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Peds Hematology recap
Terms in this set (54)
This is anemia caused by increased RBC destruction
What are the intrinsic (inherited) hemolytic anemias?
sickle cell, thalassemia, G6PD deficiency, and hereditary spherocytosis
What are the extrinsic (acquired) hemolytic anemias?
DIC, TTP, HUS, and hypersplenism
Reticulocytosis is a common finding in what?
How is sickle cell anemia diagnosed?
Hgb S on hemoglobin electrophoresis
This is a microcytic anemia with normal or increased Fe and no response to Fe treatment
On electrophoresis there is decreased Hgb A, increased Hgb A2, and increased HgbF. What does this indicate?
Episodic hemolytic anemia associated with sulfa drugs, fava, beans, and infections is most likely what?
What are the 3 etiologic causes of anemia?
increased blood loss, increased RBC destruction, and decreased RBC production
An anemia workup includes what?
CBC with RBC indices, peripheral blood smear, and bone marrow biopsy (not usually done, but is the gold standard)
If reticulocytes are not increased in response to anemia, what does this indicate?
deficient production of RBC's
What is a normal MCV?
B12 and folate deficiency are causes of what type of anemia?
What is the most common cause of B12 deficiency?
pernicious anemia (malabsorption)
Pallor, glossitis, stomatitis, and peripheral neuropathy are all suggestive of what?
B12 deficiency anemia
What are the 3 most common causes of microcytic anemia?
iron deficiency, thalassemia, and anemia of chronic disease
Iron deficiency anemia is most commonly due to what?
In iron deficiency anemia, you would expect serum iron to be ________, ferritin to be ________, and TIBC to be __________
decreased; decreased; increased
This is due to decreased production of globin chains
Heinz bodies are associated with what?
Why do patients with beta thalassmia major become symptomatic at 6 months?
that is when HgbF declines
Lead poisoning can cause what type of anemia?
acquired sideroblastic anemia (plumbism)
Abdominal pain with constipation, neurologic symptoms (fatigue, learning disabilities), and anemia are suggestive of what?
What will a peripheral smear show if a child has lead poisoning?
microcytic hypochromic anemia with basophilic stippling and ringed sideroblasts in bone marrow
Sickle cell anemia is what type of genetic disorder?
What is the first step in managing a sickle cell pain crisis?
IV hydration and oxygen
Thrombocytopenia, microangiopathic hemolytic anemia, kidney failure, neuro symptoms, and fever are included in the pentad of what condition?
thrombotic thromocytopenic purpura (TTP)
TTP is due to an antibody against what?
ADAMTS13 (von Willibrand factor cleaving protease)
What is the treatment of choice for TTP?
HUS is usually preceded by what?
Enterohemorrhagic E coli 0157:H7
This is an acquired, abnormal isolated thrombocytopenia of idiopathic cause
ITP often follows what?
Hemophilia A is a deficiency in what clotting factor?
Hemophilia A and B both cause a prolonged _____
Hemarthrosis occurs in 80% of patients with ________
Hemophilia B is a deficiency of what clotting factor?
This is the most common hereditary bleeding disorder
Von Willebrand disease
Mucocutaneous bleeding and petechiae could indicate what?
von willebrand disease
How is von willebrand disease managed?
DDAVP and cryoprecipitate
This malignancy peaks at age 20 and presents with painless lymphadenopathy.
What are systemic B symptoms?
cyclical fevers, night sweats, and weight loss
Reed sternberg cells are pathognomonic for what?
Non Hodgkin's occurs in what age group? What lymph nodes are most commonly affected?
> 50; peripheral
This is malignancy of WBC's that causes bone marrow proliferation of abnormally large amounts of WBC's
This is the most common childhood malignancy
acute lymphocytic leukemia
Philadelphia chromosome diagnoses what leukemia?
chronic myeloid leukemia
This is an acquired myeloproliferative disorder with overproduction of all 3 stem cell lines (RBC's, plts, WBC's)
What is the treatment of choice for polycythemia vera?
Neutropenia severity is often characterized based on what?
Absolute neutrophil count is equal to what?
WBC x (PMC + bands)/100
This is an abnormally low level of neutrophils
After hematologic malignancies, what is the most common childhood malignancy?
What is given to patients with a CNS tumor and increased ICP?
What is the preferred approach for most childhood brain tumors to obtain tissue and bulk removal?
open surgical procedure
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