Ipertensione Polmonare nelle malattie ematologiche
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The document discusses pulmonary hypertension (PH) in hematological diseases such as myeloproliferative neoplasms (MPNs). Several studies found high rates of PH in patients with MPNs like primary myelofibrosis, essential thrombocythemia, and polycythemia vera. The mechanisms contributing to PH in MPNs include thrombocytosis, non-hepatic hematopoiesis, enhanced angiogenesis, and portal hypertension. Current recommendations for managing PH associated with MPNs include ruling out other causes, documenting elevated pulmonary pressures, and considering low-dose whole lung irradiation. Some research also links PH with myelofibrosis-like changes in the bone marrow.
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5 Embolie Pulmonaire.pdf
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Sickle cell disease and anaeshesia
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2. Patients with sickle cell disease are at high risk for complications during surgery due to phenomena like hypoxia, acidosis, dehydration, and hypothermia that can trigger sickling.
3. Perioperative management aims to prevent sickling and its complications through measures like adequate hydration, oxygenation, transfusion if needed, and temperature control.
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Polycythemia
This document discusses polycythemia, which is an increase in red blood cells. It can be primary (polycythemia vera) or secondary to other causes like tissue hypoxia or abnormal erythropoietin production. Polycythemia vera is a clonal disorder associated with a JAK2 mutation and is typically managed through phlebotomy to reduce red blood cell mass and prevent complications like thrombosis. Symptoms can include splenomegaly, aquagenic pruritus, erythromelalgia, and hyperuricemia. Treatment may also include chemotherapy or splenectomy in severe cases.
CTEPH, Surgcal and Medical Therapy. Terapia Chirurgica e medica
This document summarizes the experience of pulmonary endarterectomy (PEA) at a hospital in Pavia, Italy. It describes that PEA is the primary treatment for chronic thromboembolic pulmonary hypertension (CTEPH), and has better long-term outcomes than lung transplantation. The Pavia center has performed over 357 PEAs since 1994 and has developed expertise in evaluating, diagnosing, and performing the surgery on CTEPH patients.
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This document summarizes the experience of pulmonary endarterectomy (PEA) at a hospital in Pavia, Italy. It describes that PEA is the primary treatment for chronic thromboembolic pulmonary hypertension (CTEPH), which can be surgically treated to remove blockages in the pulmonary arteries. The hospital has performed over 357 PEAs and seen good outcomes with low mortality and technical failure rates when performed by experienced surgeons.
Pulmonary hypertension
This document discusses the pathophysiology and diagnosis of pulmonary hypertension. It begins by defining pulmonary hypertension and describing the clinical classification system. It then covers the pathophysiology of the different groups, explaining how increases in pulmonary vascular resistance can lead to elevated pulmonary artery pressures. The diagnosis section notes that symptoms are often non-specific and outlines the steps of evaluation, including imaging, echocardiography, pulmonary function tests and right heart catheterization.
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1) Pulmonary endarterectomy (PEA) is a life-saving surgery for chronic thromboembolic pulmonary hypertension that removes blood clots from the lungs.
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GASTROINTESTINAL INFECTIONS result from the ingestion of pathogens that cause infections at the level of this tract, generally being transmitted by food, water and hands contaminated by microorganisms such as E. coli, Salmonella, Shigella, Vibrio cholerae, Campylobacter, Staphylococcus, Rotavirus among others that are generally contained in feces, thus configuring a FECAL-ORAL type of transmission.
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Organization
Academy of Oriental Medicine at Austin
Academy of Oriental Medicine at Austin
Academy of Oriental Medicine at Austin
About AOMA: The Academy of Oriental Medicine at Austin offers a masters-level graduate program in acupuncture and Oriental medicine, preparing its students for careers as skilled, professional practitioners. AOMA is known for its internationally recognized faculty, award-winning student clinical internship program, and herbal medicine program. Since its founding in 1993, AOMA has grown rapidly in size and reputation, drawing students from around the nation and faculty from around the world. AOMA also conducts more than 20,000 patient visits annually in its student and professional clinics. AOMA collaborates with Western healthcare institutions including the Seton Family of Hospitals, and gives back to the community through partnerships with nonprofit organizations and by providing free and reduced price treatments to people who cannot afford them. The Academy of Oriental Medicine at Austin is located at 2700 West Anderson Lane. AOMA also serves patients and retail customers at its south Austin location, 4701 West Gate Blvd. For more information see www.aoma.edu or call 512-492-303434.
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Selective Estrogen Receptor Modulators (SERMs): Tamoxifen is a SERM that binds to estrogen receptors, blocking estrogen from stimulating breast cancer cells. It is effective but may have side effects such as increased risk of endometrial cancer and thromboembolic events.
Aromatase Inhibitors (AIs): These drugs, including anastrozole, letrozole, and exemestane, lower estrogen levels by inhibiting the aromatase enzyme, which converts androgens to estrogen in peripheral tissues. AIs are generally preferred in postmenopausal women due to their efficacy and safety profile compared to tamoxifen.
Selective Estrogen Receptor Downregulators (SERDs): Fulvestrant is a SERD that degrades estrogen receptors and is used in cases where resistance to other endocrine therapies develops.
Combination Therapies
Combining endocrine therapy with other treatments enhances efficacy. Examples include:
Endocrine Therapy with CDK4/6 Inhibitors: Palbociclib, ribociclib, and abemaciclib are CDK4/6 inhibitors that, when combined with endocrine therapy, significantly improve progression-free survival in advanced HR+ breast cancer.
Endocrine Therapy with mTOR Inhibitors: Everolimus, an mTOR inhibitor, can be added to endocrine therapy for patients who have developed resistance to aromatase inhibitors.
Chemotherapy
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- 1. L’Ipertensione Polmonare nelle Malattie Ematologiche Corso di aggiornamento sull’ipertensione polmonare. Pavia 21 maggio 2011 Giovanni Barosi Laboratorio di Epidemiologia Clinica/Centro per lo Studio della Mielofibrosi. IRCCS Policlinico S. Matteo Foundation, Pavia, Italy
- 3. Pathogenesis of PH in hemolytic anemias
- 6. Myeloproliferative Neoplasms (MPNs) CML CNL/MCD PV ET PMF
- 7. 1. Clonal proliferation of hematopoietic stem cell (mutational events like JAK2V617F) Current Biological Pradigm of MMM
- 8. 2. Clonal cells (CD34+, megakaryocytes, monocytes) excessively produce hematopoietic, fibrogenic and angiogenic growth factors Current Biological Pradigm of MMM MO fibroblast endothelium M-CSF SCF IL-6 TGF- MYELOPROLIFERATION MYELOFIBROSIS NEOANGIOGENESIS TGF- PDGF MK CD34+ TGF- VEGF Castro-Malaspina, 1984; Le Bousse Kardiles et al, 2001; Rameshwar et al, 2000
- 10. Barosi et al. Blood 2001 Barosi et al, BJH 2003 3. CD34+ hemopoietic stem cells constitutionally migrate from bone marrow to blood and to extramedullary organs Current Biological Paradigm of MMM CD34 + in PB CD34 + in Spleen
- 11. PERIPHERAL BLOOD HEMATOPOIESIS FIBROSIS BONE MARROW A Biological Model of MMM CD34+ CELLS HEMATOPOIESIS Anemia, thrombocytopenia SPLEEN Splenomegaly Blast transformation ANGIOGENESIS ANGIOGENESIS?
- 13. PH in PMF (and MPNs) 36% TTE PMF or post ET/Post PV MF =36 Cortelezzi et al. (Leukemia, 2008) 48% TTE ET=9 PV=15 CML=3 Gupta et al. (J Natl Med Ass 2006) ET= 47.8% Reactive thrombocytosis=0% TTE ET=46 Reactive thrombocytosis=40 Altintas et al. (Leukemia & Lymphoma, 2007) Transthoracic Echo (TTE) Diagnosis of PH 41.7% PV =2 ET=14 PMF=6 CML=2 Garypidou et al. (Haematologica 2004) Incidence Disorders Author
- 17. Thrombocytosis Secretion of vasoactive cytokines Circulating TPO levels PDGF secretion Smooth muscle cells hyperplasia Pulmonary hypertension
- 18. Non-hepatosplenic extramedullary hematopoiesis Pulmonary myeloid infiltration Growth factors and cytokine secretion Pulmonary hypertension
- 20. Circulating megakaryocytes Megakaryocyte embolism Pulmonary capillary obstruction Pulmonary hypertension
- 21. Enhanced angiogenesis High serum VEGF levels, low number of circulating EPCs Pulmonary hypertension
- 24. Pulmonary vascular disease and myeloid abnormalities in PAH. Farha S et al. Blood 2011;117:3485-3493 ©2011 by American Society of Hematology