Cyndi - Week 12 - Exam 6 Flashcards by Michelle Munoz

what are the four types of hematologic disorders?

problems with RBCs
problems with bone marrow
problems with platelets
problems with clotting cascade

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what are the 3 problems with RBCs?

Sickle Cell Disease
Hemophilia
Polycythemia Vera

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what are the 2 problems with bone marrow?

Aplastic Anemia

* Multiple myeloma

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what is the one problem with platelets?

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

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what is the one problem with the clotting cascade?

• Disseminated Intravascular Clotting (DIC) - idopathic

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what are the basic diagnostics for hematological disorders?

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

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what are the labs for hematological disorders?

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

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what are the characteristics of a bone marrow biopsy?

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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what are the preferred site for a bone marrow biopsy?

• Preferred sites ‐ Posterior or anterior iliac crest, sternum

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what are the complications of bone marrow biopsies?

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

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

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

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what is sickle cell disease?

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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is sickle cell disease a recessive or a dominant trait?

RECESSIVE!

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how is sickle cell disease diagnosed?

• 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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what is the main issues with sickle cell disease?

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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what are the crises of sickle cell disease?

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

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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?

JAUNDICE

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what are the clinical manifestations of sickle cell disease?

• 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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what is the tx for sickle cell disease?

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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what is hematopoietic stem cell transplant (HSCT)?

the only curative treatment for Sickle Cell Disease

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

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

infection
graft vs host disease
immune suppression

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

CURE!

- prevention of organ damage

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

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

Study These Flashcards

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

what is aplastic anemia?

pancytopenia - marrow stops producing blood cells

what are the causes of aplastic anemia?

- congenital or acquired

what are the clinical manifestations of aplastic anemia?

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

what are the diagnostic tests for aplastic anemia?

* Labs ‐ CBC, iron, ferritin studies * Bone marrow aspiration/biopsy * ↑ yellow marrow, hypo cellular

what is the tx for aplastic anemia?

* 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

what are the complications of aplastic anemia?

- infection - bleeding - hypoxic organ damage (RBC carry o2 → ↓ RBC → necrosis)

what is hemophilia?

inherited disorder of clotting: X-link recessive

Hemophilia A+B transmitted by ______

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

hemophilia requires a defective or deficient _____ -_____

coagulation factor

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

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

what is the most common congenital bleeding disorder??

von Willebrand

what are the sxs of hemophilia?

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

what are the diagnostic tests for hemophilia?

* Clotting Factors VIII, IX, X, XII, vWF | * Platelets, PT, PTT, Thrombin, Bleeding time

what are the complications of hemophilia?

allergic or transfusion related reactions, clotting | problems, acquiring disease from blood transfusion

what is the treatment for hemophilia?

Prevent and treat bleeding, prevent complications

what is acute intervention for any bleeding in hemophilia pts?

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

what is the preferred tx for hemophilia patients?

``` 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 ```

what is multiple myeloma?

malignant cells infiltrate bone marrow and destroy bone

what is the cause of multiple myeloma?

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

what is the pathophys of mulitiple myeloma?

* 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

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

- hydrate to make sure to flush the kidneys | - ambulate to use the bone

what are the clinical manifestations of multiple myeloma?

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

what are the diagnostics for multiple myeloma?

- labs (pancytopenia, ↑ Ca2+) - radiological studies - bone marrow biopsy

what is the disease tx for multiple myeloma?

- watchful watching - corticosteroids - chemotherapy - targeted therapy - HSCT

what is the symptom treatment for multiple myeloma?

- hypercalcemia (fluids and ambulation) | - analgesics, radiation therapy, skeletal support)

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

TRUE

what are the complications of multiple myeloma?

- 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

what is disseminated intravascular coagulation?

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

what risk factors for disseminated intravascular coagulation?

sepsis, shock, birth, cancer, burns, etc

what are the CLOTTING manifestations of disseminated intravascular coagulation?

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

what are the BLEEDING manifestations of disseminated intravascular coagulation?

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

TP: what is the hallmark sxs of DIC?

bleeding without a reason

what are the diagnostic tests for DIC?

- PT, INR, PTT - thrombin time - fibrin split products (FSP) - fibrinogen - platelets - D-Dimer level ***TP***

what are the complications of DIC?

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

what is the tx for DIC?

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

Cyndi - Week 12 - Exam 6 Flashcards

(59 cards)