Heme Onc Flashcards by niki baumer

Iron def treatment (kids and adols)

iron elemental 3-6 mg/kg/day in kids

adolescents 325mg daily / BID

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physiologic nadir of HCT/Hb in baby

2 mos

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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

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Normal MCV:

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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

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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

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transmission of hereditary spherocytosis?
Exam finding?
Tx needed

autosomal dominant
Splenomegaly, may need splenectomy after 5y
need folate supp

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chronic stable hemolysis w/ exacerbations, runs in family, MCHC is HIGH

Hereditary Spherocytosis

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Periph smear showing RBC agglutination in what type of autoimmune hemolytic anemia?

Cold IgM autoimmune HA

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Direct Coombs shows what?

presence of Ab on RBCs

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Warm vs cold autoimmune anemia?

Warm: IgG: bad, occurs at body temp, splenomegaly
Cold: IgM: Liver, good.(usu from mycoplasma, HIV, or mono)

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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)

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Thalassemia and iron?

expect increased GI absorption of iron, and get iron overload even w/o transfusion in severe thal

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Hb A2 made of what and when do you see it?

alpha 2 and Delta 2: No B2…

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Hb A has what?

normal: two alpha and two beta

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alpha 2 + gamma 2 is what type of hemoglobin?

Fetal.

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cure for thal major

BMT

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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

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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

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Hemoglobin C disease?

Sickle cell

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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

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S beta thal null disease course?

severe, No Hb A, incr A2 and F, Looks very similar to SS

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WHat is dactylitis and what may it be found in?

swelling of hands / feet, may be first manifestation of sickle cell

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weird bug in sickle cell osteomyelitis?

salmonella

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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

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)

1. 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

1. pancytopenia / neutropenia, anemia but NORMAL platelets 2. short stature 3. pancreatic exocrine issues: diarrhea/steatorrhea 4. 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

1. upper limb/thumb anomaly 2. low platelets / cytopenias and anemias 3. renal abnormalities 4. short stature 5. 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

chromosome for NF2

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