Academic literature on the topic 'Chronic Myeloproliferative disorders, chronic idiopathic Myelofibrosis, Pulmonary hypertension'

Abstract The Philadelphia chromosome-negative chronic myeloproliferative disorders (CMPD), polycythemia vera (PV), essential thrombocythemia (ET) and chronic idiopathic myelofibrosis (IMF), have overlapping clinical features but exhibit different natural histories and different therapeutic requirements. Phenotypic mimicry amongst these disorders and between them and nonclonal hematopoietic disorders, lack of clonal diagnostic markers, lack of understanding of their molecular basis and paucity of controlled, prospective therapeutic trials have made the diagnosis and management of PV, ET and IMF difficult. In Section I, Dr. Jerry Spivak introduces current clinical controversies involving the CMPD, in particular the diagnostic challenges. Two new molecular assays may prove useful in the diagnosis and classification of CMPD. In 2000, the overexpression in PV granulocytes of the mRNA for the neutrophil antigen NBI/CD177, a member of the uPAR/Ly6/CD59 family of plasma membrane proteins, was documented. Overexpression of PRV-1 mRNA appeared to be specific for PV since it was not observed in secondary erythrocytosis. At this time, it appears that overexpression of granulocyte PRV-1 in the presence of an elevated red cell mass supports a diagnosis of PV; absence of PRV-1 expression, however, should not be grounds for excluding PV as a diagnostic possibility. Impaired expression of Mpl, the receptor for thrombopoietin, in platelets and megakaryocytes has been first described in PV, but it has also been observed in some patients with ET and IMF. The biologic basis appears to be either alternative splicing of Mpl mRNA or a single nucleotide polymorphism, both of which involve Mpl exon 2 and both of which lead to impaired posttranslational glycosylation and a dominant negative effect on normal Mpl expression. To date, no Mpl DNA structural abnormality or mutation has been identified in PV, ET or IMF. In Section II, Dr. Tiziano Barbui reviews the best clinical evidence for treatment strategy design in PV and ET. Current recommendations for cytoreductive therapy in PV are still largely similar to those at the end of the PVSG era. Phlebotomy to reduce the red cell mass and keep it at a safe level (hematocrit < 45%) remains the cornerstone of treatment. Venesection is an effective and safe therapy and previous concerns about potential side effects, including severe iron deficiency and an increased tendency to thrombosis or myelofibrosis, were erroneous. Many patients require no other therapy for many years. For others, however, poor compliance to phlebotomy or progressive myeloproliferation, as indicated by increasing splenomegaly or very high leukocyte or platelet counts, may call for the introduction of cytoreductive drugs. In ET, the therapeutic trade-off between reducing thrombotic events and increasing the risk of leukemia with the use of cytoreductive drugs should be approached by patient risk stratification. Thrombotic deaths seem very rare in low-risk ET subjects and there are no data indicating that fatalities can be prevented by starting cytoreductive drugs early. Therefore, withholding chemotherapy might be justifiable in young, asymptomatic ET patients with a platelet count below 1,500,000/mm3 and with no additional risk factors for thrombosis. If cardiovascular risk factors together with ET are identified (smoking, obesity, hypertension, hyperlipidemia) it is wise to consider platelet-lowering agents on an individual basis. In Section III, Dr. Gianni Tognoni discusses the role of aspirin therapy in PV based on the recently completed European Collaboration on Low-dose Aspirin in Polycythemia Vera (ECLAP) Study, a multi-country, multicenter project aimed at describing the natural history of PV as well as the efficacy of low-dose aspirin. Aspirin treatment lowered the risk of cardiovascular death, non-fatal myocardial infarction, and non-fatal stroke (relative risk 0.41 [95% CI 0.15–1.15], P = .0912). Total and cardiovascular mortality were also reduced by 46% and 59%, respectively. Major bleedings were slightly increased nonsignificantly by aspirin (relative risk 1.62, 95% CI 0.27–9.71). In Section IV, Dr. Giovanni Barosi reviews our current understanding of the pathophysiology of IMF and, in particular, the contributions of anomalous megakaryocyte proliferation, neoangiogenesis and abnormal CD34+ stem cell trafficking to disease pathogenesis. The role of newer therapies, such as low-conditioning stem cell transplantation and thalidomide, is discussed in the context of a general treatment strategy for IMF. The results of a Phase II trial of low-dose thalidomide as a single agent in 63 patients with myelofibrosis with meloid metaplasia (MMM) using a dose-escalation design and an overall low dose of the drug (The European Collaboration on MMM) will be presented. Considering only patients who completed 4 weeks of treatment, 31% had a response: this was mostly due to a beneficial effect of thalidomide on patients with transfusion dependent anemia, 39% of whom abolished transfusions, patients with moderate to severe thrombocytopenia, 28% of whom increased their platelet count by more than 50 × 109/L, and patients with the largest splenomegalies, 42% of whom reduced spleen size of more than 2 cm.

The article presents a brief review of literature which shows the characteristic of bronchopulmonary and cardiovascular diseases that most often cause secondary absolute hypoxic erythrocytosis. The information about pathogenesis of blood changes at this nosology is given. The principles of differential diagnosis between secondary absolute hypoxic erythrocytosis and chronic myeloproliferative diseases, i.e. polycythemia vera and idiopathic myelofibrosis in erythremia stage are set. As an example three clinical cases of personal experience of the authors are given: 1) the patient who developed hypoxic erythrocytosis against chronic pulmonary heart disease formed due to a combination of COPD and Pickwick syndrome and "sleep apnea"; 2) the patient admitted to hospital initially only in connection with changes in the blood (erythrocytosis) for differential diagnosis and who was revealed to have arteriovenous malformation of the lung vessels; 3) the case of primary pulmonary hypertension diagnosis in a young patient with multiple comorbidities.

Abstract Introduction: The presence of pulmonary hypertension (PH) in myeloproliferative disorders (MPD) has been evaluated in several small studies with mainly echocardiographic assessment of the right ventricular systolic pressures (RVSP). Left-sided heart disease is the most common cause of PH, and the incidence of diastolic dysfunction increases with age. Since MPD is more prevalent in older patients, it is unknown how much of PH is related to diastolic dysfunction. In addition, the presence and effect of concomitant cardiovascular diseases on overall mortality in these patients is unknown. To assess above knowledge gaps, we evaluated the relationship of PH, cardiovascular diagnoses and echocardiographic findings in a population of patient with myelofibrosis (MF). Methods: Data from MF patients who underwent formal echocardiogram from 10/1/2015 to 5/20/2017 at MD Anderson Cancer Center was collected. Clinical, radiographic, laboratory and echocardiographic data were reviewed. We performed Kaplan-Meier survival analysis focusing on cardiovascular diagnoses, laboratory values and echocardiographic variables. We determined a variable to be statistically significant from Kaplan-Meier if p-value from Log Rank (Mantel-Cox) was <0.05. Statistical software used was SPSS. Results: One hundred fifty-one patients (72 females, 79 males) were identified. The median age was 68.5 ± 14.09 years. ECOG performance status was 0 (38%), 1 (55%) and 2 (6%). Splenomegaly was present in 36%, and 40% were undergoing active treatment. Respiratory complaints included dyspnea (13%), lower extremity edema (6%) but no chest pain or syncope. Comorbid conditions included hypertension (57%), hypothyroidism (25%), dyslipidemia (23%), chronic kidney disease (12%), coronary artery disease (11%), diabetes (9%), atrial fibrillation (AF) (4%) and thromboembolic disease (7%). RVSP data was available in 103 patients. Evidence of PH by echocardiogram was found in 20%, and PH with normal diastolic function (presumptively pre-capillary PH) was found in 6%. Laboratory data revealed median hemoglobin 10.4 gm/dL (5.2-116.6), white blood cell count 8.7 x 10 K/uL (1.7-156.9), and platelets 227 K/uL (14-1432). No right heart catheterization was performed. Survival analyses revealed that a diagnosis of AF, a dilated left atrium (>34 mL/m2) and a low RVs' (<10 cm/s) (parameter of RV systolic function) were associated with a worse prognosis, and the strongest association with mortality was AF (p<0.001). The presence of other comorbidities, PH by echocardiogram, increased right ventricle size or decreased left ventricular systolic function were not statistically significant for increased mortality. Conclusion: In our cohort of MF, the incidence of pre-capillary PH on echocardiogram was 6%. The presence of AF and echocardiographic findings of left atrial dilatation and decreased RV systolic dysfunction by RVs' are strongly related to worse prognosis. Of which the presence of AF was the most robust. Traditional PH variables in echocardiogram are not related to overall prognosis in this population. Further study is needed to evaluate the impact of cardiovascular disease in MF. Figure. Figure. Disclosures Verstovsek: Celgene: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau; Italfarmaco: Membership on an entity's Board of Directors or advisory committees; Incyte: Consultancy.

Abstract In patients with myeloproliferative disorders, the increased cellular turnover may lead to hyperhomocysteinemia (HH), caused by depletion of folate and/or cobalamin (VitB12). HH represents a cause of oxidative stress, associated with a number of diseases, among which cardiovascular events play an important role. Recently, holoTC, that consist of VitB12 linked to its transport molecule, transcobalamin, has been proposed as a more reliable marker than the dosage of total serum VitB12 to detect the deficiency of this vitamin. The aim of the study was to investigate the clinical role of oxidative stress in CIMF. We performed in 67 CIMF patients (M/F: 38/29; median age 63, range 34–79), the dosage of reactive oxygen species (ROS), total antioxidant capacity (TAC), Hcy, VitB12, folates and holoTC, and correlated the results with the following clinical variables: age, sex, cytogenetics, white blood cells and platelets counts, hemoglobin and LDH levels, splenomegaly and hepatomegaly, hypertension, diabetes, smoking habit, cardiovascular events, secondary tumors, and presence of blasts in peripheral blood. Serum concentrations of VitB12, folates and holoTC were determined by AxSYM B12 or Folate microparticle enzyme immunoassay (MEIA) and new Axis-Shield HoloTC MEIA, respectively; plasma Hcy levels were measured by fluorescence polarization immunoassay (FPIA). All the panel was performed on the Abbott AxSYM analyser. CIMF patients presented increased oxidative stress (lower levels of TAC and higher levels of ROS), compared to a panel of 53 healthy volunteers (Mann Whitney U test, p<0.0001). Higher values of Hcy were detected in the group of CIMF presenting holoTC deficiency (Mann Whitney U test, p=0.03). Consistently, holoTC directly correlated with TAC (Spearman correlation, p=0.01). Interestingly, levels of holoTC and TAC were significantly lower in the group of CIMF patients with blasts in the peripheral blood (Mann Whitney U test, p=0.01 and p=0.009, respectively). Notably, levels of VitB12 and folates did not correlate with Hcy, holoTC, TAC or ROS. No correlations were found between the levels of Hcy, holoTC, TAC, ROS, VitB12 or folates and the remaining clinical variables considered. In conclusion, we demonstrated that CIMF patients present an increase in oxidative stress compared to healthy controls. We observed that holoTC depletion is associated to HH and reduced TAC, consistently with an increased oxidative potential. These findings indicate the presence of a clinically significant VitB12 deficiency, detectable only by holoTC dosage, in the presence of normal levels of total serum VitB12. The other interesting finding is the association of holoTC and TAC deficiency with the presence of blasts in the peripheral blood of the patients, suggesting a role of oxidative damage in CIMF progression. Therefore, our findings suggest that, in CIMF patients, the dosage of ROS, TAC, holoTC, and Hcy, may represent a valuable clinical aid in the diagnosis of unapparent VitB12 deficiency, and provide a rational basis to prevent and/or correct the increase in oxidative potential associated with this disease.

Abstract Introduction : Myeloproliferative neoplasms (MPN), namely polycythaemia vera (PV), essential thrombocythemia (ET) and primary myelofibrosis (PMF) have been associated with an increased risk of thrombotic and haemorrhagic events, mainly when januskinase-2 (JAK 2) V617F mutation is detected1. This association is higher for thrombotic events (TE) contributing for overall mortality in MPN patients, although the pathophysiological mechanisms remain unclear. Although age and history of thrombosis have been considered the most important factors for thrombosis risk assessment, cardiovascular risk factors have recently been included in prognostic scores for thrombosis in individuals with MPN. Aim : The primary end-point of this study was to evaluate the prevalence of thrombotic and haemorrhagic events in a population with MPN (PV, ET, PMF) harbouring the JAK-2 V617F mutation. A secondary end-point was to identify possible risk factors for these events. Methods: We retrospectively analysed the records of MPN patients with JAK-2 V617F mutation, identified between January 2010 and September 2013. Variables analysed included blood counts, gender, age at diagnosis, therapy, vascular events and cardiovascular risk (body mass index obesity-defined (WHO), diabetes, dyslipidemia, hypertension and smoking status). Multiple linear regression analysis was performed to identify the variables that best predict thrombosis after controlling for potential confounders. Variables significant in the univariate analysis were included in the regression. Analysis was performed using the SPSS version 22.0 (SPSS, Chicago, IL, USA). p-value lower than .05 was considered significant. Results: Excluding blood counts, MPN subgroups did not differ considering the studied variables, namely, thrombotic and bleeding events. In this MPN JAK-2 V617F mutated population (N=99; PV:38, ET:56, PMF:5), the prevalence of thrombotic and bleeding events was 35.4% (PV: 42.1%; ET: 30.4%; PMF: 40%) and 19.2% (PV: 15.8%; ET: 17.9%; PMF: 60%), respectively. With patients having more than one TE (1-5), there were 49 episodes, presented in either arterial (53.1%) or venous (46.9%) territories. The commonest sites of arterial thrombosis (N=26) were cerebral (50%), coronary (15.4%), peripheral arteries (15.4%) and ophthalmic (11.5%); venous thrombosis locations (N=23) were deep venous thrombosis (31.1%), superficial venous thrombosis (21.7%), pulmonary embolism (17.4%) and splanchnic (17.4%) (3 portal and 1 splenic). Prediction of TE was explained in 75% by a model including the number of thrombosis and obesity [R2 =.747; F (2, 36) = 53.255; p<.001; β (number of TE) = .410; β (obesity) = -.198]. The group with thrombosis showed lower number of platelets in comparison with the counterpart [F (1, 94) = 4.836; p= .030; η2 = .05]. None of other studied parameters differed significantly between groups with and without thrombosis. Bleeding episodes (N=28) occurred mostly under antiplatelet and/or anticoagulant therapy (N=22) and local/traumatic causes were identified in 42.9% of the events. In 2 haemorrhagic cases platelet blood count was >1x106/mL. The commonest affected sites were gastrointestinal tract (21.4%), nasal (21.4%), subdural (14.3%). In our study population, mortality was associated to older age (p= .002), male gender (p=.026), smoking (p=.007) and number of TE (p=.029). Mortality was found to be correlated with venous but not with arterial thrombosis (r=0.232, p=.021; r=0.105, p=.299, respectively). Conclusions and Discussion: In this MPN JAK-2 V617F mutated population we found higher prevalence of thrombotic events then bleeding episodes. The prediction of TE was explained in 75% by a model that includes the number of thrombosis episodes and obesity. Since JAK-2 V617F is a driver mutation in MPN acting through JAK/STAT inflammatory pathway and the finding of a TE prediction model that includes obesity, a chronic inflammatory condition, the present study underlies: 1) the importance of inflammation in the pathophysiology of thrombosis-MPN associated; 2) the relevance of the recent inclusion of cardiovascular risk factors as variables in prognostic scores for thrombosis in individuals with MPN. References Falchi L, Kantarjian HM, Verstovsek S (2017). Assessing the thrombotic risk of patients with essential thrombocythemia in the genomic era. Leukemia 31, 1845-1854. Disclosures Silva: Gilead Sciences: Research Funding; Abbvie, Gilead Sciences, Janssen, BMS: Consultancy, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Roche, Janssen, Celgene: Other: Travel Support; Roche, Janssen: Other: Institution's payment for consultancy.

Abstract Abstract 3279 Background: ITP is an autoimmune disorder characterized by increased platelet destruction and suboptimal platelet production. Romiplostim stimulates platelet production via the TPO-receptor, and is recommended for second- and third-line treatment of chronic ITP in adults. We report final data from a large prospective study of romiplostim in adults with ITP of varying duration and severity. Methods: Eligibility criteria were broad: patients ≥18 years of age, who had received prior ITP therapies (final protocol amendment: ≥1, previous amendments: ≥3), with low platelet counts (final amendment: ≤ 30 × 109/L, previous amendments: ≤ 10, ≤ 20 × 109/L) or experiencing uncontrolled bleeding. The only excluded comorbidities were: hematological malignancy, myeloproliferative neoplasms, MDS and bone marrow stem cell disorder. Romiplostim was initiated at 1 (final amendment) or 3 (previous amendments) μg/kg/week, with dose adjustments allowed to maintain platelet counts ≥50 × 109/L. Patients could continue on study until they had access to commercially available romiplostim. Rescue medications were allowed at any time; concurrent ITP therapies could be reduced when platelet counts were > 50 × 109/L. Primary endpoint was incidence of adverse events (AEs) and antibody formation. Secondary endpoint was platelet response, defined as either (1) doubling of baseline count and ≥ 50 × 109/L or (2) ≥20 × 109/L increase from baseline. Results: A total of 407 patients received romiplostim, 60% of whom were female. Median (Q1, Q3) time since ITP diagnosis was 4.25 (1.20, 11.40) years (maximum 57.1 years), with 51% of patients splenectomised and 39% receiving baseline concurrent ITP therapies. Seventy-one percent of patients completed the study, with requirement for alternative therapy and withdrawn consent the most common reasons for discontinuation (5% each). Median (Q1, Q3) on-study treatment duration was 44.29 (20.43, 65.86) weeks (maximum 201 weeks), with a total of 20,201 subject-weeks on study. Incidence and type of AEs were consistent with previous studies. The most common serious treatment-related AEs were cerebrovascular accident, headache, bone marrow reticulin fibrosis (with no evidence of positive trichrome staining for collagen and no evidence suggesting primary idiopathic myelofibrosis), nausea, deep vein thrombosis, hemorrhage and pulmonary embolism, with each reported in 2 of 407 (0.5%) patients. All other serious treatment-related AEs were each reported in one patient. Eighteen patients died; 3 deaths (hemolysis, intestinal ischaema, aplastic anemia) were considered treatment-related. No neutralizing antibodies to romiplostim or TPO were reported. Approximately 90% of patients achieved each of the platelet response definitions, regardless of splenectomy status. Overall, median (Q1, Q3) time to response was 2 (1, 4) weeks for response definition 1, and 1 (1, 3) week for response definition 2. Median (Q1, Q3) baseline platelet count was 14 (8, 21) × 109/L. After 1 week of treatment median (Q1, Q3) platelet count had increased to 42 (18, 101) × 109/L. From week 8 onwards, and excluding counts within 8 weeks of rescue medication use, median platelet counts were consistently above 100 × 109/L (range 101.0–269.5 × 109/L). Median (Q1, Q3) average weekly romiplostim dose was 3.62 (1.99, 6.08) μg/kg. Summary/conclusions: This is the largest prospective study in adult ITP reported to date. The data reported here are similar to those reported for previous romiplostim studies, with romiplostim able to safely induce a rapid platelet response in adult ITP patients with low platelet counts or bleeding symptoms. Romiplostim is an important, well-tolerated, treatment option for adult ITP patients, which significantly increases and maintains platelet counts. Adverse Event Subject Incidence Platelet Response Disclosures: Janssens: Amgen: Consultancy; Roche: Speakers Bureau; GSK: Membership on an entity's Board of Directors or advisory committees. Tarantino:Cangene corporation: Research Funding; Baxter: Research Funding; Talecris: Honoraria, Speakers Bureau; Up-to-date: Patents & Royalties; The Bleeding and Clotting Disorders Institute: Board Member. Bird:Amgen: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Novartis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; GSK: Membership on an entity's Board of Directors or advisory committees. Boccia:Amgen: Equity Ownership, Honoraria, Speakers Bureau. Lopez-Fernandez:Amgen: Membership on an entity's Board of Directors or advisory committees, Speakers Bureau. Kozak:Amgen: Membership on an entity's Board of Directors or advisory committees; Novartis: Membership on an entity's Board of Directors or advisory committees. Steurer:Amgen: Honoraria. Dillingham:Amgen Limited: Employment, Equity Ownership. Lizambri:Amgen: Employment, Equity Ownership.

Idiopathic myelofibrosis is a rare chronic myeloproliferative disease leading to extramedullary hematopoiesis (myeloid metaplasia) with splenomegaly. The liver and less frequently other organs including the lung can be involved, therefore portal hypertension is relatively common. Pulmonary hypertension (PH) is only occasionally reported, although recent studies have suggested an association between PH and myeloproliferative disorders. We present a case of PH diagnosis by echocardiography in a patient affected by idiopathic myelofibrosis with portal hypertension.

Background: Chronic myeloproliferative disorders (CMPD) seem to be associated with an increased risk for pulmonary hypertension (PH). Case Report: A patient with history of chronic idiopathic myelofibrosis (CIMF) presented with progressive dyspnea (New York Heart Association class III). Until this time he had not received specific treatment for CIMF. Echocardiography and rightheart catheterization confirmed PH. Further diagnostic procedures excluded a specific cause of PH. Therefore, primary PH was assumed. 2 years later he presented again with progressive dyspnea due to a progress of PH. A few days later the patient died from acute posterior myocardial infarction. Pathologic examination of the lung showed an obstruction of the small vessels by conglomerates of megakaryocytes. Discussion: We conclude that PH developed secondarily due to CMPD. PH should be suspected in patients with CMPD and should influence the decision for treatment of CMPD
Hintergrund: Chronische myeloproliferative Erkrankungen (CMPD) scheinen mit einem erhöhten Risiko für pulmonale Hypertonie (PH) assoziiert zu sein. Kasuistik: Ein Patient mit chronisch idiopathischer Myelofibrose (CIMF) wurde aufgrund einer progressiven Belastungsdyspnoe (New York Heart Association Stadium III) überwiesen. Bis zu diesem Zeitpunkt erhielt er keine spezifische Behandlung seiner CIMF. Echokardiographie und Rechtsherzkatheter ergaben das Vorliegen einer PH. Eine spezifische Ursache der PH konnte zunächst ausgeschlossen werden. Somit wurde das Vorliegen einer primären PH vermutet. 2 Jahre später wurde der Patient mit erneut verschlechterter Belastungsdyspnoe vorgestellt, wobei ein Progress der PH feststellbar war. Einige Tage später verstarb der Patient an einem Hinterwandinfarkt. Die Autopsie des Lungengewebes zeigte einen Verschluss der kleinen Lungengefäße durch Konglomerate von Megakaryozyten. Diskussion: Die Entwicklung der PH ist bei diesem Patienten als Folge der CMPD einzuschätzen. Das Vorliegen einer PH bei Patienten mit CMPD sollte die Entscheidung zu spezifischen therapeutischen Maßnahmen hinsichtlich der CMPD beeinflussen
Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich

Background: Chronic myeloproliferative disorders (CMPD) seem to be associated with an increased risk for pulmonary hypertension (PH). Case Report: A patient with history of chronic idiopathic myelofibrosis (CIMF) presented with progressive dyspnea (New York Heart Association class III). Until this time he had not received specific treatment for CIMF. Echocardiography and rightheart catheterization confirmed PH. Further diagnostic procedures excluded a specific cause of PH. Therefore, primary PH was assumed. 2 years later he presented again with progressive dyspnea due to a progress of PH. A few days later the patient died from acute posterior myocardial infarction. Pathologic examination of the lung showed an obstruction of the small vessels by conglomerates of megakaryocytes. Discussion: We conclude that PH developed secondarily due to CMPD. PH should be suspected in patients with CMPD and should influence the decision for treatment of CMPD.
Hintergrund: Chronische myeloproliferative Erkrankungen (CMPD) scheinen mit einem erhöhten Risiko für pulmonale Hypertonie (PH) assoziiert zu sein. Kasuistik: Ein Patient mit chronisch idiopathischer Myelofibrose (CIMF) wurde aufgrund einer progressiven Belastungsdyspnoe (New York Heart Association Stadium III) überwiesen. Bis zu diesem Zeitpunkt erhielt er keine spezifische Behandlung seiner CIMF. Echokardiographie und Rechtsherzkatheter ergaben das Vorliegen einer PH. Eine spezifische Ursache der PH konnte zunächst ausgeschlossen werden. Somit wurde das Vorliegen einer primären PH vermutet. 2 Jahre später wurde der Patient mit erneut verschlechterter Belastungsdyspnoe vorgestellt, wobei ein Progress der PH feststellbar war. Einige Tage später verstarb der Patient an einem Hinterwandinfarkt. Die Autopsie des Lungengewebes zeigte einen Verschluss der kleinen Lungengefäße durch Konglomerate von Megakaryozyten. Diskussion: Die Entwicklung der PH ist bei diesem Patienten als Folge der CMPD einzuschätzen. Das Vorliegen einer PH bei Patienten mit CMPD sollte die Entscheidung zu spezifischen therapeutischen Maßnahmen hinsichtlich der CMPD beeinflussen.
Dieser Beitrag ist mit Zustimmung des Rechteinhabers aufgrund einer (DFG-geförderten) Allianz- bzw. Nationallizenz frei zugänglich.