TWO CASES OF PULMONARY HYPERTENSION ASSOCIATED WITH CHRONIC MYELOPROLIFERATIVE DISORDERS

Respirology ◽  
2017 ◽  
Vol 22 ◽  
pp. 75-75
Keyword(s):  
Pulmonary Hypertension ◽  
Myeloproliferative Disorders ◽  
Chronic Myeloproliferative Disorders

Unexplained Pulmonary Hypertension in Chronic Myeloproliferative Disorders

CHEST Journal ◽  
2001 ◽  
Vol 120 (3) ◽  
pp. 801-808 ◽  
Author(s):  
David Dingli ◽  
James P. Utz ◽  
Michael J. Krowka ◽  
Ann L. Oberg ◽  
Ayalew Tefferi
Keyword(s):  
Pulmonary Hypertension ◽  
Myeloproliferative Disorders ◽  
Chronic Myeloproliferative Disorders

Incidence of Pulmonary Hypertension in Patients with Chronic Myeloproliferative Disorders.

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 4935-4935
Author(s):  
Ranju Gupta ◽  
Sirisha Perumandla ◽  
Yelena Patsiornik ◽  
Selva Niranjan ◽  
Anju Ohri
Keyword(s):  
Pulmonary Hypertension ◽  
Systolic Pressure ◽  
Significant Risk ◽  
Myeloproliferative Disorders ◽  
Entire Group ◽  
Form Analysis ◽  
Chronic Myeloproliferative Disorders ◽  
Entire Cohort ◽  
Doppler Study

Abstract Background: Increased incidence of cardiac involvement and pulmonary hypertension (PH) has been reported in patients with chronic myeloproliferative disorders (CMPD). Most studies are small and retrospective except one where majority of the patient had essential thrombocytosis (ET). Method: We conducted a study to assess the incidence of PH in patients with CMPD. Patients were excluded if they had secondary cause of PH. Diagnosis of PH was established if right ventricular systolic pressure (RVSP) by transthoracic echocardiography (TTE) and Doppler study was ≥ 35 mmHg. 27 patients with diagnosis of CMPD established by standard criterion were included in the study. 9 patients had ET, 14 had polycythemia vera (PV) and 3 had chronic myeloid leukemia (CML). Results: Diagnosis of PH was established in 14/27 patients. 2 patients were excluded form analysis because of poor ejection fraction on TTE, 1 with PV and 1 with CML, giving final diagnosis of PH in 12/25 (48%) patients. 9 patients were males and 16 were females. Mean age at diagnosis in the entire cohort was 56.2 years and that with and without PH was 54.5 vs. 57.7 years respectively. Mean duration of follow up was 8.7 years and that with and without PH was 7.8 vs. 9.9 years respectively. 7/9 ET, 5/14 PV AND 0/2 CML patients had PH, mostly of mild to moderate severity. All patients were asymptomatic at the time of their last visit within last 2 months. The results are depicted in the table 1. There was no relation of PH to duration of disease, platelet count and hematocrit at diagnosis or during follow up period for the entire group or specific diagnosis of ET or PV. Because of erratic and variable duration of aspirin use by individual patients, we could not determine its significance. Discussion: The results of our study are similar to the study reported by Garypidou et al with regard to ET. However they had only 2 patients with PV and both of them had no evidence of PH. In our study 5/14 PV (36%) patients had PH, indicating PV patients also have significant risk of having PH. Pathogenesis of PH can be reliably related to CMPD because: Cases of secondary PH were excluded TTE excluded cardiac causes of PH Incidence of primary PH is very low (0.2cases/100,000) and usually occurs in 3rd or 4th decade. Moreover autopsy studies have demonstrated the presence of atypical megakaryocytes and thrombotic material in the lung capillaries of pulmonary hypertension patients and CMPD. Increased level of thrombopoietin also has been demonstrated in pulmonary artery of patients with PH. Thus platelets are implicated in pathogenesis of PH in patients with CMPD. Since PH seems to be common in patients with CMPD, more studies are needed to study the long-term impact of PH on survival in these patients. Impact of therapy including platelet lowering agents and ASA on development and progression of PH also needs to be studied. Table 1 CMPD Total No (%) MeanAge at Dx(yrs) Duration of Ds(yrs) Mean Plt at Dx(k/muL) Mean Plt at fu(k/muL) Mean Hct at Dx(%) Mean Hct at fu(%) Dx-diagnosis, Ds-disease, yrs-years, Plt-platelet, fu-follow up, Hct-hematocrit, PH-pulmonary hypertension, +-present, − absent ET PH + 7/9(77.8) 56.3 7.2 877.7 488.6 41.1 36.7 ET PH − 2/9(22.2) 65.0 8.0 698.0 492.0 45.3 40.0 PV PH + 5/14(35.7) 52.0 10 528.8 320.4 53.9 43.2 PV PH − 9/14(64.3) 58.7 10.6 511.9 316.8 58.9 41.9 CML PH − 2/2 46.5 4.0 620.5 387.5 36.9 36.2

Pulmonary Hypertension in Chronic Myeloproliferative Disorders

2015 ◽  
Vol 14 (3) ◽  
pp. 150-154
Author(s):  
Sapna Bhatia ◽  
Frederick Melendres ◽  
Lana Melendres-Groves
Keyword(s):  
Pulmonary Hypertension ◽  
Cellular Proliferation ◽  
Clinical Course ◽  
Current Knowledge ◽  
Myeloproliferative Disorders ◽  
Diagnostic Methods ◽  
Clinical Implications ◽  
Chronic Myeloproliferative Disorders ◽  
Bone Marrow Histology ◽  
Heterogenous Group

Chronic myeloproliferative disorders (CMPDs) are a heterogenous group of disorders characterized by cellular proliferation of one or more hematologic cell lines in the peripheral blood. Bone marrow histology shows hypercellularity and fibrosis in most of these disorders. The incidence of CMPD is estimated between 6 and 9 new cases per 100,000 yearly. CMPDs are incurable and are associated with a variable clinical course. Pulmonary hypertension (PH) is a well-recognized complication of CMPD, and when present, portends a poorer prognosis with median survival as low as several months, though this varies by the type of PH as well as the severity of the underlying CMPD. This review will serve to summarize the current knowledge of epidemiology, pathophysiology, diagnostic methods, clinical implications, and management of PH in CMPD.

Megakaryocytes and Platelets as the Main Cause for Vascular Events in Chronic Myeloproliferative Disorders

1996 ◽  
Vol 16 (02) ◽  
pp. 151-163 ◽  
Author(s):  
W. Schneider ◽  
A. Wehmeier
Keyword(s):  
Mononuclear Cells ◽  
Buoyant Density ◽  
Myeloproliferative Disorders ◽  
Term Therapy ◽  
Volume Distribution ◽  
Bleeding Tendency ◽  
Vascular Events ◽  
Chronic Myeloproliferative Disorders ◽  
Clonal Hematopoiesis ◽  
Induced Aggregation

SummaryMegakaryocytes are part of clonal hematopoiesis in chronic myeloproliferative disorders and are responsible for most of the clinical complications in this disease. About 30-40% of patients with polycythemia vera (PV) and essential thrombocythemia (ET) suffer from thrombotic complications, and microcirculatory disorders are common. Spontaneous bleeding mainly from the gastrointestinal tract is another complication that is especially prevalent in myelofibrosis and advanced stages of chronic myeloid leukemia.In vivo, the bone marrow is hypercellular and the concentration of megakaryocytes increased with characteristic morphological abnormalities. Megakaryocytes are enlarged and ploidy is increased in PV and ET but small mononuclear cells with decreased ploidy are a feature of CML. Despite spontaneous growth in cul-ture, megakaryocytes in chronic MPD are hypersensitive to added interleukin-3, interleukin-6 and GM-CSF.Platelets released from these megakaryocytes show abnormal morphology and ultrastructure, reflected in loss of storage granules and organelles, increased volume distribution and low buoyant density. Uptake, storage and secretion of platelet dense granule constituents is abnormal, and the plasma levels of platelet specific proteins which may also include growth factors for fibroblasts are elevated. At high platelet counts, spontaneous aggregation is observed, whereas agonist-induced aggregation in vitro with adrenaline, ADP and collagen is often defective. Platelet thromboxane generation may be stimulated, and production along the lipoxygenase pathway is decreased. Abnormalities of glycoprotein receptors and decreased fibrinogen binding have been reported but their clinical significance is uncertain. Several observations suggest that not only receptor defects but ineffective intracellular signalling may be responsible for platelet function abnormalities.No single underlying defect has been discovered that could explain this variety of pathological findings. Moreover, a combination of intrinsic megakaryocyte abnormalities and increased susceptibility of platelets to activation makes it difficult to differentiate secondary phenomena from effects of clonal hematopoiesis. How-ever, there are some clinical guidelines for therapy.Most elderly patients will be treated with cytoreductive therapy. Alkylating drugs and 32P have been shown to be leukemogenic, but even hydroxyurea may have a 10% incidence of leukemia induction after long-term therapy. Therapy with platelet-inhibitory drugs is often not sufficient to control thrombosis, and may aggravate a bleeding tendency, so that younger patients with PV and ET are increasingly treated with anagrelide or interferon alpha (A-IFN). Anagrelide is a quinazolin derivative that specifically inhibits megakaryocytopoiesis, while A-IFN may suppress clonal hematopoiesis by an unknown mechanism.

scholarly journals Soluble angiogenic factors: Implications for chronic myeloproliferative disorders

2002 ◽  
Vol 69 (3) ◽  
pp. 159-163 ◽  
Author(s):  
C. Musolino ◽  
L. Calabro' ◽  
G. Bellomo ◽  
F. Martello ◽  
B. Loteta ◽  
...  
Keyword(s):  
Myeloproliferative Disorders ◽  
Angiogenic Factors ◽  
Chronic Myeloproliferative Disorders
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