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Öğe Atypical skin metastases from early tubular breast carcinoma(Wiley-Blackwell, 2008) Cil, Timucin; Altintas, Abdullah; Pasa, Semir; Isikdogan, Abdurrahman[Abstract Not Available]Öğe BREAST CARCINOMA METASTASIS TO THE TAIL OF PANCREAS(Aves, 2009) Cil, Timucin; Altintas, Abdullah; Pasa, Semir; Urakci, Zuhat; Isikdogan, AbdurrahmanThe most common distant metastasis of breast cancer are detected in liver, lung, bone and brain. However, atypical organ metastasis is not uncommon. Isolated pancreas metastasis can be seen rarely as well. There have been only seven cases described in the literature. Here, we report extremely a rare case of breast cancer, which metastasizes to the pancreas tail. The patient has diagnosed as breast cancer four years ago, gradual increases in serum levels of CEA and Ca15-3 were detected (129 (0-4,3ng/ml) and 84 U/ml (0-25 U/ml) respectively) in routine follow-up. Although she had no symptoms, contrast-enhanced abdominal computerized tomography (CT) scan revealed a mass of 81mm x 79mm x 68mm, with a necrozis in the tail of the pancreas. No other metastatic sites were detected by gastric endoscopy, lung CT scan or bone scintigraphy. Percutan transabdominal biopsy from tail of pancreas was showed that metastasis of breast carcinoma. We propose that, when an increase is detected in tumor markers, such as CA15-3 or CEA, without any detectable metastasis in common metastatic organs, should be investigated other atypical metastatic areas such as pancreas.Öğe A case of essential mixed cryoglobulinemia and associated acquired von-Willebrand disease treated with rituximab(Springer, 2009) Pasa, Semir; Altintas, Abdullah; Cil, Timucin; Danis, Ramazan; Ayyildiz, Orhan; Muftuoglu, EkremCurrent treatment options of essential mixed cryoglobulinemia (EMC); include immunosuppressive approaches, such as corticosteroids, cyclophosphamide, plasma exchange, other cytotoxic drugs in moderate to severe manifestations. Some controlled studies have been carried out to assess the efficacy of anti-CD20 monoclonal antibody, rituximab in patients with hepatitis C (HCV) related cryoglobulinemia (CG) and in patients with autoimmune disorders. Recent trials and some case reports demonstrate a beneficial role for rituximab in HCV related mixed CG. Although, the published evidence for treatment of EMC with rituximab is restricted to case reports, which have shown positive results. Several diseases include lymphoproliferative and myeloproliferative disorders, solid tumors, immunological disorders, cardiovascular disorders and some drugs associated with acquired von Willebrand disease (avWD). CG, which is a kind of immune complex disease, may be related with development of autoantibodies to various autoantigens. In this present case report, we showed the efficacy of rituximab in a 21-year-old female patient, suffered from neuropathy and arthralgia related with EMC, and developed avWD, presented with mucosal bleeding associated with CG. von Willebrand factor activity of our patient also increased with controlling the underlying disease, EMC by rituximab. This case demonstrate that rituximab may be an effective treatment option in EMC and avWD mainly related to CG.Öğe A Case of neuroleptic malignant syndrome accompanied to an atypical antipsychotic agent: Risperidone(Elsevier Science Bv, 2008) Pasa, Semir; Sayhan, Mustafa Burak; Boyraz, Taylan; Urakci, Zuhat; Altintas, Abdullah[Abstract Not Available]Öğe A Case of Polycythemia Vera Accompanied with Neurofibromatosis Type 1(Akad Doktorlar Yayinevi, 2010) Pasa, Semir; Altintas, Abdullah; Bayan, Kadim; Tuzun, Yekta; Cil, Timucin; Ayyildiz, OrhanClassical myelofibrosis syndromes (MPS) most frequently occur in adults, but MPS unique to childhood also exist. Such syndromes include juvenile chronic myelomonocytic leukemia (JMML), the MPS of monosomy 7 in childhood, familial chronic myeloid leukemia (CML), the transient MPS of infants with trisomy 21, and childhood forms of myelofibrosis. Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disorder with pigmentary abnormalities and are predisposed to benign and malignant neoplasms, mostly in children. MPSs were disproportionately common among children with NF1. Herein we reported a case of polycythemia vera (PV) accompanied to NF1 in an adult patient. The co-existance of NF1 and childhood MPS is a well known condition. The same relation was not demonstrated in adulthood NF1 patients according to our screening of the literature. Most of the reported cases were define a relation with JMML and monosomy 7 syndrome. Probably this is the first case of PV, a type of classic adult MPS accompanied to NF1.Öğe Cranial Involvement in a Follicular Lymphoma Patient with Systemic Complete Remission After Yttrium-90-Ibritumomab-Tiuxetan Treatment: Scientific Letter(Ortadogu Ad Pres & Publ Co, 2008) Cil, Timucin; Altintas, Abdullah; Pasa, Semir; Kilinc, Ilhan; Isikdogan, AbdurrahmanNon-Hodgkin's lymphoma is a heterogeneous group of lymphoproliferative disorders with varying patterns of behavior and treatment responses. Advances in understanding of tumor biology have made it possible to exploit novel treatment strategies such as monoclonal antibodies and their conjugation with radioimmunotherapy was recommended in patients with first relapse of low-grade lymphomas such as follicular lymphoma after rituximab containing chemotherapy. Treatment with radioimmunotherapy such as yttrium-90-ibritumomab-tiuxetan utilizes a monoclonal anti-CD20 antibody to deliver beta-emitting yttium-90 to the malignant B-cells. Clinical trials have demonstrated its efficacy, which is largely independent of the intrinsic activity of the anti-CD20 antibody. Relapses of nodal lymphomas usually occur in extra lymphoid areas. Central nervous system (CNS) involvement develops in less than 1% of patients with lymphomas. Herein, we are presenting a rare case of CNS involvement of follicular lymphoma in a patient who has achieved systemic complete remission after yttrium-90-ibritumomab treatment.Öğe Cytosine-arabinoside induced bradycardia in patient with non-Hodgkin lymphoma: A case report(Taylor & Francis Ltd, 2007) Cil, Timucin; Kaplan, M. Ali; Altintas, Abdullah; Pasa, Semir; Isikdogan, Abdurrahman[Abstract Not Available]Öğe Ectopic lingual thyroid as a rare cause of primary hypothyroidism - A case report(Lippincott Williams & Wilkins, 2008) Pasa, Semir; Beyaz, Coskun; Arikan, Senay; Altintas, Abdullah; Gokalp, Deniz; Cil, Timucin; Tuzcu, AlparslanEctopic lingual thyroid gland is all uncommon abnormality of migration of the thyroid gland. Although it is uncommon, it is often found in the region of the foramen cecum as a lingual thyroid at the base of the tongue in patients whose gland fails to descend. The true incidence of lingual thyroid is unknown. Treatment depends on the presence of symptoms. In this case report, we described a 17-year-old mail who presented with growth retardation due to severe hypothyroidism associated with a lingual thyroid gland. He did not complain of pressure symptoms. We recommended regular follow-up visits to monitor this state of thyroid function and the size and shape of the gland. We wanted to note that ectopic thyroid tissue should be taken into consideration in the differential diagnosis of all lingual and pharyngeal masses, and in the differential diagnosis of hypothyroidism.Öğe The effects of nonsteroidal anti-inflammatory drugs on platelet function and severity of upper gastrointestinal haemorrhage(Springer, 2009) Pasa, Semir; Bayan, Kadim; Kucukoner, Mehmet; Tuzun, Yekta; Altintas, Abdullah; Cil, Timucin; Danis, RamazanNonsteroidal anti-inflammatory drugs (NSAIDs) cause gastrointestinal (GI) damage primarily due to the inhibition of prostaglandin synthesis in gastric mucosa, which is an important factor in mucosa protection. Platelets are a cardinal feature of vascular repair. A variety of angiogenic stimulators are stored in platelets and are released during clotting at the wound. When there is a defect in any of these functions and/or platelet number, haemostasis is usually impaired and there may be an associated increased risk and severity of bleeding. While the mechanism of mucosal injury and bleeding are well documented with the use of NSAIDs, very little is known about the platelet function abnormalities and their effects on severity of upper GI bleedings. We performed a prospective analysis of 49 patients who had a history of NSAIDs use to investigate the association between the platelet function impairment associated with NSAIDs and severity of upper GI haemorrhages. Thirty-six of 49 patients (73.5%) had deteriorated platelet function. Mean severity score of patients with deteriorated platelet functions was 3.39, and that of patients with normal platelet functions was 2.46. Mean severity score was statistically significantly higher in patients with deteriorated platelet functions. In conclusion, impaired platelet functions associated with NSAIDs may cause more severe upper GI bleeding. Clinicians should be alert for GI complications especially in older patients and in those with a history of ulcer bleeding.Öğe The efficacy of rituximab in patients with splenectomized refractory chronic idiopathic thrombocythopenic purpura(Springer, 2009) Pasa, Semir; Altintas, Abdullah; Cil, Timucin; Danis, Ramazan; Ayyildiz, OrhanThe most difficult problem a physician encounters is the management of patients with idiopathic thrombocytopenic purpura (ITP), who has persistent severe thrombocytopenia after failure of initial treatment with glucocorticoids and splenectomy. Most of the patients refractory to corticosteroids and splenectomy will become refractory to other available agents, such as intravenous immunoglobulin (IVIg), danazol or chemotherapy. In this study, we investigated the effect of rituximab on 17 splenectomized refractory chronic ITP patients. Here, we showed that the anti-CD20 antibody, rituximab, induces a clinically significant response in severe chronic ITP patients, who are unresponsive to other therapeutic options. After sixth month, 10 out of 14 responders were still maintaining their durable and significant platelet responses (platelet counts > 50 x 10(9)/l), without requirement to any other ITP medication. Therefore, we suggest that, rituximab is an effective treatment option in splenectomized refractory or relapsed ITP patients. Rituximab was well tolerated without severe side effects.Öğe Factor V Leiden and G20210A prothrombin mutations in patients with recurrent pregnancy loss: data from the southeast of Turkey(Springer, 2007) Altintas, Abdullah; Pasa, Semir; Akdeniz, Nurten; Cil, Timucin; Yurt, Murat; Ayyildiz, Orhan; Batun, SabriFactor V Leiden (FV-Leiden) and prothrombin gene mutations (FII G20210A) are well-established independent risk factors for thrombosis. In the recent years, many studies have suggested that these mutations are associated with an increased risk of recurrent pregnancy loss (RPL). We aimed to investigate the prevalence of these molecular defects in subjects with a history of early RPL. One hundred and fourteen women with three or more consecutive unexplained first-trimester miscarriages were compared to 185 parous women with uncomplicated pregnancies from the same ethnic origin. The presence of FV-Leiden and FII G20210A mutations was assessed by polymerase chain reaction analysis. Overall, 11 out of the 114 women with early RPL (9.6%) had either FV-Leiden or FII G20210A mutation, as compared with 16 out of the 185 women with normal pregnancies (8.6%; p=0.756). The prevalence of FV-Leiden mutation was 7.9% (9/114) in patient group, compared with 7% (13/185) in control group (p=0.780). One hundred and two patients were primary and 12 were secondary aborters. All FV-Leiden positive cases were primary aborters (8.8%; 9/102, p=0.584). Concerning the FII G20210A, two out of 114 (1.7%) were first-trimester RPL (primary aborters) and three out of 185 (1.6%) controls were carriers of the FII G20210A mutation (1.7 vs 1.6%, p=0.931). The results obtained from patients with first-trimester RPL and the control group have no statistical significant differences in the prevalence of FV-Leiden and FII G20210A mutations. These results suggest that mutations have no role in etiology of first-trimester recurrent abortions.Öğe Familial Mediterranean fever accompanied by ankylosing spondylitis: Case report(Ortadogu Ad Pres & Publ Co, 2008) Altintas, Abdullah; Pasa, Semir; Cil, Timucin; Ayyildiz, OrhanArthritis is the second most common form of familial Mediterranean fever (FMF) attacks; lower extremity joints, such as knees and ankles, are frequent involvement sites for FMF. Sacroiliitis and spinal involvement is the hallmark of ankylosing spondylitis (AS) and a possible but infrequent feature of FMF. About 10% of the patients experience protracted attacks, usually knees or hips are involved, but sacroiliitis occurs only in 2%. We present and discuss a rare case of FMF accompanying to AS. The diagnosis of FMF must be made on clinical grounds. The combination of irregularly recurrent attacks comprising short, febrile episodes of abdominal pain, pleuritic chest pain or arthritis in children or young adults are the main features of diagnosis. FMF should be considered in the differential diagnosis in patients with inflammatory involvement of the lumbar spine and arthritis in the knees or ankles.Öğe Familial Mediterranean fever gene mutations in the Southeastern region of Turkey and their phenotypical features(Taylor & Francis Ltd, 2008) Pasa, Semir; Altintas, Abdullah; Devecioglu, Bilge; Cil, Timucin; Danis, Ramazan; Isi, Hilmi; Bayan, KadimFamilial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent inflammatory attacks of serosal membranes. Several studies have focused on the differences between frequency of the mutations and their phenotypical manifestations. The aim of this study was to evaluate whether or not this phenotypical variation is associated with the existence of particular mutations. Twelve MEFV(Mediterranean fever) gene mutations were investigated in 119 patients suffering from FMF. Heterozygote M694V(21/119), heterozygote E148Q(21/119), homozygote M694V(17/119) and heterozygote V726A(12/119) mutations were the most common mutations. Patients were grouped according to the presence of the M694V mutation: group I was M694V/M694V, group II was M694V/others, and group III was other/other. Mean severity scores for the groups were 13.94 +/- 4.10, 10.79 +/- 3.01 and 8.31 +/- 2.26, respectively. There were statistically significant differences between the mean severity scores of groups I and II(p = 0.029), groups I and III(p < 0.0001), and groups II and III(p < 0.0001). Diagnosis of amyloidosis was established in four(23%) patients of group I, and three(8%) patients of group II, but in none of the patients in group III. There was also a statistically significant difference between groups I and III(p = 0.046), but not between groups II and III(p = 0.083) and groups I and II(p = 0.317) in terms of amyloidosis development. In conclusion, we found a higher disease severity score and higher prevalence of amyloidosis in FMF patients who were M694V mutation carriers. Many ethnic groups live in Anatolia and more ethnic origin-based studies are needed to determine the real effect of these mutations on disease severity and amyloidosis.Öğe Fetal Loss in a Patient with Acute Myeloblastic Leukemia Associated with FLAG-IDA Regime(Akad Doktorlar Yayinevi, 2009) Pasa, Semir; Altintas, Abdullah; Cil, Timucin; Ayyildiz, OrhanAcute leukemia in pregnancy offers a unique management dilemma in the absence of clear guidelines. There is some transporters localized into the placental trophoblasts plays an important role in limiting the passage of substrate drug. Herein, we reported a case of fetal loss in a pregnant patient in 34 gestational weeks, with acute myeloblastic leukemia who treated with high dose cytarabine plus fludarabine chemotherapy, to discuss the effects of cytotoxic agent on fetus. There were many reports about pregnancies complicated with AML treated with standart dose cytosine arabinoside and antracyclin combination. We suggest that high dose chemotherapy, mainly FLAG-IDA regime may cause fetal death, although in nearby term periods. Understanding the role of drug transporters in moderating transplacental passage of substrates has important clinical implications for choosing specific drugs to achieve therapeutic objectives.Öğe Hepatitis B virus reactivation induced by Yttrium-90-ibritumomab-tiuxetan(Taylor & Francis Ltd, 2007) Cil, Timucin; Altintas, Abdullah; Tuzun, Yekta; Pasa, Semir; Isikdogan, Abdurrahman[Abstract Not Available]Öğe Lamivudine for the prevention of hepatitis B virus reactivation in hepatitis-B surface antigen (HBSAG) seropositive cancer patients undergoing cytotoxic chemotherapy(Informa Healthcare, 2008) Cil, Timucin; Altintas, Abdullah; Pasa, Semir; Bayan, Kadim; Ozekinci, Tuncer; Isikdogan, AbdurrahmanHepatitis B virus (HBV) is one of the major causes of chronic liver disease worldwide. Cancer patients who are chronic carriers of HBV have a higher hepatic complication rate while receiving cytotoxic chemotherapy (CT) and this has mainly been attributed to HBV reactivation. In this study, cancer patients who have solid and hematological malignancies with chronic HBV infection received the antiviral agent lamivudine prior and during CT compared with historical control group who did not receive lamivudine. The objectives were to assess the efficacy of lamivudine in reducing the incidence of HBV reactivation, and diminishing morbidity and mortality during CT. Two groups were compared in this study. The prophylactic lamivudin group consisted of 37 patients who received prophylactic lamivudine treatment. The historical controls consisted of 50 consecutive patients who underwent CT without prophylactic lamivudine. They were followed up during and for 8 weeks after CT. The outcomes were compared for both groups. Of our control group (n= 50), 21 patients (42%) were established hepatitis. Twelve (24%) of them were evaluated as severe hepatitis. In the prophylactic lamivudine group severe hepatitis were observed only in 1 patient (2.7%) of 37 patients (p0.006). Comparison of the mean ALT values revealed significantly higher mean alanine aminotransferase (ALT) values in the control group than the prophylactic lamivudine group; 154:64 (p0.32). Our study suggests that prophylactic lamivudine significantly decreases the incidence of HBV reactivation and overall morbidity in cancer patients during and after immunosuppressive therapy. Further studies are needed to determine the most appropriate nucleoside or nucleotide analogue for antiviral prophylaxis during CT and the optimal duration of administration after completion of CT.Öğe Long-term conventional interferon alpha in combination with lamivudine for chronic hepatitis B: Data from Turkey(H G E Update Medical Publishing S A, 2007) Yilmaz, Serif; Bayan, Kadim; Dursun, Mehmet; Canoruc, Fikri; Pasa, Semir; Saka, GuenayBackground/Aims: Standard interferon or lamivudine monotherapy has been shown to induce a low response rate in patients with chronic hepatitis B infection. Genotype D represents almost the whole of chronic HBV infection of Turkish population. The aim of this study was to evaluate the efficacy and safety of the long-term interferon-alpha plus lamivudine on these patients, and thereafter the co-effect of maintenance therapy by lamivudine. Methodology: This prospective study was carried out between the late 1999 and 2005. A total of 37 (24 HBeAg-positive and 13 HBeAg-negative) patients were enrolled in the study. These patients received standard interferon-alpha (9/10 MU) three times sc. a week plus lamivudine 100mg po. daily, for 52 weeks. After the interferon discontinuation, lamivudine monotherapy was assigned to be given until 4-6 months after the occurrence of HBeAg seroconversion in the HBeAg-positive patients and at least three years in HBeAg-negative patients. Response-1 was defined as the response at the end of combination therapy at the 52nd week, and Response-2 as response at the end of the follow-up period under lamivudine monotherapy. An intention-to-treat analysis was performed. Results: Patients' follow-up ranged between 7-67 months, with a mean duration of 29.64 +/- 14.01 months. Twenty-six patients (70.3%) had a Response-1, both virological and biochemical. A biochemical Response-2 was achieved in 24 patients (64.9%), while virological Response-2 in 17 (45.9%). Response-1 and Response-2 were similar between HBeAg-positive and HBeAg-negative patients (p=0.262 and p=0.734, respectively). HBeAg seroconversion was achieved only in 8 (33.3%) of HBeAg-positive patients. Clinical resistance to lamivudine developed only in 9 (24.3%) of the patients. Decompensation or hepatocellular carcinoma did not observe in any case. Conclusions: This study showed the efficacy of the 'long-term' anti-viral maintenance along with the combination therapy in genotype D predominant chronic hepatitis B patients. A low clinical resistance rate to lamivudine was achieved.Öğe Prevalence and clinical significance of elevated antinuclear antibody test in children and adult patients with idiopathic thrombocytopenic purpura(Springer, 2007) Altintas, Abdullah; Ozel, Abdulkadir; Okur, Nilufer; Okur, Nurettin; Cil, Timucin; Pasa, Semir; Ayyildiz, OrhanBackground To determine the clinical significance of the antinuclear antibody (ANA) test in children and adult patients with idiopathic thrombocytopenic purpura (ITP). Method We conducted a retrospective analysis of 365 children and 108 adult patients with ITP. Patients found to have positive ANA were regularly followed-up by an experienced hematologist until December 2006 for development of symptoms indicative of autoimmune disorder. Mean follow-up 3.6 years (range: 2.1-7 years) for all patients. At the time of diagnosis of ITP, patients with connective tissue diseases (CTD) were excluded. Out of 365 childhood ITP; 301 (82.4%) patients were acute, 64 (17.6%) patients were chronic ITP. ANA titer :1:80 were positive in 33 (9.04%) of 365 patients with childhood ITP; 21 patients (6.9%) were in acute, and 12 patients (18.7%) were in chronic group. Out of 108 adult patients with ITP; 31 (28.7%) patients were acute and 77 (71.3%) patients were chronic ITP cases. ANA titer >= 1:80 were positive in 36 (33.3%) of 108 patients with adult ITP; 12 patients (38.8%) were in acute, and 24 patients (31.2%) were in chronic group. At the end of follow-up period Sjogren's syndrome (SS) was diagnosed in only one adult chronic ITP cases. None of the other ANA positive patients developed SLE or other CTD. Conclusions Our data demonstrated that ANA positivity is often found in adult and children patients with ITP, and indicate that the detection of ANA positivity is not enough to identify those patients with ITP who are at risk of developing SLE or other CTD. There is a statistically significant difference in terms of ANA positivity between childhood acute and chronic ITP patients. We think that ANA positivity may be an indicator in terms of chronicity for childhood ITP. However, large-scale studies should be considered to determine the significance of ANA positivity and their utility in differentiating acute from chronic ITP.Öğe Prothrombin Time, Activated Thromboplastin Time, Fibrinogen and D-Dimer Levels and von-Willebrand Activity of Patients with Sheehan's Syndrome and the Effect of Hormone Replacement Therapy on These Factors(Akad Doktorlar Yayinevi, 2010) Pasa, Semir; Altintas, Abdullah; Tumer, Cemil; Demircin, Mustafa; Cil, Timucin; Bayan, Kadim; Danis, RamazanIncreased mortality due to atherosclerotic cardiovascular disease has been described in adult patients with hypopituitarism, although the precise underlying mechanisms remain undetermined. Various abnormalities of coagulation and fibrinolysis occur in patients with thyroid diseases. Conversely, there are conflicting reports concerning the effects of growth hormone replacement on coagulation and fibrinolytic pathways in hypopituitary adults, and there is no existing data on the effects of hypocortisolism on thrombotic and fibrinolytic systems. The same controversial data were also obtained in studies which evaluate the effects of estrogen replacement therapy on cardiovascular events in post-menopausal women. The aim of this study was to investigate the effects of Sheehan's syndrome (SS), which is a common cause of hypopituitarism, on haemostatic factors and to assess the effects of L-thyroxin, prednisolone and conjugated estrogen / medroxyprogesterone acetate replacement on these factors. Prothrombin time (PT), activated thromboplastin time (aPTT), fibrinogen and D-dimer levels, and von-Willebrand factor (vWF) activity were compared among 32 patients with SS and 35 control subjects (CS) with similar age. A shorter PT and aPTT, higher fibrinogen and d-dimer levels, and similar vWF activity were determined in patients with SS as compared with CS. In addition, it was determined that hormone replacement treatment did not have a significant effect on coagulation parameters except the fibrinogen and d-dimer levels.Öğe Pulmonary hypertension in patients with essential thrombocythemia and reactive thrombocytosis(Taylor & Francis Ltd, 2007) Altintas, Abdullah; Karahan, Zulkuf; Pasa, Semir; Cil, Timucin; Boyraz, Taylan; Iltumur, Kenan; Ayyildiz, OrhanIncreased incidence of pulmonary hypertension ( PH) has been reported in patients with chronic myeloproliferative disorders. The exact incidence of PH in essential thrombocythemia ( ET) is unknown. Most of the reported literature consists of case reports or small studies. We designed this study to asses the incidence of PH in patients with ET and reactive thrombocytosis. Previously or newly diagnosed 46 patients with ET, and 40 patients with reactive thrombocytosis secondary to iron deficiency anemia were found to be eligible for this study. Diagnosis of PH was established via transthoracic echocardiography. PH was found in 22 ( 47.8%) out of 46 patients with ET. Seven patients with PH were newly diagnosed ET, 5 patients with PH were in low, and the other patients with PH were in intermediate or high risk category. We found statistically significant difference in terms of platelet counts between ET patients with PH and without PH ( p = 0.027). None of the patients with reactive thrombocytosis had PH. In conclusion, PH appears to be common in patients with ET. Therefore, all patients with ET should be evaluated for PH. Larger and prospective studies are required to clarify the long-term impact of PH on the survival of these patients. Future studies are also needed to determine whether cytoreductive treatment and aspirin prevent the development of PH, and to determine the effects of cytoreductive treatments and aspirin on the prognosis of PH. The effect of PH on ET prognosis should also be determined in low risk ET patients.