Hematological Pathologies Research Articles

Epidemiology of invasive fungal disease by filamentous fungi in the period 2005 to 2015, in a university hospital in Santiago, Chile

Invasive fungal disease (IFD) due to filamentous fungi is increasingly common. To study the epidemiology of EFI in hospitalized adults in our center. Retrospective study of adult patients of a university hospital in Santiago, Chile, with EFI due to filamentous fungi between January 2005 and December 2015. 125 episodes were identified, being 48% proven, 40% probable and 11% possible according to EORTC/MSG criteria, overall incidence was 0.47/1,000 admissions, 57% male patients and age 50 ± 16 years. 66.4% had hematological pathology, 11.2% solid organ transplant, 11.2% rheumatology diseases, 11.2% other conditions. The risk factors were neutropenia 44%, corticosteroid therapy 21%, immunosuppressants 13%. The most frequent mould identified were Aspergillus spp (53.6%), Mucorales (16%), Fusarium spp (8.8%), Alternaria spp (5.6%) and other filamentous (3.2%). All received antifungals, 82% monotherapy, 18% combined therapy, there was surgical defocation in 90% of mucormycosis. The overall mortality was 42%. When comparing 2005-2009 vs 2010-2015, there was a significant increase in incidence and a tendency to lower mortality in the second period. Over a period of 10 years, we observed an increase in the incidence of EFI by filamentous, aspergillosis was the most frequent etiology and the overall mortality was 42%.

Raynaud’s phenomenon—an update on diagnosis, classification and management

Raynaud's phenomenon (RP) is used to describe a symptom complex caused by digital vascular compromise. RP is a clinical diagnosis. The typically episodic nature of RP has resulted in a reliance upon patient self-report for diagnosis. The term 'primary RP' is generally applied when no underlying pathology can be demonstrated. Whilst 'primary RP' is currently considered a distinct disorder, there is evidence that the term may comprise several entities that include a functional vasospastic disorder, a physiologically appropriate thermoregulatory response, subclinical atherosclerosis and 'cold intolerance'. Optimal management may differ depending on cause. The term 'secondary RP' encompasses a broad range of rheumatological, haematological, endocrinological and vascular pathology. RP can range from relatively benign but intrusive vasospasm, to the progressive obliterative microangiopathy of systemic sclerosis (SSc), in which severe digital ischaemia can threaten tissue viability. SSc has formed the focus of much of the research into RP but, consistent with most medical symptom complexes, the aetiopathogenesis of RP varies greatly dependent on cause. Vasospasm within the digital macro- and microvasculature occurs in SSc, but digital ischaemia is further compounded by a progressive obliterative microangiopathy. Recent work exploring the patient experience of SSc-RP is challenging the 'episodic' paradigm of 'Raynaud's', with important implications for clinical trials utilising diary-based patient-reported outcome instruments for assessing Raynaud's symptoms. This review shall examine the causes, pathogenesis, clinical features, classification and management of RP. A practical approach to the evaluation and management of RP is outlined, highlighting important knowledge gaps and unmet research needs where applicable. Key Points • Raynaud's phenomenon is a symptom complex related to digital vascular compromise secondary to broad-ranging pathology. • Raynaud's phenomenon, as currently classified, likely encompasses a number of aetiopathogenic processes. • Raynaud's phenomenon causes significant disease-related morbidity in autoimmune rheumatic diseases such as systemic sclerosis.

The impact of tick-borne pathogen infection in Indian bovines is determined by host type but not the genotype of Theileria annulata

Tick-borne pathogens (TBP) are a major source of production loss and a welfare concern in livestock across the globe. Consequently, there is a trade-off between keeping animals that are tolerant to TBP infection, but are less productive than more susceptible breeds. Theileria annulata is a major TBP of bovines, with different host types (i.e. exotic and native cattle breeds, and buffalo) displaying demonstrable differences in clinical susceptibility to infection. However, the extent to which these differences are driven by genetic/physiological differences between hosts, or by different parasite populations/genotypes preferentially establishing infection in different host breeds and species is unclear. In this study, three different bovine host types in India were blood sampled to test for the presence of various TBP, including Theileria annulata, to determine whether native cattle (Bos indicus breeds), crossbreed cattle (Bos taurus x Bos indicus breeds) or water buffalo (Bubalus bubalis) differ in the physiological consequences of infection. Population genetic analyses of T. annulata isolated from the three different host types was also performed, using a panel of mini- and micro-satellite markers, to test for sub-structuring of the parasite population among host types. We discovered that compared to other host types, “carrier” crossbreed cattle showed a higher level of haematological pathology when infected with T. annulata. Despite this finding, we found no evidence for differences in the genotypes of T. annulata infecting different host types, although buffalo appeared to harbour fewer mixed parasite genotype infections, indicating they are not the major reservoir of parasite diversity. The apparent tolerance/resistance of native breed cattle and buffalo to the impacts of T. annulata infection is thus most likely to be driven by host genotype, rather than differences in the parasite population. Our results suggest that an improved understanding of the genetic factors that underpin disease resistance could help to ameliorate future economic loss due to TBP or tropical theileriosis.

Leucemia mieloide aguda en adulto mayor comórbido. A propósito de un caso

Caso clínico: paciente masculino de 81 años de edad, con cuadro de 24 horas de evolución secundario a caída, caracterizado por somnolencia, desorientación en persona, tiempo y espacio, incontinencia urinaria y dificultad para la deambulación, motivo por el cual se le efectúa una tomografía simple de cráneo encontrándose hematomas subdurales bilaterales, presenta episodios de sangrado recurrente y leucocitosis sostenida , en frotis de sangre periférica se observó 28% de blastos por lo que se realiza una biopsia de medula ósea descubriéndose un cariotipo medular compatible con leucemia mieloide aguda, debido a sus características clínicas y a su mala evolución fue catalogado como paciente paliativo. Conclusiones: la leucemia mieloide aguda es una patología hematológica cuya evolución al no ser detectada genera un alto grado de mortalidad, sobre todo en el adulto mayor comórbido.

PB1981 RESULTS OBTAINED FROM CLINICAL TRIALS PHASE I/II IN HEMATOLOGICAL MALIGNANCIES. CLINICAL EXPERIENCE OF A SINGLE CENTER: HOSPITAL 12 OCTUBRE.

Background:Early phase clinical trials (phases I / II) represent a key element in the process of translation of knowledge, obtained in basic research, towards its application in clinical practice. Phase I studies are designed to evaluate the drug's safety and toxicity at different dose levels and determine drug pharmacokinetics and pharmacodynamics. In contrast, phase II trials focus on clinical effectiveness. In some cases, phase I studies are perceived as a high risk procedure for the achievement of an uncertain benefit.Therefore, it seems appropriate to perform an analysis of the results obtained in early stage trials, in order to achieve a more objective risk/benefit assessment in this context.Aims:The main objective of the study was to analyze the safety and benefit of clinical trials in early stages in hematology. The results of overall survival (OS), progression free survival (PFS) and major toxicities between the phase I and phase II trials were compared.Methods:Data was extracted from medical histories of 262 patients with different hematological pathologies. These patients were admitted within one of the 66 phase I / II clinical trials opened between 2011 and 2018 at the Hospital 12 de Octubre. Data obtained was analyzed in terms of clinical response, toxicity and survival. We excluded 39 cases due to screening failure and 1 case due to withdrawal of the informed consent.Results:Considering the whole sample of patients, both in phase I (n = 77) and in phase II (n = 145), an overall response rate was verified (including complete and partial responses) in 66% of the patients. In 24% the disease remained stable and in 10% of cases it was refractory. The discontinuation rate of the studies in the whole series was 35.5%, mainly due to progression of the disease.Overall survival (OS) was 34.6 months, while the progression‐free survival (PFS) was 31.6 months. There were significant differences in PFS between the group of patients in phase I (22.5 months) and phase II (35.7 months) (p = 0.004), but there were no differences in OS (p = 0.405). 33,7% and 32,4% of patients enrolled in phase I and phase II studies died, respectively. The main cause of death was progression.Up to 39% of the patients included had some type of grade 3 or 4 toxicity, being hematological toxicity the most frequent(up to one third of the patients). In this regard, there were no significant differences between phase I and phase II trials, with 32.5% and 26.2% grade 3 or 4 toxicity, respectively.Summary/Conclusion:When comparing phase I and phase II trials, we found a higher PFS in phase II studies. This difference is probably due to the fact that part of the patients in phase I trials have received suboptimal doses of treatment, as the main objective of these studies is to warrant safety. However, when analyzing safety data, the differences between both groups in terms of grade 3 or higher toxicity are minimal and not statistically significant. In addition, there were no differences of OS between phase I and II trials.imageThere are few reports in this regard that can be used as a reference. However, the results obtained in our center offer a hopeful outlook in the future of therapeutics in Hematology, confirming the existence of a significant clinical benefit in patients included in early phase clinical trials.

PB2266 HOW TO IMPROVE HEMATOLOGIC REFERRAL? ANALYSIS AND RECOMMENDATIONS

Background:Incidental laboratorial abnormalities detected in asymptomatic persons are increasingly becoming a frequent motive for referral to a haematological consultation, overloading outpatient units that are run by small teams. These referrals, along with the subsequent acceptance by the triage manager and further haematological investigation by a haematologist, originate from the fear of missing incipient severe haemopathies.Aims:To evaluate the adequacy and the result of these referrals to ascertain the hypothesis that a large proportion of the cases have no indication for specialized haematological care.Methods:Retrospective analysis of patients referred to the Haematology consult of a central hospital over a 2‐year period (Jan/16‐Dec/17), excluding the ones who already had a known diagnosis. We evaluated the reason for referral, the final diagnosis and at a second stage we tried to quantify the referrals which could be managed in primary care, and their origin.Results:Out of 1162 evaluable patients (236 still in study), the majority (65%) came from Primary Health Care. The motive, in 85% of the cases, consisted in blood count abnormalities (59% cytopenias). In 13% of the cases, the initial abnormality no longer existed upon arrival. Finding an underlying pathology was possible in only 48% of the cases. From these, 14% could have been managed in Primary Health Care (e.g. iron deficiency; vitamin deficits) and 86% were haematologically relevant. As for the referrals which could have been avoided (no underlying haematological pathology or unconfirmed motive) the most frequent causes were cytopenias (anaemia 33%, leukopenia 15%, thrombocytopenia 12%) and they originated from Primary Health Care (72%).Summary/Conclusion:In this sample, we confirmed that a high proportion of referrals are manageable in Primary Health Care, particularly cytopenias, part of which are no longer present at the first haematological evaluation. The probability of a haematological investigation resulting in a clinically relevant diagnosis is very low, seemingly not justifying the consult time, the costs for the system or the personal costs for the patient. The elaboration of specifically designed recommendations for Primary Health Care and the implementation of interaction/discussion networks of pre‐triage cases are indispensable measures to optimize the haematological referral process.

Quality and determinants of anticoagulation therapy by AVK in elderly subjects: study of a continuous series of 155 patients hospitalized in geriatric medicine

AVK could be prescribed in elderly patients over 75 years for the prevention of thromboembolic complications of atrial fibrillation (AF). The objective of this study was to study the quality of the anticoagulation balance by AVK and its determinants. Inclusion of all patients ≥ 75 years of age treated with AVK for an AF hospitalized in the acute geriatric department of the University hospital of Dijon. The balance of the AVK treatment was determined by the input INR and the calculation of the TTR. The last four INRs prior to admission were retrospectively collected for its calculation. Each patient had a standardized geriatric evaluation (EGS): lifestyle, MMSE, nutritional status (albumin), polypathology (Charlson), level of dependence (ADL-IADL). Bleeding complication were collected. 155 patients aged over 75 years (87±5.6 years, 88 women and 67 men) were included. The mean TTR was 55.4±31.2%. Only 46% of patients had a correct TTR (≥ 75%). The balance was significantly worse in women than in men (49.3±29.5 vs 60.1±31.8%; p=0.0326), and in case of haematological pathology (41.7±27.1 vs 57.2± 9.8; p=0.047) but better with high BMI (r=0.416, p=0.001). No EGS parameters were associated with the quality of anticoagulation. The control of AVK therapy is insufficient in geriatric elderly subjects. No modifiable explicative geriatric factor has been identified. If AVK remains a therapeutic option, direct oral anticoaulants should be considered as the first choice.

Blockage of autophagic flux is associated with lymphocytosis and higher percentage of tumoral cells in chronic lymphocytic leukemia of B cells.

Autophagy has lately emerged as an important biological process with implications in several hematological pathologies. Recently, a growing body of evidence supports a putative role of autophagy in chronic lymphocytic leukemia; however, no definitive clue has been established so far. To elucidate this issue, we have developed a pilot study to measure autophagic flux in peripheral blood mononuclear cells from chronic lymphocytic leukemia patients, and explored its correlation with classical clinical/analytical parameters. Thirty-three chronic lymphocytic leukemia patients participated in the study. Autophagic flux in peripheral blood mononuclear cells was determined by western blot measuring the levels of the proteins p62 and lipidated LC3. Moreover, p62 mRNA levels were analyzed by RT-qPCR. Lymphocytosis and the percentage of tumoral lymphocytes in chronic lymphocytic leukemia patients statistically correlate with a blocked autophagic flux. Alterations in autophagic flux could play an important role in the physiopathology of chronic lymphocytic leukemia.

CARDIOTOXICITY AND METHODS OF ITS DIAGNOSIS IN HEMATOLOGY PATIENTS (REVIEW)

The article is devoted to the modern concept of cardiotoxicity, induced chemotherapy in patients with hematological pathology and methods of its diagnosis. The study of cardiotoxicity is one of the current trends in oncology and oncohematology, as the side effects of chemotherapy drugs on the cardiovascular system affect the quality and life expectancy of patients, regardless of the prognosis associated with oncohematological disease. While modern strategies for the treatment of oncohematological diseases make it possible to achieve stable remissions and increase the duration and quality of life of patients, such successes can be offset by an increase in mortality in this group of patients due to the development of side effects of chemotherapy in relation to the cardiovascular system. In the review problems and prospects of cardiotoxicity diagnosis in patients with hematological diseases on the background of chemotherapy are presented at modern level of medical science development. The use of such methods as three-dimensional echocardiography, echocardiography with determination of global longitudinal myocardial deformation, stress echocardiography with determination of contractile reserve, determination of troponin and natriuretic peptide levels for diagnosis of cardiotoxicity makes it possible to detect manifestations of cardiotoxicity at an earlier stage and thus will reduce mortality in patients with oncohematological profile receiving chemotherapy for non-hematological reasons.

Досвід впровадження прогресивних форм навчання у вищу медичну освіту

The significant demand of changes in the medical education system bases on two factors: a prominent increase of the medical information`s volume and a rapid scientific progress in the understanding of medical facts, phenomena, etc. Therefore, the informatization of continuous medical education should become the principal platform of these changes, especially in the context of the current reform of the medical sphere. In addition to modular higher education, another very effective remote learning technology through the Internet is widely provided. Technologies such as electronic universities, academies, schools, libraries, training portals, educational electronic resources, virtual round tables, seminars, conferences, symposiums, lectures, practical classes, laboratory works, work-shops have become popular. Multimedia and interactive learning tools, in addition to traditional forms, can enhance the quality of education by using of modern computer technologies (educational CDs, E-books, atlases, presentations, films, MCQs, etc.) in the educational process. The implementation of modern views on the development of medical science and practice is the introduction of adjustments in the training and professional development of medical workers with the approach of their education to international standards. One of the effective forms of distribution of personal pedagogical experience is such a modern form of methodical work as a workshop. The opportunity to demonstrate their workshops and attend such classes of colleagues is one of the types of informational education for modern doctors. The effectiveness of the workshop on the topic "Differential diagnosis of the anemic syndrome" with students and young scientists in the framework of the International Medical and Pharmaceutical Congress of Students and Young Scientists was analyzed in present work. The workshop was held in an integrated style, in the form of the combination of lecture and practical class. The scientific and practical event was evaluated positively according to all the criteria; the participants suggested the directions for further improvement of differential diagnostics of hematological pathology in the internal diseases.

Kahler's Disease Revealed by a Fracture of the Humeral Diaphysis

Kahler's disease or multiple myeloma is a malignant hematological pathology, characterized by the malignant proliferation of plasma cells. Reportin g a case of a humeral shaft fracture in a non - traumatic context that revealed Kahler's disease during paraclinical investigations. Management involves histological confirmation of the pathology, osteosynthesis of the fracture by static centromedullary nail ing, with medical treatment made of biphosphonate combined with a chemotherapy protocol targeting the disease. The management of a pathological fracture is a diagnostic emergency, but never an operating emergency. A diagnosis of certainty must be made befo re any intervention.

Cerebrovascular Disorders Associated with Ph-Negative Myeloproliferative Diseases

Background. Cerebrovascular disorders continue to be among the most common and socially significant worldwide. Among multiple reasons for circulatory disturbances special importance is attached to hemorheology and hemostasis disorders occurring also in patients with Ph-negative myeloproliferative diseases (MPD). Aim. To investigate typical characteristics of the course of cerebrovascular disorders in patients with Ph-negative MPD. Materials & Methods. The trial included 169 adult patients with neurological diseases. Among them the main group consisted of 104 patients aged 34 to 55 years (median 48.5 years) with Ph-negative MPD diagnosed at the National Research Center for Hematology. The control group consisted of 65 patients aged 51 to 58 years (median 55.5 years) with cerebrovascular diseases without concomitant hematological pathology. Results. The incidence of acute ischemic strokes was 26.2 % in polycythemia vera (PV), 20.5 % in essential thrombocythe-mia (ET), and 8.7 % in primary myelofibrosis (PMF). Conclusion. An acute ischemic stroke with a concurrent thrombotic occlusion of one of the major head arteries is a criterion for ruling out Ph-negative MPD. To identify cerebral lesions in patients with Ph-negative MPD (PV, ET, PMF) MRI of the brain is recommended.

Current Issues in Surgical, Hematologic, and Cytologic Pathology.

Current Issues in Surgical, Hematologic, and Cytologic Pathology.

Totally Laparoscopic Partial Splenectomy in a Child with Hamartoma

Abstract Background: To date laparoscopic surgery plays a key role in the surgical treatment of not only splenic hematologic pathologies but also of solid pathologies such as benign and malignant t...

Transfusion practices in geriatric short stay unit before and after the French national health authority guidelines of 2014.

Retrospective descriptive study in two geriatric short stay units, including patients aged 80 years-old or over, transfused in 2012 and 2015. 103 patients were included. More than 30% patients had a chronic heart failure, and there was no significant difference on general characteristics between the groups in the two years. Compared to 2012, the transfused population in 2015 was more fragile with a higher Charlson comorbidity index (p=0.005). The main symptoms of anemia bad tolerance were cardiovascular symptoms. The average pre-transfusion hemoglobin concentration was 7.9 g/dL in 2015, 8 g/dL in 2012 (p=0.63). By 2015, 72.3% transfusions respected the hemoglobin thresholds recommended in guidelines, compared to 50% in 2012 (p=0.023). Transfusion thresholds in our study were lower than those recommended; 13 adverse reactions were identified, 12 of them were heart failure. There was no significant difference in transfusion benefit between the two years. This study helped describe profile of elderly transfused patients, their geriatric characteristics and the transfusion data, without showing any changes in transfusion practices following the guidelines, despite a more fragile population in 2015. It seems difficult, because of the diversity in the geriatric population, to have a single threshold of hemoglobin recommended, only non-specific symptoms of intolerance and to consider only the cardiovascular comorbidities to decide whether or not to provide a transfusion.

Refractory and Relapse Predictive Biomarkers in Hodgkin Lymphoma and Potential Directed-Therapy Targets

Despite 90% stage I Hodgkin Lymphoma (HL) patients can respond to current systemic therapy, this drops to 60%, when diagnosed in advanced stages. Nevertheless and independently of the lymphoma stage, the real challenge when treating these patients, is the refractory and relapsed disease. There is no molecular biomarker to identify patients that would be non-responsive to conventional treatment or that would relapse. Furthermore, rescue chemotherapy schemes for refractory and relapsed patients, associate with acute and late toxicity high risk. This highlights the need to deeper understand the HL molecular biology and the screening for predictive biomarkers as well as potential therapeutic directed-targets.We have previously reported that HL relies on the alternative NFkB pathway, mediated by RelB and NIK, to survive. Depletion of either RelB or NIK by shRNAs or pharmacological NIK inhibitors induce HL cell death. ChIP-Seq analysis uncovered RelB target genes showing RelB bound to BCL2 promoter. A significant downregulation of BCL2 mRNA and protein levels, following RelB or NIK knockdown was observed, indicating that RelB regulates BCL2 expression in human HL cell lines.Our molecular studies suggested that NFkB alternative pathway constitutive signaling could at least partially explain the non-responding HL cases. We aimed to analyze whether mediators of this pathway could be useful as predictive biomarkers and would represent potential targetable factors in both refractory and relapsed patients.We analyzed NIK and BCL2 citoplasm expression in Hodgkin Reed-Sternberg cells (HRS) in lymphatic node biopsies of 96 patients by inmunohistochemistry [50 female Md age and (range) 59 (6-82), 46 male 42 (9-78)]. The univariate analysis showed no correlation between NIK or BCL2 expression and the prognosis clinical and pathological parameters, neither the molecular markers routinely assayed. A positive correlation was found between NIK and BCL2 expression (p=0.01). NIK and BCL2 correlated with lack of response to conventional therapy and both early and late disease progression.The analysis of survival, applying the Kaplan-Meier Curves, showed > 60% NIK positive HRS cells associated with shorter Disease Free Survival (DFS) [Log Rank Test (p=0.000)] and predicted overall survival (OS) as well [Log Rank Test (p=0.01)]. Furthermore, > 60% BCL2 positive HRS cells correlated with poor prognosis in terms of OS [Log Rank Test (p=0.002)]. The statistical significance was maintained in the multivariate analysis [Cox Regression and Logistic Regression (p=0.001)].NIK and BCL2 performed successfully as useful predictive markers to identify refractory or risk of relapse HL patients at diagnosis. They represent attractive molecules to further analyse their potential as directed-therapy targets, since we have already reported that HL is sensitive to NIK inhibitors and BCL2 blockers have already been approved for clinical use in other hematological pathologies. DisclosuresZerga:Bristol Myers Squibb: Other: Conference fees; Janssen: Other: Conference fees; Roche: Other: Conference fees; Takeda: Other: Conference fees.

Severe Aplastic Anemia (SAA) in Colombia: Characteristics and Treatment in 6 Centers

IntroductionAcquired severe aplastic anemia (SAA) is a low frequency hematologic disease associated with significant morbidity and mortality. The destruction by immunological mechanisms of hematopoietic progenitor cells appears to play a fundamental role in the pathophysiology of the disease, therefore, immunosuppressive treatment (IST) is one of the pillars of management. In Colombia and Latin-America there is lack of information about the characteristics of patients with SAA and the effectiveness of IST and other treatments. The main objective of this study was to assess the outcomes of a group of patients treated in different centers in Colombia during the last five years.Materials and methodsThis is a retrospective observational study, carried out within 6 institutions of 3 Colombian cities (Bogota, Medellín, Bucaramanga) of patients treated from 2013 to 2018. An anonymized database was constructed. All patients diagnosed with SAA and treated with IST as first line of therapy were included.ResultsWe analyzed the data of 37 patients; 22 of them were women. The average age was 37 years old (with a range of 7-68 years). The median time since the onset of the symptoms to the time of diagnosis was 88 days (with a range of 7-655 days). Clastogenic effect of diepoxybutane (DEB) tests were available only in 9 patients (7 of which were positive); Paroxysmal Nocturnal Hemoglobinuria (PNH) clone was detected in 9 of the 27 tested, all of which were under 5%. Mean leukocytes, hemoglobin concentration, and platelets at the moment of diagnosis were 3.090, 8.5 and 22.000. Bacterial infection was the most important clinical manifestation at diagnosis (in 16 of the 37 patients); 19 out of 37 patients had more than 5 red blood cell transfusions before the first treatment. As first line therapy, 25 patients received IST (most by antithymocyte globulin (ATG) of which 10 were treated with rabbit ATG, 12 with horse ATG and 3 combined with Eltrombopag); 4 patients had cyclosporin and prednisone, 2 had allogeneic transplant and 6 received other therapies. Those treated with IST patients, 8 had a complete response (CR) (32%), 6 had partial response (PR) (24%), 8 had no response (32%). 5 received a second course of IST and 3 had CR. 3 patients were lost in follow up. At a mean follow up of 18.7 months (1.7-65), 44% were alive in CR, 26% in PR, 9% were in relapse and 21% died. Causes of death were SAA related infection in 5 patients, transplant related in 1 and non-disease related in 1.ConclusionsThis is the first multicenter report of SAA treatment in Colombia, it includes children and adults, resulting in a very heterogeneous population. This preliminary information shows that there is a significant delay in the diagnosis and initiation of treatment in patients, most patients have more than 5 transfusions before treatment. This could, in part, explain the apparent low response rates to IST in comparison to that of what have been reported in literature. A larger registry of hematologic pathologies is required so we can confirm these findings, start proposing strategies applicable in our country to achieve better outcomes in these patients and planning clinical trials to answer questions relevant to our population. DisclosuresNo relevant conflicts of interest to declare.

Case 3: Premature Infant with Anemia and High-Output Cardiac Failure

A female singleton is delivered at 30 6/7 weeks’ gestational age via cesarean for preterm labor and breech presentation. The mother is a 37-year-old grand multipara in her 10th pregnancy. The pregnancy is notable for severe fetal anemia, high-output cardiac failure with normal cardiac anatomy, polyhydramnios, and a placental chorioangioma diagnosed at 26 weeks of gestation. Evaluation for congenital infections is negative. At 25 and 28 weeks’ gestation, the fetus had received in utero red blood cell transfusions for severe fetal anemia. At birth, the infant has respiratory depression requiring intubation and ventilation. Apgar scores are 7, 6, and 8 at 1, 5, and 10 minutes, respectively, and her birthweight is 1,416 g. The neonate is noted to have a protuberant abdomen with significant hepatosplenomegaly, diffuse subcutaneous edema, petechiae, and ecchymoses. The infant requires significant ventilator support for the first few weeks after birth. Chest radiography shows no evidence of pleural or pericardial effusion. Echocardiography shows normal cardiac segmental anatomy with significant biventricular hypertrophy, though blood pressure is maintained. Significant anemia, thrombocytopenia, and coagulopathy are noted over the first days after birth, for which the newborn requires multiple blood products. Profound jaundice necessitates multiple double-volume exchange transfusions. Thrombocytopenia and coagulopathy gradually improve over the first week after birth. However, the infant develops significant cholestasis, reaching a peak direct bilirubin of 12.5 mg/dL (213.8 μmol/L) at 2 weeks of age. The unique constellation of findings in this case, anemia, thrombocytopenia, high-output failure, and cholestasis, presented a broad differential diagnosis. The differential included congenital heart disease and vascular malformations, infectious etiologies, metabolic and genetic disorders, and various renal, hepatic, gastrointestinal, and hematologic pathologies. Sepsis evaluation was performed on admission and …

The sonographic measurement of the spleen: A clinical audit

AbstractIntroductionThe spleen is an intra‐abdominal organ that increases in size (splenomegaly) due to a variety of hepatic and haematological pathologies. The measurement of its size is crucial for patient treatment and monitoring. This project sought to determine a standard technique for the sonographic measurement of the spleen through a retrospective clinical audit.MethodsSerial splenic measurements from 250 patients were retrospectively assessed for caliper placement and visualisation/technique. The measurements were graded according to caliper placement and splenic visualisation on ultrasound. Patients included had serial abdominal ultrasounds over the 3 years prior to audit.ResultsA literature review was performed to establish a benchmark for splenic measurement. This uncovered a wide variation in reported normal splenic length measurement values. The key point from the data collected along with the literature review is that a normal measurement of the spleen is 10–12 cm. Consistent results were found regarding the method sonographers used to measure the spleen.DiscussionTo standardise measurement method, the spleen should be imaged with the patient in the right posterior oblique position in the optimal respiratory phase for individual patient habitus.

Flow Cytometry With Inflorescence-An Accessible Method in the Study of Qualitative and Quantitative Modifications of Blood Platelets in Immune Thrombocytopenic Purpura

The immune thrombocytopenic purpura (ITP) is a polymorphic and hematologic pathology, in terms of both clinical manifestations and etiopathogenic mechanisms, which bring on this disease. Establishing the type of thrombocytopenia, which can either be primary or secondary, peripheral or central, is essential for a further proper therapeutic conduct. The current means of diagnosis include a large variety of hematologic, immunologic and biochemical explorations, from the traditional ones to the latest methods, either genetical or molecular. The purpose of this paper is to emphasize the importance of a classical method of diagnosis, used in the primary triage of patients suffering from thrombocytopenia, highly valuable in the diagnosis process: the analysis of the mean platelet volume (MPV) and of the platelet distribution width (PDW), parameters revealed by the CBC (complete blood count). Evaluating the size of the platelets is a useful tool in the differential diagnosis between immune thrombocytopenic purpura and hereditary macrothrombocytopenia. The platelet distribution width and the mean platelet volume are read simultaneously, in order to differentiate the immune peripheral thrombocytopenia from the central one. The classical, viable and accessible method facilitates the measurement of MPV and PDW with the automatic analyzer, which functions according to the principle of flow cytometry with inflorescence, by using a LASER semiconductor and hydrodynamic focusing.