HEME/ONC peds Flashcards
What are part of the MYELOBLASTS?
Basophil, Neutrophil, Eosinophil
What is part of the lymphblasts?
Lymphocytes
What is part of the monblasts?
Monocytes
What is the most common childhood hematologic problem?
Anemia
This clinical presentation represents? Pallor, fatigue, dizzy, poor growth, exercise intolerance, tachycardia, murmur, HA, syncope,
Jaundice = hemolysis Splenomegaly = hemolysis, splenic congestion
Anemia
What facts of history are looked at in anemia?
Predisposing diet Blood loss Known congenital problems Family hx Abnormal newborn metabolic screen
What does this describe?
Tachycardia, FLOW MURMUR, poor exercise tolerance, HA, fatigue, irritable, poor feeding
More severe, hemolysis
Acute Anemia
This type is very well tolerated in children
Tachycardia, flow murmur
Chronic Anemia
Historical clues for anemia
nutrition, growth and development
- sx of chronic dz = malabsorption, blood loss
- hx of jaundice
- ethnic background
- Fam hx of splenomegaly, splenectomy, anemia, jaundice
- Patient age
chronic disease or hypothyroid, Fe deficit
poor growth
aplastic anemia
congenital anomaly
leukemia, HUS
petechiae, purpura
hemolysis, HIV, JRA
jaundice
leukemia, sickle cell, hemoglobinopathy, spherocytosis
splenomegaly
what type is thalassemia and sickle cell?
hemoglobinopathy
impaired red cell production, increased red cell destruction, and blood loss causes what?
anemia in children and infants
What are the 2 best labs?
CBC and Retic count
What does it mean for retic count to be elevated?
Bone marrow response to anemia,
1-2% retics in circulation normally
size of rbc?
MCV
density of hemoglobin in RBC?
MCHC
range of size of mature RBC?
RDW
What is the best indicator in an iron study?
FERRITIN
Also in this : Serum FE, TIBC
What is low red blood cell production indicate?
low retic count
Parvovirus B19 and Diamond-Blackfan are what types of anemia?
low retic anemia
What does normal / elevated retic represent?
Bilirubin raised or normal
What if Bilirubin is raised?
hemolysis (beta thalassemia, sickle cell)
What if Bilirubin is normal?
Blood loss or ineffective erythropoiesis
red cell aplasia, respond to steroids, BMT curative, low retic
Diamond Blackfan anemia
EPO deficinecy
chronic renal failure, lot retic count
membrane defects - hereditary spherocytosis , enzyme 6gpd deficit, hemoglibinopathies, increased retic count
intrinsic hemolysis
auto-immune hemolytic anemia, DIC ,IV hemolysis, increased retic count
extrinsic hemolysis
Blood loss, increased retic count
Loss
Most common anemia in childhood
- term infants are born with enough iron for 4-5 months
- poor diet intake of iron (cows milk)
pallor , fatigued, irritable, poor growth
- micryoctytic, hypochromic, low MCV
- if neutropenic or thromboyctopenic –> check bone marrow
- oral __ is 6mg/kg/day
Fe deficit anemia
what is the treatment of iron deficit anemia?
oral supplements
- increased retic 3-4 days
- increased hemoglobin 4-30 days
- increased iron stores 1-3 months
b12 deficit in what diet?
vegan diet
folate deficit in what diet?
goat’s milk, no veggies
chrons dz, and pernicious anemia
smooth tongue, beefy red, irritable, weak, paresthesia, unsteady gait
b12 deficit
celiac dz
folate deficit
Anemia of inflammation/chronic illness, arrested development, infiltration of bone marrow space
Nomoschromic, normocytic Anemia
congenital - fanconi anemia, Diamond- blackfan
acquired- parvovirus 19 infection
arrested development of cell
2-10 year olds at diagnosis, congenital anomalies 75% of time (skeletal, cafe au lait, renal microcephaly, thrombocytopenia, neutropenia –> pancytopenia
- rocker bottom feet, dangling thumbs, short
autosomal recessive
tx : transfusions ,bone marrow transplant
underproduction, congenital aplastic anemia, fanconi
birth - 1 year, respond to steroid, short, AD
ONLY RBC LINES AFFECTED
underproduction congenital hypoplastic anemia, Diamond Blackfan
underproduction - acquired
pancytopenia with hypocellular bone marrow
- sulfonamides, nsaids, anticonvulsants rxns
- infections (hep, hivm syphilis, mono)
- 50% idiopathic
- sx: anemia, thrombocytopenia, fevers. NO HSM, or jaundice
- death from hemorrhage or infection
Tx: transfusions, immune modulators
TX: transfusions, immune modulators
Increased destruction
isoiz - hemolysis
–> rh/abo/ minor blood groups, hemolytic dz of the newborn
- Congenital hemolysis
- -> sphercytosis, G6PD deficit, pyruvate kinase deficit, hemoglobinopathy
- Infections (bacterial, viral, protozoa)
- Autoimmune hemolytic anemia
- Thrombotic microangiopathy
sphercytosis, G6PD deficit, pyruvate kinase deficit, hemoglobinopathy, these are what?
Congenital Hemolysis
increased destruction
hemolytic disease of the newborn
mom rh- / baby rh +
- mom makes anti-D antibodies that hemolyzes fetal cells
- coombs test –> direct = antibodies on rbc, indirect = antibodies in sera
Jaundice within the FIRST 24 HOURS of life.
RBC membrane defects
hereditary spherocytosis
- splenic sequestration of abn cells
- anemia, jaundice, splenomegaly
- gallstones 60-70% adults
- increased retic
- smear shows spherocytes, normoctyic, hyperchromic
most frequent inherited enzyme defect
- x linked (males) recessive
- anemia, HYPERBILIRUBINEMIA, jaundice
- episodic hemolysis with oxidative stress
RBC ezyme defect : G6PD deficit
2nd most common RBC enzyme defect?
Pyruvate kinase deficit
deficit in the production of globin chains (alpha and beta) , beta not present for first 4 months,
- MICROCYTIC AND HYPOCHROMIC
- severe = pallor, HSM
Hemoglobinopathy
Thalassemia
high prevalence in central Africans, mediterranean, indians, middle east, carribean
- Increased retic count ( normocytic, macroctyic)
- smears have sickle cell and target cells
sx: 3-4 months of life, pallor fatigue, jaundice. increase risk of gallstones, splenomegaly d/t sequestration
recurrent vasocclusion and tissue ischemia
recurrent abdominal / msk pain
Sick Cell Disease
Decreased production
Increased consumption
Impaired function
Disorders of platelets
Hemophilias
Vitamin K deficit
DIC
Disorders of coagulation
Petechiae, purpura, bruising, hemorrhage, GI bleed, frequent/prolonged epistaxis, heavy menses, hemarthrosis
SX of bleeding disorders
Bleeding to the skin and mucous membranes is characteristic of what bleeding disorders?
There’s petechiae, and ecchymoses
Platelets and blood vessel bleeding disorder
Bleeding into soft tissue muscle and joints is of what bleeding disorders
Hemophilia or other disorders of coagulation proteins
Thrombocytopenia has what 3 things?
decreased production
increased consumption
impaired function
infiltrative dz : leukemia, fanconi anemia
decreased production in thrombocytopenia
immune thrombocytopenic purpura, DIC, kasaback - merritt syndrome
increased consumption (more common) in thrombocytopenia
von willebrand dz
impaired fxn in thrombocytopenia
low progenitor cells/ thrombopoietin
- congenital viral syndrome (cmv, rubella, hiv, parvo)
- marrow dz - congenital leukemia, neuroblastoma, fanconi anemia
decreased platelet production
platelet destruction/ consumption
in peds- most often immune mediated –> ITP
- can also be consumptive coagulopathy (dic, kasabach-merritt syndrome)
- clots
- infections ,shock, asphyxia
What is the most common bleeding disorder of childhood?
ITP
what are inherited bleeding disorders?
Hemophilia a,b
vWB
what are acquired bleeding disorders?
DIC
Liver Dz
Henoch Schonlein Purpura (HSP)
age 2-5
-often follows viral infection: MEASLES, rubella, varicella, EBV, influenza, parvo
- LOW PLATELETS, petechiae, ecchymosis, OTHERWISE HEALTHY KID
-
Immune Thrombocytopenia Purpura (ITP)
Factor 8 Factor 9 (christmas dz) - PROLONGED PTT, normal PT - Present in infancy with excessive bleeding after circumcision, or bruising after IM injection - Joint bleeding
HEMOPHILIA (coagulation disorder)
Factor 8 deficit (mild to severe)
- mild d/t trauma
- severe d/t spontaneous bruising, soft tissue bleeding
- PROLONGED PTT, nl PT
- x-linked males
- TX: Desmopressin
Hemophilia A (Classic)
x-linked
factor 9 deficit
prolonged ptt, nl pt
Tx: Factor 9
Hemophilia B (christmas dz)
most common inherited bleeding disorder among caucasians - autosomal dominant
- easy bruising and epistaxis from childhood
- prolonged bleeding time, nl plt count
TX: desmopressin, IV or intranasal
Von Willebrand Disease
platelet consumption, coagulation goes away
DIC
major synthesis of prothrombin, fibrinogen, clotting factors, anticoags
LIVER DZ
autoimmune mediated vasculitis –> severe abdominal pain
Henoch Schonlein Purpura
nonthrombocytic : henoch-schonlein purpura, sepsis, trauma
child with petechiae or purpura
thrombocytopenic: immune thrombocytopenia purpura, leukemia, DIC
child with petechiae or purpura
acquired disorders: vitamin k deficit, liver disease, thrombocytopenia
child with abnormal bleeding to soft tissues, mucocutaneous, following surgery
inherited disorders: hemophilias, von willebrand dz
child with abnormal bleeding into soft tissues, mucocutaneous, following surgery
generally uncommon in children
clotting disorders
most common hereditary cause of a predisposition to thrombus
factor V leiden mutation
unconjugated (indirect)
- increased production
- impaired uptake
- decreased conjugation
- Increased enterohepatic recirculation (infancy)
these are all causes of jaundice
conjugated (direct)
- impaired excretion into bile
- biliary obstruction
- hepatocyte destruction (hemolytic dz of newborn)
all causes of jaundice
what catalyzes bilirubin conjugation in liver?
Glucuronyl transferase
build up of unconugated bilirubin –> increased enterohepatic reuptake to blood
most healthy infants dont require intervention for jaundice
Physiologic Jaundice
1Physiologic – breast milk jaundice
2Hemolytic – RH, ABO, G6PD, spherocytosis,
3Polycythemia – T21, too viscous, not flowing properly through blood vessels
4Hematoma / hemorrhage – scalp injury, excessive bruising
5Glucuronyl transferase defect – (unable to conjugate) - inherited
ddx of unconjugated hyperbilirubinemia
decreased transcription of mRNA –> less enzyme
rx: no treatment needed except in neonate with excessive hyperbilirubinemia
gilbert’s syndrome (genetic cause - transferase gene)
type 1 gene mutation –> no enzyme activity
Rx: liver transplant
type 2: gene mutation –> diminished activity
Rx: phenobarbital, phototherapy
Crigler-Najjar Syndrome (genetic cause - transferase gene)
less common -bad sign
- always abnormal
signs of liver disease: biliary atresia, cholestasis
conjugated hyperbilirubinemia
what is the most common cause of death by dz in children?
Cancer
what is the most common CA in childhood?
ALL
What is the second most common CA in childhood?
Brain tumor
What is the most common solid tumor (CA) in childhood?
Brain tumor
What cancerous tumor in the abdomen crosses the midline?
Neuroblastoma
These are symptoms of what? Fatigue, bruising, anorexia, petechiae, malaise, bleeding, HA, pain, fever, vomit, abnormal lump/mass, vision changes, weight loss, pallor, night sweat
Cancer
What is in a CA workup?
CBC w/ diff
- Peripheral blood smear
- CXR
- Biopsy
- CT of solid tumors
- CSF : Leukemia, NH lymphoma, CNS tumors
Uncontrolled proliferation of immature lymphcytes (lymphblasts)
- Peak age 4, 85% 2-10 yr
- Boys > girls
- White > Black
- Lymphoblasts seen on blood smear and BM bx
-
Acute Lymphoblastic Leukemia (ALL )
What does the CXR for ALL look like?
mediastinal widening
anterior mediastinal mass
tracheal compression from lymphadenopathy
What xrays are taken for ALL?
spine and long bones - demineralization, periosteal elevation
induction - immune suppression
- consolidation : chemotherapy
- maintenance : methotrexate, vincristine, steroids
duration = 2-3 years with 80% cure rate
ALL therapy
CBC: anemia, thrombocytopenia, neutropenia
- malignant immature myeloblasts
- poor prognosis than ALL
- aggressive initial therapy with toxic substances
- 80% achieve remission, high relapse rate
- cure rate 50%
- AUER RODS
Acute Myeloid Leukemia (AML)
rare leukemia
- peripheral smear has ___ cells in all stages of maturation with few blasts
- philadelphia chromosome, very poor prognosis
- translocation of chr 9 and 22
- bone pain, fever, night sweats, fatigue can be asx
- HSCT only cure (bone marrow transplant)
- 50-80% survival rate
Chronic Myelogenous Leukemia CML
malignant proliferation of lymphoid cells
- arising from lymph nodes, thymus, spleen
- 3rd most common malignancies in kids
- hodgkin dz more common at 50%
- non hodgkin is collective group
Lymphomas
which hodgkin presents more in younger children?
Non hodgkin disease
which hodgkin presents more in adolescence or >50?
hodgkin disease
what clinical signs relate to both NH and H lymphoma?
lymphadenopathy
painless cervical or supraclavicular adenopathy, mediastinal mass
- fatigue, night sweats, cough, sob , anorexia, weight loss, fever
- CBC ,CXR
- BIOPSY : REED STERNBERG CELLS
- TX: chemo
- 5 year survival rate 90%
Hodgkin Essential
- arise in any site of lymphoid tissues (bone, marrow, CNS, skin, tests)
- more malignant
- cough, dyspnea, orthopnea, swelling of face, lymphadenopathy, mediastinal mass, pleural effusion
- abdominal pain, distension, constipation, abd mass, ascites, hepatosplenomegaly
- fever, neuro deficits, skin lesions
- CBC, CXR, BIOPSY
- TX: Chemo
Non Hodgkin Essentials
morning headache, vomiting, papilledema
classic triad of brain tumors
4th ventricular mass
medulloblastoma
neural crest tissue of sympathetic ganglia or adrenal medulla
- most common solid neoplasm outside CNS
- 50% dx before 2y, 90% by 5y
- most common: abdominal pain or mass that EXTENDS BEYOND MIDLINE
- bone pain suggests mets
- mets to : bone, marrow, lymph nodes
- cbc, plain xrays : stipled calcifications of tumor, patho fractures
- urine catecholamines (90%)
- Bx is definitive and for staging
TX: chemo to shrink tumor and surgical resection
Neuroblastoma
Where are mets of neuroblastoma?
Bone
Bone marrow
Lymph nodes
- nephroblastoma
- from immature cells of kidney
- encapsulated tumor from kidney
- affect 2-5 year old, rarely older 6
- Does NOT cross midline, abdomen increases, PAINLESS abd mass
- CXR OR CT = PULMONARY METS
TX: surgical resection then chemo
Wilms Tumor
- from embryonic retinal cells
- 90% diagnosed
Retinoblastoma
- rhabdomyosarcoma
- most common soft tissue ___ in children
- peaks = 2-5 and adolescence
- Younger pt : head, neck, GU
- Older pt : extremities, trunk, male GU
- Male > female
Sarcomas
head, neck, GU are locations for sarcoma of what age?
younger patients
extremities, trunk, male GU are locations for sarcoma of what age?
older patients
symptoms result from disturbances in normal growth fxn due to tumor growth
- orbital : proptosis, decreased extraocular motion
- nasal mass: chronic otitis, ear discharge
- bladder : hematuria, urinary obstriction, pelvic mass
- xrays, ct, mri
TX: surgery if possible, chemo, radiation
Rhabodomyosarcoma
most frequent in adolescents - peaks with growth spurts
- pain, lump, swelling
- cure rate 75%+ w/ no mets
- tissue biopsy definitive
Sarcomas - Bone
- 60% cases
- adolescents/ young adults
- diagnosed during growth spurt
- epiphysis, metaphysis most common
- pain, palpable mass
- xray : lytic lesion with calcifications around soft tissue
- surgery chemo
Osteosarcoma
- toddlers to young adults
- femur and pelvis most common
- pain, swelling
- systemic sx: fever, weight loss
- chemo/surg, chemo/radiation
Ewing Sarcoma
Short term side effects of chemotherapy
Bone marrow suppression = anemia, thrombocytopenia, bleeding, neutropenia
Immunosuppression = infection
Undernutrition from gut mucosal damage, nv, anorexia, alopecia
Late effects of cancer therapy
60% will have some disability
Complications
- growth : pituitary gland
- endocrine/ growth = thyroid, pituitary
- cardiopulm = pulm fibrosis, cardiomyopathy
- renal = hemorrhagic cystitis, bladder fx
- neurospsych = non verbal tasks, math, short term memory
- second malignancies
