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Polycythemia Vera Complications

Polycythemia Vera (PV): Complications High numbers of cells & platelets ↑ blood viscosity Polycythemia Vera Hematological disorder in which JAK2 mutations in hematopoietic cells result in increased RBC production. See corresponding Calgary Guide slide “Polycythemia Vera: Pathogenesis” ↑ Blood cell volume Presence of increased numbers of platelets creates a hypercoagulable and prothrombotic state ↑ Risk of venous & arterial thrombosis ↑ Systemic vascular resistance Impaired/“sluggish” blood flow ↓ Perfusion to small vessels and ↓ oxygen delivery to throughout body Arterial clots in arms and legs or in vessels leading to brain prevent oxygen delivery to cells Systemic hypertension ↑ Turnover of hematopoietic cells (RBCs, WBCs, platelets) Fatigue Transient visual disturbances Neurological symptoms (e.g. headache, dizziness, tinnitus, concentration problems) Breakdown of nucleic acids during cell turnover ↑ uric acid levels in blood Lysing cells release lactate dehydrogenase (LDH) into bloodstream ↑ Spleen activity to filter and dispose of old blood cells Splenomegaly Authors: Caitlin Bittman Noriyah Al Awadhi Yan Yu Peter Duggan* Reviewers: Maharshi Gandhi Kevin Zhan Michelle J. Chen Paul Ratti Merna Adly Crystal Liu Kareem Jamani* Man-Chiu Poon* Lynn Savoie* * MD at time of publication Extramedullary hematopoiesis Pancytopenia Clots in the portal vein, splenic vein, & mesenteric vein are unusual & highly suggestive of PV Clots in cardiac arteries prevent O2 delivery to cardiac tissue Myocardial infarction Venous blood moves more slowly and is less pressurized compared to arterial blood. Combined with hypercoagulable state, clots are likely to form in deep veins (usually of the legs) Deep vein thrombosis Clot breaks off and travels through the inferior vena cava & right heart into the pulmonary arteries Pulmonary embolism Stimulation fibroblasts in the bone marrow Uric acid accumulates & precipitates in blood Hyperuricemia ↑ Serum LDH Limb ischemia Ischemic stroke & transient ischemic attacks Erythromelalgias (burning pain in the extremities and erythema due to poor perfusion and transient microvascular occlusion) Compensatory vasodilation in the capillaries of the skin Plethora/rusted or “ruddy” complexion, particularly noticeable on the face, palms, nailbeds, & mucous membranes Gout Uric acid stones Advanced Disease Progression Abnormal blood cells produced in PV release various growth factors and cytokines Production of excess collagen and other fibrous materials Hematopoietic cells in bone marrow replaced with fibrous tissue over time Bone marrow increasingly unable to produce healthy blood cells Myelofibrosis (rare type of chronic blood cancer; considered late-stage progression of PV characterized by bone marrow fibrosis) Bone marrow failure Chronic bone marrow hyperactivity leads to exhaustion and/or damage of hematopoietic cells Legend: Pathophysiology Mechanism Sign/Symptom/Lab Finding Complications Published Aug 7, 2012, updated Feb 22, 2025 on www.thecalgaryguide.com