Hematology/ ONcology Flashcards
3 possibilities for causes of anemia
reduced production
increased destruction
loss of RBCs
in what type of anemia will MCV be low?
iron deficiency
what type anemia is due to red cell aplasia?
Diamond Blackfan anemia
is “normal” reticulocyte count in the face of anemia normal?
No, it is abnormal
how to you tx diamond blackfan anemia?
steroids
BMT is curative
if reticulocyte count is increased and there is anemia what is happening?
hemolysis
blood loss
what are intrinsic problems that cause hemolysis leading to anemia?
membrane defects- hereditary spherocytosis
enzyme-G 6PD deficiency
Hemoglobinopathies
what is the most common childhood hematologic problem?
anemia
what does jaundice with anemia indicate?
hemolysis
where do all RBCs go to die?
spleen
presents with Pallor, fatigue, dizziness, poor growth, exercise intolerance
Tachycardia, murmur, headache, syncope
anemia
anemia w/ lymphadenopthy can indicate what?
leukemia
HIV
JRA
anemia with splenomegaly can indicate what?
leukemia
sickle cell
hemoglobinopathy
spherocytosis
what are the three cell lines?
red cells
white cells
platelets
when 2 cell lines are depressed what is there a higher likelihood for?
leukemia
what do schistocytes indicate?
HUS
what can blister cells indicate?
G6PD
should iron deficiency show up early in life?
no, have iron stores from mom for first 5-6 months of life
what causes iron deficiency anemia in kids 6-24 months
poor dietary intake (ie cows milk)
tx for iron deficiency anemia
6 mg/ kg/ day divided TID
why does cows milk lead to iron deficiency anemia?
calcium in cows milk binds the iron
what are 2 macrocytic anemias?
Folate and B 12 deficiency
what does irritable, weakness, paresthesias, unsteady gait indicated deficiency in
Vit B 12
congenital aplastic anemia. Will have congenital anaomlaies, thrombocytopenia. diagnosed in 2-10 year olds
fanconi’s
just affects red cell lines. shows up at birth- 1 year.
diamond black-fan
what are 2 congenital anemias
fanconi
diamond black fan
what can cause acquired anemias
idiosyncratic rxn to meds
after an infection
idiopathic (mostly)
in acquired anemias are there HSN or jaundice?
No
what causes isoimmunization hemolysis?
Rh/ABO/ minor blood groups
what are congenital hemolysis?
sphereocytosis
G6PD deficiency
pyruvate kinase deficiency
hemoglobinopathies
autosomal dominant. Managed with splenectomy. cells look like spheres.
Hereditary spherocytosis
why do you take the spleen out with hereditary spherocytosis
so the spleen stops killing the red blood cells
Most frequent inherited enzyme defect
X-linked recessive (males)
Anemia, hyperbilirubinemia* jaundice
Episodic hemolysis with oxidative stress
G6PD deficiency
what is the 2nd most commonly inherited enzyme defect
pyruvate kinase deficiency
when do symptoms of beta thalassemias show up?
4 months due to fetal hemoglobin
when do symptoms of sickle cell onset?
3-4 months
what will you have with sickle cell dz?
splenomegaly due to sequestration
recurrent episodes of vasoocclusion and tissue ischemia
what should be given for prophylaxis for sickle cell dz?
penicillin (pneumococcal, meningococcal, H. flu)
what are 3 general categories of platelet disorders
Decreased production
Increased consumption
Impaired function
what are 3 disorders of coagulation
Hemophilias
Vitamin K deficiency
DIC
what measure the intrinsic pathway?
aPTT
what measures the extrinsic pathway?
PT
kids show up with a lot of petechiae and purpura what do you suspect?
ITP
what causes decreased production leading to thrombocytopenia
infiltrative diseases (affect bone marrow) congenital production defects viral infection?
large vascular venous malformation
Kasabach-Merritt syndrome
what cause increased consumption leading to thrombocytopenia (more common)
ITP
DIC
Kassabach-Merrit syndrome
what causes thrombocytopenia by impaired function
Von Wilebrand dz class test- bleeding time
in peds what is the most common bleeding disorder?
ITP
what are 3 acquired bleeding disorders?
DIC
liver dz
henoch Schonlein Pupura (HSP)
what age range is ITP most common in?
2-5
what does ITP most often follow?
viral infection
how do you tx ITP
steroids
IVIG in severe situations
will there be LAD or HSM with ITP
No
what will platelet counts be below with ITP
<10,000
What will be increased in the bone marrow with ITP?
megakaryoctyes
factor VIII deficieicny is what?
Hemophilia A
factor IX deficieincy is what
hemophilia B or
Christmas Dz
hemophilias will have prolonged what?
aPTT
Tx for hemophilia A (classic)?
Factor VIII
desmopressin releases endothelial stores of factor VIII and vWf
Tx for hemophilia B (christmas dz)?
Factor IX
Most common inherited bleeding disorder among Caucasians – autosomal dominant
Von Willebrand Dz
Tx for Von Wilebrand Dz?
Desmopressin (causes release of VWF from endothelial stores)
what is an autoimmune mediated vasculitis.
Henoch schonlein purpura
what causes cholestasis?
IV fluids (TPN live on trashes your liver)
what contains all the clotting factors and can help with DIC?
fresh frozen plasma
what do you give for a low fibringoen?
cryoprecipitate
what is a physiologic cause of unconjugated hyperbilirubinemia?
breast milk jaundice
what enzyme catalyzes bilirubin conjugation in liver.
Glucuronyl transferase (low levels at birth)
what is a condition where an infant often has polycythemia at birth?
trisomy 21
caused by Abnormal gene promoter leading to decreased transcription of mRNA less enzyme
Gilbert’s syndrome
what are sxs of gilbert’s syndrome?
life-long episodes of mild juandice
enhanced neonatal juandice
tx for gilbert’s syndrome
no tx except in neonate w/ excessive hyperbilirubinemia
who is Gilbert’s syndrome more common in?
males
are LFTs normal with Gilberts
yes, except direct bili
syndrome with no enzyme activity leading to severe unconjugated hyperbilirubinemia starting at th-5th day of life
Type 1
Crigler-Najjar Syndrome
How do you tx type 1 crigler-najiar syndrome?
liver transplant
what type of Crigler-Najjar Syndrome has diminished activity?
Type 2
how do you tx Crigler-Najjar Syndrome
type 2?
phenobarbital
conjugated hyperbilirubinemia is a sign of what?
liver dz
what happens with biliary atresia
conjugate the bilirubin but you can’t excrete it. Will have impeded bile acid flow
what does a wide mediastinum indicate?
thymus, lymphadenopathy
2 main cancers in peds population
leukemias
brain tumors
what is the most common death by disease in children?
cancer
what are the 2 main things to do for a CA workup?
CBC with diff
peripheral blood smear
what is the most common of the leukemias?
acute lymphoblastic leukemia (ALL)
when is ALL common?
ages 2-10
what conditions have a higher incidence of ALL
T21
NF type 1
Fanconi’s anemia
what will you see with ALL on chest xray?
mediastinal widening or anterior mediastinal mass or tracheal compression from lymphadenopathy.
are bone marrow transplants (HSCT) common with ALL?
no, chemo usually cures
which has a worse prognosis- ALL or AML?
AML
what leukemia can have translocation of chromosome 9 and 22 (Philadelphia chromosome)
Chronic Myelogenous Leukemia
what is the only curative intervention for CML?
HSCT
where do lymphomas arise?
lymph nodes
thymus
spleen
what is the most common lymphoma?
Hodgkin disease
where will you find a mass with hodgkin lymphoma?
cervical, supraclavicular adenopathy or mediastinal
what cells will be present with hodgkin?
Reed Sternberg cells (malignant B cells)
Tx for hodgkin dz?
chemo + los dose radiation
what will a child with hodgkin present with?
fatigue cough SOB anorexia weight loss fever night sweats
are hogkin’s or NH more malignant?
NK
Tx for NH lymphoma?
chemotherapy
what is the second most common cancer in kids?(more common solid tumor)
brain tumors
what is the classic triad of brain tumors in kids?
morning headache, vomiting, papilledema
what is the most common brain tumor of childhood?
astrocytoma (also known as gliomas)
symptoms that go along with mass in supratentorial area?
seizures
hemiplegia
focal neurological signs
What symptoms will you have with an craniopharyngioma (midline tumor)
bitemporal hemianopsia
pituitary failure (growth failure)
diabetes insipids
weight gain
what would a tumor in the cerebellar and IVth ventricle present with?
truncal ataxia
coordination difficulties
abnormal eye movements
What would a brainstem tumor present like?
cranial nerve defects
pyramidal tract signs
ataxia
often no raised ICP
does cerebellar astrocytoma have high or low cure rate?
high cure rate
what brain tumors have a very poor prognosis?
brainstem gliomas
glioblastoma
with kidney problem what diagnostic should you start with?
Ultrasound
what does a neuroblastoma do that a nephroblastoma doesn’t?
crosses the midline
what is the most common solid neoplasm outside the CNS?
Neuroblastoma
when you hear abdominal pain and masses what should you suspect?
neuroblastoma
Tx for neuroblastoma
surgical resection with chemo
what is the definitive diganostic tool for neuroblastoma?
biopsy
large tumor in the kidney that doesn’t cross the midline.
Wilms tumor
what is the 2nd most common abdominal tumors in children?
Wilms tumor
when are Wilm tumors common (age wise)?
2-5, unusual after age 6
who can wilms tumors been more common in?
Kids with hypospadias, GU malformations and others
is a Wilms tumor painless or painful?
painless
Tx for wilms tumor.
surgical resection/ exploration then chemo
what other physical symptoms may be present with a Wilms tumor?
hematuria and HTN
neuroectodermal malignancy” arising from embryonic retinal cells
Retinoblastoma
most common sign of retinoblastoma.
white pupillary reflex
tx for retinoblastoma
radiation, enucleation (take out eye)
prognosis depends on spread beyond globe
when is a retinoblastoma usually diagnosed?
before age 3
Diagnostics for retinoblastoma
MRI
most common soft tissue sarcoma in childhood
Rhabdomyosarcoma
when are the 2 peaks for sarcomas in children?
2-5 years and adolescents
most common locations for sarcomas in younger patients.
head, neck, and GU
most common locations for sarcomas in older patients
extremities
trunk
male GU
Diagnostics for Rhabdomyosarcoma
Xrays
Ct
MRI
(look for tumor and mets)
Tx for rhabdomyosarcoma
surgery
chemo
radiation
when are osteosarcomas and ewing sarcomas most often seen
growth spurts
is osteosarcoma or Ewing sarcoma more commonly seen?
osteosarcoma
