Pediatric Hematology Flashcards
hemophilia and their correlated clotting factor
- hemophilia A: factor VIII
- hemophilia B: factor XI
s/s of hemophilia
- hemarthrosis
- bruising
- easy bleeding
- only in males
diagnosis of hemophilia
- low clotting factor
- prolonges PTT
treatment of hemophilia A
- mild: desmopressin
- moderate/severe: factor VIII concentrate
treatment of hemophilia B
factor IX concentrate
patient education fo hemophilia
avoid contact sports
MC inherited bleeding disorder
von Willebrand disease
Types of von Willebrand disease
- Type I: quantity deficient
- Type II: quality impaired
- Type III: severe absent vWF
s/s of von willebrand
- bleeding from trivial wounds
- oral cavity bleeding
- excessive menstrual bleeding
Treatment of von Willebrand Disease
desmopressin
hemophilia C
factor XI deficiency
treatment of factor XI deficiency
factor XI or FFP
s/s of immune thrombocytic purpura
sudden appearance of petechial rash, bruising, or bleeding in a healthy kid
diagnosis of ITP
isolated thrombocytopenia
management of ITP
- initial management is watchful waiting
- severe: steroids first line, then IVIG
etiology of TTP
ADAMTS13 deficiency
presentation of TTP
- neurologic sx
- fatigue
- petechiae
diagnosis of TTP
- anemia
- thrombocytopenia
- schistocytosis
treatment of TTP
plasma exchange with FFP
etiology of HUS
E.coli
s/s of HUS
- gastroenteritis
- lethargy
- seizures
- HTN
- renal failure
- edema
diagnosis of HUS
- elevated BUN/Cr
- anemia
- thrombocytopenia
- ecoli in stool
treatment of HUS
- typical: supportive
- not caused by ecoli (atypical): plasma exchange
etiology of DIC
- sepsis
- snake bite
- cancer
- trauma
- aneurysm
s/s of DIC
blood oozing out of multiple sites
diagnosis of DIC
- prolonged PT/PTT
- thrombocytopenia
treatment of DIC
blood products
screening for anemia in kids
at 1 and 2
why do we see anemia in 12 month olds?
transition from formula to milk
s/s of anemia
pale, fatigue, shortness of breath, tachycardia
MC malignancy in children
ALL
s/s of ALL
- bone pain
- weakness
- intermittent fevers
- petechiae
- infections
diagnosis of ALL
- lymphoblasts on smear
- pancytopenia
technique for diagnosis of ALL
bone marrow aspirate
treatment of ALL
- phase 1: induction chemo
*phase 2: chemo and radiation
*phase 3: maintenance oral chemo and mtx and oral steroids
s/s of AML
- flu like symptoms
- fatigue
- poor appetite
- bleeding
- infection
diagnosis of AML
blasts on smear
treatment of AML
- phase 1: induction chemo
- phase 2: intrathecal chemo and radiation
- phase 3: high dose chemo
S/S of Hodgkin’s Lymphoma
- painless cervical lymphadenopathy
- mediastinal mass
- constitutional sx
diagnosis of hodgkin’s lymphoma
biopsy of node shows reed sternberg cells
Treatment of Hodgkin’s Lymphoma
chemo
S/S of Non-Hodgkin’s Lymphoma
- night sweats
- cough
- SOB
- weight loss
- swollen lymph nodes
MC site of Non-Hodgkin Lymphoma
abdomen
types of non-hodgkin lymphoma
- burkitt: abd pain, distention
- lymphoblastic: ALL like sx
- large cell: doesn’t spread as quickly
Diagnosis of Non-Hodgkin’s lymphoma
lymph node biopsy
Treatment of Non-Hodgkin’s lymphoma
chemo and sometimes radiation
triad of brain tumor presentation
- morning HA
- vomiting
- papilledema
MC glioma of childhood
astrocytoma
diagnosis of brain tumors
MRI with confirmation via biopsy
brain stem gliomas
- middle of brainstem
- challenging to treat
ependymoma
- lining of the ventricles
- blocks CSF flow
MC malignant brain tumor
Medulloblastoma
location of neuroblastoma
adrenal medulla
s/s of neuroblastoma
- bone pain
- fixed, solid, abdominal mass extending beyond midline
treatment of neuroblastoma
surgery, chemo, radiation
s/s of lead poisoning
- weakness
- irritability
- weight loss
- vomiting
- ataxia
- HA
- retarded development
screening for lead poisoning
blood test at 1 and 2
treatment of lead poisoning
- levels over 45: chelation with succimer
- levels over 70: hospitalize
drugs used for chelation of lead
- calcium disodium EDTA
- dimercaprol
- succimer
- d-penicillamine
neutropenia levels
- mild: ANC>1000 and <1800
- moderate: ANC>500 and <1000
- severe: ANC<500
etiologies of neutropenia
- insufficient production
- shift in neutrophils from circulating pool to marginal blood
- destruction due to autoimmune disorders
- medication
complications of neutropenia
infection
when to refer to heme for neutropenia?
ANC below 1000
management of neutropenia
- myeloid growth factors
- if med caused, stop med
- if autoimmune, steroids