Hematology/Oncology Flashcards
three reasons for anemia
reduced production
increased destruction
loss of blood
When would you expect a low retic count?
Iron def anemia
ALL
Diamond Blackfan anemia
when would you expect a high retic count?
Hemolysis
Bloos loss
Intrinsic hemolysis
hereditary spherocytosis
G6PD deficiency
hemoglobinopathies
Extrinsic hemolysis
AIHA
DIC
IV hemolysis
most common childhood hematologic problem
anemia
Kid comes in with sxs of anemia and is jaundice and splenomegaly. What kind of anemia do you think?
hemolytic
Kid comes in with tachycardia, flow murmur, poor exercise tolerance, headache, fatigue, irritability, poor feeding and syncope
acute anemia
This anemia is well tolerated in kids, may show up with tachycardia or a flow murmur
chronic anemia
peripheral blood smear shows us
size, shape, inclusions
iron studies include
serum Fe, ferritin, TIBC
hypochromic, microcytic RBCs
Fe deficiency
schistocytes associated with
HUS
Blicter cells associated with
G6PD
Sickle cells associated with
Hgb SS
spherocytes associated with
autoimmune hemolytic
most common anemia in childhood
Fe deficient anemia
treat Fe deficiency anemia
oral Fe 6mg/kg/day divided TID
macrocytic (megaloblastic) anemia
Folate
B12 deficiency
B12 absorbed where
terminal ileum (watch with crohns)
Folate absorbed where
jejunum (watch with celiac)
2-10yr olds at diagnosis. Congenital anomalies in ~ 50% (skeletal, renal, microcephaly, hypogenitalia) pancytopenia (AR)
Fanconi
birth – 1 yr, responds to steroids. (prednisone 2mg/kg/day) short stature and congenital anomalies in ~ 30% pt. (AD/AR)
Diamond Black-Fan
pancytopenia with hypocellular bone marrow. Sx related to anemia or thrombocytopenia, fevers. No HSM, no jaundice
acquired aplastic anemia
bone marrow not making enough stuff
test for isoimmunization
Coombs
direct looks for ab on RBC
indirect looks for ab in sera
AD disorder, splenic sequestration of abnormal cells. Anemia, jaundice, and splenomegaly occur
hereditary spherocytosis
treat hereditary spherocytosis
take out spleen
most frequent inherited enzyme defect
G6PD deficiency
X-linked recessive (males)
Anemia, hyperbilirubinemia* jaundice
Episodic hemolysis with oxidative stress
G6PD
2nd most common inherited enzyme defect
pyruvate kinase deficiency
Quantitative deficiency in production of globin chains (alpha or beta)
thalassemia
Associated with Hb S, elevated retic count (normo or macrocytic)
sickle cell disease
disorders of coagulation
hemophilias
vitamin k deficiency
DIC
most common type of thrombocytopenia
increased consumption
Idiopathic thrombocytopenic purpura
types of increased consumption thrombocytopenia
ITP
DIC
Kasabach-Merritt syndrome
most common bleeding d/o of childhood
ITP
Petechaie, ecchymoses, decreased platelet count, otherwise healthy child. Often follows a viral infection (rubella, varicella)
ITP
treat ITP
IVIG best response
steroids good response
hemophilia A
factor VIII
hemophilia B
factor IX
Christmas disease
treat factor VIII deficiency
desmopressin (or order factor VIII)
treat factor IX deficiency
give factor IX
Most common inherited bleeding disorder among Caucasians – autosomal dominant
von willebrand
treat VWD
desmopressin
clotting d/o’s are uncommon in kids T or F
TRUE
Diseases in which you have a defect in glucuronyl transferase
gilbert’s
crigler naijar
Abnormal gene promoter leading to decreased transcription of mRNA, leading to less GT enzyme
Gilbert’s
treat Gilbert’s
No treatment needed except in neonate with excessive hyperbilirubinemia
type 1 crigler-naijar
gene mutation- no GT activity- letha
type 2 crigler naijar
diminished GT activity, treat with PB
two main cancers seen in peds
leukemia
brain tumors
Most common cause of death by disease
cancer
most common leukemia
ALL
least common leukemia
CML
associated with philadelphia chromosome
chronic myelogenous leukemia
associated with philadelphia chromosome
chronic myelogenous leukemia
most common lymphoma
Hodgkin
most common lymphoma
Hodgkin
painless lymphadenopathy, mediastinal mass, fatigue, cough DOB, anorexia, occurs mostly in teenagers
hodgkin
biopsy lymph node in hodgkin to look for what
Reed Sternberg cells (malignant B cells)
common nodes hodgkin
cervical or supraclavicular
classic triad brain tumor
morning HA, vomiting, papilledema
most common tumor of childhood
astroytoma
neural crest tissue of sympathetic ganglia or adrenal medulla
most common solid neoplasm outside CNS
neuroblastoma
most common presentation for a neuroblastoma
abdominal pain or mass
2nd most common solid tumor, 1st most common abdominal tumor
neuroblastoma
second most common abdominal tumor
wilms
increasing size of abdomen with PAINLESS abdominal mass. Hematuria or HTN may be present
wilms
most common sign for a retinoblastoma
white pupillary reflex
most common soft tissue sarcoma in childhood
rhabdomyosarcoma
common place for rhabdomyo in younger pt
head, neck, GU
common place for rhabdomyo in older pts
extremities, trunk, male GU
who gets sarcomas most
adolescents- peaks with growth spurt