Heme

Question 1

Microcytic anemias

Answer 1

(<80) → iron deficiency,thalassemia, lead tox, neoplastic

Question 2

Normocytic anemias

Answer 2

(80-100) → acute blood loss, anemia of chronic dz, hemolyic, renal dz

Question 3

Macrocytic anemias

Answer 3

(>100) → hemolytic, alcohol, liver dz, aplastic anemia, hypothyroidism, drug effect, myseldysplasia

Question 4

Megaloblastic anemias

Answer 4

(>110) → folate deficiency, B12 deficiency

Question 5

When should you screen for lead poisoning

Answer 5

Screen at 10-14 mo and 2 yo

Question 6

Tx for lead poisoning

Answer 6

Enviro control, education

10-19 → deu
20-45→ enviro eval +
45-69 → med and enviro intervention including chelation (EDTA or DMSA)
>70 → medical emergency Dimercaprol followed b EDTA x 5 days

Question 7

Presentation of lead poisoning

Answer 7

Acute (>60) → HA, abd pain, loss of appetite, constipation, acute encephalopathy

Chronic → cognitive impairment, ADHD

Question 8

Hemophilia presentation

Answer 8

Easy bruising, intramuscular hematomas, hemarthroses → joint destruction, spontaneous hemorrhaging

Question 9

Hemophilia A

Answer 9

factor VIII deficiency

MC than B and is more likely to be severe

Question 10

Hemophilia B

Answer 10

factor IX deficiency

Question 11

Hemophilia tx

Answer 11

Early diagnosis, prevent trauma

Prompt mgmt of bleeding, recombinant factors, cryoprecipitate

AVOID anticoag, aspirin and NSAIDs

Question 12

ALL presentation

Answer 12

Fatigue, anorexia, lethargy, pallor, bone pain, fever, bleeding, bruising, petechiae, lymphadenopathy, hepatosplenomegaly, bone tenderness,testicular swelling, septicemia

Question 13

What leukemia is mc

Answer 13

ALL 5x mc than AML

Question 14

Leukemia tx

Answer 14

Remission induction: 1 wk anthracycline and cytosine arabinoside

Postremission therapy: several more courses of high dose cytrabine chemo, allogenic stem cell transplant or autologous stem cell transplant

Infection prevention→ isolation and abx

Platelet transfusion for bleeding

RBC trans for anemia

Question 15

Juvenile Chronic Myelogenous Leukemia (JCML) presentation

Answer 15

<2 yo

Skin lesions (eczema, xanthoma, café au lait spots), lymphadenopathy, splenomegaly

Question 16

Juvenile Chronic Myelogenous Leukemia (JCML) tx

Answer 16

Complete remission w/ stem cell transplant

Majority relapse with overallsurvival of 25%

Question 17

Lymphoma presentation

Answer 17

MC childhood cancer in 15-19 yo

Fever, night sweats, weight loss, loss of appetite, cough, dysphagia, dyspnea, lymphadenopathy (lower cervical and supraclavicular), hepatosplenomegaly, mediastinal mass

Question 18

reed-sternberg cells (owl eyes)

Answer 18

Hodgkin lymphoma

Question 19

Lymphoma tx

Answer 19

Radiation for stage I or II dz

Chemo for stage III or IV

Question 20

Neutropenia w/u

Answer 20

ANC < 1500

Mild: 1000-1500

Moderate: 500-1000

Severe: <500

Agranulocytosis: <200

Question 21

Neutropenia presentation

Answer 21

suspectibility ot bacterial infection

Stomatitis, gingivitis, recurrent OM, cellulitis, pneumonia and septicemia

Skin and oral cavity mc affected

Question 22

Juvenile pernicious anemia, red beefy tongue

Answer 22

vit B12 def anemia

Question 23

tx for vit B12 def anemia

Answer 23

Vit B12 IM monthly

Oral therapy is CI

Question 24

tx for iron def anemia

Answer 24

Ferrous sulfate 3-6 mg/kg/day x 8 wks after a nrml Jgb level is obtained

Question 25

tx for folate def anemia

Answer 25

Parenteral folic acid (CI if co-existing vit B12 deficiency anemia) Eat more green veggies, fruits

Question 26

Appears after 6 mo (when HbF↓) Anemia, vaso-occlusive (leg ulcers, stroke,priapism, pain crises), hand foot syndrome (swollen), splenomegaly, infection, cardiac enlargement, short stature, delayed puberty, gallstones/jaundice

Answer 26

Sickle Cell Disease

Question 27

Sickle Cell Disease tx

Answer 27

Infection prevention → pneumococcal vaccine, meningococcus, pphx Pcn by 4 m until 5 yo Hydration and analgesics for painful crisis Priapism → exchange transmusion, hydroxyurea

Question 28

Hemolytic Disease of the Newborn presentation

Answer 28

Hemolytic anemia Fetal hydrops→ large placenta, ↑ unconjugated hyperbilirubinemia, abd distension,scalp or skin edema, purpura, cyanosis

Question 29

Hemolytic Disease of the Newborn etiology

Answer 29

Maternal blood comes in contact w/ fetal blood cells → maternal Ab produced against Rh Ag → subsequent preg w/ Rh + → hemolysis

Question 30

Thalassemia tx

Answer 30

Monthly transfusion of packed RBC to maintain Hgb >10 Splenectomy if requiring >240 mL/k of pRBC/yr

Question 31

Aplastic Anemia

Answer 31

pancytopenia (RBC WBC and platelets) | Fatigue, infections, bleeding

Question 32

Aplastic Anemia tx

Answer 32

Platelet and RBC transfusion Immunosupp drugs → antilymphocyte globulin, antithymocyte globulin, cyclosporine Growth factors→ EPO, G-CDF, GM-CSF Stem cell trasnplant is definitive cure but required chemo and/or radiation

Question 33

Thrombotic Thrombocytopenic Purpura (TTP) presentation

Answer 33

Fever, microangiopathic hemolytic anemia, thrombocytopenia, abnrml renal fn, neurologic signs

Question 34

TTP tx

Answer 34

Plasmapheresis, corticosteroids, splenectomy

Question 35

HUS presentation

Answer 35

Hemolytic anemia, thrombocytopenia, acute renal failure Hx of bloody diarrhea

Question 36

HUS tx

Answer 36

Fluid mgmt, dialysis, plasmapheresis

Question 37

ITP presentation

Answer 37

Abrupt onset of petechial, purpura and epistaxis Usually 1-4 wks after viral infection

Question 38

ITP tx

Answer 38

If platelet <20 admit IVIG or anti-Rho Ab Splenectomy Platelet transfusion

Question 39

DIC presentation

Answer 39

Bleeding, petechia, ecchymoses, hemolysis

Question 40

DIC tx

Answer 40

Treat underlying cause replace plateleys, cryoprecipitate, FFP Heparin prevents consumption of coag factors

Question 41

Von Wilebrand Disease presentation

Answer 41

Easy bruising, heavy or prolonged menstruation, freq or prolonged epistaxis, prolonged bleeding after inj

Question 42

Von Wilebrand Disease w/u

Answer 42

aPTT nrml or prolonged Prolonged bleeding time Nrml PT Abnrml factor VIII , platelet count and aggregation

Question 43

Von Wilebrand Disease tx

Answer 43

Usually no therapy necessary Avoid unnecessary trauma DDAVP and favtor VIII for surgery if needed Cryoprecipitate recommended only in life threatening emergencies d/t risk of HIV and hepatitis infection

Question 44

What is MC posterior fossa tumor of childhood

Answer 44

Cerebellar Astrocytoma

Question 45

Prognosis of Cerebellar Astrocytoma

Answer 45

Good prognosis 5 yr survival >90%

Question 46

Medulloblastoma presentation

Answer 46

Intracranial HRN, ataxiam sx evolving in few weeks Papilledema is absent

Question 47

Craniophryngioma presentation

Answer 47

Short stature or other endocrine associated problems are common initial signs May compress optic nerve or obstruct CSF flow

Question 48

Neuroblastoma presentation

Answer 48

Adrenals, retroperitoneal and abd are MC

Question 49

Neuroblastoma tx

Answer 49

Surgical resection followed by radio + chemotherapy

Question 50

Meduloblastoma tx

Answer 50

Surgical resection followed by irradiation 5 yr survival is >80%