Problems of hemostasis

1. Problems of hemostasis Megan McClintock, MS, RN Fall 2011

2. Thrombocytopenia • Platelets < 150,000 • Causes – inherited or acquired (pg 678-679) • Immune – ITP • Shortened circulation – TTP, DIC, HIT, splenomegaly • Turbulent blood flow – hemangiomaa, valve problems • Decreased production – chemotherapy, viral infxn, sepsis, alcoholism, aplastic anemia, malignancy, radiation • Food, Drug, Herbal causes – pg 679

3. Immune Thrombocytopenic Purpura (ITP) • Most common acquired thrombocytopenia • Autoimmune disease • Platelets survive < 8 days • Gradual onset with transient remissions • Tx • Nothing if asymptomatic • Corticosteroids • Splenectomy • IVIG • Thrombopoietin receptor agonists • Platelet transfusions (only for life-threatening hemorrhage • Amicar (antifibrinolytic) for severe bleeding • Avoid ASA and other meds that affect platelets

4. Thrombotic Thrombocytopenic Purpura (TTP) • Medical emergency!!! • Uncommon syndrome with clumping of platelets, leading to microthrombi in small vessels • Almost always associated with HUS • Causes – idiopathic, drug toxicity, pregnancy/preeclampsia, infxn, autoimmune disorders • S/S – hemolytic anemia, thrombocytopenia, neuroprobs, fever with no infxn, renal probs • Tx • Stop the underlying disorder, remove causative agent • Plasmapheresis daily • Corticosteroids • Immunosupressants (ie. Cytoxan, cyclosporine) • Splenectomy • No platelet transfusions!!!

5. Heparin-Induced Thrombocytopenia (HIT) • Patho – formation of abnormal antibodies that activate platelets • Major complication is venous thrombosis (DVT, PE) • Other complications – stroke, kidney damage, MI • S/S – rarely have bleeding (platelets stay above 60,000), new or worsening thrombosis, decreased platelet count • Tx – have to protect from thrombosis and not reduce the platelet count further (no warfarin, no platelet transfusion) • Stop heparin (even line flushes) • Maintain anticoagulation with direct thrombin inhibitors • Only start Coumadin if platelets > 150,000 • For severe clotting, plasmapheresis, protamine sulfate, thrombolytics, surgery to remove clots • No platelets • Never give heparin to them again

6. Signs and Symptoms • **Usually asymptomatic (unless platelets < 50,000) • Bleeding (nosebleeds, gums, petechiae, purpura, bruising) • Prolonged bleeding after routine procedures • May have normal PT and aPTT • Complications • Spontaneous hemorrhage (platelets < 20,000) • Internal bleeding • Vascular thrombosis

7. Treatment • Platelet transfusions only if platelets < 10,000 OR active bleeding Acquired (usually from another underlying condition or therapy – ie. Leukemia) • Identify the cause and treat the disease • Remove the causative agent • If cause unknown, corticosteroids • Platelet growth factor and thrombopoietin (stimulates the bone marrow)

8. Nursing Care/Teaching • Discourage use of OTC meds that can cause thrombocytopenia (ie. ASA, NSAIDs) • Teach pt to notify dr even for minor nosebleed, gum bleeding, new petechiae • Avoid injections if possible • If injections unavoidable, use small-gauge needles for injections and apply pressure for 5-10 minutes • Suppression of menses • Soft toothettes • No lemon glycerin swabs • Soft, bland, non-acidic foods • Use electric razor • Avoid high-impact activities

9. Nursing care/teaching (cont.) • Blow nose gently • Prevent constipation • Do not pluck body hair • No tattoos or body piercing • No tampons • Check with dr before invasive procedures (ie. Dental cleaning, manicure, pedicure) • Call dr for black BMs, black vomit or urine, bruising, petechiae, bleeding, headache, change in vision, stroke symptoms

10. Hemophilia and von willebrand disease • X-linked recessive genetic disorder caused by defective or deficient coagulation factor • *Hemophilia A (classic or Factor VIII deficiency) • Hemophilia B (Christmas disease, Factor IX deficiency) • von Willebrand disease (deficiency of von Willebrand coagulation protein) • Hemophilia is transmitted by female carriers but displayed almost exclusively in men (von Willebrand is seen in both genders)

11. Signs & Symptoms • Slow, persistent, prolonged bleeding from minor trauma • Delayed bleeding after minor injuries • GI bleeding from ulcers and gastritis • Subcutaneous hematomas that can lead to nerve compression • Hemarthrosis leading to joint injury/deformity

12. treatment • Preventive care!!!! • Replacement of deficient clotting factors (not FFP) with active bleeding and before surgery • DDAVP (IV, SC, Intranasal) for minor bleeding and dental procedures • Antifibrinolytics (Amicar) with oral bleeding, nosebleeds, menses • Gene therapy (experimental)

13. Complications • Development of inhibitors to factors VIII or IX • Transfusion-transmitted infectious diseases (ie. HIV, Hep B, Hep C) • Allergic reactions • Thrombotic complications with factor IX • With von Willebrands, can develpalloantibodies • Starting factor replacement too late • Stopping factor replacement too soon • Treat minor bleeding for 72 hours, longer for surgery or traumatic injury

14. Nursing Care/Teaching • Genetic counseling • Stop topical bleeding as quickly as possible (Gelfoam, fibrin foam, thrombin) • Administer specific coagulation factor • If joint bleeding, totally rest the joint, pack in ice, treat pain without NSAIDs/ASA, once bleeding stops, ROM and PT; no weight bearing until healed • Watch for life threatening complications • Refer to local chapter of National Hemophilia Society • Immediate medical attention for severe pain/swelling of muscles/joints, head injury, swelling in neck/mouth, abdominal pain, hematuria, melena, skin wounds • Careful daily oral hygiene • Non contact sports only • Wear gloves when doing household chores • Wear a Medic Alert tag

15. Disseminated Intravascular Coagulation (DIC) • Complex systemic thrombohemorrhagic disorder • Clotting is abnormally initiated and accelerated using up all of the clotting factors and platelets leading to uncontrollable bleeding (consumptive coagulopathy) • Not a disease, but a complication • Always secondary to an underlying disorder (most common is septic shock and trauma) (pg 687) • Almost always causes organ failure • Can also have chronic DIC in which the body compensates (seen in malignancies, autoimmune diseases)

16. Sequence of events

17. Signs & Symptoms • No well-defined sequence of events • Unexplained bleeding • Pallor, petechiae, purpura • Oozing blood • Hematomas • Weakness • Malaise • Fever • Figure 31-9 (pg 687) • Respiratory – tachypnea, hemoptysis, orthopnea • GI – bleeding, abd distension, bloody stools • Urinary – hematuria • Neuro – vision change, dizziness, headache, change in LOC, irritability • MS – bone/joint pain • Thrombotic s/s – cyanosis, necrosis, PE, ARDS, ECG changes, paralytic ileus, oliguria

18. Diagnosis & Treatment • D-dimer assay is the best test (increased) • FSPs (fibrin split products) increased • FDPs (fibrin degradation products) increased • Schistocytes on blood smear • Treatment is controversial • If chronic and no bleeding, no treatment • If bleeding, provide support with blood products and treat the primary disorder • Only use platelets, cryoprecipitate, FFP if life-threatening hemorrhage • If signs of thrombosis, use heparin (controversial)

19. Nursing Care • Identify the development of DIC quickly • Early detection of bleeding and microthrombi • Administer blood and blood products correctly • Same care as for those with thrombocytopenia (see previous slides)

20. Neutropenia • Reduction in neutrophils (have to know the absolute neutrophil count – ANC) • ANC < 1000, severe if ANC < 500 • Even more important is the rapidity of the decrease of the ANC, degree of neutropenia, and the duration • Causes – clinical consequence of disease, side effect of certain drugs • *Most common cause is iatrogenic from chemo/immunosuppressants

21. Signs & Symptoms • Risk of infection from pathogens and normal body flora • Won’t have the normal signs of infection (ie. Redness, heat, swelling) • *Even low grade fever (100.4 F or 38 C) is critical • *Minor c/o pain or symptoms (ie. Sore throat, ulcers in the mouth, diarrhea, vaginal discharge, shortness of breath, cough) must be reported immediately • Commonly infected with bacteria, fungi, viruses • Must have a differential count to confirm neutropenia

22. Nursing Care • Monitor closely for infection and early septic shock • If fever 100.4 or higher, draw two blood cultures and start IV antibiotics immediately (w/in 1 hour) • Watch for fungal infxn if neutropenia prolonged • GCSF to prevent neutropenia • *Handwashing!!!! • Private room (maybe HEPA filtration) • No uncooked meat, pepper, unwashed fruits/veggies • Avoid crowds (wear a mask in public areas) • Bathe or shower daily • Don’t garden or clean up after pets • No fresh flowers in the room

23. Leukemia • Malignant disorders affecting blood and blood-forming tissues • Cause – unknown, genetics and environment play a role • Classified as acute or chronic (r/t cell maturity and disease onset), also by type of leukocyte involved (myelogenous or lymphocytic) • AML – much more common in adults, abrupt onset, very sick • ALL – most common type in kids, usu. have fever, can be abrupt or insidious, may have CNS symptoms • CML – have Philadelphia chromosome, usu. has a chronic stable phase and then an acute, aggressive phase leading to death • CLL – most common type in adults, B cells, lymph node enlargement, can lead to non-Hodgkin’s lymphoma

24. Signs & Symptoms • Vary (pg 695) • Anemia • Thrombocytopenia • Neutropenia • Late in disease can have: • Splenomegaly • Hepatomegaly • Lymphadenopathy • Bone pain • Meningeal irritation • Oral lesions

25. Diagnosis & Treatment • Peripheral blood and bone marrow exam determines type and subtype • LP, CT to look for leukemic cells outside of blood and bone marrow • Combination drug therapy to treat during all cell cycles, minimize drug toxicity, decrease drug resistance • With CLL, watchful waiting • If high WBCs (>100,000), leukapheresis and hydroxyurea to prevent stroke

26. chemotherapy • Induction – aggressive tx to destroy leukemic cells and bring about remission • Pts can get critically ill • Intensification – high dose therapy given immediately after induction for several months • Consolidation – eliminate remaining leukemic cells that may not be clinically/pathologically evident • Maintenance – given every 3-4 weeks (rarely done in AML) • May also give corticosteroids, radiation, intrathecal meds (ALL), may prepare for bone marrow transplant or hematopoietic stem cell transplant

27. Nursing Care • Many physical and psychosocial needs • Fear of unknown, death • Stress with the abrupt onset of disease • Provide hope • Be an advocate (help them understand, ask questions, manage side effects) • Care for neutropenia, thrombocytopenia, anemia, skin, GI issues, nutrition, etc. (discussed in MS I) • Be knowledgeable of the drugs • Refer to support groups • Encourage vigilant follow-up care

28. Hodgkin’s Lymphoma • Overgrowth of Reed-Sternberg cells in the lymph nodes • Cause – unknown, but genetics, exposure to occupational toxins, and infxn with EBV and/or HIV play a role • Originates in one location but can infiltrate other organs • S/S – insidious, painless enlargement of cervical, axillary, or inginual lymph nodes; weight loss, fever, night sweats (B symptoms); after ingestion of alcohol have rapid onset of pain at site of disease; can have other symptoms based on site of disease

29. Diagnosis and staging • Peripheral blood analysis (microcytic, hypochromic anemia, leukocytosis) • Lymph node biopsy • Bone marrow exam • Xray exam (defines sites of the disease) • CT & MRI (staging the disease)

30. Treatment & Nursing Care • Intensive chemo • Do not need maintenance chemo once a remission is achieved • Have a high risk of developing a secondary malignancy in the future (ie. AML, non-Hodgkin’s lymphoma, solid tumor) • Prognosis is better than most cancers • Nursing care is similar to leukemia

31. Non-Hodgkin’s Lymphoma • Originate in B-cells or T-cells (B-cells more common) • Cause – unknown, more common with HIV, those who have had immunosuppressants, chemo, or radiation; EBV infection • No hallmark sign, but always involve lymphocytes and look a lot like leukemia • Burkitt’s lymphoma is the most highly aggressive • S/S – can originate outside the lymph nodes, can spread unpredictably, and have widely disseminated disease at time of dx; *painless lymph node enlargement; may have B symptoms

32. Diagnosis, Staging & Treatment • Very similar to Hodgkin’s lymphoma, but may do more diagnostic studies since NHLs are more disseminated • Same staging as Hodgkin’s but more focus on the histologic subtype • Prognosis is not as good as for Hodgkin’s • Tx – chemo, radiation • *More aggressive lymphomas are more responsive to treatment and more likely to be cured • Many pts relapse several times

33. Multiple Myeloma • Cancerous plasma cells infiltrate the bone marrow and destroy bone, produce abnormal amts of immunoglobulin • Usually develops in older, African American men • S/S – insidious, skeletal pain is late in the disease, diffuse osteroporosis, hypercalcemia, Bence Jones proteins in the urine, high protein levels leading to renal failure, anemia, thrombocytopenia, neutropenia • High levels of beta-microglobulin and low levels of albumin are associated with poor prognosis

34. Treatment and Nursing Care • Seldom cured • Chemo with corticosteroids • Ambulation • Adequate hydration • Weight bearing • Biphosphonates given IV monthly • Radiation therapy • Allopurinol and Lasix • Be watchful and careful to prevent pathologic fractures • Braces • NSAIS with opioids • Recognition and treatment of infection • Dialysis due to myeloma-induced renal failure

35. Splenomegaly • When enlarged it doesn’t work as well and can lead to a decrease in blood cells • S/S – may be asymptomatic, may have abd pain, early satiety • Splenectomy is done to increase blood cell count or for splenic rupture • Care – can affect lung expansion, cause significant pain, cause anemia, thrombocytopenia, leukopenia • Post splenectomy can have immunologic deficiencies leading to a lifelong risk for infection; recommend Pneumovax, influenza vaccine