Heme/Onc Flashcards
Hemorrhage and hemolysis will show what on a CBC?
high reticulocyte count
When does a normal physiologic nadir occur in Hgb?
8-10wks of life
What is the DDx for microcytic anemia?
Fe def. anemia, thalassemia, sickle cell anemia, chronic dz, lead intox, copper def.
What it the most common cause of anemia?
Iron deficiency
When do we screen for iron def. anemia?
12-15mos
What is the Tx for iron def. anemia?
4-6mg/kg/d iron
When do we screen for lead poisoning?
12 months in high risk populations
How might lead poisoning might present?
Pica, abd pain, CNS change, microcytic/hypochromic anemia, basophilic stippling
When is chelation indicated in lead poisoning?
blood lead level >45ug/dL, re-test in 48hrs.
Alpha thalassemia causes what fatal condition?
hydrops fetalis–missing 4 genes –/–
What are sx’s of hemoglobin H dz(–/-a)?
alpha thalassemia variant-3 of 4 genes missing, chronic hemolytic anemia, neonatal jaundice
When does Beta thalassemia present?
btwn 4-12mos of life when nml hgb should be replacing fetal hgb
What are sx’s B-thalassemia major?
skeletal changes/extrameduallary hematopoeisis, splenomegaly, liver/gallbladder dz, aplastic crisis, cardiopulmonary d/o
What’s the tx of B-thalassemia major?
chronic transfusions, chelation from iron overload
What is the life span of sickle cells?
17days vs 120 in normal RBCs
What are manifestation of sickle cell?
dactylitis, hemolytic anemia, vaso-occlusive crisis, acute chest syndrome, chronic pain, priapism, hepatobiliary dz, bone pain/infarction, functional asplenia
When does splenic infarction occur?
2-4yo
Sickle cell tx?
iron, folate, transfusion, chelation, hydroxyurea (promotes Hgb F), stem cell transplant
What is the MC RBC enzyme defect that causes hemolytic anemia?
G6PD deficiency
What are sx’s of G6PD Def.?
neonatal jaundice, dark urine, no sx’s, , gallstones, splenomegaly
What lab findings do you expect w/oxidant stress in G6PD Def?
hemolytic anemia, bite cells, Heinz bodies, elevated ESR, spherocytes
Tx of G6PD Def.?
remove stressor, no fava beans, transfusion, avoid sulfas/nitrofurantoin
What is autoimmune hemolytic anemia?
acute, self limited hemolysis following an infection or drugs
Sx’s of hemolytic anemia?
jaundice, fatigue, pallor, dark urine, splenomegaly
Labs in autoimmune hemolytic anemia?
normochromic/normocytic anemia, elevated reticulocytes, LDH, AST, urobilinogen, + Coomb’s test
Tx for autoimmune hemolytic anemia?
> 80% resolve, IVIG, steroids, transfusion
Which primary combined immunodeficiency causes eczema, eosinophilia, staph abscess, ostepenia, fractures
hyper IgE syndrome
MC bleeding d/o in childhood?
Idiopathic thrombocytopenic purpura
MC tumor of infancy?
neuroblastoma
How does neuroblastoma present?
abd mass, miosis, anhydrosis, ptosis, constitutional sx’s, dancing eyes, dancing feet
Second most common abdominal tumor?
Wilm’s tumor/nephroblastoma
How does Wilm’s present?
smooth abd. mass, HTN, microscopic hematuria, usually around age 3
MC malignancy in childhood?
acute lymphoblastic leukemia ALL
How does ALL present?
fever, bone pain, petechiae, HSM, LAD, respiratory sx’s…bone marrow is gold standard Dx
