Peds Heme/onc

Front 1

Microcytic, hypochromic anemia

Back 1

Fe def,

thalassemia, (incr RBC count)

lead toxicity (stippled RBCs)

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Normocytic, normochromic anemia

Back 2

Acute blood loss

Hemolytic (schistocytes)

Anemia of chronic dz (can also be microcytic/hypochromic)

Hemoglobinopathy (target cells)

Renal dz (acanthocyte - spiked cell membrane)

Front 3

Macrocytic (MCV > 100) normochromic

Back 3

AI hemolytic (spherocytes)

EtOH

Liver dz (round, target, echinocyte (burr cell), acanthocyte (spiked cell membrane))

Aplastic anemia

Hypothyroidism

Drugs effect (hydroxyurea)

Myelodysplasia

Front 4

Megaloblastic (>110)

Back 4

Folate def (incr homocysteine)

B12 def (incr MMA & homocysteine, decr cobalamin, Schilling test for pernicious anemia)

Front 5

Anemic kid w/ 0% retic count

Back 5

Transient anemia of childhood - should spontaneously return in 30-60 days; give transfusions in meantime

Front 6

Dz's leading to iron def anemia?

Anemia of chronic dz?

Back 6

Iron def: CF, JRA

ACD: JRA, SLE, UC, cancer, renal dz

Front 7

pathophys of anemia from lead poisoning

Back 7

Lead inhibits enzymes involved in heme production & inhibits iron utilization

Front 8

Basophillic stippling = ?

Back 8

Lead poisoning

Front 9

Lead poisoning Tx?

Back 9

> 70 ug/dL: Dimercaptrol (BAL) + EDTA

45-70: EDTA or Succimer (DMSA)

Front 10

Drugs causing folate def

Back 10

Phenytoin, MTX

Vit C def

Front 11

Megaloblastic anemia + neuro Sx

Back 11

B12 def - demyelination of DCML + lateral columns

Front 12

Pernicious anemia = ____ def

Back 12

B12 (req IF for abs in ileum)

Front 13

Anemia + neutropenia + osteoporosis + ataxia

Back 13

Copper deficiency

Front 14

What does haptoglobin do? When is it decr?

Back 14

haptoglobin binds free hemoglobin (Hb) - the complex is removed in the spleen.

Decr in intravascular hemolysis - free Hg binds haptoglobin, lowering haptoglobin levels. Relatively nl in extravascular hemolysis, since MOs phagocytose the Hg & bring it to the spleen.

Front 15

Infections seen in SCD

Back 15

S. pneumo - penicillin to PPx

H. flu

Salmoneal (osteomyelitis)

Front 16

Kid req freq transfusion w/ skull bossing + maxillary overgrowth

Back 16

B-thal major - extra medullary hemopoiesis

Front 17

Heinz bodies + bite cells

Back 17

G6PD def

Front 18

Drugs causing G6PD hemolysis. Diet?

Back 18

ASA

Sulfonamides

Cipro

Anti-malarials

Also fava beans

Front 19

Increased LDH = ?

Back 19

Hemolysis, heart failure, hypothyroidism, and lung, liver, bone disease; elevated w/ some cancers

Front 20

Where does destruction in hereditary spherocytosis occur? coombs test result?

Back 20

Spleen - consider splenectomy in kids > 5 yo

Coombs test negative (structural not immune mediated destruction)

Front 21

Morning hemoglobinuria = ?

Tx?

Back 21

Paroxysmal nocturnal hemoglobinuria - defect of RBC membrane

Tx: prednisone, BMtx if severe

Front 22

Schistocytes, spherocytes, helmet cells

Thrombocytopenia, nl PT & aPTT

Abnl renal fxn

Neuro Sx

What is the Dx? Tx?

Back 22

TTP - VWF multimers deposited in microvasculature lead to microangiopathic hemolytic anemia.

Sx include FAT RN (Fever, anemia, thrombocytopenia, renal dysfxn, neuro abnl)

Tx: plasmapheresis, CS, splenctomy

Front 23

Diarrhea or URI followed 10 days later w/ hemolytic anemia, thrombocytopenia, ARF.

Cause?

Tx?

Back 23

HUS

E. coli 0157H7 produces shiga-like toxin

Tx: dialysis, plasmapheresis if neuro comp. Don't give Abx (toxin mediated)

Front 24

URI followed by low platelets, otherwise nl CBC. Cause? Tx?

Back 24

ITP - 50% preceded by viral illness (mono, mumps, rubella, varicella), rest unknown etiology

Tx: 80% recover spontaneously, give IVIG or CS if severe, splenectomy if chronic / severe

Platelet transfusions not helpful

Front 25

Incr PT, aPTT

Decr fibrinogen & platelets

Incr fibrin degradation products & D-Dimer

Causes?

Tx

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DIC

-Meningoccemia, septic shock

-Incompatible transfusion

-RMSF

-Snake bite

-Acute promyelocytic leukemia

Tx: Platelets, FFP,

heparin to prevent consumption of coag factors

Front 26

Rx for recurrent SCA pain crises

Back 26

Hydroxyurea: increases fetal Hg

Dose-limiting SE: myelosuppression

Front 27

Epistaxis, , easy bruising, prolonged bleeding, increased aPTT, nl platelet count

Tx?

Back 27

vWD - def of vWF +/- VIII

Tx with DDAVP, factor VIII concentrate

Front 28

Hemophilia A = ?

B=?

What tests are abnl?

Sx?

Back 28

A = VIII def

B = IX def

aPTT 2-3x higher than nl

IM hematomas, hemarthroses, easy bruising

Front 29

What leads to increased PT?

Back 29

PT measures extrinsic system

Factor deficiencies

DIC, Liver failure

Vit K def, fat malabs

Circulating anticoagulants

Warfarin use

Front 30

Causes of increased aPTT?

Back 30

PTT measures intrinsic system

vWD, hemophilia

Factor def , circ anticoagulants

Heparin use, high dose warfarin

Front 31

What platelet levels lead to spontaneous bleeds?

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<20k

<10k: indication for platelet transfusion

Front 32

Deficiencies of what proteins leads to hypercoag state?

Back 32

Def of protein S, protein C, antithrombin III or Factor V Leiden

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What Ht level should you give a transfusion?

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<8; 8-10 if Sx'tic

Front 34

Inherited & acquired causes of methemoglboinemia? Tx?

Back 34

Inherited: Cytochrome b5 reductase def

Acquired: Nitrites (contaminated water), xylocaine/benzocaine (teething gel), sulfonamides, benzene, aniline dyes, potassium chlorate

Tx: IV methylene blue if methemoglobin level > 30%; if unresponsive hyperbaric O2 or oral ascorbic acid

Front 35

Bilateral pulmonary edema & ARDS-like picture after transfusion - what causes this type of reaction?

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Transfusion-related acute lung injury (TRALI) - antigranulocyte Ab from donor product

(that donor shouldn't donate plasma products)

Front 36

Nonhemolytic, febrile transfusion reaction - what causes this type of reaction?

Back 36

Antibodies to granulocytes or cytokines in plasma

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Delayed hemolytic transfusion reaction - what causes this type of reaction?

Back 37

Ab to minor blood group proteins from prior transfusion (Kidd, Duffy, Rh, Kell)

Front 38

Allergic transfusion reaction - what causes this type of reaction?

Back 38

Ab to plasma proteins

Front 39

Photsensitivity, numbness/tingling, loss of DTR, red urine, severe cramps and pain

Tx?

Back 39

Prophyria

Acute: hydration, maintain electrolytes (tend to see low Na), IV hematin, pain control

Long-term: avoid EtOH, some Rx; use sunscreen; high CHO diet

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Syndromes with increased leukemia risk (5 major)

Back 40

Down's

Fanconi's

Wiskott-Aldrich

AT

Agammaglobulinemia, immune def

Bloom's

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Reed-Sternberg cells =

Back 41

Hodgkin's lymphoma