Heme Onc_UW Flashcards
What confirms dx of leukemia?
bone marrow biopsy with greater than 25%
Most common pediatric renal malignancy?
Nephroblastoma
Wilms tumor is aka
Nephroblastoma
Burr cells are seen in?
Spiculated RBCs of similar size with regularly spaced projections. Seen in uremia or artifact of preparation
Pappenheimer bodies.
Seen in sideroblastic anemia. Results from phagosomes that engulf excessive amounts of iron.
Heinz bodies seen in
G6PD deficiency
Heinz bodies are
when Hb oxidizes in GPD deficiency, they precipitate into these heinz bodies.
Universal screening for iron deficiency anemia
complete CBC 9-12 months
Tx for iron deficiency anemia
empiric oral iron therapy. Recheck Hg in 4 weeks, if it has risen by 1g/dL, continue for 2-3 months.
Tx for ITP
If only skin manifestations: observe, regardless of platelet count. If bleeding, then regardless of platelet count give IVIG or glucocorticoids
S pneumo, H influenze and N. meningitidis have what in common?
Encapsulated bacteria
Common vaccinations of childhood that use live attentuated viruses?
Measles, mumps, rubella and chicken pox
What can prevent pneumococcal sepsis in sickle cell pateints
Pneumococcal vaccine and pencillin propylaxis (kids should get it till age 5)
Common vaccinations of childhood with bacterial toxoids
Tetanus and diptheria
Epistaxis, visible nasal mass, nasal obstruction
Juvenile angiofibroma
Side effects of hydroxyurea treatment
Suppresses the bone marrow. Can cause thrombocytopenia, leukopenia and anemia. but these effects are generally temporary and reversible but can predispose patient to infection.
MOA of hydroxyurea
Increases proportion of fetal hemoglobin so that the proportion of HgS is reduced and chances of sickling reduce
What bones are frequently affected in osteonecrosis due to sickle cell anemia?
Humeral and femural heads
What is anistocytosis
Blood cells of unequal size
Lab findings for iron deficiency anemia
Decreased MCV, decreased transferrin, anistocytosis => increased RDW, decreased MCH, decreased trasnferrin saturation, increased TIBC. Smear shows small hypochromic rbcs.
MCHC is elevated or depressed in hereditary spherocytosis? Why?
It’s elevated. Due to cellular dehydration and membrane loss, the MCHC is high.
What are the diagnostic tests for hereditary spherocytosis?
Osmotic fragility test with acidified glycerol lysis test and abnormal eosin-5-maleimide binding test (flow cytometry)
What is the typical manifestation of hereditary spherocytosis
Coombs negative hemolytic anemia, jaundice and splenomegaly
Genetic pattern of hereditary spherocytosis
75% A/D, 25% spontaneous
What are lab findings/diagnostic tests for hereditary spherocytosis
Imp finding is elevated MCHC. Due to cell dehydration and lower cell volume, this is elevated. Spherocytes on peripheral blood smear, coombs negative. Diagnostically do the acidfied glycerol lysis test and the eosin 5 maleimide binding test.
How is vitamin K related to factors 2,7,9,10 and factors C and S.
Vitamin K is a cofactor for the enzyme gamma glutamyl carboxylase that adds carboxyl groups to the glutamate residues of several coagulation factors such as 2, 7, 9, 10 and C and S. These extra carboxyl groups add the factors affinities for platelets
Most common primary bone tumor in children
Osteosarcoma
Osteosarcomas are found most commonly in
Metaphyses of long bones: proximal humerus, distal femur, proximal tibia.
PE findings and X -ray findings for osteosarcoma
Large tender mass. X-ray finding: sun-burst periosteal pattern and “codman” triangle.
Lymphoblast histology/staining
Lack peroxidase positive material, often contain +PAS staining material, 95% immunostain for TdT. (TdT is only present in pre B and T lymphocytes)
Example of clotting disorders
Hemophilia A and B
Activated PTT time in hemophilias
Increased activated PTT
Examples of platelet defects
Von Willebrand and Bernard Soulier Syndrome
Ewing’s sarcoma is often confused with
Osteomyelitis because of the intermittent fevers, leukocytosis, malaise, joint swelling
Characteristic x-ray findings for Ewing’s sarcoma
Lytic, central bone lesion with periosteal reaction leading to onion skin appearance, followed by mottled appearance/moth eaten and extension into soft tissue
Cobalamin deficiency presents as
Macrocytic anemia
Most common inherited bleeding disorder
Impaired Von Willebrand factor
Primary dose limiting side effect of hydroxyurea
Myelosuppression
Brainstem is composed of
Midbrain, pons, medulla
Pineal gland secretes
Melatonin
What is parinaud syndrome
Downward gaze preference (sun setting gaze) with limited upward gaze, ptosis, upper eyelid retraction, pupillary abnormalities
What are the clinical sx of pineal gland mass
Parinaud syndrome, obstructive hydrocephalus, precocious puberty (if the pineal gland mass is a germ cell tumor that secretes b-HCG)
What are the clinical sx of obstructive hydrocephalus
papilledema, vomiting, headache, ataxias
What kind of masses can arise from pineal gland?
variety but most common is the germ cell tumor
Wht is the triad of hemolytic uremic sydnrome
renal failure, thrombocytopenia and microangiopathic hemolytic anemia
clinical ppt of HUS
abdominal pain and diarrhea followed by bloody diarrhea. Within 5-7 days => anemia, thrombocytopenia and renal insufficiency.
PE of HUS
Pallor, jaundice (hemolysis), edema, petechiae, HTN
What is the characteristic peripheral smear of HUS
Schistocytes and large platelet
Lead poisoning causes what kind of anemia
Microcytic anemia. Basophilic stippling on peripheral blood smear.
Triad of Wiskott Aldrich Syndrome
Eczema, thrombocytopenia (from decreased platelet production), hypogammaglobulinemia leading to recurrent bacterial infections.
How does polycythemia lead to cyanosis?
High red blood cell mass and hyperviscosity leads to cyanosis
What is the definition of neonatal polycythemia
Hematocrit greater than 65%
Wht are the risk factors for neonatal polycythemia
Increased erythropoiesis from Maternal diabetes, maternal hypertension, IUGR, smoking. Or erythrocyte transfusion from delayed cord clamping or twin twin transfusion
Clinical presentation of neonatal polycythemia
Neurologic manifestations (irritability, jitteriness), respiratory distress and hypoglycemia.
