Peds Hematologic/Immune disorders

Response to hypoxia, ie Heart disease, High altitude 

• Neutrophils (bacteria)
• – Eosinophils (allergic response and parasites)
• – Basophils (contain histamine, secrete heparin) 

• Monocytes/macrophages (phagocytize)
• Lymphocytes (found in BM, spleen, thymus..)
• • T cells (cell-mediated immunity)
• • B cells (humoral immunity) 

• 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 

• Hgb < 11 g/dL 
• pale mm and conjunctiva
• Easy fatigability
• irritability
• Extreme pallor
• tachycardia
• Treatment:
• – Reverse the process causing anemia!
• – Replace deficiency
• – Severe: O2, bed rest, replace blood factors and products 

In chronic anemia, growth retardation is likely.

• –  Preventable
• –  Adolescents especially at risk due to rapid growth and poor eating habits 
• –  Most common hematological disorder

• Vitamin C aids in Fe absorption.
• Will also stain both baby and adult teeth, so give at the back of throat and rinse afterward. 

Usually in response to hypoxia, so keep away from high altitude or situations which may decrease O2 supply-->sickle cell crisis.

microvascular occlusions --> Ischemia, infarcts, and tissue death 

• 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 

Chronic damage to spleen impares it's ability to filter bacteria --> frequent bact. nfxns. (leading cause of death for children with SCD)

• – Any event that increases body’s need for O2 or alters transport of O2
• -trauma
• -infection, fever
• -physical and emotional stress
• -increased viscosity of blood dt dehydration
• -hypoxia
• -high altitude, poorly pressurized flights, hypoventilation,
• -vasoconstriction dt hypothermia

• 1. Vaso-occlusive
• 2. Accute Chest Syndrome
• 3. CVA
• 4. Splenic Sequestration
• 5. Aplastic/Aplasia
• 6. Priapism

• fever, pain, tissue swelling. Can occure in bones, lungs, liver/spleen, brain, penis, extremeties (dactylitis)
• Tx:
• Hydration, especially if a crisis is expected.
• Opiods
• NSAIDS

• Emergency!
• • Leading cause of death in SCD patients older than 10 yrs
• • Causes: Infection, infarction, or pulmonary fat embolus
• SnSs: 
• • Fever, chest or back pain
• • Decreased 02 Sats, cough, dyspnea 
• Tx:
• – Antibiotics/steroids
• – Pain/fever control/ O2
• – Maintenance fluids/rehydration 
• --Pulmonary toilet
• Diagnostics/monitoring: CXR

• Emergency!
• Occurs in 7% of children with SCD
• SnSs
• – Convulsions/slurred speech
• – Ataxia/weakness/paralysis
• Tx
• – Immediate
• • Symptoms
• • Exchange/Transfusion
•  – Later
• • Chronic transfusion program
• • Neuropsychfollow-up 

• Infarction of spleen at microvascular level --> – Blood pools in the spleen --> SnSs:
• Rapidly enlarging abdomen with left-sided pain
• Profound anemia
• hypovolemia
• shock

• – Occurs primarily in children< 4 yrs of age
• – Life threatening-death can occur within hours
• • High mortality

• Temporary cessation of bone marrow function --> ↓production and increased destruction of RBCs --> SnsS:
• profound anemia
• pallor

• Triggered by viral infection or depletion of folic
• acid

• Treatment: PRBC transfusions
• treat infection
• monitor closely

• Persistent, painful, unwanted erection
• • Stuttering: Multiple short episodes
• • Severe prolonged: Lasting > 24hrs

High incidence of sexual dysfunction

• Treatment
• • Hydration/opioids
• • Exchange.transfusion
• • Avoid temperature extremes 

• Aggressive treatment of infection
• Hydration
• Protection from cold exposure
• Frequent transfusions (Concern of Fe overload, ie hemosiderosis --> chelation therapy
• SupportiveCare
• – Pain relief
• – Oxygen 

• • Acquired
• – 50% idiopathic, but may be from:
• – Activated suppressor lymphocytes
• – Drugs/toxins/chemicals
• – Radiation
• • Congenital
• – Fanconi’s anemia
• • Diagnosed by chromosome analysis 

• • v RBCs--> Pallor & weakness
• • v Platelets --> Petechiae/bruising
• • v WBCs --> Infections, mouth sores

**No hepatosplenomegaly, which is good because you can r/o leukemia 

• • 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 

• Depends on type of treatment and severity of disease but...
• there is a high risk of AML (Acute Myelo Leukemia)

• – 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 “classic hemophilia”
• • Deficiency of factor VIII
• • 80% of hemophilia cases

• –B
• • Caused by deficiency of factor IX
• • Also known as “christmas disease”
• • 15% of hemophilia cases 

• • 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

• • 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

• • 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 +

• 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)

• 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) 

• 
• Apart form platelets, all blood count labs normal. 
• No hepatosplenomegally
• Looks like an otherwise healthy child. 

• • 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 

• • 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.)

• S/S: lymphadenopathy, hepatosplenomegaly, oral
• candiasis, chronic or recurrent diarrhea, FTT, DD 

• 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