Clinical Features and Risk Factors Analysis for Hemorrhage in Adults on ECMO

Background: The use of extracorporeal membrane oxygenation (ECMO) to support critically ill patients with cardiorespiratory dysfunction has increased over the last decades. However, hemorrhagic complications occur frequently during ECMO support, and this has a significant impact on morbidity and mortality. Thus, this study aimed to identify the risk factors for hemorrhage in patients receiving ECMO.Methods: Our retrospective study included 60 patients, who were admitted to the Taihe Hospital in Shiyan City, Hubei Province, China from February 2017 to October 2020. About 18 patients developed hemorrhagic complications, and 42 patients did not demonstrate such complications. Data regarding patient demography, laboratory tests, and clinical manifestations prior to ECMO were collected to analyze their clinical features. Univariable and multivariable logistic analyses were used to explore the risk factors for hemorrhage in adults on ECMO. The receiver operating characteristic (ROC) curve was used to evaluate the predictive value of the binary logistic model. The amount of blood transfusions was compared between the two groups, and the activated partial thromboplastin time (APTT), platelet count, and hemoglobin level before the initiation of ECMO.Results: Logistic analysis showed that a longer duration of ECMO support, higher APTT, and lower platelet count prior to ECMO were independent risk factors for hemorrhage in adults on ECMO. In addition, we found that the cannula site was the most common bleeding site. Most bleeding events occurred within the first 3 days of ECMO therapy. After the ECMO initiation, APTT was prolonged while the platelet count and hemoglobin levels were decreased. The amount of blood transfusion was significantly higher in the hemorrhage group than in the non-hemorrhage group.Conclusions: Clinicians should evaluate the risk of hemorrhage based on the coagulation function of patients, underlying disease, and the duration of ECMO support. In the first 3 days during ECMO support, special attention should be given to the cannula site, mucosal, and dermal regions, and digestive tract to detect any signs of hemorrhage. Moreover, increasing the platelet count transfusion threshold and accurately determining the amount of blood transfusion required may prevent bleeding events.

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Clinical Features and Risk Factors Analysis for Hemorrhage in Adults on ECMO

10.22541/au.160624518.82192740/v1 ◽

2020 ◽

Author(s):

Wenwen Hu ◽

Meifang Wang ◽

Wei Chen ◽

Lin Chai ◽

Elaine Lai-Han Leung ◽

...

Keyword(s):

Risk Factors ◽

Clinical Features ◽

Clinical Manifestations ◽

Underlying Disease ◽

Ecmo Support ◽

Bleeding Events ◽

Logistic Analysis ◽

Factors Analysis ◽

Red Blood Cell Count ◽

Risk Of Hemorrhage

Background: The use of extracorporeal membrane oxygenation (ECMO) to support critically ill patients with cardiorespiratory dysfunction is increasing over the last decades. However, hemorrhagic complications remain occurring frequently during ECMO support, which have a significant impact on morbidity and mortality. Methods: A retrospective study was performed on the 60 patients, who were admitted to the Taihe hospital in Shiyan City, Hubei Province from February 2017 to October 2020. All those were rescued with ECMO. Including 18 patients developed hemorrhage complications and 42 patients did not. Demographic, laboratory tests, clinical manifestations prior to ECMO were collected to analysis the clinical features. Univariable and multivariable logistic analysis methods were used in our study to explore the risk factors for hemorrhage in adults on ECMO. Results: There were significant differences between the hemorrhage group and no-hemorrhage group in duration of ECMO support, mode of ECMO, red blood cell count, hemoglobin, platelet count, serum creatinine. Particularly, multivariate logistic analysis showed that the longer duration of ECMO support and the higher activated partial thromboplastin time (APTT) prior to ECMO were independent factors for hemorrhage in adults on ECMO. In addition, we found that the mortality of hemorrhagic patients was higher than no-hemorrhagic patients. Cannula site was the most common bleeding site. Most bleeding events occurred within the first three days of ECMO therapy. Conclusions: Clinicians should evaluate the risk of hemorrhage based on patients’ coagulation function, underlying disease as well as the duration of ECMO support. Especially in the first three days during ECMO support. Attempting to wean from ECMO early whenever feasible is also effective to reduce the occurrence of hemorrhage. Special attention should be given on cannula site, mucosal, dermal and digestive tract to alert hemorrhage.

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Seroprevalence and Associated Risk Factors of Hepatitis B Virus Infection Among Children in Enugu Metropolis

Virology Research and Treatment ◽

10.1177/1178122x18792859 ◽

2018 ◽

Vol 9 ◽

pp. 1178122X1879285 ◽

Cited By ~ 1

Author(s):

Maryann Chinenye Ezeilo ◽

Godwill Azeh Engwa ◽

Romanus Ifeanyi Iroha ◽

Damian Chukwu Odimegwu

Keyword(s):

Risk Factors ◽

Hepatitis B Virus ◽

Blood Transfusion ◽

Hepatitis B ◽

Clinical Manifestations ◽

Hbv Infection ◽

Age Group ◽

History Of Surgery ◽

B Virus ◽

History Of

Background: Though measures are being put in place for the management of Hepatitis B virus (HBV) infection in Nigeria, children remain the most vulnerable to develop chronic hepatitis. Routine screening in children is therefore necessary for effective control. However, the performance of the commonly used immunochromatographic test (ICT) strips has been challenging. Also, identifying the risk factors of transmission in this age group is of importance for the implementation of preventive measures. Hence, the goal of this study was to assess the test performance of the routinely used ICT strip and identify the associated clinical manifestations and risk factors of HBV. Methods: A cross sectional study involving 270 children below six years of age was conducted at ESUTH and Favor Child Pediatrics Hospital in Enugu, Nigeria. The subjects were screened for HBV by ICT and ELISA assays and a structured questionnaire was used to obtain participants data including demographic, socioeconomic, signs and symptoms, risk factors and vaccination. Results: BBased on ELISA, 31 out of 270 children were positive for HBV with an infection rate of 11.5%. ICT kit showed a low sensitivity of 51.6% in diagnosing HBV but was highly specific (100%) and accurate (94.4%). HBV infection was not associated with sex (χ2: 0.209; p = 0.401). The prevalence of HBV infection was similar in all the age group and HBV infection was not associated (χ2: 2.099; p = 0.914) with age group. All the clinical manifestations were not associated ( p > 0.05) with HBV infection. Blood transfusion, shared items, tattoo marks and history of surgery associated significantly ( p < 0.05) with HBV infections having odd ratios of 4.247, 4.224, 3.134 and 3.195 respectively. The vaccination rate was 55.2% (159/270) and only 3 (1.1%) out of 159 vaccinated subjected contracted the infection (OR: 0.068, p < 0.0001). Conclusions: HBV was prevalent (11.5%) in children below six years old in Enugu metropolis. Moreover, the routinely used ICT test was less reliable than ELISA in diagnosis HBV infection. More so, shared items, blood transfusion, tattooing and history of surgery were potential risk factors while vaccination served as a protective factor against the infection.

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Safety of Lumbar Punctures in Adult Oncology Patients with Thrombocytopenia

Blood ◽

10.1182/blood.v126.23.1141.1141 ◽

2015 ◽

Vol 126 (23) ◽

pp. 1141-1141 ◽

Cited By ~ 1

Author(s):

Shuoyan Ning ◽

Brent Kerbel ◽

Jeannie Callum ◽

Yulia Lin

Keyword(s):

Risk Factors ◽

Platelet Count ◽

Platelet Transfusion ◽

Conflicts Of Interest ◽

Tertiary Care ◽

Bleeding Risk ◽

Von Willebrand Disease ◽

Oncology Patients ◽

Platelet Counts ◽

Hemorrhagic Complications

Abstract Introduction: Lumbar puncture (LP) is a frequently performed diagnostic and therapeutic intervention in adult oncology patients. While thrombocytopenia is common in this patient population, the minimum "safe" platelet count required for LPs is unknown. Recent guidelines from the AABB (American Association of Blood Banks) recommend a pre-procedure platelet count of 50 x 109/L. However this recommendation is largely based on expert opinion, and there remains a paucity of studies in the adult oncology literature to address this important question. Methods: We retrospectively reviewed all oncology patients ≥18 years who underwent 1 or more LPs over a 2 year period at a single tertiary care institution to determine 1) the range of platelet counts at which LPs are performed; 2) the rate of traumatic taps; and 3) the rate of hemorrhagic complications. Laboratory, clinical, and transfusion information were extracted through the Laboratory Information System, chart review, and blood bank database, respectively. Thrombocytopenia was defined as a platelet count of < 150 x 109/L. Pre-LP platelet counts were those collected ≤24 hours from, and closest to the time of the LP. The following bleeding risk factors were documented: end stage renal disease; platelet dysfunction; von Willebrand disease; hemophilia. Anticoagulation, anti-platelet, and non-steroidal inflammatory use was also recorded, with accuracy limited by the study's retrospective nature. All patients with coagulopathy were excluded (INR ≥ 1.5, aPTT ≥ 40, fibrinogen ≤ 1.0). Traumatic tap was defined as 500 or more red blood cells per high-power field in the cerebrospinal fluid. A follow up of 1 week after LP was used to capture any hemorrhagic complications. Results: From January 2013 to December 2014, 135 oncology patients underwent 369 LPs; 64 (47.4%) patients were female, and the mean age was 59 years (range 20-87). 119 (88.1%) patients had a primary hematological diagnosis. 113 (30.6%) LPs were performed in thrombocytopenic patients. 28 (7.6%) procedures had a pre-procedure platelet count of ≤ 50 x 109/L, with 18 receiving a single platelet transfusion on the day of the LP. Of these 18 transfusions, only 1 had a post-transfusion platelet count available prior to LP with no improvement in platelet count (33 x 109/L). 15 transfusions had post-LP platelet counts within 24 hours of the transfusion (8 below 50 x 109/L with lowest 14 x 109/L), 1 had post-LP platelet count within 24-48 hours (54 x 109/L) and 1 did not have a post-transfusion platelet count. Traumatic taps occurred in 17 (15.0%) LPs in patient with thrombocytopenia, compared to 26 (11.0%) LPs in patients with a normal platelet count (fisher's exact test P=0.39). There was 1 traumatic tap in a patient with a pre-LP platelet count of ≤ 50 x 109/L; however, this patient received a pre-LP platelet transfusion for a platelet count of 42 x 109/L and had a post-LP platelet count of 66 x 109/L. Presence of bleeding risk factors did not increase the risk of a traumatic tap (present in 48.8% of traumatic taps vs. 88.3% of non-traumatic taps). There were no hemorrhagic complications. Conclusion: Among this cohort of adult oncology patients undergoing diagnostic and therapeutic LPs, there were no hemorrhagic complications. There was no significant increase in traumatic taps in patients with thrombocytopenia or bleeding risk factors. While platelet transfusions were frequently administered for patients with a platelet count of ≤ 50 x 109/L, post-transfusion platelet counts were infrequently assessed prior to the procedure. Our findings question whether a platelet transfusion threshold of 50 x 109/L is necessary for lumbar puncture.Table 1.Platelet Count Pre-LP(x109/L)Number of LPsNumber of Traumatic TapsNumber of Hemorrhagic Complications0-90N/AN/A10-2030021-5070051-1003380101-1495270> 150242270Unknown1400< 50 x 109/L and received platelet transfusion on day of LP181*0Total369430*There was one traumatic tap in a patient with a platelet count of 42 x 109/L who received a platelet transfusion pre-LP. The post transfusion platelet count was 66 x 109/L. Disclosures No relevant conflicts of interest to declare.

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Clinical features of dengue hemorrhagic fever and risk factors of shock event

Paediatrica Indonesiana ◽

10.14238/pi46.3.2006.144-8 ◽

2016 ◽

Vol 46 (3) ◽

pp. 144 ◽

Cited By ~ 1

Author(s):

Rismala Dewi ◽

Alan Roland Tumbelaka ◽

Damayanti Rusli Sjarif

Keyword(s):

Risk Factors ◽

Platelet Count ◽

Clinical Features ◽

Dengue Hemorrhagic Fever ◽

Univariate Analysis ◽

Hemorrhagic Fever ◽

Chi Square ◽

Logistic Regression Method ◽

Chi Square Test ◽

Level Of Significance

Background Dengue hemorrhagic fever (DHF) can lead to highmorbidity and mortality. Its clinical features vary from time to time.Many studies were performed to determine the risk factors of se-vere dengue infection.Objective To find out clinical features and risk factors for predict-ing the likelihood of shock in DHF.Methods A retrospective cohort study was conducted in all con-firmed DHF children who were hospitalized at the Department ofChild Health, Cipto Mangunkusumo Hospital within the period ofJanuary 1, 2003 until June 30, 2004. Risk factors for developmentof shock were analyzed using chi-square test and multiple logisticregressions with a level of significance of <0.05.Results A total of 101 patients, consisted of 47 males and 54females were enrolled in this study. Mean age was 6.5 (SD 3.6)years, ranged from 5 months to 15 years. About 31.7% patientshad grade III DHF, 30.7% grade II, and 26.7% grade IV (including1 patient with encephalopathy). Shock was more frequent amongpatients aged between 6-10 years, female, under-nourished, bodytemperature <38°C, hematocrit level 46-50 vol%, and platelet count<20 000/ml. During year 2003-2004, there was increased numberof patients who developed shock. Based on univariate analysis,hepatomegaly, high hematocrit value, and thrombocytopenia wereconsidered significantly different. Among those variables analyzedwith multiple logistic regression method, only hepatomegaly andthrombocytopenia were identified as predictors of shock.Conclusion There was an alteration on clinical features of DHFin our hospital in 2003-2004 period compared to the previous years.Hepatomegaly and platelet count <50 000/ml are independent riskfactors of shock among DHF patients

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Nosological Dilemma and Clinical Polymorphism of the Catatonia Phenomenon

Psychiatry ◽

10.30629/2618-6667-2020-18-2-61-70 ◽

2020 ◽

Vol 18 (2) ◽

pp. 61-70

Author(s):

P. O. Borisova

Keyword(s):

Clinical Features ◽

Affective Disorders ◽

Selection Criteria ◽

Scientific Literature ◽

Clinical Manifestations ◽

Underlying Disease ◽

Cochrane Library ◽

Distinctive Features ◽

Somatic Diseases

Objective: to provide the description of researchers’ views evolution and provide the review of modern scientific literature on nosological affiliation and clinical features of catatonia. Material and methods: Scopus, PubMed, Cochrane Library, eLIBRARY databases were searched for literature (44 000 papers) using the keyword ‘catatonia‘. Based on the selection criteria, the search has been found the clinical cases, cohort and random studies, dedicated to psychopathologic description of catatonia, accompanied by affective disorders, autism and hysteria. 50 papers issued between 2000 and 2020 were selected for the analysis. Conclusions: catatonia, traditionally treated as a symptom of schizophrenia, currently is predominantly interpreted as a “transnosological syndrome”, which accompanies various (both mental and somatic) diseases. The clinical manifestations of catatonia in affective disorders, autism and hysteria have their own distinctive features, also the presence of catatonic symptoms is associated with a greater severity of the underlying disease, low quality of remissions, which explains why the experts are wary of the endogenic factors.

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Clinical Features and High-risk Factors Prediction of Multiple Fiberoptic Bronchoscopy Therapy of Plastic Bronchitis in 269 Children

10.21203/rs.3.rs-154560/v1 ◽

2021 ◽

Author(s):

Tongqiang Zhang ◽

Lihua Zhao ◽

Jiafeng Zheng ◽

Linsheng Zhao ◽

Xiaojian Cui ◽

...

Keyword(s):

Risk Factors ◽

Clinical Features ◽

Clinical Manifestations ◽

Multivariate Logistic Regression Analysis ◽

Fiberoptic Bronchoscopy ◽

Plastic Bronchitis ◽

Pleural Thickening ◽

High Risk Factors ◽

Mycoplasma Pneumonia ◽

Flexible Fiberoptic Bronchoscopy

Abstract Background. To analyze the clinical features of children with plastic bronchitis (PB) and identify the risk factors of multiple flexible fiberoptic bronchoscopy (FOB) therapy.Methods. Retrospective analysis was performed on 269 PB children from 2016 to 2019, 144 cases were in single FOB group, 125 cases were in the multiple FOB group. The clinical manifestations, laboratory datas, imaging findings and management were investigated. The different features were compared between the single FOB group and multiple FOB group.Results. A total of 269 PB children were collected with a mean age of 6.7 ± 2.8 years. 257 (95.5%) cases were diagnosed as Mycoplasma pneumonia (MP) infection. The mean duration of fever was 10.6 ± 3.7 days. All the patients presented with fever, and 62 (23.0%) suffered from hypoxemia, 144 (53.5%) had extrapulmonary complications. Higher levels of ESR, CRP, PCT, IL-6, LA, LDH, FER and D-dimer were observed. The proportion of pulmonary consolidation, segmental or lobar atelectasis, pleural effusion and pleural thickening were 97.4%, 46.5%, 47.9% and 63.2%, respectively. Furthermore, multivariate logistic regression analysis showed that N% >75.5%, LDH >598.5U/L, and D-dimmer>0.45mg/L were independent isk factors for multiple FOB therapy. Conclusions. MP is a significant pathogen of PB in children. Patients with PB are more likely to suffer from persistent fever, excessive inflammation and severe radiological findings. N% >75.5%, LDH >598.5U/L and D-dimmer > 0.45mg/L may be predictors of multiple FOB treatment.

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Association of clinical features, comorbidities and laboratory profile with outcomes among dengue patients admitted in a tertiary care hospital, Delhi NCR

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20212509 ◽

2021 ◽

Vol 9 (7) ◽

pp. 1918

Author(s):

Mohit Tiwari ◽

Abhishek Tibrewal ◽

Varun S. Pichika ◽

Narinder P. Singh ◽

Pankaj N. Choudhary ◽

...

Keyword(s):

Blood Transfusion ◽

Clinical Features ◽

Tertiary Care Hospital ◽

Wide Spectrum ◽

Dengue Infection ◽

Tertiary Care ◽

Clinical Manifestations ◽

Chronic Obstructive ◽

Care Hospital ◽

Laboratory Profile

Background: Dengue fever is an endemic disease across multiple countries. Dengue infection results in a wide spectrum of non-specific clinical manifestations with unpredictable clinical course and outcome. Objective of the study was to understand the association of different clinical features, comorbidities and laboratory profile with outcomes (ICU use, ventilation use and blood transfusion) among dengue patients admitted in a tertiary care hospital in Delhi, National Capital Region.Methods This cross-sectional study included 75 dengue patients with fever <1 week confirmed based on NS-1 antigen and/or IgM antibody positivity. Descriptive analysis was used.Results: Gender was not significantly associated with the outcomes. The duration of fever was significantly higher among those with ICU use (median: 6 versus 4 days; p=0.005), ventilator use (median: 5.5 versus 4.0 days; p=0.049] and blood transfusion (median: 6 versus 4 days; p=0.013). Dengue patients with co-morbidities (diabetes, hypertension, or chronic obstructive pulmonary disease) or co-infection had a significantly higher odds of the outcomes. The platelet level was significantly lower while liver enzymes were significantly higher among those with the outcomes.Conclusions: The clinical features, comorbidities and laboratory profile can help in identifying critical patients for ICU admission and timely intervention to improve outcome.

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Invasive aspergillosis in adult patients with rheumatic diseases

Modern Rheumatology Journal ◽

10.14412/1996-7012-2020-4-16-22 ◽

2020 ◽

Vol 14 (4) ◽

pp. 16-22

Author(s):

V. I. Mazurov ◽

A. M. Lila ◽

O. V. Shadrivova ◽

M. S. Tonkoshkur ◽

M. S. Shostak ◽

...

Keyword(s):

Risk Factors ◽

Respiratory Failure ◽

Invasive Aspergillosis ◽

Rheumatic Diseases ◽

Lupus Erythematosus ◽

Clinical Manifestations ◽

Underlying Disease ◽

Control Group ◽

Group 2 ◽

Group 1

Objective: to study risk factors for invasive aspergillosis (IA), its etiology, clinical manifestations, and treatment efficiency in patients with rheumatic diseases (RD).Patients and methods. The first study of proven and probable IA (EORT/MSGERC, 2019) was conducted in 18 patients with RD, who accounted for 3% of all adult IA patients (n=699) included in the 1998–2020 registry of the Department of Clinical Mycology, Allergology, and Immunology, I.I. Mechnikov North-Western State Medical University (Group 1). This group comprised 56% women; the median age was 59 [21; 75] years. Group 2 (a comparison group) included 610 adult hematology patients with IA (median age, 45 [18; 79] years; 42% women). A prospective case-control study was conducted to identify risk factors for IA in patients with RD: 36 rheumatic patients without IA (median age, 58 (18–79) years; 61% women) (a control group).Results and discussion. Patients with RD were found to often develop IA in the presence of anti-neutrophilic cytoplasmic antibody-associated vasculitis (granulomatosis with polyangiitis and microscopic polyangiitis) and systemic lupus erythematosus (50 and 16%, respectively). It was shown for the first time that the likelihood of IA in patients with RD increases with prolonged (median 14 days) lymphocytopenia during RD treatment (odds ratio 13.0; 95% confidence interval, 3.3–50.3). The main causative agents of IA were A. fumigatus (50%) and A. niger (29%). IA was more severe in Group 1 than in Group 2: in the resuscitation and intensive care units, there were 44 and 18%, respectively (p=0.01). Group 1 versus Group 2 more frequently had respiratory failure (61 and 37%, respectively; p=0.03), hemoptysis (28 and 7%; p=0.0001), chest pain (17 and 7%; p=0.04), and cardiac involvement (11 and 1%; p=0.0001), and less frequently had fever (67 and 85%; p=0.01). The common site of IA was the lung (83%); the characteristic feature detected by computed tomography (CT) is pulmonary cavitation (44%). Antifungal therapy was used in 89% of Group 1 patients; the overall 12-week survival was 69%.Conclusion. In patients with RD, it is difficult to differentiate between the progression of the underlying disease, adverse drug reactions, infectious complications, or a combination of these disorders due to the similarity of their clinical manifestations. When RD patients with infectious syndrome and respiratory failure develop prolonged lymphocytopenia during combination therapy, AI should be suspected and lung CT, bronchoscopy, and mycological examination of the material obtained by bronchoalveolar lavage be done.

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Clinical features, risk factors, and outcome of cerebral venous thrombosis in Tehran, Iran

Journal of Neurosciences in Rural Practice ◽

10.4103/0976-3147.185512 ◽

2016 ◽

Vol 7 (04) ◽

pp. 554-558 ◽

Cited By ~ 2

Author(s):

Samira Yadegari ◽

Askar Ghorbani ◽

S Roohollah Miri ◽

Mohammad Abdollahi ◽

Mohsen Rostami

Keyword(s):

Risk Factors ◽

Magnetic Resonance ◽

Clinical Features ◽

Sinus Thrombosis ◽

Oral Contraceptive Pill ◽

Clinical Signs ◽

Signs And Symptoms ◽

Underlying Disease ◽

Tertiary Referral Hospital ◽

D Dimer

ABSTRACT Introduction: Despite increasing the use of magnetic resonance imaging (MRI), cerebral venous sinus thrombosis (CVST) has remained an under-diagnosed condition. In this study, characteristics and frequency of various risk factors of CVST patients in a tertiary referral hospital were closely assessed. Methods: Patients with an unequivocal diagnosis of CVST confirmed by MRI and magnetic resonance venography during 6 years of the study were included. All data from the onset of symptoms regarding clinical signs and symptoms, hospital admission, seasonal distribution, medical and drug history, thrombophilic profile, D-dimer, neuroimaging, cerebrospinal fluid findings, mortality, and outcome were collected and closely analyzed. Result: A total of 53 patients with female to male ratio of 3.07 and mean age of 33.7 years were included in the study. Headache and papilledema were the most frequent clinical features (44 and 36 patients, respectively). An underlying disease (diagnosed previously or after admission) was the most common identified risk factor for CVST in both females and males (21 patients). A total of 15 women used the oral contraceptive pill (OCP) where 12 of them had simultaneously other predisposing factors. Overall, 19 patients (36%) had more than one contributing factor. D-dimer had a sensitivity of 71.4% in CVST patients. The mortality of patients in this study was 3.7% (n = 2). Focal neurologic deficit and multicranial nerve palsy were associated with poor outcome which defined as death, recurrence, and massive intracranial hemorrhage due to anticoagulation (P = 0.050 and 0.004, respectively). Conclusion: Unlike most of the CVST studies in which OCP was the main factor; in this study, an underlying disease was the most identified cause. Considering the high probability of multiple risk factors in CVST that was shown by this study, appropriate work up should be noted to uncover them.

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Prevalence,Type, and Risk Factors for Bleeding in a Large Cohort of Myeloproliferative Neoplasm Patients

Blood ◽

10.1182/blood.v124.21.3207.3207 ◽

2014 ◽

Vol 124 (21) ◽

pp. 3207-3207

Author(s):

Elizabeth M Kander ◽

Sania S. Raza ◽

Zheng Zhou ◽

David Dittmann ◽

Juehua Gao ◽

...

Keyword(s):

Risk Factors ◽

Platelet Count ◽

Blood Group ◽

Jak2 V617f ◽

Older Age ◽

Bleeding Events ◽

Mutational Status ◽

The Mean

Abstract Background: The BCR-ABL negative myeloproliferative neoplasms (MPN), including polycythemia vera (PV), essential thrombocythemia (ET) and myelofibrosis (MF), share an increased risk of thrombotic and hemorrhagic complications. The risk of bleeding and thrombosis is less defined in MPN-Unclassifiable (MPN-U). Risk factors for hemorrhage are less defined compared to thrombosis, but acquired von Willebrand disease (aVWD) and thrombocytosis have been implicated. Because patients (pts) with CALR mutations have higher platelet counts compared to JAK2 V617F mutated pts, bleeding rates may be increased in the former group. Our aim was to define the prevalence and bleeding type and to evaluate whether aVWD, thrombocytosis, mutational status or treatment history associated with bleeding in a large cohort of MPN pts. Methods: The Northwestern University Electronic Data Warehouse identified MPN pts ≥18 years, last seen between 2005 and 2013. MPN was diagnosed based on clinical features, histopathology, and mutational status. A significant bleeding event was defined by a need for medical evaluation. Mutations in exon 9 of CALR were evaluated by PCR and fragment analysis. aVWD was defined by vW antigen (ag) and/or ristocetin cofactor activity below the laboratory reference range for blood type (<40% for blood group O or <53% for non blood group O; type defined by vW ag: activity ratio). Associations were tested using Fisher’s exact test; p<0.05 was considered statistically significant. Results I: Baseline characteristics 351 MPN pts were identified: 142 with ET (40.4%), 118 with PV (33.6%), 62 with MF (17.7%), 4 with MDS/MPN (1.1%), and 25 with MPN-U (7%). Median follow-up was 5 years, median age at diagnosis was 54.7 years, and 58.1% were female. 73.3% were treated with aspirin, and 51.6% were treated with hydroxyurea. JAK2 V617F was identified in 178/288 patients (61.8%). Among 110 JAK2 V617F-negative samples, residual DNA was available in 46 and 15/46 (32.6%) were CALR mutated, including primarily type 1 (52-bp deletion; c.1092_1143del, N=11), type 2 (5-bp insertion; c.1154_1155insTTGTC, N=3) mutations, and 1 with a 69-bp deletion. CALR mutated patients included 10/29 ET (34.5%), 2/7 MF (28.6%), and 3/9 MPN-U (33%). To date, 34 patients in the cohort (9.8%) progressed to MF and 18 (5.2%) had leukemic transformation. Results II: Bleeding complications 55 pts (15.6%) experienced 62 bleeding events, while thrombotic events were reported in 84 cases (23.9%). 19% of cases had both bleeding and thrombosis. In bleeding pts, the mean white blood count (WBC) at the time of diagnosis was 15.0 x 109/L, mean platelet count 803 x 109/L, and mean hemoglobin 12.6 x 109/L. Bleeding occurred at a median of 2 years after diagnosis. Gastrointestinal bleeding (GIB) was the most common type in 28/55 pts (50.9%), including 10 upper GIBs, 9 lower GIB, and 9 cases involving an unspecified location. Bleeding episodes from mucocutaneous sites were noted in 17/55 pts (30.9%; 9 with epistaxis, 1 with gum bleeding, and 7 with vaginal/uterine bleeding). There were 6 pts with intracranial hemorrhage. The mean platelet count at the time of bleeding was 473 x 109/L. Bleeding events were significantly more common in MPN-U (32%) than MF (19%), ET (10.6%), or PV (15.3%), (p=0.0163). Bleeding was associated with older age at diagnosis (59.4 yrs in bleeding pts vs 53.9 yrs in non-bleeders, p=0.03). There was no association between bleeding and mutational status (JAK2 V617F, CALR), gender, or aspirin use. The median ristocetin activity was 94% (N=38), vW ag 110% (N=37) and factor VIII activity 94% (N=28). Of 38 pts, there were 7 (18.4%) cases of VWD; 6 were acquired (5 with type 2, 1 with type 1) and 1 was congenital, respectively. Two of 7 pts with VWD (28.6%) had bleeding events. Conclusions Consistent with prior reports, bleeding was less prevalent than thrombosis, associated with older age and, uniquely MPN-U, an entity with a less defined natural history compared to ET, PV, or MF. VWD was infrequently tested for, even less commonly identified, and less represented in bleeding cases. The role of routine testing for aVWD requires further definition. We did not find any association between bleeding and aspirin use or mutational status. However, a limited number of CALR mutated patients may have precluded recognition of any association, and future studies with a larger sample of CALR mutated patients are needed to identify its impact on bleeding. Disclosures No relevant conflicts of interest to declare.

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