Ankle hemophilic arthropathy: literature review

xlomafota13 shared this article with you from Inoreader Ankle hemophilic arthropathy: literature review Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):206-216. eCollection 2021. ABSTRACT Hemophilia is a bleeding disorder characterized by the deficiency of a coagulation factors. The hemarthrosis is the most common and earliest manifestation. Repeated hemarthrosis over time causes the development of hemophilic arthropathy. Among most involved joints, the ankle is the one where much uncertainty remains about the best course of action in managing the various degrees of hemophilia manifestations. These manifestations range from simple acute swelling and pain to devastating deformity. The purpose of our review is to draw a comprehensive picture of ankle hemophilic arthropathy epidemiology, pathophysiology, clinical symptoms and signs, radiological features and all the treatments available at present days. This review confirms that the first line of treatment considered should be the replacement therapy of the coagulation deficient factors that, preventing hemarthrosis, stops the development and progression of ankle's joint damage. The treatments proposed in literature for advanced stage of arthropathy are many and vary according to the severity of the case. They range from conservative ones such as physiotherapy, orthosis, intra-articular injections, laser therapy, external beam radiation therapy, radio-synovectomy and oral drug to invasive surgical treatment such as ankle arthrodesis and total ankle replacement. Whatever is the chosen treatment, according to the arthropathy severity we believe that it must be carried out in reference centers for foot and ankle surgery assisted by expert hematologists. PMID:34322283 | PMC:PMC8303020 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Safety and efficacy of azathioprine in immune thrombocytopenia

xlomafota13 shared this article with you from Inoreader Safety and efficacy of azathioprine in immune thrombocytopenia Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):217-226. eCollection 2021. ABSTRACT BACKGROUND: Immune thrombocytopenia (ITP) is a benign hematological disorder characterized by low platelet counts in peripheral blood and spectrum of various bleeding manifestations. Azathioprine is one of the effective, readily available, and affordable immunosupressants available for ITP management in developing countries. We aimed to study the efficacy and long-term safety profile of our patients with ITP who were treated with azathioprine. METHOD: This was a retrospective, single-center study conducted at a tertiary care hospital in Northern India. The patients who had received at least one line of therapy before receiving azathioprine were included in this study. All patients received oral azathioprine at a dose of 1 mg/kg/day (50 mg or 100 mg tablet formulations were used), which was increased up to 2 mg/kg/day depending upon the response and adverse effe cts. RESULT: Sixty-three patients were analyzed. Their median age was 28 years (range 15-68); 29/63 patients (46.03%) were females. The median duration from diagnosis to azathioprine initiation was 539 days (323 days-980.5 days). The patients included in the study had received a median of 3 (range 1-6) prior lines of therapies; 38/63 patients (60.32%) had received ≥3 prior therapies. Six patients (9.5%) had relapsed after splenectomy, and 16 patients (25.4%) had relapsed after receiving rituximab. The mean baseline platelet count was 10000/μL. The median time to response was 95 days (90 days-not reached) and the cumulative overall response rate (complete and partial response) at day 90 was 38.1%. Only one patient achieved complete response with azathioprine in our study. The cumulative rate of relapse at five years was 21.2%. Twenty-six patients stopped azathioprine after achieving some response (CR/PR) with Azathioprine for a median duration of 1067.5 days (range: 236 days -2465 days). They were followed up for a median of 870 days (range: 392 days-1928 days), and twelve of them relapsed. Twenty-six patients (26/63, 41.27%) reported one or more adverse events while on azathioprine. Leucopenia was the most frequent adverse event, followed by anemia and hepatobiliary laboratory abnormalities. Serious adverse events (grade ≥3 CTCAEv4) were noted in three patients (4.7%). One patient succumbed to severe sepsis multiorgan dysfunction while being on treatment. CONCLUSION: We conclude that azathioprine has a good response rate in chronic ITP patients. It is well-tolerated with minimal and manageable side effects. PMID:34322284 | PMC:PMC8303009 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

The non-leukemic T cell large granular lymphocytic leukemia variant with marked splenomegaly and neutropenia in the setting of rheumatoid arthritis - Felty syndrome and hepatosplenic T cell lymphoma mask

xlomafota13 shared this article with you from Inoreader The non-leukemic T cell large granular lymphocytic leukemia variant with marked splenomegaly and neutropenia in the setting of rheumatoid arthritis - Felty syndrome and hepatosplenic T cell lymphoma mask Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):227-237. eCollection 2021. ABSTRACT T cell large granular lymphocytic (T-LGL) leukemia is a rare type of mature T cell neoplasm. The typical features of T-LGL leukemia include an increased number of large granular lymphocytes in the peripheral blood, cytopenia (most commonly neutropenia), and mild-to-moderate splenomegaly. Up to 28% of patients with T-LGL leukemia have rheumatoid arthritis (RA). This study reports ten atypical cases (seven women and three men, median age 60.5 years) of RA-associated T-LGL leukemia presenting with lymphopenia, severe neutropenia, and marked splenomegaly. The weight of the spleens ranged from 892 to 2100 g (median 1100 g). Bone marrow histology and differential counts of bone marrow aspirates revealed no peculiarities in nine of ten cases. The red pulp of the spleen was expanded and showed moderate to strong infiltration by medium-sized slightly pleomorphic lymphocytes i n nine cases and subtle infiltration in one. Although lymphocytic infiltration involved both cords and sinusoids, it was more apparent within the splenic cords. The white pulp was preserved and contained prominent germinal centers in eight patients and was atrophic in two patients. Immunohistochemically, malignant lymphocytes were CD3+, CD43+, and CD4- in all cases and TIA-1+ in nine out of ten. TCRαβ positivity and TCRγδ positivity was observed in six and four cases out of ten, respectively. All ten patients had T cell clonality in the spleen tissue, but in three cases it was absent in both blood and bone marrow. STAT3 mutations in the spleen tissue were detected in three of ten cases. In all eight cases studied, neither isochromosome 7q nor trisomy 8 was detected in the spleen tissue. Cases of RA-associated T-LGL leukemia with low LGL count in the peripheral blood, neutropenia, and marked splenomegaly present a diagnostic challenge and can be misdiagnosed as Felty's syndrome o r hepatosplenic T cell lymphoma. PMID:34322285 | PMC:PMC8303016 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Incidence of lupus anticoagulant in hospitalized covid-19 patients

xlomafota13 shared this article with you from Inoreader Incidence of lupus anticoagulant in hospitalized covid-19 patients Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):317-324. eCollection 2021. ABSTRACT BACKGROUND: 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). METHODS: 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. RESULTS: 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%) pediat ric 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. CONCLUSION: 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. PMID:34322296 | PMC:PMC8303017 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Graves' disease patients with iron deficiency anemia: serologic evidence of co-existent autoimmune gastritis

xlomafota13 shared this article with you from Inoreader Graves' disease patients with iron deficiency anemia: serologic evidence of co-existent autoimmune gastritis Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):238-247. eCollection 2021. ABSTRACT BACKGROUND: 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. METHODS: 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), Helicobacter pylori antibodies (H. pylori ab), mean serum gastrin levels] were examined. RESULTS: 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. CONCLUSIONS: A significant subset of patients presenting with GD may suffer from IDA due to concurrent autoimmune atrophic gastritis. PMID:34322286 | PMC:PMC8303011 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Prevalence of positive factor V Leiden and prothrombin mutations in samples tested for thrombophilia in Saudi Arabia

xlomafota13 shared this article with you from Inoreader Prevalence of positive factor V Leiden and prothrombin mutations in samples tested for thrombophilia in Saudi Arabia Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):255-260. eCollection 2021. ABSTRACT Venous thromboembolism (VTE) is a multifactorial disease that results from the interaction of both inherited and acquired risk factors. The complications of these risk factors often lead to significant morbidity and mortality. There are many inherited thrombophilia risk factors, such as factor V Leiden (FVL) and prothrombin gene mutation (PT). The prevalence of these mutations varies among geographical locations and ethnic groups. OBJECTIVES: This is a retrospective analysis of laboratory data aimed to estimate the laboratory-based frequency of FVL and PT mutations and assess the concordance between the coagulation assay and FVL molecular test. METHODS: The study reviewed the frequency of positive blood samples tested by molecular and functional-based techniques. The demographic and laboratory data of patients tested in molecular and coagulation laborator ies at the Institute for Thrombophilia were reviewed and analyzed. RESULTS: A total of 1524 samples were tested for FVL, 1023 for PT, and 1057 for APCR. Results showed that 90 (5.9%) patients were positive for FVL, 30 (2.93%) for PT mutations, and 95 (8.99%) had low APCR, while 38 (3.69%) patients had low APCR with no FVL mutation. CONCLUSION: This study reports high positive results among patients tested as part of thrombophilia workup or screening for other clinical conditions associated with the increased risk of thrombosis. The limitation of this study was that it had minimal clinical correlation because the data were collected retrospectively from laboratory records. PMID:34322288 | PMC:PMC8303010 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Evaluation of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab in Turkey: a multicenter retrospective analysis

xlomafota13 shared this article with you from Inoreader Evaluation of clinical characteristics of patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab in Turkey: a multicenter retrospective analysis Via American Journal of Blood Research Am J Blood Res. 2021 Jun 15;11(3):279-285. eCollection 2021. ABSTRACT 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. PMID:34322292 | PMC:PMC8303018 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

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.