Figures
- FIGURE 1.
Contrast enhanced computed tomography of a mural thrombus in the main pulmonary artery trunk (arrow) in a patient with chronic thromboembolic pulmonary hypertension. a) Coronal view and b) axial view.
- FIGURE 2.
a) Pulmonary veno-occlusive disease (PVOD) with partial thrombotic occlusion of the lumen of a medium-sized vein (haematoxylin and eosin stain, 200× original magnification). b) An area simulating a capillary haemangiomatosis in a patient with PVOD, showing capillary proliferation with interstitial thickening and hemosiderin deposits (haematoxylin and eosin stain, 200× original magnification). c) Thrombotic occlusion of medium-sized veins (Weigert's elastic stain 100×). d) Almost complete thrombotic occlusion of the lumen of a small vein (haematoxylin and eosin stain, 200× original magnification). Figure courtesy of Dr. A. Cavazza (Unit of Pathology, IRCCS-Arcispedale S. Maria Nuova di Reggio Emilia, Reggio Emilia, Italy).
Tables
- TABLE 1
Updated clinical classification of pulmonary hypertension (from the fifth World Symposium on pulmonary hypertension, Nice, 2013)
Disease Classification Pulmonary arterial hypertension (PAH) 1 Idiopathic PAH 1.1 Heritable 1.2 BMPR2 1.2.1 ALK1, endoglin (with or without hereditary haemorrhagic telangiectasia), SMAD9, CAV1, KCNH3 1.2.2 Unknown 1.2.3 Drug and toxin-induced 1.3 Associated with: 1.4 Connective tissue diseases 1.4.1 HIV infection 1.4.2 Portal hypertension 1.4.3 Congenital heart diseases 1.4.4 Schistosomiasis 1.4.5 Pulmonary veno-occlusive disease and/or pulmonary capillary haemangiomatosis 1′ Persistent pulmonary hypertension of the newborn 1″ Pulmonary hypertension owing to left heart disease 2 Left ventricular systolic dysfunction 2.1 Left ventricular diastolic dysfunction 2.2 Valvular disease 2.3 Congenital/acquired left heart inflow/outflow tract obstruction and congenital cardiomyopathies 2.4 Pulmonary hypertension owing to lung diseases and/or hypoxia 3 Chronic obstructive pulmonary disease 3.1 Interstitial lung disease 3.2 Other pulmonary diseases with mixed restrictive and obstructive pattern 3.3 Sleep disordered breathing 3.4 Alveolar hypoventilation disorders 3.5 Chronic exposure to high altitude 3.6 Developmental abnormalities 3.7 Chronic thromboembolic pulmonary hypertension 4 Pulmonary hypertension with unclear multifactorial mechanisms 5 Haematological disorders: chronic haemolytic anaemia, myeloproliferative disorders, splenectomy 5.1 Systemic disorders: sarcoidosis, pulmonary Langerhans cell histocytosis, lymphangioleiomyomatosis, neurofibromatosis, vasculitis 5.2 Metabolic disorders: glycogen storage disease, Gaucher disease, thyroid disorders 5.3 Others: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH 5.4 BMPR2: bone morphogenetic protein receptor protein type 2; ALK1: activin receptor-like kinase type 1; CAV1: caveolin-1. Reproduced from [4] with permission from the publisher.
- TABLE 2
World Health Organization classification of the chronic myeloproliferative diseases
Myeloproliferative neoplasms (MPN) 1 Chronic myelogenous leukaemia, BCR-ABL1 positive 1.1 Polycythaemia vera 1.2 Essential thrombocythaemia 1.3 Primary myelofibrosis 1.4 Chronic neutrophilic leukaemia 1.5 Chronic eosinophilic leukaemia, not otherwise specified 1.6 Mast cell disease 1.7 Unclassifiable myeloproliferative neoplasms 1.8 Myeloid and lymphoid neoplasms with eosinophilia and abnormalities of PDGFRA, PDGFRB and FGFR1 2 Myelodysplastic syndromes/myeloproliferative neoplasms 3 Chronic myelomonocytic leukaemia 3.1 Juvenile myelomonocytic leukaemia 3.2 Atypical chronic myeloid leukaemia, BCR-ABL-negative 3.3 Unclassifiable myelodysplastic syndromes/myeloproliferative neoplasms 3.4 Myelodysplastic syndromes 4 Acute myeloid leukaemia 5 PDGFRA: platelet-derived growth factor receptor α; PDGFRB: platelet-derived growth factor receptor β; FGFR1: fibroblast growth factor receptor 1. Information from [5].
