Chronic Myeloproliferative Diseases Research Articles

Chronic myeloproliferative disease

Chronic myeloproliferative disease

Time Relationship between the Occurrence of a Thromboembolic Event and the Diagnosis of Hematological Malignancies.

Venous and arterial thromboembolism (VTE/ATE) often coexist with onco-hematologic diagnosis. This study aimed to assess the time relationship between the diagnosis of VTE/ATE and blood cancers. The second aim was to identify VTE/ATE risk factors related to the type of hematology disease and cardiac history. A total of 1283 patients underwent cardio-oncology evaluation at the Institute of Hematology and Transfusion Medicine in Warsaw from March 2021 through March 2023 (2 years), and 101 (7.8%) cases were identified with VTE/ATE. ATE compared with VTE significantly occurred more often before the diagnosis and treatment of hematologic malignancy: 33/47 (70.2%) vs. 15/54 (27.8%), p < 0.0001. The risk of a VTE episode is exceptionally high in the first months after the diagnosis of an onco-hematological disease and the initiation of anticancer treatment. The higher frequency of VTE was associated with acute myeloid leukemia (17 cases/270 patients/6.30%/p = 0.055), acute lymphocytic leukemia (7 cases/76 patients/9.21%/p = 0.025), and chronic myeloproliferative disease (7 cases/48 patients/14.58%/p = 0.0003). Only the risk of VTE was significantly increased before (OR = 6.79; 95% CI: 1.85-24.95; p = 0.004) and after diagnosis of myeloproliferative disease (OR = 3.12; 95% CI: 1.06-9.16; p = 0.04). ATEs occur more often than VTE before a diagnosis of blood cancer. The risk of VTE is exceptionally high before and after diagnosis of chronic myeloproliferative disease.

The investigation of janus kinase 2 and calreticulin mutations in patients with essential thrombocytosis

Aims: The aims of this study were to investigate the frequency of Janus Kinase 2 (JAK2) and calreticulin (CALR) mutations in patients with essential thrombocytosis (ET) and primary myelofibrosis (PMF) and to compare the data of each group with JAK2 and CALR mutations (+) and (-). Methods: The research group consisted of 80 patients with chronic myeloproliferative disease (CMPD) followed in Gazi University Hospital of Medical Faculty, Hematology Clinic. Results: Of the patients included in the study, 66.2% had ET and 33.8% had PMF. JAK2 mutation (+) was detected in 60% and CALR mutation (+) was found in 22.5% of the patients. JAK2 mutation (+) was detected in 60.4% of patients with ET and 59.3% of patients with PMF. In JAK2 (-) patients, CALR was detected as (+) in 11 patients (52.4%) with ET and 7 patients (63.6%) with PMF. Conclusion: Studies in the literature have shown that the incidence of CALR mutations in patients with CMPD is between 28% and 80% and that the mutation is mostly seen in patients with ET. As a result of our study, it was concluded that the laboratory findings of patients with CALR mutations (+) were similar to those in the literature and were more common in women and ET patients.

A Clinical Case of Timely Diagnosis and Successful Treatment of Budd-Chiari Syndrome With Fulminant Cytolysis in the Setting of a First-time Diagnosed Myeloproliferative Disease.

The article presents a clinical case of peculiarities of clinical manifestations, diagnostic and therapeutic approaches of undiagnosed chronic myeloproliferative disease, on the background of which Budd-Chiari syndrome (BCS) developed. The results of clinical course, examination, and treatment of a patient with BCS as a manifestation of the hidden course of primary myelofibrosis with the presence of somatic mutation (V617F) in Janus-tyrosine kinase-2 (JAK2) gene in myeloid cells are presented. Standard clinical and laboratory examinations, and cytomorphologic and histologic examination of bone marrow were used. The diagnosis of BCS was confirmed by ultrasound (US) Doppler examination of the portal system vessels. Symptomatic therapy resulted in insignificant positive results. The analysis of this clinical case showed that the development of BCS was due to a chronic myeloproliferative disease that was not diagnosed before the development of thrombosis. Hepatic vein thrombosis was accompanied by the development of fulminant cytolytic syndrome. Along with symptomatic therapy, patient K., female, 32 years old, underwent transjugular intrahepatic portosystemic shunting 1 month after the first symptoms of BCS appeared, which contributed to a significant clinical effect. Seven years after the installation of 4 transjugular intrahepatic portosystemic shunts, the patient's condition remains satisfactory. The uniqueness of this clinical case lies in the presence of 2 serious diseases at the same time: myeloproliferative pathology (primary myelofibrosis) JAK2-positive variant and BCS. Timely diagnosis of both hematological diseases and their complication in the form of hepatic vein thrombosis with fulminant cytolytic syndrome allowed timely prescription of adequate treatment with a good clinical response.

Comparison of Polystemia Vera Patients Diagnosed Before and After the Updated Diagnostic Criteria

Polycythemia vera is a chronic, clonal and progressive myeloproliferative disease characterized by leukocytosis, thrombocytosis and splenomegaly with increased erythroid series. In this study, we aimed to compare the clinical and laboratory features of patients diagnosed with PV according to the 2008 and 2016 WHO diagnostic criteria and the complications that developed during follow-up, and to retrospectively evaluate the impact of the change in diagnostic criteria on real life. A total of 100 polycythemia vera patients with positive JAK2-V617F mutation were enrolled in the study, 50 between 2011-2015 and 50 between 2016-2020. When the two groups were compared in terms of laboratory values at the time of diagnosis, hemoglobin, hematocrit and red blood cell count were significantly higher in the group diagnosed before 2016 (p=0.036). When patients were evaluated for complications of thrombosis, hemorrhage, progression to myelofibrosis, leukemic transformation, and transformation of myelodysplastic syndrome at last follow-up, it was found that 19 (38%) patients in the pre-2016 group and 5 (10%) patients in the post-2016 group developed complications (p=0.002). The number of patients who developed a myelofibrosis complication was significantly higher in the pre-2016 group (p=0.006). The number of patients who developed thrombosis was higher in the pre-2016 group, but the difference did not reach a statistically significant level (p=0.055). About other complications, there was no difference between the two groups (p &amp;gt; 0.05). In summary, our study found a significant difference only in the number of patients with myelofibrosis complications. Further studies with more patients will be needed to determine the impact of the 2016 change in diagnostic criteria on other complications.

Polistemi Vera ve Esansiyel Trombositemi Tanısı Alan Hastalarda Elektrokardiyografik ve Ekokardiyografik Bulgularının Değerlendirilmesi

Background/Aims: Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative diseases. Along with the increase in myeloproliferative cell lines, they can cause various clinical outcomes such as arterial and venous thrombosis, pulmonary hypertension, and myocardial infarction. This study was designed to evaluate the cardiac effects of these diseases via electrocardiography and echocardiography. Methods: A total of 50 patients with a diagnosis of PV, 50 patients with a diagnosis of ET, and 50 healthy individuals as a control group were included in this study. Data on patient demographics were recorded in all subjects. All patients and control subjects had electrocardiography (ECG) recordings and routine transthoracic echocardiographic examination. Pulse wave velocity (PWV) was assessed with a Holter blood pressure device. Results: In total, 50 PV patients, 50 ET patients, and 50 control group were included in the study. The demographic characteristics of the PV, ET and control groups were similar. The PR interval was significantly shorter in control subjects than in PV and ET patients (p:0.007, p:0.024). Although the measured values were within normal limits, diastolic posterior wall thickness was significantly lower in the control group compared to PV and ET patients (p:0.019, p:0.009). PWV was significantly higher in ET patients compared to the control group (p:0.012). Conclusion: In this study, evaluating the effects of PV and ET on electrocardiography and transthoracic echocardiography; ECG parameters used to predict ventricular arrhythmias (QT, QTc, Tp-Te, Tp-Te/QT) and Pulmonary Artery Pressure showed no significant change, in opposition to existing literature. Nonetheless, similar to previous publications, PV and ET were found to negatively affect the diastolic function parameters on transthoracic echocardiography. While the aortic stiffness was significantly higher in ET patients compared to the control group, no significant difference was noted between PV patients and control subjects in terms of aortic stiffness.

Диаметр оболочек зрительного нерва при хронических миелопролиферативных заболеваниях

Aim: to evaluate the optic nerve sheath diameter (ONSD) in patients with chronic myeloproliferative disorders (MPD) using ultrasound (US). Patients and Methods: the study included 32 patients (64 eyes) with established chronic MPD and 28 gender- and age-matched healthy volunteers (56 eyes) without chronic MPD. Several patients had a history of cardiovascular diseases, which may be related to long-term use of specific target therapy. All patients underwent a comprehensive eye examination, which included eye and orbit ultrasonography, as well as laboratory tests such as complete blood count (CBC) and coagulation test. Results: the majority (81.3%) of patients had either unilateral or bilateral increases in ONSD. Furthermore, ONSD was found to be significantly greater in patients with chronic MPD who also had increased white blood cell count, platelet count, and fibrinogen levels, as well as decreased hemoglobin levels, compared to those without a history of chronic MPD. The correlation was stronger in patients with chronic MPD than in the control group. Correlations were found between ONSD and all studied hematologic parameters. A moderate negative correlation was found between ONSD and hemoglobin, a weak positive correlation between ONSD and WBC count, and a moderate positive correlation between ONSD and platelet and fibrinogen in patients with chronic MPD. Conclusion: an increase in ONSD was detected by eye and orbit US in patients with chronic MPD who also had hematologic abnormalities. This symptom may be an early sign of vascular complications secondary to long-term specific treatment. An increase in ONSD can illustrate increased intracranial pressure. For patients with chronic MPD, it is recommended to have more detailed follow-up to prevent vision loss and life-threatening complications in the central nervous system due to disease progression, in particular, those with an increase in ONSD. KEYWORDS: chronic myeloproliferative diseases, optic nerve sheath diameter, ultrasound, blood rheology, vascular complications, chronic myeloid leukemia, true polycythemia, essential thrombocythemia, intracranial hypertension. FOR CITATION: Yunusova E.M., Mukhamadeev T.R., Bakirov B.A. Optic nerve sheath diameter in chronic myeloproliferative disorders. Russian Journal of Clinical Ophthalmology. 2024;24(2):55–59 (in Russ.). DOI: 10.32364/2311-7729-2024-24-2-2.

Is Ruxolitinib an Effective and Safe Therapy for Chronic Myeloproliferative Diseases?

Is Ruxolitinib an Effective and Safe Therapy for Chronic Myeloproliferative Diseases?

Investigation of Blood Supply to the Macular Region of the Retina in Chronic Myeloproliferative Diseases

Chronic myeloproliferative diseases (CMPD) are tumor diseases of hematopoietic tissue with increased proliferation of one or more lines of myelopoiesis. Quite often with this pathology there are find references to damage to the organ of vision. With CMPD changes in the composition of peripheral blood are observed, leading to changes in the rheology of blood and contributing to the deterioration of microcirculation of eye tissues. In addition, abnormal proliferation of hematopoietic cells and prolonged specific therapy weaken the body’s immune system, contributing to the development of systemic inflammation. These processes can lead to deterioration of blood supply to eye tissues, in particular in the macular region. Purpose. To evaluate vessel density (VD) in the superficial and deep vascular plexuses of retina and the area of foveolar avascular zone (FAZ) using optical coherence tomography with angiography function (OCTA) in patients with CMPD. Patients and methods. The study included 25 patients with CMPD (50 eyes) and 20 volunteers (40 eyes), comparable in gender and age, without a history of hemoblastosis. All the examined patients underwent comprehensive ophthalmological diagnostics, including OCTA. Results. According to the results, patients with CMPD revealed a significant decrease in VD in all sectors (except the foveolar zone) in both studied vascular plexuses, as well as a significant increase in area of FAZ compared to the control group. Conclusion. A decrease in VD in the vascular plexuses of the retina, as well as an increase in area of FAZ, are signs of deterioration of retinal blood supply and retinal ischemia. It is possible that these changes may lead to violations of blood rheology, as well as chronic systemic inflammation observed in patients with CMPD. Deterioration of blood flow and prolonged retinal hypoxia can contribute to serious visual impairment of patients. In this regard, patients with CMPD require more detailed monitoring, possibly with correction of the main treatment.

MYELOPROLIFERATIVE DISEASES AS A CAUSE OF ACUTE CEREBROVASCULAR ACCIDENT

Among the hemorheological forms of thrombophilia, chronic myeloproliferative diseases (CMPD) play an important role: polycythemia vera, essential thrombocythemia, primary myelofibrosis, the complications of which are arterial and venous thrombosis, including acute cerebrovascular accident (ACV). Identification of changes in the hemogram in such patients requires a thorough examination to exclude CMPD: monitoring of hemogram parameters in dynamics, hemostasiological examination, and determination of the level of erythropoietin, spleen size, and molecular-genetic study. Patients with chronic MPD and stroke need to start cytoreductive therapy (α-interferons, hydroxyurea) in a timely manner, conduct active long-term antithrombotic treatment (anticoagulants, antiplatelet agents) in order to prevent thromboembolism and improve the prognosis of the disease.

The atherogenic index of plasma complicates the thrombotic tendency of chronic myeloproliferative disorders: A retrospective cohort study

Objectives: Chronic myeloproliferative diseases (CMPD) are neoplastic disorders leading to hypercoagulability and thrombosis. The critical hemostatic abnormalities include alterations in the blood viscosity and a history of recent thrombus. The aim of this study is to assess the interrelationships among the atherogenic index of plasma (AIP) and thromboembolism of CMPD with JAK2 V617F mutation. &#x0D; Methods: Ninety-two patients diagnosed as CMPD with JAK2 V617F mutation and 73 controls were included into the study. The patients were evaluated for the presence of any venous or arterial thromboembolic events. AIP was calculated by using the formula log (Tg/HDL) from serum triglyceride and high-density lipoprotein values.&#x0D; Results: The study group consisted of 30 patients (33%) with myelofibrosis (MF), 42 patients (46%) with polycythemia vera (PV) and 20 patients (21%) with essential thrombocythemia (ET). Two study groups were similar in terms of sex, age and other comorbidities (p &gt; 0.05). CMPD group had higher levels of right blood cell count (RBC), red blood cell distribution width (RDW), platelets (PLT), hemotocrit (Hct) and AIP. Univariate and multivariate logistic regression analysis revealed that platelet count, RBC and AIP were independent predictors for thrombosis in both groups. The comparison of ROC curve analysis disclosed that AIP was superior to platelet count and RBC in predicting thrombosis.&#x0D; Conclusion: AIP can be used to determinate higher risk of thromboembolism in patients with CMPD. As a reliable and ‘easy-to-assess’ diagnostic tool, AIP could be useful for the determination of thrombotic events in CMPD clinicobiological disease course.

Total Skin Treatment with Helical Arc Radiotherapy.

For widespread cutaneous lymphoma, such as mycosis fungoides or leukemia cutis, in patients with acute myeloid leukemia (AML) and for chronic myeloproliferative diseases, total skin irradiation is an efficient treatment modality for disease control. Total skin irradiation aims to homogeneously irradiate the skin of the entire body. However, the natural geometric shape and skin folding of the human body pose challenges to treatment. This article introduces treatment techniques and the evolution of total skin irradiation. Articles on total skin irradiation by helical tomotherapy and the advantages of total skin irradiation by helical tomotherapy are reviewed. Differences among each treatment technique and treatment advantages are compared. Adverse treatment effects and clinical care during irradiation and possible dose regimens are mentioned for future prospects of total skin irradiation.

THE VALUE OF MUTATIONS IN THE ASXL1 GENE IN THE ONCOGENESIS OF ACUTE MYELOID LEUKEMIA IN ADULTS

The aim was to evaluate the significance of the ASXL1 gene mutations in the oncogenesis of acute myeloid leukemia (AML) in adults. Materials and methods. Bone marrow and peripheral blood samples of 49 patients with AML aged from 18 to 84 years, treated at the Sverdlovsk regional hematological center, were examined, including those with a morphological variant of AML M0 – 3, M1 – 4, M2 – 19, M3 – 6, M4 – 10, M5 – 1, M6 – 1, M7 – 1, blast plasmacytoid dendritic cell neoplasm – 1, acute hybrid leukemia – 2, AML after chronic myeloproliferative disease (CMPD) – 2. Mutations in the ASXL1 gene were detected in peripheral blood and bone marrow samples by direct RNA sequencing (n=26) and high-throughput DNA sequencing (n=23) on automatic genetic analyzers ABI Prism 310 and MiSeqDX, respectively. Results. It was found that pathogenetically significant mutations of the ASXL1 gene were detected in 16.3% of samples with AML M0, M2, M4, AML from CMPD and acute hybrid leukemia. The distribution of ASXL1 anomalies was as follows: deletion n. Del (1755;2007) — 4, duplication c.1934dupG — 3, non-synonymous transversion c.1348A&gt;T — 1. Along with lesions of the ASXL1 gene, quantitative and structural chromosomal aberrations were determined in these samples, including del(5)(q13), t(8;21)(q22;q22), t(9;22)(q34;q11), as well as point mutations in the CEBPA, IDH2, NRAS, RUNX1, SRSF2, TET2 genes, internal tandem duplication in the FLT3 gene. This indicates the cooperation of two or more mutational events in the oncogenesis of ASXL1-positive AML, which can determine the different reaction of the tumor to chemotherapeutic treatment and the prognosis of the disease.

Dyspnea as a symptom of progressive chronic myeloproliferative disease: a rare case of hemorrhagic alveolitis and pulmonary fibrosis, due to extramedullary erythropoiesis

Dyspnea as a symptom of progressive chronic myeloproliferative disease: a rare case of hemorrhagic alveolitis and pulmonary fibrosis, due to extramedullary erythropoiesis

Right portal vein thrombosis from idiopathic cause: case report

The aim of this study was to describe the case report of a 33-year-old girl with portal vein thrombosis. J.S.D.F., 33 years old, female, previously healthy, with a personal and family history negative for thromboembolic phenomena, without comorbidities, started with severe abdominal pain in the mesogastric region, radiating to HCD and back, associated with vomiting. Smoking active for about 20 years and using OC for 2 years. In view of the symptoms, she sought emergency services, where the first suspicion would be pancreatitis. Initial tests plagued leukocytosis. GUS of the total abdomen with doppler and CT of the abdomen showed the presence of thrombus in the right portal vein, with no other alterations. Rocefin® and metronidazole as an initial measure in the service began in addition to full coagulation with Clexane®. It showed clinical and laboratory improvement, with a reduction of leukocytes. The patient remained hospitalized in a hospital for 22 days to investigate the etiological cause. After of hospitalization, the patient was released with medical discharge, with guidelines for seeking the hematology service. During the investigation consultations, the results of the requested tests were negative for the diagnostic suspicions of nocturnal paroxysmal hemoglobinuria and chronic myeloproliferative disease. After 6 months of follow-up, she was discharged from the hematology service because she did not present a suspected disease and classified the cause of thrombolytic event as idiopathic associated with the combined use of OC and smoking.

A RARE ASSOCIATION IN A CASE WITH HEREDITARY SPHEROCYTOSIS: SPECTRIN BETA (SPTB) AND JAK-2 MUTATION

Five types of gene variants are seen in hereditary spherocytosis (ANK, SPTB, SPTA1, SLC4A1, EPB42). JAK2 V617F mutation; is most common seen in bcr-abl negative chronic myeloproliferative diseases. As a result of NGS performed before splenectomy, SPTB c.4973+2T&gt; C and JAK-2 c.1849G&gt;T p.(Val617Phe) mutations were detected. Co-occurrence of these two mutations requires special attention in terms of the management of thrombocytosis and side effects that may occur after splenectomy.

Kaposi’s sarcoma in HIV-negative patient with primary myelofi brosis

Introduction. Kaposi’s sarcoma (КS) is a multi-focal malignant tumor of vascular origin with a predominant lesion of the skin, involving internal organs and lymph nodes. Epidemic and immunosuppressive variants of the disease are considered to be the most studied and common. The main reason for the development of the immunosuppressive variant is the use of immunosuppressive drugs in patients after transplantation of solid organs. Descriptions of the development of Kaposi’s sarcoma in patients with diseases of the hematopoietic system are rare.Aim — to describe a rare combination of a malignant tumor of vascular origin (Kaposi’s sarcoma) and a chronic myeloproliferative disease: primary myelofi brosis.Main findings. To verify the diagnosis of Kaposi’s sarcoma in a patient with primary myelofi brosis, the following were used: histological, immunohistochemical studies of skin biopsies and the polymerase chain reaction method with detection of a fl uorescent signal in “real time” in biological material samples (peripheral blood, smear from the mucous membrane of the oropharynx, skin biopsies from lesions) with quantitative determination of the activity of the human herpes virus 8. A comprehensive examination made it possible to establish the fi nal diagnosis of multiple malignant neoplasms and recommend treatment.

Polisitemi Veralı Hastalarda Dinamik Thiol/Disulfit Dengesi ve İskemi Modifiye Albumin Düzeyleri

Aim: Polycythemia vera is a chronic myeloproliferative disease characterized by increased red cell mass and JAK2 mutation positivity. Transformation to myelofibrosis and acute leukemia is possible in patients with polycythemia vera. Oxidative stress causes DNA damage and might be a reason for malignant transformation. Thiol molecules can prevent the harmful effects of oxidative stress. Therefore, in this study, we aimed to analyze the state of thiol homeostasis in patients with polycythemia vera. Material and Methods: Thirty-one patients with polycythemia vera and 80 healthy volunteers were included in this study. Serum samples of the cases were stored until the end of the study. Native thiol, total thiol, disulfide, and ischemia modified albumin levels were determined. Results: The mean ischemia modified albumin (1.09±0.21 vs 0.67±0.08; p&amp;lt;0.001, mean disulfide (23.5±6.1 vs 10.7±2.6; p&amp;lt;0.001), the mean disulfide/native thiol ratio (5.6±1.1 vs 3.1±1.2; p&amp;lt;0.001), the mean disulfide/total thiol ratio (5.0±0.9 vs 2.9±1.0; p&amp;lt;0.001), the mean native thiol (418.9±80.6 vs 371.4±103.7; p=0.024), the mean total thiol (466.0±89.8 vs 393.0±105.5; p=0.001) and the mean disulfide/total thiol ratio (89.8±1.8 vs 94.1±2.0; p&amp;lt;0.001) were found higher in polycythemia vera patients. Ischemia modified albumin levels were also higher in high-risk polycythemia vera patients. Patients on ruxolitinib therapy had higher native thiol, total thiol and disulfide levels, and higher disulfide/native thiol and disulfide/total thiol ratios. Conclusion: Oxidative stress markers are still high in patients with polycythemia vera who were under treatment. Besides, ruxolitinib may be helpful to decrease oxidative stress in these patients.

Erectile Dysfunction after Allogeneic Hematopoietic Stem Cell Transplant: Results from a Single Center Experience

▪Erectile dysfunction (ED) is a multidimensional disturbance of the erectile response involving organic, relational and psychological components, which has been reported to affect a significant proportion of patients after allogeneic hematopoietic stem cell transplant (HSCT). Although predictors for ED have been established in the general population, few studies have addressed which variables are associated with ED in HSCT patients.Aims of the study were to establish prevalence and extent of ED in 55 consecutive patients transplanted in our Institution between 2003 and 2015, and to identify within socio-demographic, medical and psychological domains eventual ED predictors.Patients were enrolled in the study upon informed consent. Inclusion criteria were age ≥ 18 years, continuous disease remission, and ≥ 6 months from HSCT. Exclusion criteria were concurrent malignancy, major psychiatric disorder or mental retardation, and hormonal replacement.ED was assessed and measured by the International Index of Erectile Dysfunction (IIEF15).Patient socio-demographics were collected by an ad hoc schedule.Medical data were obtained from hospital records. The andrological visit included testicular measure, penis and genital skin inspection, and penile ultrasound and elastosonography. Free testosterone (FT), inhibin B, follicle stimulating hormone (FSH), luteinizing hormone (LH), prolactin, thyroid stimulating hormone (TSH), free triiodothyronine 3 (FT3), FT4, cortisol, and adrenocorticotropic hormone (ACTH) levels were measured.Anxiety was quantified by the State-Trait Anxiety Inventory (STAI), depression by the Zung Self-rating Depression Scale (SDS).Differences between groups were analyzed by the non-parametric Mann-Whitney test and the Fisher's exact test when testing quantitative and categorical variables, respectively. Logistic regression analysis was used to estimate the association of the variables predictive for ED, when including into the association model the relevant covariates. Chronic graft versus host disease (cGVHD) was diagnosed according to NIH criteria.Fifty-two (94.5%) of the 55 patients eligible for the study returned the self-administered questionnaires and underwent the andrological visit: 32 had acute leukemia, 7 non-Hodgkin lymphoma, 4 myelodysplastic syndrome, 4 chronic myeloproliferative disease, 3 multiple myeloma, 2 B cell chronic lymphocytic leukemia.Median time from HSCT to study assessment was 19 (6-108) months. Thirty-nine patients (75%) had hypogonadism, while 10 (19.2%) presented genital cGVHD, with post-HSCT phimosis in 7 cases, meatal scarring in 2, lichen sclerosis-like lesions in 1. At penile ultrasound and elastosonography, 12 (23%) and 7 (13.5%) patients had a medium-hard and hard penile elasticity.FT and inhibin B were below the range limit in 34 (65.4%) and 33 (63.5%) patients, while FSH was above in 47 (90.4%), thus indicating a large prevalence of gonadal failure. Three patients (5.7%) had a mild hypotyrodism. No prolactin, cortisol or ACTH alterations were observed.Forty-seven (90.4%) of the 52 participants declared a stable sexual partner: the 5 patients [median age 42 (19-50) years, 1 with cGVHD] who reported not having any sexual activity due to partner absence were excluded from the analysis of ED predictors according to IIEF15 requirements.ED was reported by 31/47 (66%) patients: it was severe in 42% cases, moderate in 22.6%, moderate to mild in 6.4% and mild in 29%. There was no difference between patients with and without ED in terms of time from HSCT [25 (6-108) vs 15 (6-79) months, p=0.32].In univariate analysis higher age, low education level, and cGVHD (excluding the isolated genital one) were significantly associated to ED, while the same variables in addition to hypertension maintained their significance in the subgroup of patients with moderate-severe ED only (Table 1). In a logistic regression model, age and cGVHD were the only variables independently associated with ED (any grade, p=0.02 and moderate-severe, p=0.0001, respectively).Our study confirms that ED is a frequent long-term complication of HSCT, with cGVHD being a major predictor. We suggest that an early screening of sexual dysfunction and a close partnership between HSCT clinicians and andrologists could help to implement reliable prevention strategies and/or prompt interventions for patients suffering from ED. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Spleen Stiffness Measurement by Using Shear-Wave Elastography as a Predictor of Progression to Secondary Myelofibrosis.

Polycythemia vera (PV) and essential thrombocythemia (ET) are chronic myeloproliferative diseases that can transform to secondary myelofibrosis (SMF). In this study, we evaluated spleen stiffness using shear-wave elastography (SWE) as a predictor of progression to SMF. Participants were grouped as healthy volunteers (HVs), PV/ET patients, and SMF patients. Participants' spleen sizes, spleen stiffness values, bone marrow fibrosis degrees, and the other parameters were evaluated. Spleen stiffness values and spleen sizes were compared between groups. Of the 121 participants included in this study, 52 patients were HVs, 52 patients were PV and/or ET patients, and 17 patients were SMF patients. In terms of age and sex, there was no difference between groups. Splenic parenchymal stiffness median values by using SWE were found to be 0.82 m/s in HVs, 1.41 m/s in PV/ET patients, and 2.32 m/s in SMF patients (P < 0.001). In terms of median length of the spleen, the difference between groups was significant (P < 0.001). In addition, we found a significant positive correlation between spleen stiffness and bone marrow fibrosis degree (P < 0.001, r = 0.757). However, in multivariate analysis, there was no strong independent risk factor for spleen stiffness. In this study, we showed that measurement of spleen stiffness using SWE can distinguish SMF from PV/ET patients and HVs. Therefore, we believe that SWE may be used as a noninvasive and easily accessible method to check the fibrotic progression of bone marrow in PV and ET patients to monitor the transformation to SMF, and enables to detect fibrosis in early phase.