Milan, Italy, June 12 – 15, 2014

Bleeding disorders (congenital and acquired) PB2035 RADIOISOTOPE SYNOVECTOMY IN PATIENTS WITH HAEMOPFILIA: SINGLE CENTER EXPERIENCE 1 * 1 1 1 2 1 PB2033 T Patiroglu , E Unal , M Ozdemir , F Mutlu , M Kula , M Karakukcu , S Doganay 3 CIRCUMCISION IN CHILDREN WITH CONGENITAL FACTOR DEFICIENCY 1Department of Pediatric , 2Department of Nuclear Medicine, Z Salcioglu 1,* H Sayilan Sen 1, D Tugcu 1, A Akcay 1, G Keskindemirci 1, 3Department of Pediatric Radiology, Erciyes University Medical Faculty, Kayseri, G Aydogan 1, F Akici 1, M Gokce 1, N Aktay Ayaz 1, U Guvenc 2, Z Mahmud 2 Turkey 1Pediatric Hematology and Oncology Clinic, 2Pediatric Surgery Clinic, Istanbul Kanuni Sultan Suleyman Education and Research Hospital, Istanbul, Turkey Background: Haemophilic arthropathy represents the most common clinical manifestation of , related to recurrent haemarthoses and chronic Background: Circumcision is the most frequently performed surgical prosedure synovitis. Haemophilic arthropathy is radiologically characterized by destructed for religious reasons in Turkey. articulation, bone deformities, complete closure of intra-articular spaces. Aims: Examination of hemostasis tests carefully before circumcision leads to Surgical synovectomies are not cost-effective procedures whereas radioisotope the recognition of patients with diathesis. We evaluated 158 records synovectomy (RS) is both less invasive and inexpensive procedures. It is of patients diagnosed as congenital factor deficiency (CFD), underwent accepted that RS is the gold-standart therapy before surgical synovectomy circumcision in our clinic between 1990-2014, retrospectively. Aims: We aimed to investigate the efficacy and complications of RS in the Methods: Information of patients were retrieved from patient records and from patients with haemophilia. the records contained in the data-processing environment introduced in 2005. Methods: Twenty five RS were performed in 12 patients with and Circumcisions performed with an open method, with dorsal slit technique. B, age ranging 6-21 mean (11 years) at the time of RS,for last five years. We glue was not used during circumcision procedures. All of the factor preparations preferred to use Yttrium 90 (Y 90 ) for knees and ankles, and Erbium 169 (Er 169 ) were administered as bolus via the peripheral vein. No catheters were used. for elbows. Radioisotopes such as Y 90 and Er 169 were injected intra-articularly Results: Of the patients, 84 (53.2%) had Hemophilia A, 45 (28.5%) FVII for treating target joints and chronical synovitis. deficiency, 11 (6.9%) Hemophilia B, 4 (2.5%) vWF deficiency, while 4 (2.5%), Results: We have evaluated our experience for knees (n:12), for ankles (n:9) 3 (1.9%), 3 (1.9%), 2 (1.3%), 2 (1.3%) had fibrinogen, FV, FXI, FX, FXIII and for elbows (n:4) in total 25 RS procedures for 12 patients. After RS joint deficiencies retrospectively. Age of patients ranged from 2 to 24 years (mean were decreased for all patients. Radioisotop injections were three 14.4 years). Thirty-four asymptomatic patients were diagnosed before times repeated in right ankle of one patient. We have observed local circumcision (21.5%). In 35 patients circumcision were applied without any after RS in three patients. For elbows, RS with Er 169 seems to be safe replacement therapy (22.2%). We used FVIII in 80 patients, FIX in 11 treatment method. patients, vWF+FVIII in 4 patients, fresh frozen plasma in 13 patients, rFVIIa in Summary and Conclusions: We had observed that RS was a safe and 10 patients, PCC in 2 patients and fibrinogen in 3 patients during surgical efficient therapeutic strategy. interventions . Replacement therapy was achieved with single dose in 36 Key Words: Haemophilia A and B, Haemophilic arthropathy, radioisotope patients for circumcision (29.3%). Also in 87 patients 2-10 doses replacement synovectomy. therapy was needed (70.7%). In 35 patients antifibrinolytic agents were used also (28.5%). Bleeding during and after surgical procedures were observed in nine patients (5.6%). Transfusion was needed in two patients (1.3%). PB2036 Thrombotic events were not observed. Antibody occurrence was not detected in these patients. No delay in surgical wound healing was noted. LUPUS ANTICOAGULANT SYNDROME WITH Summary and Conclusions: Children with CFD can be safely circumcised in SEVERE BLEEDING IN A 4-YEAR-OLD CHILD a center with experience In our study, we evaluated preparation before H Lahlou 1,* I Beukinga 1, O Pradier 1 circumcision factor replacement prior to surgery and postoperative processes 1Laboratory of hematology, Hopital Erasme, Université libre de Bruxelles, in patients. Brussels, Belgium

Background: Lupus anticoagulant is an acquired inhibitor which is commonly PB2034 associated with thromboembolic manifestations. But in some rare cases, the antibody has affinity to prothrombin and patients have hemorrhagic tendency; ACQUIRED HEMOPHILIA A WITH IMMUNE IN sometimes they may present with severe bleedings, depending on their factor ADOLESCENT PATIENT II level. The association of acquired factor II deficiency and lupus anticoagulant 1 * 1 1 1 1 HS Kim , SL Jin , SM Hahn , JW Han , CJ Lyu is recognized as “Lupus Anticoagulant Hypoprothrombinemia Syndrome” 1 Department of Pediatric Hematology and Oncology, Yonsei University Health (LAHS). System, Seoul, Korea, Republic Of Aims: We report a case of a 4-year-old boy with severe bleeding due to LAHS, with spontaneous recovery. Background: Acquired hemophilia A (AHA) is rare autoimmune disorder Results: A 4-year-old boy was admitted in pediatric emergency for macroscopic caused by autoantibodies against factor VII (FVIII) in the hematuria. He had no other symptom. Physical examination was normal. Initial nonhemophilic population. The age distribution of autoantibodies typically laboratory evaluation consisted of a urine examination that confirmed hematuria biphasic with small peak between 20 and 30 years (mainly postpartum (32833 red cells/µL and 75 leucocytes/µL, without infection) and blood tests inhibitors) and a major peak in patients aged 70 to 80 years. The incidence of revealed a decreased PT (40%) and a prolonged APTT (69.9 sec (24.0 – 35.0 AHA in children is very rare. Indeed, the incidence in children under 16 years sec)). count was normal (279 G/L). The measure of clotting factors has been estimated to be 0.045 per million/year compared with 14.7 per showed an isolated factor II deficiency (20%). Lupus anticoagulant was positive. million/year in the elderly over 85 years. Also, immune thrombocytopenia is an C-reactive protein was 10 mg/L (<10 mg/L). An abdominal echography was acquired immune-mediated disorder caused by increased destruction of performed to exclude other hemorrhagic sites and showed signs of gastro- opsonized by anti-platelet autoantibodies. intestinal infection. The patient was diagnosed with LAHS related to gastro- Aims: We experienced a case of AHA with thrombocytopenia caused by intestinal infection. Two days after his admission, hematuria disappeared. Ten autoantibody in 18 years old boy. weeks later, a control blood evaluation showed that all parameters became Methods: He without any previous bleeding history presented easy on normal. We performed a specific research of antiprothrombin antibodies (INOVA lower extremities and he had diagnosed AHA. A initial activated partial Quantalite) what demonstrated increased rates of IgG and IgM thromboplastin time (APTT) was 136 seconds, the level of FVIII was 0.6% and antiphosphatidylserine antiprothrombin (Table 1). FVIII inhibitor was 13.1 Bethesda Units (BU). Moreover, platelet counts were low (34,000/uL), and we proved platelet-associated autoantibody. Other autoimmune diseases were ruled out. He was instantly treated with oral Table 1. prednisolone (1mg/kg/d) and added to oral cyclophosphamide (2mg/kg/d) one Patient Normal values week later due to elevation of FVIII inhibitor (14.7 BU). antiphosphatidyl serine IgG 50,78 U/ml <11 U/ml About seven weeks later after treatment, FVIII inhibitor was Results: antiphosphatidyl serine IgM 17,49 U/ml <25 U/ml disappeared and APTT was normalized, we started to tapering off the antiphosphatidyl serine IgA 2,82 U/ml <20 U/ml medication. During treatment of inhibitor eradication, platelet counts were also antiphosphatidylserine/prothrombine IgG >150 U/ml 30 U/ml increased and normalized. antiphosphatidylserine/prothrombine IgM >150 U/ml <30 U/ml Summary and Conclusions: We report our experience as first successful antibody eradication for FVIII inhibitor and platelet-associated autoantibody simultaneously in adolescent AHA patient. Summary and Conclusions: LAHS was first described in 1960. In 2011, the 74 cases reported between 1960 and 2011 had been reviewed (Mazodier K,

haematologica | 2014; 99(s1) | 775 19 th Congress of the European Hematology Association

Arnaud L, Mathian A et al. Lupus Anticoagulant Hypoprothrombinemia patient had gastrointestinal hemorrhage, one patient had hematuria. Six Syndrome, report of 8 Cases and Review of the Literature. Medicine 2012; 91: patients with afibrinogenemia and three patients with factor XIII deficiency had 251-260). LAHS mostly affects young women. But sometimes it occurs in ICH. Four patients diagnosed preoperatively and three patients postoperatively children. It is mainly related to autoimmune diseases, in particular systemic after circumcision and tonsillectomy as RBD. The patients were administered lupus erythematosus, or to acute viral infection. However, few cases of drug- fresh frozen plasma, cryoprecipitate and fibrinogen concentrates. All patients induced LAHS or lymphoma-associated are reported. Clinical features and the survived, but four of them with ICH had sequelae such as decrease in severity of bleeding are correlated to the factor II level. According to the review intellectual capacity and motor deficit. of the 74 cases reported in the literature, some patients were asymptomatic, Summary and Conclusions: This registry provides a comprehensive but 89% of them had hemorrhagic manifestations with severe bleedings in information and could be an important resource for hematologists. 51%. Macroscopic hematuria was present in 15% of patients. Key Words: Rare bleeding disorders, registry. In children, it is generally a transient situation which does not need any treatment, and clinical and biological features usually normalize in few days except when the LAHS is revealing an autoimmune disease or when there are PB2039 severe complications in relation to the bleedings. STUDY OF THROMBIN GENERATION IN PATIENTS WITH IMMUNE THROMBOCYTOPENIC RG Mihaila 1,* A Olteanu 2, D Lienerth 2, A Catana 2, O Flucus 2, C Bus 2, RC Cipaian 1 PB2037 1Faculty of Medicine, Lucian Blaga University of Sibiu, 2Emergency County DIRECT APTT RATIO (PLATELINLS / ACTINFS) PERMITS TO QUICKLY Clinical Hospital Sibiu, Sibiu, Romania AND RELIABLY DETECT BLEEDING RELATED FACTOR DEFICIENCY WHEN ISOLATED PROLONGATION OF APTT IS FOUND IN PEDIATRIC Background: Although thrombocytopenic, patients with primary immune PRE-OPERATIVE SC thrombocytopenia rarely have severe bleeding episodes, mainly because their R Li 1,* C Swaelens 1, F Vandermijnsbrugge 1, B Cantinieaux 1 blood platelets are hyperfunctional. 1Laboratory of Haematology, Institut Jules Bordet et CHU Saint Pierre, ULB, Aims: We aimed to study if the level of thrombin generation is another factor Brussels, Belgium that may explain the rarity of bleeding in patients with primary immune thrombocytopenia. Background: Isolated prolongation of activated partial thromboplastin time Methods: We made a pilot study on a group of 10 patients with primary immune (aPTT) found during paediatric pre-operative screening could be due to thrombocytopenia found in the records of the Hematology Service of coagulation factor deficiency or more frequently to nonspecific inhibitor like Emergency County Clinical Hospital Sibiu, who presented consecutively to lupus anticoagulant (LA). clinical and biological control in January 2014. We retained age, peak of Aims: The aim of our study is to evaluate the clinical values of an additional thrombin generation (obtained wiht alpha Ceveron device), the platelet count, aPTT with ActinFS and/or a mixing aPTT study to identify the bleeding blood hemoglobin level, the hematocrit, white blood cells number, international related factor deficiency (BRFD) like Factor VIII, Factor IX or Factor XI normalised ratio (INR), and the activated partial thromboplastin time. The peak deficiency. of thrombin generation of these patients was compared with that of a control Methods: During 4 years, an aPTT with ActinFS and a mixing aPTT were group - 10 voluntarily subjects without any known disease. The results were added for 308 paediatric patients in which an isolated prolongation of aPTT with analyzed with arithmetic mean, standard deviation, Student’s t test, and PlatelinLS was found among 9048 pre-operative screening. A direct aPTT ratio Pearson test, and they have allowed conclusions that we hope to have between PlatelinLS and ActinFS was calculated. For 156 patients (50.6%), this implications for clinical practice. prolonged aPTT was confirmed in a second sample. Measurements of bleeding Results: The mean age of surveyed patients was 59.11+/-15.14 years. The related factors (FVIII, FIX and FXI), Factor XII and the LA research were average peak of thrombin generation in patients with ITP was 166.57+/48.88, performed respectively in 141,131 and 88 samples. and in the witnesses - 123.29+/-20.64; the difference is statistically significant Results: We found 17 BRFD, 26 FXII deficiency and 64 positive LA. A (p <0.05). The thrombin generation peak in patients with ITP was inversely prolonged ActinFS had a significant association with BRFD (P<0.0001) while correlated with age (r=-0.316), platelet count (r=-0.672), hemoglobin (r=-0.756), a corrected mixing study had not. The direct aPTT ratio had a significant hematocrit (r=-0.433) and INR (r=-0.294); it did not correlate with the number association not only with BRFD (P<0.0001) but also with positive LA (P<0.05). of leukocytes and activated partial thromboplastin time. In applying a cut-off value of this ratio as <1,29, the sensitivity and specificity Summary and Conclusions: Patients with ITP have an average peak of for BRFD were 82% and 76% respectively, while they were 59% and 93% thrombin generation significantly higher than that of witnesses. The lower the using ActinFS alone. Normal ActinFS was found in 7 mild BRFD cases with age of patients with ITP, the higher the peak of thrombin generation. The higher factors levels superior to 30%, five of 7 had a direct ratio <1,29. the peak of thrombin generation in patients with ITP, the smaller the Summary and Conclusions: The direct aPTT ratio between PlatelinLS and hemoglobin, hematocrit and INR values. The smaller the platelet count in ActinFS without mixing study permits to identify quickly and reliably a BRFD for patients with ITP, the higher the peak of thrombin generation - another reason an isolated prolongation of aPTT found in paediatric pre-operative screening. for them to present bleeding rarely.

PB2038 PB2040 SINGLE CENTER REGISTRY OF RARE BLEEDING DISORDERS FROM HAEMORRHAGIC SYNDROME DUE TO PRIMARY HYPERFIBRINOLYSIS TURKEY AS THE FIRST CLINICAL SIGN OF PROSTATE CANCER T Patiroglu 1,* M Ozdemir 1, M Karakukcu 1, E Unal 1 Z Cvetkovic 1,* A Kulic 2, B Cvetkovic 3, V Libek 2 1Department of Pediatric Hematology, Erciyes University Medical Faculty, 1Department of hematology, 2Blood bank department, 3Department of urology, Kayseri, Turkey Clinical Hospital Center Zemun, Belgrade, Serbia

Background: Deficiencies of coagulation factors (except factor VIII and factor Background: Hematologic disorders are commonly seen in patients with IX) are generally much rarer than the hemophilias. prostate cancer (PCa). The most frequently observed disorder in PCa patients Aims: The purpose of this poster presentation is to review the disorders in is acute or chronic disseminated intravascular coagulation (DIC). The incidence terms of clinical manifestations and complications from hemorrhage. of DIC complication in PCa rages from 13% to 30%, and can be presented as Methods: Records at Erciyes University Medical School, department of catastrophic bleeding or various thrombotic events. In contrast, bleeding pediatric hematology, were reviewed from 1998 to 2012 and 31 patients with symptoms in the setting of primary hyperfibrinolysis in this malignancy are seen rare bleeding disorders (RBD) were evaluated. The data were collected based in only 0.40% to 1.65% patients, usually provoked by surgical procedures. It is on age at diagnosis, familiy history of disease, bleeding sites, treatment supposed that metastatic PCa cells produce high levels of urokinase type strategies and complications of disease. plasminogen activator. Laboratory diagnosis of hyperfibrinolysis is based on the Results: Ten patients had afibrinogenemia (plasma fibrinogen level<100 increase of biomarkers like D-Dimer; fibrinogen split products, plasminogen, mg/dl), five had factor V deficiency, three had factor VII deficiency, five had and euglobulin lysis test (ELT). But, these tests are not specific for primary , three had factor XI deficiency, and the last five had factor hyperfibrinolysis and they are also elevated in other pathological condition. XIII deficiency, respectively. The mean age of the patients was 6.41 years ( Aims: We reported the diagnosis and treatment of a rare case of haemorrhagic ranging from 3 months to 13 years) at diagnosis. Twenty two out of 31 patients syndrome caused by primary hyperfibrinolysis, which was the first clinical were male, and nine were female. Out of 31 patients, three with symptom of metastatic prostate cancer. afibrinogenemia, two with factor V deficiency, two with factor X deficiency, and Methods: The patient’s blood was collected in vacuum tubes containing 3.2% four with factor XIII deficiency coming from two diferrent families were siblings. sodium citrate and lithium heparin.Thrombelastometry analysis was performed Among all RBDs, the most common sites of bleeding were skin and mucus with the Rotem ® analyzer. Routine coagulation test was performed with IL membranes. Furthermore, 11 patients had intracranial hemorrhage ( ICH), one ELITE PRO ®and ACL 300 ® analyzer.

776 | haematologica | 2014; 99(s1) Milan, Italy, June 12 – 15, 2014

Results: A 64-year-old male was admitted to our hospital with large Aims: In this work, we describe our experience with three cases of indolent NHL haematomas: in the right pectoral and axillary area, size 20x7 cm, right with isolated prolonged aPTT and PT. hemiabdomen size, 30x30 cm and left lumbal area, size 25x5 cm. Methods: Case 1 : A 72 year-old man referred to our Institution because of developed two days before hospitalization. Patient denied neither subjective recurrent epistaxis and abnormalities of coagulation tests PT INR 2.5, aPTT symptoms nor any medication. Performed labory analises showed mild ratio 2.73. No history of bleeding disorders, recent surgery or new drug intake normocytic anaemia of chronic disease (Hb 108g/L, MCV 90fL, RBC were reported. Spleen enlargement, with a large focal lesion, and pancytopenia 3.72x10 12 /L, Hct 0.331, Plt 419x10 9/L, WBC 9.5x10 9/L, Neutrophils 60%; serum (Leukocytes 2800/ μL; Haemoglobin 9 g/dL; Platelets 82,000/ μL) were iron 12,9µmol/L, ferritin 7820 μ/ L), highly elevated alkaline phosphatase observed. Moreover, it was present a reduction of Factor VIII, Factor II, Factor (1390U/L), lactate dehydrogenase (1740U/L) and prostate specificic antigen V, Factor VII, Factor IX, Factor X and Factor XI activity (FVIII:C 16%; FII: 44%; (above 150ng/mL). Viral test vere negative (HBs Ag, anti HCV and anti HIV). FV:8%; FVII: 11%; FIX: 10%; FX:30%; FXI:27%) and positivity of antibodies Initial coagulation testing: protrombin time and activated partial thromboplastin against coagulation factors. Lymphoid infiltrates were observed in bone marrow time were within the normal range (14.4s and 31.9s, respectively), as well as examination. Moreover, increased PET/CT splenic uptake and in a supraclavear anti-thrombin III (86.6%), while fibrinogen level was very low (1.068 g/l) and D- lymphonode was observed. FNAB in the supraclavear lymphonode, performed Dimer assay result was 1122 ng/mL. Results obtained by rotation after an injection of 60 μg/Kg recombinant Factor VIIa enabled the NHL CD5- thrombelastometry have pointed on primary . First line treatment for CD22+lambda+ diagnosis. The patient underwent to six administrations of patient was combined administration of tranexamic acid (3x500 mg intravenous) chemotherapy with CEOP and achieved complete remission with coagulation and transfusion of ten units of cryoprecipitate (400 ml). Next day, fibrinolytic parameters and factor activity normalization. Then, during followup, 11 months function measurements by rotation thrombelastometry were within the normal after the end of frontline treatment, the patient came to our Department for the ranges. APTEM test wasn’t registered pathological fibrinolysis. Fibrinogen level onset of abdominal bleeding and relapse of the lymphoproliferative disease. was normalized within two days (2.4 g/l). There were no newly developed After surgical treatment, the patient underwent to R-FN chemotheapy treatment hematomas. Transrectal biopsy of prostate was successfully performed without (6 courses), with complete remission of disease. After the end of treatment, he any haemorrhagic complications, revealing prostate adenocarcinoma-G3, underwent to Rituximab-based maintenance treatment with persistence of Gleason score 9. Further examination confirmed the laboratory findings of normality of coagulation and factor activity parameters. Case 2 : A 62 year-old metastatic bone disease. Afterwards, the patient was referred to the oncologist woman came to our observation for lymphoadenopathy, hepatoplenomegaly, for further treatment with androgen deprivation therapy together with oral anemia and lymphocitosis. She didn’t present any personal or familial history tranexamic acid. of bleeding disorders and didn’t take any drug. Laboratory tests showed: PT INR Summary and Conclusions: This case represent primary hiperfibrinolysis with 3.26, aPTT ratio 4.92; there was also a reduction of factor VIII activity, Factor as the first clinical sign of previousy undiagnosed metastatic II activity, Factor VII activity, Factor IX activity, Factor activity X and Factor PCa. Rotation thrombelastometry in this severe complication helps to achieve activity XI (FVIII:C: 2.3%; FII: 32%; FVII: 47%; FIX: 1%; FX:43%; FXI:1%) and prompt and proper diagnosis.The management of hyperfibrinolysis was done appearance of antibodies against many coagulation factors. PET/CT revealed within a short period of time thanks to adequate diagnostic procedure. increased uptake at axillary and inguinal lymphonodes and spleen. Bone marrow analysis showed a lymphoid infiltrate and enabled the diagnosis of NHL CD5-CD22+lambda+. The patient underwent to six courses of R- PB2041 Fludarabine, and after the second administration it was observed a PT and aPTT normalization. Case 3 : A 80 year-old man came to our observation INTRACRANIAL HEMORRHAGE IN HEMOPHILIA PATIENTS because of pancytopenia and abnormalitites of coagulation tests: PT INR 2.28, K Lee 1,* aPTT ratio 3.2. No story of previous bleeding disorders. The activity of various 1Pediatrics, Kyungpook National University School Of Medicine, Daegu, Korea, coagulation factors was reduced : FII 54% FV 68% FX 60%. PET/CT Republic Of documented splenomegaly with increased splenic uptake. Bone marrow examination enabled the NHL CD20+CD19+CD5-CD22+CD23-lambda+ Intracranial hemorrhage (ICH) in hemophilia patients is the most Background: diagnosis. The patient underwent to R-Chlorambucil courses, after which a common cause of death in Korea. Early suspicion and prompt treatment of ICH normalization of PT and PTT was observed. is a very important for saving their life and minimizing neurologic sequelae. All patients of this study underwent to Rituximab-based maintenance We investigate the prognosis of the hemophilia patients with ICH who Results: Aims: treatment to prevent the emergence of lymphoproliferative clone. registered in Daegu & Kyungpook area. Summary and Conclusions: The onset of an acquired coagulation disorder Methods: We evaluated the clinical courses, laboratory findings, brain image, can be an useful diagnostic and progonostic marker of immunological effect of treatment and prognosis of ICH. Nine of 161 patients registered in our impairment due to an underlying lymphoprolipherative disease. In particular, Department of Pediatrics suffered 16 episodes of ICH for 10 years. maintenance treatment may actively control the neoplastic clone reducing the Results: All were male hemophilia A patients (severe; 5, moderate; 3 and mild; risk of bleeding. 1) with median age of 48 months at diagnosis of ICH. Two patients who had inhibitor became negative and one patient who had found inhibitor on annual routine follow-up has had inhibitor until now. The median time interval from first symptom to hospital visit was 7 hours. Chief complaints were vomiting in 6 patients, headache in 4, seizure in 3 and mental change in 1. All patients except one were initially given factor VIII concentrate, 50 units/kg and then continuous infusion, 2-3 units/kg/hour. One patient who had factor VIII inhibitor was given factor IX concentrates, 100 units/kg with activated prothrombin complex (FEIBA), 75 units/kg at every 12 hours. All except one with hematoma in cerebellar vermis and the third ventricle are alive without any neurologic sequela Summary and Conclusions: It is desirable to have early treatment, prophylaxis, regular follow-up and patient education about abnormal symptoms to reduce the complications of them.

PB2042 ACQUIRED HEMOPHILIA IN ELDERLY PATIENTS WITH NON HODGKIN’S LYMPHOMA C Cerchione 1,* S Luponio 1, R Della Pepa 1, I Migliaccio 1, G Beneduce 1, N Pugliese 1, E Seneca 1, E Cimino 2, A Coppola 2, AM Cerbone 2, A Tufano 2, M Notarangelo 1, I Peluso 1, G Di Minno 2, F Pane 1, A De Renzo 1 1Hematology, 2Reference Centre for coagulation disorders, AOU “Federico II”, Napoli, Italy

Background: Acquired hemophilia is a rare disorder characterized by autoantibodies against circulating coagulation factors. Patients often have not history of bleeding disorder and present spontaneous hemorrhage, an isolated prolonged aPTT and PT and antibodies against coagulation factors. It has an incidence of 0,2-1 cases/million/year. This condition may be associated in 50% of cases with autoimmune disease, solid tumor, lymphoproliferative disorders and pregnancy.

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