Cyndi - Week 12 - Exam 6 Flashcards

1
Q

what are the four types of hematologic disorders?

A
  • problems with RBCs
  • problems with bone marrow
  • problems with platelets
  • problems with clotting cascade
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2
Q

what are the 3 problems with RBCs?

A
  • Sickle Cell Disease
  • Hemophilia
  • Polycythemia Vera
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3
Q

what are the 2 problems with bone marrow?

A
  • Aplastic Anemia

* Multiple myeloma

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4
Q

what is the one problem with platelets?

A

• Immune Thrombocytopenic Purpura (ITP) - brusing - small platelet cells

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5
Q

what is the one problem with the clotting cascade?

A

• Disseminated Intravascular Clotting (DIC) - idopathic

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6
Q

what are the basic diagnostics for hematological disorders?

A
  • labs
  • CT of liver, spleen, lymph nodes
  • biopsies of bone marrow and lymph nodes (cancers → swelling in nodes)
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7
Q

what are the labs for hematological disorders?

A
  • CBC
  • Blood type
  • Iron studies - Iron, TIBC (total iron binding capacity), Ferritin
  • Coagulation, clotting studies
  • Blood smear
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8
Q

what are the characteristics of a bone marrow biopsy?

A

Sterile procedure
• Bone marrow needle is inserted into the cortex of bone
• Severe pain by the patient during the aspiration
• Direct pressure to site after needle is removed
• May have the patient lie on the site if possible for 1 hour to compress
• Homeostasis is the goal

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9
Q

what are the preferred site for a bone marrow biopsy?

A

• Preferred sites ‐ Posterior or anterior iliac crest, sternum

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10
Q

what are the complications of bone marrow biopsies?

A
  • Hemorrhage and infection are rare
  • Position patient on side 30‐60 min.
  • Tenderness can last for 3‐4days
  • Analgesics
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11
Q

TEST: what nursing intervention can help with hemorrhaging after a bone marrow biopsy?

A

put the pt on that side in order to put pressure on the wound

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12
Q

what is sickle cell disease?

A

inherited autosomal disorder
- causes RBCs to “sickle”

  • More than 2 million Americans have SCT ‐ (1/12 have SCT)
  • 1 in 500 African American births SCD
  • 1 in 36,000 Hispanic American births
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13
Q

is sickle cell disease a recessive or a dominant trait?

A

RECESSIVE!

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14
Q

how is sickle cell disease diagnosed?

A

• labs - CBC, peripheral blood smear, hgb electrophoresis
• newborn screening
***pt may be interested in genetic counseling if trying to start a family

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15
Q

what is the main issues with sickle cell disease?

A

main issue is ORGAN HYPOXIA

  • reversible in the early stages with oxygen
  • blockage of perfusion
  • self-perpetuating hypoxia
  • more sickling of RBCs
  • triggers (infection, dehydration, stress, pregnancy) causes exacerbations
  • person lives anemic
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16
Q

what are the crises of sickle cell disease?

A
  • severe exacerbation
  • painful - ischemia occurring in multiple areas
  • vasospasms
  • can lead to organ damage, MODS (multiorgan dysfunction syndrome), and death
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17
Q

TEST: when sickled cells in the system need to be cleaned up by the spleen, they begin to lyse because they can’t keep up, what will the pt look like?

A

JAUNDICE

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18
Q

what are the clinical manifestations of sickle cell disease?

A
• Pain
• Fever
• Swelling
• Tenderness
• Tachypnea
• Hypertension
• Nausea, vomiting
• Signs of organ perfusion problems
(↓ U.O., pain, altered LOC,
respiratory distress, chest pain, hand or foot pain, etc.)
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19
Q

what is the tx for sickle cell disease?

A

maximize oxygenation, monitor organ perfusion, minimize complications
• O2 and rest
• Treat pain – usually opiates
• Lots of IVF – flush out occlusive areas of cells
• Blood transfusion
• Infection – prevent, avoid triggers, and treat asap
– Get immunizations
– Prophylactic antibiotics may be used
• Medications ‐ hydroxyurea
• Hematopoietic Stem Cell Transplant (HSCT)
• Genetic counseling

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20
Q

what is hematopoietic stem cell transplant (HSCT)?

A
  • the only curative treatment for Sickle Cell Disease

- Sources of stem cells: bone marrow , peripheral, or cord blood

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21
Q

what are the risks of hematopoietic stem cell transplant (HSCT)?

A
  • infection
  • graft vs host disease
  • immune suppression
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22
Q

what are the benefits of hematopoietic stem cell transplant (HSCT)?

A
  • CURE!

- prevention of organ damage

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23
Q

what are the two types of dones for hematopoietic stem cell transplant (HSCT)?

A
  • allogeneic - someone else is the donor
    • Matched sibling donors have the best outcome
    • Families members should be encouraged to have cord blood collected and stored
  • autologous - the pt him/herself is the donor
    • Healthy bone marrow taken from pt before marrow ablation
    • Rate of rejection very low and immune system response much quicker
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24
Q

T/F All patients with SCD should be evaluated for HSCT

A

TRUE
• Severity of disease can be underestimated – damage is occurring
• Allogeneic HSCT is not only curative in SCD, but abates progressive organ
dysfunction, including CNS complications

25
Q

what is aplastic anemia?

A

pancytopenia - marrow stops producing blood cells

26
Q

what are the causes of aplastic anemia?

A
  • congenital or acquired
27
Q

what are the clinical manifestations of aplastic anemia?

A
  • Abrupt or insidious onset: fatigue, pallor, tachycardia, tachypnea, bleeding
  • S/s of suppression of blood cells ‐ infection
28
Q

what are the diagnostic tests for aplastic anemia?

A
  • Labs ‐ CBC, iron, ferritin studies
  • Bone marrow aspiration/biopsy
  • ↑ yellow marrow, hypo cellular
29
Q

what is the tx for aplastic anemia?

A
  • ID and remove causative factor if possible
  • Supportive care (fluids, O2, blood transplant)
  • Bone marrow stimulant meds – Filgrastim, Epoetin - helps body create RBCs
  • HSCT – bone marrow transplant
  • Immunosuppressive therapy possible
  • Corticosteroids
30
Q

what are the complications of aplastic anemia?

A
  • infection
  • bleeding
  • hypoxic organ damage (RBC carry o2 → ↓ RBC → necrosis)
31
Q

what is hemophilia?

A

inherited disorder of clotting: X-link recessive

32
Q

Hemophilia A+B transmitted by ______

A

female
• 50% of sons have the disease
• 50% of daughters are carriers of disease
• Daughters with the disease usually have a milder case

33
Q

hemophilia requires a defective or deficient _____ -_____

A

coagulation factor

34
Q

what are the 3 different coagulation factors that can be defective or deficient?

A
  • A: 85‐90% ‐ missing Factor VIII
  • B: 10‐15% ‐ missing Factor IX
  • von Willebrand – missing von Willebrand factor
35
Q

what is the most common congenital bleeding disorder??

A

von Willebrand

36
Q

what are the sxs of hemophilia?

A
Bleeding:
• Slow persistent oozing
• Delayed bleeding
• Uncontrolled hemorrhage
• Bleeding in GI tract (dark tarry stools), nose, kidneys, brain, joints, or skin
37
Q

what are the diagnostic tests for hemophilia?

A
  • Clotting Factors VIII, IX, X, XII, vWF

* Platelets, PT, PTT, Thrombin, Bleeding time

38
Q

what are the complications of hemophilia?

A

allergic or transfusion related reactions, clotting

problems, acquiring disease from blood transfusion

39
Q

what is the treatment for hemophilia?

A

Prevent and treat bleeding, prevent complications

40
Q

what is acute intervention for any bleeding in hemophilia pts?

A
  • First aid for minor cuts
  • Replace deficient clotting factors during a bleeding episode
  • If joint bleeding occurs, pack it in ice and rest it
41
Q

what is the preferred tx for hemophilia patients?

A
Factor Replacement Therapy
• Plasma derived or recombinant products
• DDAVP – Vasopressin – increases VIII, and vWF
• Prophylaxis vs “on demand”
• Decreased use of FFP nowadays
• Antifibrinolytic therapy
• Monoclonal antibodies
42
Q

what is multiple myeloma?

A

malignant cells infiltrate bone marrow and destroy bone

43
Q

what is the cause of multiple myeloma?

A

unknown cause, may be exposure to chemicals, radiation, or genetic factors

44
Q

what is the pathophys of mulitiple myeloma?

A
  • excess plasma B cells - produce abnormal antibodies (ineffective, don’t fight infection, infiltrate the bone marrow, show up in the urine (bence-jones proteins)
  • excess cytokine production destroys bone as well
  • develops slowly (usually not diagnosed until disease is advanced)
  • bone destruction → calcium is released → worried about kidneys
45
Q

TP: what are the simple nursing interventions that will help the kidneys in multiple myeloma?

A
  • hydrate to make sure to flush the kidneys

- ambulate to use the bone

46
Q

what are the clinical manifestations of multiple myeloma?

A
• skeletal pain
• bone degeneration 
- osteoporosis 
- pathological fractures 
-  hypercalcemia (bone loss)
- renal, GI, and neuro manifestations
47
Q

what are the diagnostics for multiple myeloma?

A
  • labs (pancytopenia, ↑ Ca2+)
  • radiological studies
  • bone marrow biopsy
48
Q

what is the disease tx for multiple myeloma?

A
  • watchful watching
  • corticosteroids
  • chemotherapy
  • targeted therapy
  • HSCT
49
Q

what is the symptom treatment for multiple myeloma?

A
  • hypercalcemia (fluids and ambulation)

- analgesics, radiation therapy, skeletal support)

50
Q

T/F Multiple myeloma is seldom cured , but treatment can relieve sxs, produce remission, and prolong life-preventing complications a major focus

A

TRUE

51
Q

what are the complications of multiple myeloma?

A
  • uric acid accumulation (gout)
  • renal tubular necrosis
  • pathological fractures
  • pain and peripheral neuropathy
  • infection (d/t ↓ antibodies TP: shouldn’t be around )
  • depression and psychological stress
  • death
52
Q

what is disseminated intravascular coagulation?

A

not a disease, a physiological response to disease, infection, or insult

  • abnormally initiated and accelerated clotting
  • after clotting factors depleted, unstoppable bleeding
  • can be acute or chronic situation
53
Q

what risk factors for disseminated intravascular coagulation?

A

sepsis, shock, birth, cancer, burns, etc

54
Q

what are the CLOTTING manifestations of disseminated intravascular coagulation?

A

cyanosis, hemorrhagic necrosis, ↓ urine output, sxs of PE, MI, DVT, stroke

55
Q

what are the BLEEDING manifestations of disseminated intravascular coagulation?

A

petechiae, purpura, pallor, oozing blood through skin and orifices, hematoma, sxs of hypovolemia, visual changes, joint pian, hematuria, rectal bleeding, ALOC

56
Q

TP: what is the hallmark sxs of DIC?

A

bleeding without a reason

57
Q

what are the diagnostic tests for DIC?

A
  • PT, INR, PTT
  • thrombin time
  • fibrin split products (FSP)
  • fibrinogen
  • platelets
  • D-Dimer level TP
58
Q

what are the complications of DIC?

A

organ ischemia or infarction, hemorrhage, ARDS, multiple organ dysfunction syndrome (MODS), death

59
Q

what is the tx for DIC?

A

• frequent assessments - early diagnosis
• supportive care (blood products, plts, cryoprecipitate, FFP)
• anticoags (IV heparin)
- FIX the underlying cause (sepsis? ATB)