American journal of blood research最新文献

{"title":"Ibrutinib in patients with relapsed/refractory mantle cell lymphoma: a real-life, retrospective, multicenter trial on behalf of the \"RTL\" (regional Tuscan lymphoma network).","authors":"Emanuele Cencini,&nbsp;Bianca Mecacci,&nbsp;Francesca Morelli,&nbsp;Francesco Ghio,&nbsp;Ilaria Romano,&nbsp;Silvia Birtolo,&nbsp;Federico Simonetti,&nbsp;Valentina Zoi,&nbsp;Sabrina Moretti,&nbsp;Emanuela Sant'Antonio,&nbsp;Annarosa Cuccaro,&nbsp;Simone Santini,&nbsp;Sofia Kovalchuk,&nbsp;Sara Galimberti,&nbsp;Monica Bocchia,&nbsp;Alberto Fabbri","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Relapsed or refractory (R/R) mantle-cell lymphoma (MCL) patients have a poor prognosis and their management is challenging, in absence of a golden standard as salvage treatment. Bruton's tyrosine kinase inhibitor ibrutinib represents an effective treatment for R/R MCL patients. We investigated ibrutinib efficacy and safety in daily clinical practice, together with factors that could predict disease outcome.</p><p><strong>Patients and methods: </strong>We retrospectively analyzed 69 consecutive R/R MCL patients managed in 10 Tuscan onco-hematological centers. The treatment regimen consisted of oral, continuous, single-agent ibrutinib, maximum dosage of 560 mg once per day, until disease progression.</p><p><strong>Results: </strong>Overall response rate was 62.3%, with a CR rate of 39.1%. After a median follow-up of 15.6 months, 40/69 patients (58%) were alive, the main cause of death was progressive disease (PD, 22/69 cases, 31.9%). Median progression-free survival (PFS) and overall survival (OS) were 17 and 34.8 months. Inferior PFS was associated with >1 prior line of therapy and B symptoms. Ibrutinib refractoriness was associated with inferior OS, median OS after ibrutinib failure was only 5 months.</p><p><strong>Discussion and conclusion: </strong>In this real-life setting ibrutinib treatment prolonged survival in R/R MCL patients, without unexpected adverse events. Patients receiving ibrutinib as 2^nd line regimen had the most favorable outcome.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 4","pages":"373-383"},"PeriodicalIF":0.0,"publicationDate":"2021-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8446834/pdf/ajbr0011-0373.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39431702","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Graves' disease patients with iron deficiency anemia: serologic evidence of co-existent autoimmune gastritis.","authors":"Andrew G Gianoukakis,&nbsp;Shelly Gupta,&nbsp;Theresa N Tran,&nbsp;Patrick Richards,&nbsp;Marelle Yehuda,&nbsp;Sarah E Tomassetti","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Graves' disease (GD) has been associated with iron deficiency anemia (IDA). Atrophic gastritis leads to IDA and has been associated with autoimmune thyroid disease. This study prospectively determined the prevalence of atrophic gastritis markers and the relationship between these markers and markers of IDA in GD subjects.</p><p><strong>Methods: </strong>Newly diagnosed GD patients (90) and controls (41) were studied. Of the newly diagnosed GD patients, 65 were consecutively enrolled and identified with GD irrespective of anemia, 25 had GD and IDA. Thyroid function, hematologic indices, and atrophic gastritis markers [parietal-cell antibodies (PCab), <i>Helicobacter pylori</i> antibodies (<i>H. pylori</i> ab), mean serum gastrin levels] were examined.</p><p><strong>Results: </strong>GD patients presenting with IDA were twice as likely (64% vs. 32%, P=0.049) to harbor PCabs when compared to all other GD subjects. Unselected GD subjects (n=65) had significantly higher PCab (37% vs. 7%, P<0.001) compared to controls. Gastrin levels were significantly elevated in all GD subjects compared to controls (105 vs. 39 pg/ml, P<0.0001). This difference was magnified in PCab+ subjects (202 vs. 64 pg/ml, P=0.003). In all GD subjects, PCabs were associated with increased gastrin levels (202 vs. 75 pg/ml, P=0.0004) and lower ferritin levels (52 vs. 95, P=0.05). In GD anemic subjects, PCabs were associated with lower mean corpuscular volume (75 vs. 81, P=0.001). Gastrin levels correlated inversely with ferritin levels in all GD subjects and positively with TIBC in GD anemic subjects.</p><p><strong>Conclusions: </strong>A significant subset of patients presenting with GD may suffer from IDA due to concurrent autoimmune atrophic gastritis.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"238-247"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303011/pdf/ajbr0011-0238.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Haemoglobin cut-off values for the diagnosis of anaemia in preschool-age children.","authors":"Ehab Hamed,&nbsp;Mohamed Ahmed Syed,&nbsp;Bayan Faleh Alemrayat,&nbsp;Syed Hammad Anwar Tirmizi,&nbsp;Ahmed Sameer Alnuaimi","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>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).</p><p><strong>Objectives: </strong>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.</p><p><strong>Design, settings, and participants: </strong>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 disease or who have altered biological parameters indicating a non-healthy child.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusion: </strong>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.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"248-254"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303014/pdf/ajbr0011-0248.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254655","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nivolumab in relapsed/refractory Hodgkin lymphoma: towards a new treatment strategy?","authors":"Emanuele Cencini,&nbsp;Monica Bocchia,&nbsp;Alberto Fabbri","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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 avoiding 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.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"261-265"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303013/pdf/ajbr0011-0261.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"SARS-CoV-2 infection in a pediatric acute leukemia patient on chemotherapy and concurrent sofosbuvir/velpatasvir for HCV.","authors":"Amitabh Singh,&nbsp;Akriti Gera,&nbsp;Aroonima Misra,&nbsp;Sumit Mehndiratta","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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 possible role even in immune-compromised children.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"286-289"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303012/pdf/ajbr0011-0286.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254621","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"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.","authors":"Sumeet Mirgh,&nbsp;Archana Sharma,&nbsp;Mohammad Rizwan Mohammad Anwar Shaikh,&nbsp;Kirti Kadian,&nbsp;Narendra Agrawal,&nbsp;Vishvdeep Khushoo,&nbsp;Pallavi Mehta,&nbsp;Rayaz Ahmed,&nbsp;Dinesh Bhurani","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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. AML 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.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"290-302"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303019/pdf/ajbr0011-0290.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254622","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A new FLT3 inhibitor with two cases: the gilteritinib experience.","authors":"Istemi Serin,&nbsp;Mehmet Hilmi Dogu,&nbsp;Gulben Erdem Huq,&nbsp;Osman Yokus","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Introduction: </strong>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 phenotype. The bone marrow biopsy aspiration performed on the 14^th 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 28^th 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 14^th 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 28^th day, control bone marrow aspiration was evaluated as remission.</p><p><strong>Discussion and conclusion: </strong>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.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"271-278"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303015/pdf/ajbr0011-0271.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incidence of lupus anticoagulant in hospitalized covid-19 patients.","authors":"Tarek Owaidah, Mahasen Saleh, Amelita M Aguilos, Abdulllah Al Amri, Khalid Maghrabi, Mustafa Owaidah, Khawar Siddiqui, Khalid Alsaleh, Randa Alnounou","doi":"","DOIUrl":"","url":null,"abstract":"<p><strong>Background: </strong>Procoagulant profile of 2019-nCoV/SARS-CoV-2 has been well documented over the last year. Perturbance in coagulating factors has also been reported in Covid-19 patients, including increased d-dimers and reports of lupus anticoagulant (LA).</p><p><strong>Methods: </strong>The current study aimed to identify the incidence of positivity of lupus anticoagulant in Covid-19 patients and analyze the association between LA and D-dimer in predicting thrombosis and mortality in one-hundred and five hospitalized adult (age >14 years) patients and forty-three hospitalized pediatric (age <14 years) patients with a confirmed diagnosis of Covid-19 between June 2020 and September 2020.</p><p><strong>Results: </strong>Twenty-one (20%) adult patients were tested positive for PTT LA, of which nine (8.6%) turned out to be confirmed positive for LA through StaClot and DRVVT Ratio tests. Six (14%) pediatric patients were positive for PTT LA, and only one (2.3%) had positive StaClot. Median D-dimer at admission was positively correlated with age and CRP among adult patients and was significantly higher in expired cases (P=0.001). No association between any of the coagulation tests and thrombosis or mortality was observed in the pediatric cohort.</p><p><strong>Conclusion: </strong>We report an increased incidence of LA in Covid-19 patients, yet we didn't find any association between thrombotic events or mortality, probably due to the small sample size.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"317-324"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303017/pdf/ajbr0011-0317.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254624","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Evaluation of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab in Turkey: a multicenter retrospective analysis.","authors":"Fatma Keklik Karadag,&nbsp;Mustafa Nuri Yenerel,&nbsp;Mehmet Yılmaz,&nbsp;Hava Uskudar,&nbsp;Vildan Ozkocaman,&nbsp;Tülin Firatli Tuglular,&nbsp;Fuat Erdem,&nbsp;Ali Unal,&nbsp;Orhan Ayyildiz,&nbsp;Gülsüm Ozet,&nbsp;Melda Comert,&nbsp;Emin Kaya,&nbsp;Mesut Ayer,&nbsp;Ozan Salim,&nbsp;Birol Guvenc,&nbsp;Hakan Ozdogu,&nbsp;Özgur Mehtap,&nbsp;Mehmet Sonmez,&nbsp;Nil Guler,&nbsp;Sibel Hacioglu,&nbsp;İsmet Aydogdu,&nbsp;Ozlen Bektas,&nbsp;Selami Kocak Toprak,&nbsp;Lale Kaynar,&nbsp;Munci Yagci,&nbsp;Salih Aksu,&nbsp;Anil Tombak,&nbsp;Volkan Karakus,&nbsp;İrfan Yavasoglu,&nbsp;Birgul Onec,&nbsp;Mehmet Ali Ozcan,&nbsp;Levent Undar,&nbsp;Rıdvan Ali,&nbsp;Osman Ilhan,&nbsp;Guray Saydam,&nbsp;Fahri Sahin","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>Paroxysmal nocturnal hemoglobinuria (PNH) is a rare X-linked genetic disorder. On the contrary to its name, it is a multisystemic disease and various symptoms other than hemoglobinuria could be occurred. It could be life threatening especially because of thromboembolic events. In the last decade, a terminal complement inhibition with eculizumab approved with promising results for PNH patients. We conducted this study to evaluate the long term experience of eculizumab therapy from Turkey for the first time. Our cohort included 138 patients with PNH treated with eculizumab between January 2008 and December 2018 at 28 centers in Turkey. Laboratory and clinical findings at the time of diagnosis and after eculizumab therapy were recorded retrospectively. The median age was 39 (range 18-84) years and median granulocyte PNH clone size was 74% (range 3.06-99.84%) at the time of diagnosis. PNH with bone marrow failure syndrome was detected in 49 patients and the rest of 89 patients had classical PNH. Overall 45 patients (32.6%) had a history of any prior thrombotic event before eculizumab therapy and only 2 thrombotic events were reported during the study period. Most common symptoms are fatigue (75.3%), hemoglobinuria (18.1%), abdominal pain (15.2%) and dysphagia (7.9%). Although PNH is commonly related with coombs negativity, we detected coombs positivity in 2.17% of patients. Seven months after the therapy, increased hemoglobin level was seen and remarkably improvement of lactate dehydrogenase level during the treatment was occurred. In addition to previous studies, our real life data support that eculizumab is well tolerated with no serious adverse events and improves the PNH related findings.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"279-285"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303018/pdf/ajbr0011-0279.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254620","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Hyperhomocysteinemia-related lung disease and hemolytic anemia with bone marrow features masquerading as myelodysplasia.","authors":"Masayoshi Yamanishi,&nbsp;Atsushi Tamura,&nbsp;Takashi Miyoshi,&nbsp;Shinsaku Imashuku","doi":"","DOIUrl":"","url":null,"abstract":"<p><p>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 <i>MTHFR</i> gene associated with chronic alcoholism-induced folate deficiency. Pulmonary lesions showed ground-glass opacity and there was pleural effusion. The patient was managed successfully 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.</p>","PeriodicalId":7479,"journal":{"name":"American journal of blood research","volume":"11 3","pages":"266-270"},"PeriodicalIF":0.0,"publicationDate":"2021-06-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8303007/pdf/ajbr0011-0266.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"39254658","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}