Peds Hematologic/Immune disorders
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When might a patient experience polycythemia?
Response to hypoxia, ie Heart disease, High altitude
What are granulocytes and what is their function?
- Neutrophils (bacteria)
- – Eosinophils (allergic response and parasites)
- – Basophils (contain histamine, secrete heparin)
What are basophils and what are their functions?
- Monocytes/macrophages (phagocytize)
- Lymphocytes (found in BM, spleen, thymus..)
- • T cells (cell-mediated immunity)
- • B cells (humoral immunity)
What are some pediatric norms for a CBC?
- RBC 4.5-5.5
- Hgb 11.5-15.5 (age dependent)
- Hct 35% -45%
- RBC Indexes (MCV, MCH, MCHC)
- Reticulocytes 0.5-1.5% (immature RBCs)
- WBC 4.5-13.5
- Diff WBC
- ANC (>1000) (Formula involving WBC, Segs, Bands)
- Plt 150-400
What are some SnSs of RBC anemia and what are its consequences, and how can you treat it?
- Hgb < 11 g/dL
- pale mm and conjunctiva
- Easy fatigability
- Extreme pallor
- – Reverse the process causing anemia!
- – Replace deficiency
- – Severe: O2, bed rest, replace blood factors and products
In chronic anemia, growth retardation is likely.
What are some basic things to know about anemia caused by poor diet?
- – Preventable
- – Adolescents especially at risk due to rapid growth and poor eating habits
- – Most common hematological disorder
Why would you give Vit C to an anemic pt and what do you need to know about its administration?
- Vitamin C aids in Fe absorption.
- Will also stain both baby and adult teeth, so give at the back of throat and rinse afterward.
Why do RBCs in pts with SCD "curl up"?
Usually in response to hypoxia, so keep away from high altitude or situations which may decrease O2 supply-->sickle cell crisis.
What is a sickle cell crisis?
microvascular occlusions --> Ischemia, infarcts, and tissue death
What are some physiological consequences of SCD?
- Occur in tissues (large and small)
- Cause chronic damage to liver, spleen, heart, kidneys, eyes and bones
- Pulmonary infarction -->acute chest syndrome
- Cerebral infarction -->stroke
- Sickled cells are more likely to be destroyed -->jaundice
Why are SCD pts considered immunocompromised?
Chronic damage to spleen impares it's ability to filter bacteria --> frequent bact. nfxns. (leading cause of death for children with SCD)
What can trigger a sickle cell crisis?
- – Any event that increases body’s need for O2 or alters transport of O2
- -infection, fever
- -physical and emotional stress
- -increased viscosity of blood dt dehydration
- -high altitude, poorly pressurized flights, hypoventilation,
- -vasoconstriction dt hypothermia
What are the types of sickle cell crises?
- 1. Vaso-occlusive
- 2. Accute Chest Syndrome
- 3. CVA
- 4. Splenic Sequestration
- 5. Aplastic/Aplasia
- 6. Priapism
SnSs and treatment of Vaso-occlusive crisis?
- fever, pain, tissue swelling. Can occure in bones, lungs, liver/spleen, brain, penis, extremeties (dactylitis)
- Hydration, especially if a crisis is expected.
What is Acute Chest Syndrome? (ACS)
- • Leading cause of death in SCD patients older than 10 yrs
- • Causes: Infection, infarction, or pulmonary fat embolus
- • Fever, chest or back pain
- • Decreased 02 Sats, cough, dyspnea
- – Antibiotics/steroids
- – Pain/fever control/ O2
- – Maintenance fluids/rehydration
- --Pulmonary toilet
- Diagnostics/monitoring: CXR
What do you need to know about a SCD related CVA?
- Occurs in 7% of children with SCD
- – Convulsions/slurred speech
- – Ataxia/weakness/paralysis
- – Immediate
- • Symptoms
- • Exchange/Transfusion
- – Later
- • Chronic transfusion program
- • Neuropsychfollow-up
What is Splenic Sequestration?
- Infarction of spleen at microvascular level --> – Blood pools in the spleen --> SnSs:
- Rapidly enlarging abdomen with left-sided pain
- Profound anemia
- – Occurs primarily in children< 4 yrs of age
- – Life threatening-death can occur within hours
- • High mortality
What is Aplastic Crisis (aka aplasia)?
- Temporary cessation of bone marrow function --> ↓production and increased destruction of RBCs --> SnsS:
- profound anemia
- Triggered by viral infection or depletion of folic
- Treatment: PRBC transfusions
- treat infection
- monitor closely
What is Priapism?
- Persistent, painful, unwanted erection
- • Stuttering: Multiple short episodes
- • Severe prolonged: Lasting > 24hrs
High incidence of sexual dysfunction
- • Hydration/opioids
- • Exchange.transfusion
- • Avoid temperature extremes
How do you treat SCD?
- Aggressive treatment of infection
- Protection from cold exposure
- Frequent transfusions (Concern of Fe overload, ie hemosiderosis --> chelation therapy
- – Pain relief
- – Oxygen
What is Aplastic Anemia?
- • Acquired
- – 50% idiopathic, but may be from:
- – Activated suppressor lymphocytes
- – Drugs/toxins/chemicals
- – Radiation
- • Congenital
- – Fanconi’s anemia
- • Diagnosed by chromosome analysis
What are SnSs of Aplastic Anemia?
- • v RBCs--> Pallor & weakness
- • v Platelets --> Petechiae/bruising
- • v WBCs --> Infections, mouth sores
**No hepatosplenomegaly, which is good because you can r/o leukemia
How you do Tx Aplastic Anemia?
- • Remove exposure to hazardous drugs/toxins
- • HCT (Hematopoietic cell transplant, aka bone marrow t/p)
- • Non-HCT treatment:
- – Antithymocyte Globulin (ATG)
- – Methylprednisone
- – Cyclosporin
- – Cytokines
- • G-CSF/GM-CSF
- • Epogen/IL-3
Aplast Anemia prognosis?
- Depends on type of treatment and severity of disease but...
- there is a high risk of AML (Acute Myelo Leukemia)
What is Hemophilila?
- – Group of hereditary bleeding disorders that result
- from deficiencies of specific clotting factors
- – X-linked congenital bleeding disorder
- • Protein deficiency affects normal clotting
- • Occurs among all races
A vs. B type Hemophelia...
- – A “classic hemophilia”
- • Deficiency of factor VIII
- • 80% of hemophilia cases
- • Caused by deficiency of factor IX
- • Also known as “christmas disease”
- • 15% of hemophilia cases
What are some SnSs for both types of hemophelia?
- • Excessive/prolongedbleeding–-> Life-threatening bleeds
- • Tongue/throat
- • CNS: Signs of bleeding
- • Tingling/pain/swelling
- • Hemarthrosis: Bleeding into joint spaces of knee, ankle, elbow--> dyarthrosis (impaired mobility)
- • Ecchymosis: large subdural hematoma (like purpura, but larger
How do you Tx Hemophelia?
- • Prevent Bleeding!
- – Close supervision, dental, shaving, pressure for 15” and ice to vasoconstrict any ooze
- • DDAVP (Vasopressin): Synthetic form of ADH (antidiuretic hormone)
- – Increases Factor VIII and vWF (von Willebrand's factor)
- • Replace missing clotting factors
- • Transfusions of other blood products to prevent hemorrhage
What is the prognosis like for Hemophelia?
- • Excellent with appropriate management by multidisciplinary center
- • Gene therapy is hope for the future
- • Need Hepatitis C screening
- • HIV no longer an issue
- – Many adult hemophiliacs treated prior to 1986 are HIV +
What is von Willebrand's 's Disease?
- Hereditary bleeding disorder (auto dom)
- Involves deficiency of vWf (carrier for factor VIII): Necessary for platelet adhesion
- Signs/Symptoms: easy bruising, epistaxis, gingival bleeding, excessive bleeding with lacerations or surgery
- Treatment: infusion of vWB protein concentrate
- DDAVP infusion before bleeding event
- Other notes: Avoid aspirin and NSAIDS (increases bleeding time and decreased plt)
What is ITP?
- Immunte Thrombocytopenia Purpura (tells you the disease and characteristic symptom.)
- • Acquired hemorrhagic disorder resulting in excess destruction of circulating platelets
- – Platelet count less than 100K
- – Shortened platelet life span
- – Anti-platelet activity
- –^Megakaryocytes in bone marrow
- • History of viral infection within 2-4 weeks
- • Present with ecchymosis/petechiae/epistaxis (looks like child abuse)
Actue vs. Chronic ITP
- Apart form platelets, all blood count labs normal.
- No hepatosplenomegally
- Looks like an otherwise healthy child.
Tx for ITP?
- • Prevention
- – Safety precautions
- – Quiet play
- • Steroids
- • IV Ig
- 80-90% resolve within 4 months
- 95% resolved by 6 months
- Chronic if lasts > 6 months
- Recurrence in children is rare
- Not associated with any further disease or malignancy
Nursing care of the child recieve blood transfussion?
- • Hemolytic Reactions
- • Febrile Reactions
- • Allergic Reactions
- • Circulatory Overload (run transfussion over 4 hours)
- • Air Emboli
- • Hypothermia
- • Electrolyte Disturbances (potassium is released as aged, stored blood lyses RBCs. If infused too fast, [potassium] may be too high. Also, make sure the replacement blood is relatively fresh.)
What are the SnSs of childhood HIV/AIDS?
- S/S: lymphadenopathy, hepatosplenomegaly, oral
- candiasis, chronic or recurrent diarrhea, FTT, DD
What are SCIDS and Wiscott-Aldrich Syndrome?
- SCIDS-Severe Combined Immunodeficiency Syndrome
- – absence of both humoral (B) and cell-mediated (T) immunity
- – Genetic
- – Chronic infections very early in life
- – Treatment: PREVENT INFECTION and Stem Cell Transplant
- Wiskott-Aldrich Syndrome
- – X-linked recessive disorder
- – Poor prognosis
- – Clotting issues
- – Also affects B and T lymphocytes
- – Triad of abnormalities:Thrombcytopenia, eczema, and immunodeficiency
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