Polycythemia and Platelet Disorders
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Questions and Answers

In the context of polycythemia vera, which of the following pathophysiological mechanisms most directly contributes to the manifestation of pruritus post-shower?

  • Amplified sensitivity of cutaneous nerve endings to thermal stimuli secondary to JAK2 mutation-induced cytokine dysregulation. (correct)
  • Enhanced prostaglandin synthesis in epidermal cells stimulated by rapid temperature changes.
  • Direct activation of the complement cascade leading to mast cell degranulation in response to altered blood viscosity.
  • Increased histamine release from basophils due to elevated erythropoietin levels.

A patient with a platelet count of 30,000/μL is scheduled for an elective surgical procedure. Which preemptive intervention strategy would be most appropriate to mitigate the risk of intraoperative bleeding, considering the potential for platelet refractoriness?

  • Preoperative administration of recombinant human thrombopoietin (rhTPO) to stimulate platelet production.
  • Administer a single dose of high-dose intravenous immunoglobulin (IVIG) 24 hours prior to the procedure.
  • Initiate a continuous infusion of aminocaproic acid (Amicar) 12 hours prior to the procedure and continue until 24 hours postoperatively.
  • Schedule a pre-procedure platelet crossmatch to identify compatible donors and administer HLA-matched platelets. (correct)

Considering the immunosenescence observed in older adults, which of the following age-related changes most significantly impairs the efficacy of vaccination against novel viral pathogens?

  • Diminished production of acute phase proteins, leading to impaired activation of the complement system.
  • Enhanced generation of long-lived plasma cells, providing sustained humoral immunity from prior vaccinations.
  • Reduced diversity in the T-cell receptor repertoire, limiting the ability to mount effective responses to new antigens. (correct)
  • Increased activity of regulatory T cells (Tregs), suppressing excessive inflammatory responses to vaccines.

A patient on chronic warfarin therapy with a consistently therapeutic INR of 2.5 presents with an acute ischemic stroke. After recombinant tissue plasminogen activator (rtPA) administration, which strategy is most critical for mitigating the risk of hemorrhagic conversion?

<p>Maintaining a strict blood pressure control regimen with a target systolic blood pressure of &lt;140 mmHg. (B)</p> Signup and view all the answers

Following a splenectomy, a patient is prescribed prophylactic antibiotics. Which of the following mechanisms contributes most significantly to the heightened risk of overwhelming post-splenectomy infection (OPSI) despite antibiotic prophylaxis?

<p>Compromised opsonization of encapsulated bacteria due to diminished synthesis of IgM antibodies. (A)</p> Signup and view all the answers

During a bone marrow biopsy, the patient experiences a vasovagal response resulting in transient hypotension and bradycardia. Which immediate intervention is most appropriate to address this complication?

<p>Elevate the patient's lower extremities and administer supplemental oxygen via nasal cannula. (D)</p> Signup and view all the answers

In the context of anemia, which of the following compensatory mechanisms contributes most directly to the sensation of dyspnea experienced by affected individuals?

<p>Augmented alveolar ventilation rate, decreasing the partial pressure of carbon dioxide in arterial blood. (C)</p> Signup and view all the answers

A patient with sickle cell disease presents with acute chest syndrome. Which therapeutic intervention targets the central mechanism underlying the pathogenesis of this complication?

<p>Exchange transfusion to reduce the proportion of sickled hemoglobin (HbS) and prevent further vaso-occlusion. (A)</p> Signup and view all the answers

Which of the following pathophysiological processes is primarily responsible for the neurological manifestations observed in patients with pernicious anemia?

<p>Impaired synthesis of myelin sheath components secondary to vitamin B12 deficiency, leading to axonal degeneration. (C)</p> Signup and view all the answers

In autoimmune idiopathic thrombocytopenic purpura (ITP), which cellular mechanism contributes most directly to the accelerated destruction of platelets?

<p>Phagocytosis of platelets by splenic macrophages via Fc receptor-mediated recognition of platelet-bound IgG autoantibodies. (B)</p> Signup and view all the answers

During a blood transfusion, a patient develops acute respiratory distress, hypotension, and frothy sputum. Which complication is most likely occurring, and what is the most appropriate immediate intervention?

<p>Transfusion-related acute lung injury (TRALI); stop the transfusion and provide respiratory support, including possible intubation and mechanical ventilation. (A)</p> Signup and view all the answers

In a patient with polycythemia vera undergoing phlebotomy, which laboratory parameter should be most closely monitored to guide the frequency and volume of blood removal?

<p>Hematocrit levels to maintain a target range and minimize hyperviscosity. (A)</p> Signup and view all the answers

A patient with thrombocytosis is being treated with cytoreductive therapy. Which of the following mechanisms of action is most likely to result in a reduction in platelet count without significantly affecting other hematopoietic cell lines?

<p>Inhibition of Janus kinase 2 (JAK2) signaling, selectively targeting megakaryocyte proliferation. (D)</p> Signup and view all the answers

Considering the age-related changes in the immune system, which of the following vaccinations would be of greatest importance for an elderly patient to prevent morbidity and mortality?

<p>Varicella vaccine to prevent shingles reactivation. (C)</p> Signup and view all the answers

A patient on warfarin therapy has an elevated INR of 6.0 and no active bleeding. Which agent, in addition to holding the next dose of warfarin, would be the most appropriate to administer?

<p>Oral phytonadione (vitamin K) to reverse the effects of warfarin. (C)</p> Signup and view all the answers

A patient who underwent a splenectomy five years ago presents with a fever, chills, and a productive cough. Which of the following organisms is the most likely cause of this patient's infection?

<p>Streptococcus pneumoniae. (D)</p> Signup and view all the answers

Following a bone marrow biopsy, a patient reports persistent pain at the biopsy site despite administration of analgesics. Which of the following interventions would be most appropriate to assess for a potential complication?

<p>Assess the biopsy site for signs of bleeding, hematoma, or infection. (A)</p> Signup and view all the answers

In a patient with chronic anemia, which compensatory mechanism would be least effective in maintaining adequate tissue oxygenation during periods of increased physical activity?

<p>Increased erythropoietin production to stimulate red blood cell production. (E)</p> Signup and view all the answers

A patient with sickle cell disease presents with an acute vaso-occlusive crisis. Which of the following interventions has the highest priority in managing this patient's pain?

<p>Initiating patient-controlled analgesia (PCA) with intravenous opioids. (B)</p> Signup and view all the answers

A patient with pernicious anemia is started on intramuscular vitamin B12 injections. Which of the following assessment findings would indicate an improvement in the patient's neurological status?

<p>Improved vibratory sense in the lower extremities. (A)</p> Signup and view all the answers

A patient with autoimmune idiopathic thrombocytopenic purpura (ITP) is refractory to first-line treatments. Which of the following second-line therapies aims to reduce platelet destruction by inhibiting Fc receptors on macrophages?

<p>Intravenous immunoglobulin (IVIG) to block Fc receptors. (A)</p> Signup and view all the answers

During a blood transfusion, a patient experiences flushing, urticaria, and wheezing. Which of the following actions should the nurse prioritize?

<p>Discontinuing the transfusion and administering antihistamines and epinephrine. (C)</p> Signup and view all the answers

In the management of polycythemia vera, which of the following therapeutic interventions is specifically aimed at reducing the risk of thrombotic events associated with elevated hematocrit levels?

<p>Phlebotomy to reduce red blood cell mass and decrease blood viscosity. (C)</p> Signup and view all the answers

For a patient with essential thrombocythemia who is at low risk for thrombosis, what is the recommended initial treatment approach primarily aimed at preventing platelet aggregation?

<p>Low-dose aspirin to inhibit thromboxane A2 production and reduce platelet activation. (B)</p> Signup and view all the answers

Considering the hematologic changes associated with aging, which intervention is most important to prevent complications related to reduced blood volume and lower plasma protein levels in older adults?

<p>Monitoring for signs of dehydration and promoting adequate hydration. (C)</p> Signup and view all the answers

When providing dietary education to a patient on warfarin therapy, which of the following instructions is most critical to ensure the stability of their international normalized ratio (INR)?

<p>Maintain a consistent daily intake of vitamin K-rich foods. (B)</p> Signup and view all the answers

A patient who has undergone a splenectomy is at increased risk of infection. Which of the following vaccinations is most important for preventing severe infections in this patient population?

<p>Meningococcal vaccine to prevent invasive meningococcal disease. (B)</p> Signup and view all the answers

Following a bone marrow biopsy, a patient experiences significant bleeding from the insertion site despite direct pressure. Which immediate intervention should the nurse implement to control the bleeding?

<p>Applying a topical hemostatic agent such as thrombin or fibrin sealant to the site. (C)</p> Signup and view all the answers

In a patient with severe anemia, which physiological adaptation is most likely to exacerbate pre-existing cardiac conditions, increasing the risk of heart failure?

<p>Increased heart rate and cardiac output to maintain oxygen delivery. (E)</p> Signup and view all the answers

A patient with sickle cell disease develops splenic sequestration crisis. Which intervention has the highest priority to prevent hypovolemic shock and death?

<p>Administering packed red blood cell transfusions to correct the anemia. (C)</p> Signup and view all the answers

A patient with pernicious anemia is non-compliant with vitamin B12 injections and develops severe neurological deficits. Which of the following long-term complications is most likely to result from this non-compliance?

<p>Irreversible peripheral neuropathy and cognitive impairment. (D)</p> Signup and view all the answers

A patient with autoimmune ITP is being treated with corticosteroids. Which of the following adverse effects of long-term corticosteroid use is most likely to complicate the management of their thrombocytopenia?

<p>Increased susceptibility to infections. (B)</p> Signup and view all the answers

During a blood transfusion, a patient becomes hypotensive and develops diffuse bleeding from the IV site. Which of the following complications is the most likely cause?

<p>Hemolytic transfusion reaction causing disseminated intravascular coagulation (DIC). (E)</p> Signup and view all the answers

In the management of a patient with polycythemia vera who is at high risk for thrombosis, which of the following agents is most effective in reducing the risk of thrombotic events by suppressing the production of blood cells in the bone marrow?

<p>Hydroxyurea to suppress bone marrow activity. (B)</p> Signup and view all the answers

For a patient with essential thrombocythemia who is also positive for the JAK2 V617F mutation, which therapeutic strategy directly targets the underlying molecular abnormality to reduce thrombotic risk?

<p>Employing JAK2 inhibitors to target the mutated kinase and reduce cellular signaling. (A)</p> Signup and view all the answers

Given the changes in immune function associated with aging, which preventive measure is most important for reducing the risk of morbidity and mortality from infectious diseases in elderly patients?

<p>Ensuring age-appropriate vaccination to boost immune responses to common pathogens. (B)</p> Signup and view all the answers

Flashcards

Polycythemia

Condition with an excess of RBCs, thickening the blood.

Polycythemia Vera

A rare blood disorder where the bone marrow makes too many RBCs due to a genetic mutation (JAK2).

Platelet Count Implications

Normal range: 150,000-450,000. Below 150,000 increases bleeding risk; above 450,000 increases clotting risk.

Warfarin

An anticoagulant that prevents clots but carries a risk for bleeding; requires consistent Vitamin K intake.

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Spleen Function & Splenectomy Implications

Produces lymphocytes/macrophages, filters RBCs, stores platelets. Removal increases infection/clot risk.

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Bone Marrow Biopsy

Needle inserted into iliac crest to sample bone marrow for diagnosing hematologic disorders.

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Anemia - Clinical Manifestations

Reduction in RBCs, hemoglobin, or hematocrit, leading to fatigue, weakness, and paleness.

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Sickle Cell Disease

Genetic disorder with abnormally shaped RBCs, causing pain and reduced oxygen carrying capacity.

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Pernicious Anemia

Vitamin B-12 deficiency due to lack of intrinsic factor, leading to large, dysfunctional RBCs.

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Autoimmune Idiopathic Purpura (ITP)

Low platelet count as a result of autoimmune response, causing bruising and bleeding.

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Administering Blood - Implications

Restoration of blood levels but with risks of reaction, infection or overload; requires careful verification and monitoring.

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Thrombocytopenia

Low platelet count, increasing the risk of bleeding.

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Study Notes

  • Study notes for Exam 2 – Spring 2025

Polycythemia

  • Condition characterized by thickened blood due to excess RBCs.
  • Increases risk of stroke and clotting.
  • Caused by a genetic mutation or myeloproliferative disorder, where the bone marrow produces too many RBCs.

Polycythemia Vera

  • Rare blood disorder resulting from a genetic mutation (JAK2) in the bone marrow, leading to excess RBC production.
  • Manifestations: fatigue, headaches, dizziness, tinnitus, hypertension, thrombosis, pruritus after showers, and a ruddy complexion.

Platelet Count

  • Normal range: 150,000-450,000.
  • Thrombocytopenia: Platelet count below 150,000.
  • Risks of thrombocytopenia: increased bleeding risk, potential for spontaneous or internal bleeding, and risks during surgery or birth.
  • Treatment for thrombocytopenia: platelet transfusion, corticosteroids, or splenectomy.
  • Thrombocytosis: Platelet count above 450,000.
  • Risks of thrombocytosis: increased clotting risk, risk for DVT, stroke, PE, and paradoxical bleeding.
  • Treatment for thrombocytosis: aspirin or cytoreductive therapy.

Older Adults and the Immune System

  • Hematologic changes: decreased blood volume, lower plasma protein levels, aging bone marrow leading to fewer RBCs, reduced antibody levels and responses, and falling hemoglobin levels.
  • Immune system changes: reduced cell function, increased inflammatory response, decreased adaptive immune response, and increased autoimmune activity.

Warfarin Therapy

  • Anticoagulant used to prevent DVT, atrial fibrillation, and PE.
  • Risk for bleeding.
  • Avoid NSAIDs.
  • Maintain consistent Vitamin K intake to stabilize INR.
  • High vitamin K foods: dark leafy greens, green tea, liver, and asparagus.
  • Increased bleeding risk with alcohol, cranberry juice, ginger, garlic, turmeric, and fish oil.

Spleen

  • Functions: produces lymphocytes and macrophages, filters and maintains RBCs, and stores platelets and blood.
  • Splenectomy: removal of the spleen due to trauma, cancer, or sickle cell anemia.
  • Implications for splenectomy: increased risk of infection and blood clots, possible change in CBC, and risk of splenosis.

Bone Marrow Biopsy

  • Needle inserted into the iliac crest to collect a bone marrow sample.
  • Used to diagnose hematologic disorders, evaluate low blood counts, and assess metastatic cancer.
  • Nurse's role: patient education, obtaining informed consent, preparation, assisting provider/support patient.
  • Post-biopsy teaching: pain management (avoid NSAIDs), activity restrictions, site care, signs of complications (bleeding, infection, pain), and follow-up.

Anemia

  • Reduction in number of RBCs, hemoglobin, or hematocrit.
  • Clinical manifestations: fatigue, weakness, paleness, dizziness, SOB, chest pain, cold extremities, tachycardia, hypotension, heart murmurs, angina, dyspnea, increased RR, confusion, vertigo, tinnitus, and restless leg syndrome.

Sickle Cell Disease

  • Genetic blood disorder with abnormally shaped, rigid, crescent-shaped RBCs.
  • Alters ability to carry oxygen efficiently.
  • Interventions for sickle crisis: oxygen therapy and monitoring, pain management (opioids, NSAIDs, PCAs), antibiotics, hydration (IV fluid or ringers), and emotional support.
  • Triggers for sickle cell crisis: alcohol, dehydration, pregnancy, infection, and hypoxia.

Pernicious Anemia

  • Vitamin B-12 deficiency due to lack of intrinsic factor.
  • Leads to larger, dysfunctional RBCs.
  • Treatment: Vitamin B12 replacement through IM injections, oral supplements, or nasal spray; treat underlying autoimmune conditions.

Autoimmune Idiopathic Purpura (ITP)

  • Low platelet count due to an autoimmune response.
  • Manifestations: easy or excessive bruising (purpura and ecchymosis), petechiae, prolonged bleeding, heavy menstrual bleeding (menorrhagia), nosebleeds (epistaxis), gum bleeds, and internal bleeding.

Blood Administration

  • Implications: restoration of blood levels and treatment of disorders.
  • Risks/complications: allergic reaction, febrile non-hemolytic transfusion reaction (FNHTR), hemolytic transfusion reaction, transfusion-related acute lung injury, circulatory overload, iron overload, and infection.
  • Nurse’s responsibility: verify order, assess patient, confirm blood product/type/patient identity, prepare equipment and infusion rate, monitor vitals and complications, and document.
  • Procedure: verify order, patient, and blood compatibility; prepare equipment; connect blood and start transfusion at a slow rate for the first 15 minutes; monitor vitals every 15 minutes; check for reaction and adjust transfusion rate (usually 2-4 hours).
  • Febrile non-hemolytic transfusion reaction (FNHTR): Caused by body's reaction to WBCs in transfused blood - Treat with acetaminophen and slow the transfusion.
  • Hemolytic transfusion reaction: Immune system attacks transfused blood - Medical Emergency.

Thrombocytopenia

  • Low platelet count.

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Study notes covering polycythemia and platelet disorders. Polycythemia is characterized by thickened blood due to excess RBCs, increasing the risk of stroke and clotting. Platelet count abnormalities, including thrombocytopenia and thrombocytosis, pose bleeding and clotting risks respectively.

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