Peds Heme-Oncology

Clinical Values

Acquired congenital hemolytic anemia

• -hemolytic disease of the newborn (HDN)
• -maternal antibodies directed against fetal antigens
• -fetus is RH+ and mom is RH-
• -we get antigens against it from mom which causes hemolysis of newborn’s RBC
• -fairly rare, less than 10% of the time we get incompatibilities of RH between mom and baby
• -it’s not the first time around that its a problem, its the second time because at that point, mom has developed antibodies
• -we give the mom Rhogam to prevent mom from making antibodies so that if she does get pregnant again, the baby won’t get this
• -aka erythroblastosis fetalis
• -babies are very jaundice and anemic (moms antibodies have attacked blood cells)
• -hyperbilirubinemia: bilirubin levels are elevated bc of dead RBC

Disseminated intravascular coagulopathy

• Presentation:
  • – increased uncontrolled bleeding
    
  • – anemia from excess bleeding
    
  • – organ damage
    
  • – tissue hypoxia and necrosis
  • – excessive bruising
    
  • – hematuria
    
  • – oozing injection sites
    
  • – mild GI bleeding
• Precipitating factors:
  • – burns, trauma
    
  • – sepsis, shock
    
  • – cancer, hypoxia
    
  • – liver disease, NEC

Hemochromatosis

• Path:
  • – conservation of iron causes cellular damaged long term complications
• Long term effects:
  • – splenomegaly
    
  • – cardiac complication
    
  • – jaundice
    
  • – gallbladder diseases
    
  • – liver enlargement
    
  • – growth retardation
    
  • – skeletal changes
    
  • – endocrine complications
    
  • – cirrhosis

Hemolytic disease of the newborn

Hemophilia

• Path:
  • -inherited blood disorder
  • -could have a hemorrhagic stroke, nosebleeds, bruise easily
  • – hemarthrosis – most common disease found in kids with hemophilia
    • -warm, painful, swollen joints bc they are bleeding into joints
  • -GI bleeds: coffee-ground emesis, black tarry stools
  • -any kind of renal damage: have cola-colored urine
  • -usually can recognize hemophilia by the time they are 3-4Presentation:
• Types:
  • Hemophilia A
    • -factor VIII deficiency
      
    • -von Willebrand disease
  • Hemophilia B
    • -factor IX deficiency
      
    • -Christmas disease
  • Hemophilia C
    • -factor XI deficiency
• Management:
  • -do not give anything that can cause bleeding, no aspirin, no NSAIDs
  • -give blood transfusions, give whatever clotting factor they are missing (factor VIII is when they’re missing factor VIII, so we give them that)
  • -Significant bleed = give a dose of plasma

Iron deficiency anemia

• Etiology:
  • -most common thing we will see when talking about blood disorders of childhood/infancy
  • -from lack of iron intake or blood loss (lose iron stores)
  • -babies can’t tell us when something hurts so we have to look for irritability weakness, decreased activity from what they usually are, lack of interest from playing because they are tired

Idiopathic thrombocytopenic purpura (ITP)

• Path:
  • – antibodies against own platelets
    
  • – bleeding, bruising for no reason
    
  • – mucosal bleeding from nose, mouth, gums, cuts, etc.
    
  • – normally get after a virus (about 1-3 weeks after)
    
  • – increased destruction of platelets
  • most common bleeding disorder in children
  • -the same as an adult but sometimes will manifest itself in childhood
  • -an autoimmune disorder where platelets are being destroyed by the immune system
• Presentation:
  • -may come out with viral disease and a reaction to that
  • -may get bruising and petechiae all over
• Management:
  • -treat with steroids which decreases immune system destroying platelets, then the body will stop making the antibodies
  • -bc of this, if found in childhood there’s a good prognosis that it will resolve on its own (80% children)
  • – IVIG which resets and tricks the immune system and prevents it from making new antibodies to destroy the platelets

Leukemia

• Path:
  • -most common malignancy of childhood
  • -most of the time when a kid has leukemia, it’s ALL (acute lymphoblastic leukemia); the remainder is AML (acute myelogenous leukemia)
  • -5-year survival rate is 80% from tx
  • -having leukemia as a child puts you at higher risk for cancer as an adult
• RIsk:
  • -down syndrome is at risk for leukemia
• Presentation:
  • -children with leukemia will be tired, bruise for no reason, have intermittent fevers

Acute lymphocytic leukemia (ALL)

• Presentation:
  • – headache
    
  • – weight loss
    
  • – N/V
    
  • – bone pain
    
  • – papilledema

Acute myelogenous leukemia (AML)

• Presentation:
  • – fatigue
    
  • – bleeding
    
  • – hepatosplenomegaly
    
  • – pallor
    
  • – fever
    
  • – petechiae
    
  • – lymphadenopathy

Lymphoma

• Types:
  • Non-Hodgkin lymphoma:
    • -either nodular (in nodes themselves) or diffuse (most in childhood are diffuse)
      
    • -often diagnosed from the mediastinal mass with nodes in the chest
      
    • -treat with chemo and radiation
      
  • Hodgkin lymphoma:
    • -rare as an actual child
      
    • -usually, when seen in a child, they have a high level of Epstein Barr
      
    • -usually talking about teenagers

Lymphadenopathy Evaluation

Myeloproliferative Disorders

Sickle cell anemia

• Path:
  • manifests in children
  • -a genetic disorder, single-gene disorder
  • -instead of regular hemoglobin, we have hemoglobin S (HbS)
  • -the difference in one amino acid (valine is replaced by glutamic acid)
  • -RBC in response to either low levels of oxygen or dehydration, RBC go from normal to a sickled shape
  • -the shape will lead to issues because they get stuck places like small blood vessel
  • -often spleen stops working by the time they are 8 years old bc sickle cell infarct spleen
  • -any time you are dehydrated or deoxygenated, it triggers a sickled shape
  • -can get stuck in capillaries, spleen, head (cause stroke)
• Pathway:
  • – decreased oxygen
    
  • – hemoglobin S sickles
    
  • – cells become rigid
    
  • – obstruct capillary blood flow
    
  • – congestion of blood
    
  • – tissue hypoxia
    
  • – additional sickling
    
  • – extensive infarctions
    
  • – vaso-occlusion
• Presentation:
  • Babies first sign of sickle cell disease
    • – dactylitis (swelling of the hands and toes)
  • – Vaso-occlusive crisis
    • – stasis of blood causes ischemia and infarction
    • – fever, pain, tissue engorgement
  • – Sequestration
    • – blood pools in spleen
      
    • – profound anemia, hypovolemia, shock
  • Aplastic crisis
    • – increased destruction of fragile RBCs
      
    • – profound anemia, pallor
  • Biggest concern:
    • – acute chest syndrome
      • – type of pneumonia caused by congestion
• Types:
  • -sickle cell disease is when you inherit HbS from both parents
  • -sickle cell trait is where you get hemoglobin SA, not nearly as big of a deal, some may exhibit both trait and disease
  • -sickle cell-thalassemia disease
  • -sickle cell-HbC disease: a combination of sickle cell and HbC disease
  • -HbC is a different type of hemoglobin, hereditary, cause mild anemia, not a big deal but can be a big deal if you inherit HbC from one parent and sickle cell from the other parent bc you have anemia and sickling which can be dangerous
• Complication of Sickle cell:
  • -stroke
  • -can get stuck in eyes and get retinopathy, blindness, or hemorrhage
  • -hepatomegaly: liver enlarges
  • -autosplenectomy (the spleen is killed off)
  • -can get stuck in the kidneys and get hematuria
  • -priapism from cells getting stuck in penis
  • -pulmonary htn and HF, get stuck in the pulmonary vasculature
  • -prevention is big, make sure they stay oxygenated and hydrated, want to dilate blood vessels so sickle cells do not get stuck and cause infarction
  • -warm, IV fluids, can tolerate a lot of pain
• Management:
  • – oxygen
    
  • – treat pain
    
  • – IV fluids
    
  • – antibiotics if have fever
  • hydroxyurea
    • increases amount of fetal hemoglobin in the system and that hemoglobin replaces the sickled Hgb (prevention)
  • -if they are in crisis: we must keep them warm, hydrated, and oxygenated
  • Kids < 5 for sickle cell disease:
    • – penicillin prophylaxis
      
    • – haven’t been immunized against that bacteria yet

Thalassemias

• Path:
  • -autosomal recessive disorders
  • -have a problem with the globin chains of hemoglobin
  • – disorder of hemoglobin synthesis
    
  • – fragile RBCs with shortened life spans
    
  • – synthesis of defective hemoglobin
    
  • – results in anemia and hypoxia
    
  • – iron overload
    
  • – bone deformities
• Population Risk:
  • – Mediterranean
    
  • – African
    
  • – Asian
    
  • – Middle Eastern
• Types: 
  • -alpha thalassemia:
    • alpha chains are affected
  • -beta-thalassemia:
    • beta chains are affected
  • -if it’s major: you have both recessive chains
  • -if its minor: you only got one copy
  • -beta-thalassemia major= life-threatening
  • -alpha-thalassemia major= FATAL (usually die of HF)
  • -beta and alpha-thalassemia minor= both serious but aren’t always fatal
  • -in all of these there’s a problem with hemoglobin so they end up with anemia bc they can’t synthesize Hgb fast enough
• Presentation:
  • -will have anemia symptoms
• Management:
  • -blood transfusions to treat anemia, and may get iron overload (can cause heart and liver problems and liver failure and cancer)
  • -if they have beta-thalassemia major and don’t get treated they will usually die by 2 years old
  • -treat with regular lifetime blood transfusions (chelation treats iron overload, preventing them from getting liver failure from excess iron)
  • -may take out spleen bc it gets very enlarged from all defective RBC

Notes