant mast cell disorder. All patients were vaccinated without any immediate adverse reactions.Conclusions: None of our patients experienced repeated cutaneous reactions after the second dose. Patients with previous anaphylaxis or mastocytosis also were safely vaccinated.


Author(s):  
Opeyemi Olaoluwa Oredola ◽  
Kehinde Opeyemi Oyesomi ◽  
Ada Sonia Peter

The importance of health communication and information cannot be over emphasized especially with issues related to sickle cell disorder. Sickle cell disorder, common among Africans, has a lot of myths and misconceptions tied to it, so this chapter unearths and explores how indigenous communication can facilitate learning and understanding of the disorder majorly in rural areas and some urban areas where knowledge of the disorder is assessed low using the focus group discussion. It also reveals the importance of incorporating indigenous language and communication techniques in increasing awareness and eradicating stereotypes as regards sickle cell disorder. The findings of this chapter reflect that misinformation occurs due to lack of proper understanding of language used in sickle cell health communication-related issues. Hence, this chapter proposes that health education about the concept of SCD should be executed majorly in indigenous languages and through the indigenous media platforms.


Author(s):  
Cynthia Omoseyitan Ojomo ◽  
Taye Babaleye Babaleye

Sickle cell disorder (SCD) is a defective blood disorder that causes pain associated with blood genotypes of victims who risk dying before their 30th birthday. SCD is best controlled when victims avoid getting married to one another to prevent the spread of susceptible genotype. Many youths lack basic facts of SCD. For long, its awareness was limited to broadcast and print media. But today, social media platforms—Facebook, Instagram, Twitter, and WhatsApp—are also used to create awareness on the disease. This study attempts to determine the preferred communication channels for creating awareness on SCD among university students in South-Western Nigeria. Survey research design was adopted. Respondents were 259 students of Obafemi Awolowo University, Ile-Ife, and University of Ibadan, both in South-West Nigeria. Descriptive and inferential statistics were used for data analysis. Social media awareness was 51.6%, and conventional media was 48.4%. Thus, more of the respondents preferred awareness on SCD through social media platforms.


2021 ◽  
Author(s):  
Ajeet Kumar ◽  
Vatsal Mishra ◽  
Chandra Bhan Singh ◽  
Rashmi Patel ◽  
Siddharth Samrat ◽  
...  

Abstract Background Chronic myeloid leukaemia (CML) is a hematopoietic stem cell disorder, caused by a balanced reciprocal translocation (t(9;22) (q34;q11))that lead to the formation of BCR (Break point Cluster Region)-ABL (Abelson) fusion transcripts known as Philadelphia (Ph) chromosome. Prevalence of BCR-ABL fusion transcripts in Indian CML population is poorly understood and few studies have been reported from India. The aim of present study was to determine the frequencies as well as prognostic effects of the three fusion transcripts i.e. b2a2, b3a2 and e1a2 in an Indian population. Methods RNA was isolated from total 123 sample 27 bone marrow (BM) sample and 96 Peripheral blood (PB) sample of CML patient followed by cDNA synthesis. Real-time quantitative reverse-transcription polymerase chain reaction (qRT-PCR) was performed using TaqMan→ assay (ABI, CA, USA) to monitor BCR-ABL transcript. Results Ph' chromosome was observed in 103 patients whereas it was not detected in 20 cases. qRT-PCR revealed that the b3a2 fusion transcripts was the most common transcript in CML patients (63.41%) while b2a2 fusion transcript was present in 16.26% cases. Co-expression of b3a2+b2a2 fusion transcript was observed in 0.81% cases whereas co-expression of b3a2+e1a2 fusion transcript was found in 1.63% cases. There was no co-relation observed between b3a2 fusion transcript and platelet count. The fusion transcript b2a2 was observed in relatively younger patients compared to b3a2 fusion transcript. Although this correlation was not statistically significant. Conclusion The co-expression of BCR-ABL fusion transcripts was higher (63.41% aggregate of b3a2) in the present population in contrast to other populations reported. This finding was consistent with the frequency data reported from Sudan.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 3787-3787
Author(s):  
James E Hoffman ◽  
Marla B. Sultan ◽  
Balarama Gundapaneni ◽  
Ronald Witteles

Abstract Introduction: In cardiac amyloidosis (CA), immunoglobulin-derived light chains (AL) and transthyretin (TTR) are the most common amyloidogenic proteins infiltrating the heart. 1 Identification of the specific precursor protein leading to amyloid deposition is needed to establish the correct therapeutic approach. 2 In both AL- and TTR-related CA, an early and accurate diagnosis is critical to achieving the best treatment outcomes. TTR amyloid cardiomyopathy (ATTR-CM) is often misdiagnosed as other causes of heart failure (HF), including AL CA. 3 While almost all patients with AL amyloidosis have elevated serum free light-chain (sFLC) levels and abnormal free kappa:lambda (κ:λ) ratios, 4 patients with ATTR-CM can also have abnormal sFLC levels due to either an unrelated monoclonal gammopathy or relative κ-predominance in renal dysfunction. 2,5 Because ATTR-CM most often occurs in elderly adults and is commonly accompanied by renal comorbidity, we theorized that patients with ATTR-CM may have κ:λ ratios that approach, or exceed, the upper limit of the normal reference range (0.26-1.65 6). High light-chain ratios in these patients have the potential to increase the likelihood of misdiagnosis of a monoclonal plasma cell disorder and may lead to unnecessary referrals to hematologists and/or inappropriate treatments. To explore this theory, we evaluated κ:λ ratios in patients with biopsy-proven ATTR-CM who were enrolled in the Tafamidis in Transthyretin Cardiomyopathy Clinical Trial (ATTR-ACT). 7 Methods: In ATTR-ACT, a double-blind, placebo-controlled, randomized study, patients with biopsy-proven ATTR-CM, and without light-chain amyloidosis, received tafamidis or placebo for 30 months. In the current analysis, sFLC levels and κ:λ ratios were assessed in the intent-to-treat population (N=441), excluding patients with a prior diagnosis of monoclonal gammopathies (n=13; defined by MedDRA preferred terms: 'monoclonal gammopathy', 'plasma cell myeloma', 'plasma cell disorder', and 'hypergammaglobulinemia benign monoclonal'). Subgroup analyses were performed by estimated glomerular filtration rate (eGFR) category (≥60 vs ≥40 to <60 vs <40 mL/min/1.73 m 2). Findings were summarized using descriptive statistics (min/max, mean, median, and interquartile range [IQR]). Results: In 422 patients with ATTR-CM and available sFLC data, and without a prior diagnosis of monoclonal gammopathies, the mean κ:λ ratio was 1.45 (median, 1.20 [IQR, 0.98, 1.47]) (Figure). The ratio increased with declining renal function: eGFR ≥60 mL/min/1.73 m 2, mean, 1.25 (median [IQR], 1.11 [0.94, 1.38]); ≥40 to <60 mL/min/1.73 m 2, 1.52 (1.22 [0.99, 1.49]); and <40 mL/min/1.73 m 2, 1.62 (1.30 [1.05, 1.68)]. Conclusions: In patients with biopsy-proven ATTR-CM without known monoclonal gammopathies who were enrolled in ATTR-ACT, the mean κ:λ ratio showed a κ-predominance, often exceeding the upper range of normal in patients with more advanced kidney dysfunction. The findings suggest that individuals with ATTR-CM can have higher sFLC levels than those normally seen in the general population, and such elevations do not necessarily indicate the presence of monoclonal gammopathy of undetermined significance or AL CA. In an era when most patients with ATTR-CM and without monoclonal gammopathies are diagnosed noninvasively using bone scintigraphy, age- and renal-function-based sFLC norms are critical to ensure appropriate use of diagnostic testing modalities. References: 1. Maleszewski JJ. Cardiovasc Pathol. 2015;24:343-50. 2. Donnelly JP, et al. Cleve Clin J Med. 2017;84:12-26. 3. Witteles RM, et al. JACC Heart Fail. 2019;7:709-716. 4. Falk RH, et al. J Am Coll Cardiol. 2016;68:1323-41. 5. Geller HI, et al. Mayo Clin Proc. 2017;92:1800-5. 6. Katzmann JA, et al. Clin Chem. 2002;48:1437-44. 7. Maurer MS, et al. N Engl J Med. 2018;379:1007-16. Figure 1 Figure 1. Disclosures Hoffman: BMS, Celgene: Honoraria. Sultan: Pfizer: Current Employment, Current equity holder in publicly-traded company. Gundapaneni: Pfizer: Current Employment, Current equity holder in publicly-traded company. Witteles: Pfizer: Honoraria, Research Funding; Alnylam: Honoraria, Research Funding; Eidos: Research Funding.


2021 ◽  
Vol 21 ◽  
pp. S30
Author(s):  
Wei Yee Chan ◽  
Lara Howells ◽  
William Wilson ◽  
Emilie Sanchez ◽  
Louise Ainley ◽  
...  

2021 ◽  
Author(s):  
Annette Akinsete ◽  
Michael Ottun ◽  
Adelabu Hameed ◽  
Jorden Veeneman ◽  
Larry Ajuwon

The study aimed to assess the impact of the COVID-19 pandemic on Quality of Life (QoL) in persons living with Sickle Cell Disorder (SCD) in Lagos, Nigeria and to determine how they coped during the pandemic, particularly during the period of total lockdown with the additional SHIELDING measures to which they had to adhere. Data was collected using a standardized protocol PedsQL, Sickle Cell Disease Module version 3, designed for youth within the ages of 13 to 18 years and 19 to 35 years and their parents and guardian if underage. The survey captured data on patients pain impact, hurts, management, treatments, communication with their caregivers and their guardians perception. The survey was performed online, or face to face and telephone interview if online was not possible. Contacts of patients and parents were obtained from the database of Sickle Cell Foundation Nigeria. A total of 105 (80 patients and 25 parents) participants responded to the survey. The age distribution of respondents was highest at 56 percent in the age bracket of 13 to 18 years old. Pain crisis were very common amongst patients. The survey revealed that the type of treatment or care received at these times determined whether or not the patients visited the hospital when they had pain crises. In addition, as patients reports an increase in ill treatment they experienced in the hands of health care givers, so did the fear of accessing treatment during the COVID pandemic. It was observed that the frequency of pain crises experienced by SCD patients was proportional to the patients quality of life (the higher the frequency of pains, the worse the QoL). As a follow-up, a more detailed study would be required, as this study was limited in the capturing of the demographics, sex and number of participants; Considering the number of persons living with SCD that visit the Sickle Cell Foundation Nigeria, (approx. 3,000 patients), the number of responses in this study was low (105). It is believed that a higher number of responses would have given more information about the Sickle Cell burden and the QoL of persons living with SCD in Lagos during the COVID-19 pandemic. Lagos was the epicentre of the COVID-19 pandemic in Nigeria.


2021 ◽  
Vol 83 (3) ◽  
pp. 29-35
Author(s):  
Mayur Bhavsar ◽  
Joseph Cercone ◽  
Catherine Chiu

Waldenstrom macroglobulinemia (WM) is a rare, malignant lymphoproliferative B-cell disorder causing an excessive buildup of monoclonal protein. WM is associated with excessive buildup of IgM, which can cause blood hyperviscosity and damage many organ systems. This case report describes a patient who was followed annually but rapidly developed posterior pole and significant midperipheral hemorrhages secondary to a hyperviscosity condition of the retina. Management of this condition is dependent on macular involvement and must be co-managed with an oncologist.


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