peripheral pancytopenia w/ hypocellular bone marrow
acquired aplastic anemia
most common inherited anemia?
Congenital Aplastic Anemia- Fanconi anemia
defective DNA repair that is caused by genetic mutation- pancytopenia
congenital aplastic anemia
weakness, fatigue, pallor, frequent infections, purpura, petechiae, bleeding, neutropenia, low platelet count
acquired aplastic anemia
hx of pica w/ decreased serum ferritin, serum iron, increased TIBC
elevated RDW
iron deficiency anemia
megaloblastic anemia finding on peripheral smear
hypersegmented neutrophil
spherocytes on peripheral smear indicate
hereditary spherocytosis
a-thalassemia severity is related to?
of gene deletions
B-minor thalassemia presents?
asymptomatic, mild, microcytic hypochromic anemia
B-major thalassemia presents?
homozygous for B-globin mutation, early death
chronic hemolysis: pallor, fatigue, jaundice, & development of gallstones during childhood & adolescence
Pain due to vaso-occlusion
sickle cell
labs showing thrombocytopenia and/or neutropenia, followed by progression to pancytopenia
congenital aplastic anemia
short stature, cafe-au-lait spots, cephalic issues, developmental delay
congenital aplastic anemia
peripheral pancytopenia w/ hypocellular bone marrow
low reticulocyte count
acquired aplastic anemia
what is the most common anemia
iron deficiency anemia
heinz bodies on peripheral smear?
G6PD
osmotic fragility test?
detects elevated RBC hemolysis
for hereditary spherocytosis
what are the two diagostics for hereditary spherocytosis
spherocytes on smear
osmotic fragility test
lead poisoning shows what on peripheral smear?
basophilic stippling
primary polycythemia is
congenital erythrocytosis- elevated RBCs
secondary polycythemia is
in response to hypoxemia
most common cause of secondary polycythemia
cyanotic congenital heart disease
what is the most common bleeding disorder of childhood
idiopathic thrombocytopenic purpura
ITP etiology?
often follows viral infection
most common inherited bleeding disorder?
Von Willibrand disease
Petechiae, ecchymosis, epistaxis
ITP
hemophilia A is
factor VIII deficiency
hemophilia B is
factor IX deficiency
which hemophilia is most common
A
most common inherited thrombotic condition (in caucasians, anyways)
Leiden mutation
palpable purpura on lower extremities, arthralgias, abd. pain, renal disease
Henonch-Schonlein Purpura (HSP)
most common small vessel vasculitis
HSP
inherited thrombotic disorders (4)
protein C deficiency
protein S deficiency
antithrombin deficiency
Leiden mutation
complications of thrombotic disorders
DVT, PE
uncontrolled proliferation of immature lymphocytes
ALL
complications of tumor lysis syndrome
hyperkalemia
hyperuricemia
hyperphosphatemia
—> acute renal failure
CBC with WBC > 50,000/uL in 20% of pts
ALL
25% of pts have WBC > 100,000/uL
AML
Auer rods on peripheral smear indicate
AML
where will you see blasts on the peripheral smear
ALL & AML
Anemia, thrombocytopenia, leukocytosis, relatively few blasts
CML
most common solid tumor of childhood
brain tumor
most common brain tumor
glial tumor
astrocytomas & ependymomas
deadlier, more aggressive brain tumor
medulloblastoma
malignant marker cells in Hodgkin Disease?
Reed-Sternberg Cells
painless cervical or supraclavicular adenopathy, mediastinal mass, weight loss, fever, night sweats, fatigue, splenomegaly, hepatomegaly
Hodgkin Disease
LAD, abd pain, fevers, cough, dyspnea, weight loss, night sweats
All developing over 1-3 weeks
Non-Hodgkin Lymphoma
most common solid neoplasm outside the CNS
neuroblastoma
labs w/ anemia & increased urinary catecholamines
neuroblastoma
fever, weight loss, abdominal mass, irritability, bone pain, hematuria, HTN
nephroblastoma
nighttime bone pain
Ewing’s Sarcoma
most common soft tissue sarcoma in kids
rhabdomyosarcoma
enlarging abdomen, elevated aFP
hepatic tumor
bone tumor that occurs most often in the pelvis, long bones
minority in soft tissue
Ewing’s Sarcoma
normal platelet count
elevated PT, aPTT
decreased fibrinogen
liver disease
weakness, fatigue, purpura, petechiae, frequent infections, bleeding
acquired aplastic anemia
