Heme Onc Flashcards
Iron def treatment (kids and adols)
iron elemental 3-6 mg/kg/day in kids
adolescents 325mg daily / BID
physiologic nadir of HCT/Hb in baby
2 mos
How to distinguish Thal trait from Fe def?
thal trait: all RBC are very small so very small MCV and RDW is normal. MArked discrepancy between Hgb and MCV.
Proportional MCV/Hgb in iron def
Normal MCV:
80
1-4 year old with very low Hgb and no retic, with normal MCV
Dx and Tx?
Transient Erythroblastenia of Childhood (TEC)
No treatment needed
progressive, normochromic, macrocytic anemia in infancy or early childhood with some congenital anomaly / growth failure?
Dx
Tx
Diamond Blackfan anemia - bone marrow has no erthroid precursors
Tx: steroids, pRBCs, stem cell trp
transmission of hereditary spherocytosis?
Exam finding?
Tx needed
autosomal dominant
Splenomegaly, may need splenectomy after 5y
need folate supp
chronic stable hemolysis w/ exacerbations, runs in family, MCHC is HIGH
Hereditary Spherocytosis
Periph smear showing RBC agglutination in what type of autoimmune hemolytic anemia?
Cold IgM autoimmune HA
Direct Coombs shows what?
presence of Ab on RBCs
Warm vs cold autoimmune anemia?
Warm: IgG: bad, occurs at body temp, splenomegaly
Cold: IgM: Liver, good.(usu from mycoplasma, HIV, or mono)
hypochromasia and microcytosis in mild anemia, maybe scattered target cells should lead you to consider what?
thalassemia (less hemoglobin per RBC because decreased hemoglobin produced)
Thalassemia and iron?
expect increased GI absorption of iron, and get iron overload even w/o transfusion in severe thal
Hb A2 made of what and when do you see it?
alpha 2 and Delta 2: No B2…
Hb A has what?
normal: two alpha and two beta
alpha 2 + gamma 2 is what type of hemoglobin?
Fetal.
cure for thal major
BMT
four gamma chains called what and what is the disorder? what do they get in older yrs?
in nweborn you get 4 gammas because no alpha: alpha thal (Barts), as they get older and beta chains mature, you get B4: Hb H
Hemoglobin H disease?
In who?
What is the defect?
This is alpha thal with Asians
variable phenotype with mild/moderate hypochromatic, microcytic anemia
Also liver / spleen issues
Hemoglobin C disease?
Sickle cell
Hb SS
Hb SC
Hb S beta thal + dz
Most severe?
Hb SS. No Hb A present. A2 and F are normal, no excess alpha chains
Shortest life expectancy: 40s
S beta thal null disease course?
severe, No Hb A, incr A2 and F, Looks very similar to SS
WHat is dactylitis and what may it be found in?
swelling of hands / feet, may be first manifestation of sickle cell
weird bug in sickle cell osteomyelitis?
salmonella
lusaback merritt
hemangioma and low plts (consumed by hemangioma)
bernard soulier
GP1b def causing congenital qualitative plt defect
Fibrinogen in DIC and tx?
low, give cryo
purpose of WinRHo (Anti-D) in ITP?
for Rh+ kids w/ ITP, trick spleen into causing mild HA and attacking RBC to leave plts alone
Evans vs ITP
Evans has autoimmune attack of MORE than one cell line. ITP ONLY plts
% of ITP w/ chronic disease, and who?
20%, usu females, infants, adol
splenectomy % success in chronic ITP
only 70%… may be liver hemnolysis
platelet trf in ITP?
NO, unless ICH
chemo causing clots?
Aspariginase
PT/aPTT/plts in hemophilia and vwfD
PT normal in both
PTT long in both
plts normal in both
Diagnosis of vWF?
vwF Ag quant: < 40%
ristocetin qualitative test
check FVIII and plt function screen
most common type of VFW?
Tx
Type 1, tx w/ DDAVP, OCP, AMicar
dx in life threatening bleeding from umb stump but normal PT and PTT
Factor 13 def
incidental long aPTT but no bleeding, may clot.
Factor 12 def
tumors w/ dissem LAD?
ALL, NHL, Neuroblastoma
what stage IV disease can have an ok long term control
Stage IV Hodgkins nodular sclerosing type in teen
most important prognostic factors in Neuroblastoma,
also what?
age and stage, also n-myc
labs in tumor lysis syndrome?
Who is most at risk
Hi K, Hi Uric Acid, high phosphate, low Calcium (gets bound)
Most in new ALL or Burkitt
aplastic anemia vs ALL
if pancytopenic from aplastic anemia - no LAD, no HSM
Two tumors to screen for in Beckwith Weidemann
Wilms, Hepatoblastoma
tumor causing proptosis
rhabdomyosarcoma (NOT retinoblastoma)
mediastinal mass in teen, most likely?
Hodgkins
Abdomen vs chest for ALL B or T cell?
abdomen B cell
Thorax/ChesT: T cell
Where doesn’t neuroblastoma met to?
lung
peripheral smear in sickle cell?
Target, Howell-Jolly *splenic destruction, sickled cells, microcytes
what do you look for if you have a right sided Wilms?
US of IVC and heart (can infiltrate up into the heart!)
Cisplatin causes what tox
ototoxicity
bleomycin causes what tox?
pulm fibrosis
vincristine causes what tox
neurotox
cyclophos causes what s/e
hemorrhagic cystitis
aspariginase causes what s/e
clots and pancreatitis
ALL poor prognostic age, and poor prognostic B/T situation?
<2 bad
T cell bad, pre-B-cell good
Reed Sternberg cell in what?
Hodgkins
NHL in kids? Presents where?
masses in neck, head, abdomen
type of cancer presenting with anterior mediastinal mass / pleural effusions
T cell lymphoblastic NHL
seborrheic rash, focal bone lesion in skull, ear d/c, DI
Dx
Test
Langerhans Histiocytosis X
Skin bx, EM
Prognosis in neuroblastoma in infancy , before 12 mos
95% survival, may spontaneously regress
major prognostic features for neuroblastoma (2)
age < 1 awesome
n-myc amplification is VERY bad
2 ways neuroblastoma causes htn and most common
renal artery compression (most common) catecholamine production (VMA/HMA)
Link between neuroblastoma and what cancer?
osteosarcoma later in life
Risk of RB if parent had it in two eyes
50%
Risk of RB if parent or sibling had unilateral RB
5%
Tumor lysis syndrome in what cancers
what labs
what tx
large tumors, leukemia, Burkitts
Labs: hyperphos, hyperkalemia, hyperuricemia
Tx: hydration, alkalinize, allopurinol
4causes of anterior mediastinal mass
Ts:
thymoma, teratoma, thyroid, Terrible T cell lymphoma
never do what in patient with anterior mediastinal mass
anesthesize/intubate: mass is below vocal cords
s/e of methotrexate
oral ulcers
s/e of procarbazine
CNS
s/e of vincristine
periph neuropathy, SIADH
s/e of asparaginase
pancreatitis
TIBC in anemia of chronic disease
low
Free erythrocyte protoporphyrin in lead, iron, thal?
FEP is high in lead poisoning and iron def
FEP is normal thalassemia
Fanconi and Blackfan anemia have what MCV?
Macrocytic: >100
hypersegmented polys are ween in what anemia?
B12 def macrocytic anemia
goat milk diets lead to what kind of anemia and why
macrocytic from folate def
triad of weakness, paresthesias, sore tongue?
Treatment?
Pernicious anemia from lack of intrinsic factor
Treat with IM Cobalamin
best way to check for folate def?
erythrocyte folic acid concentration
what is the diagnosis in a hemolytic normocytic anemia but with normal retic count (1%)
Parvo - aplastic crisis, so no retics
serum haptoglabin high or low in hemolyltic anemia
low
coombs direct looks for what?
antibodies on surface of RBC. Direct antigloboulin test
A vs B type G6PD def?
Key lab features for dx / timing?
A: African AMericans, episodic
B: Mediterranean, chronic
Dx w/ heinz bodies and enzyme level BUT not right after acute hemolysis b/c retics will have large amt of G6PD
what has high MCHC?
HS (small volume cell but same amt of Hb)
etiology of HS?
spectrin deficiency/mutation
HS treatment? (Hereditary Spherocytosis)
folic acid, transfusions,
splenectomy can be curative
consider what in something that looks like Hereditary Spherocytosis but there is a normal osmotic fragility test?
Also there is increased exercise tolerance in face of anemia?
pyruvate kinase deficiency
defect in sickle cell?
Substitution for glutamic acid to valine at AA #6
PCN at what ages for children with sickle cell disease?
at least through age 5
Treatment of acute chest syndrome in sickle cell?
confirm with ABG (not Sat), and then tx w/ transfusion or exchange transfusion if relatively high HCT to start
If sickle cell and stroke are suspected, treatment steps?
Transfusion then MRI
Kids with fanconi anemia / fancony syndrome are assoc w/ what malignancy
AML / myelodysplastic syndrome
problem in diamond blackfan anemia
arrest of maturation of RBC
prob n transient erythroblastopenia of childhood (TEC)
suppression of erythroid production
how to distinguish the common asx RBC anemias, TEC and Diamond blackfan?
AGE: infants. babies 2-3 mos for DBA, and Toddlers about 2 years for TEC
Treatment of Diamond blackfan anemia?
steroids
severe bad skin infections and giant granules in neutrophils, and easy bruising, and oculocutaneous albinism
Chediak-Higashi Syndrome
Treatment for Chediak-Higashi?
BMT
Typical bugs causing infx in Chediak Higashi?
Staph aureus, Strep pyogenes, pneumococcus
Bugs that come and get you in CGD?
recurrent staph, gram negatives, Serratia, Acinetobacter
severe periodontal dz, umbilical cord stuck, and wounds still open?
LAD
Define neutropenia in infants and others?
<1500 in others
mucosal ulcerations are a clue to what blood cell prob?
neutropenia
Abx causing neutropenia
macrolides: mycins
congenital neutropenia
1 is severe
2. neutrophils look weird (2)
- severe: Kostmann
2. weird: Schwachman-Diamond, Chediak Higashi
oral lesions that come about every month in child < 10y
genetics?
cyclic neutropenia- lasts about a week q month (like menstruation)
Autosomal Dom
typical cause of infections in cyclical neutropenia?
clostridia perfringes: oral lesions
list four things in Schwachman Diamond syndrome?
Risk?
When does it present
what does CBC look like
- pancytopenia / neutropenia, anemia but NORMAL platelets
- short stature
- pancreatic exocrine issues: diarrhea/steatorrhea
- skeletal: clinodactyly/syndactyly AND Thumb problems
Risk for leukemic transformation
Presents in infancy
When to transfuse platelets in ITP?
10-20K
Possible tx for ITP?
IVIG
WinRho (antiRh-D if pt is Rh pos)
Splenectomy if bad/chronic in older kids, steroids
Anaphylactoid purpura is what?
HSP
Distinguish platelet size in Wiskott Aldrich vs ITP?
ITP has big platelets
WAS has small ones
low platelets and immunodef in boys?
Wiskott Aldrich - x linked
what is kasabach-merritt syndrome?
hemangioma traps platelets, normal marrow
List issues in Fanconi anemia/syndrome? (5)
Timing
- upper limb/thumb anomaly
- low platelets / cytopenias and anemias
- renal abnormalities
- short stature
- abnormal pigmentation
Timing is in first rew years, not infancy
Problem with platelet aggregation but normal platelet count and PT/PTT
Glanzmann throbasthenia
deficiency in vitamin K factors results in what PT/PTT issue
long PT (Extrinsic pathway: KEPT)
hemophilia has what pt/ptt issue
prolonged PTT
PT/PTT etc issues in von willebrands
long PTT, normal PT, long bleeding time
req for Factor VIII function and affects plts
nonspecific inhibition of various clotting factors and no correction with 1:1 plasma mixing. (long aptt, clotting)
lupus anticoagulant
best lab to order in suspected vwD?
ristocetin cofactor activity if suspected type 1 (most common) (type 3 has no vWF adn Type 2 has dyfunctional so Ag is helpful there)
chromosome for NF1
17
chromosome for NF2
22
elevated Hb A2 in what?
beta thal
if you have Hg A and Hg S, but A>S what do you have?
sickle trait
inhibitors that do not allow for correction of aPTT in mixing study?
heparin and lupus anticoagulant
only factor not made in liver? (Check in DIC vs liver prob)
Factor VIII (and v)
shortest half life in the warfarin / Vitk antagonist? Prob/tx?
Protein C (can cause warfarin necrosis / purpura fulminans) - cover w/ heparin 24-48 hrs
what does factor v leiden mutation cause?
APC resistance
recurrent infx, rashes, diarrhea, TCP and small platelets, high igA and low IgM
wiskott Aldrich
eczema, low platelets, immune dysfunction
Wiskott Aldrich
