Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Pediatrics > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Acute Lymphoblastic Leukemia

Two main complications?

  • -re: infection?
  • -re: side effect of chemo?
A

Acute Lymphoblastic Leukemia

Features
–nonspecific onset: bone and joint pain in lower extremities; pallor, bruising, epistaxis, petechiae, lymphadenopathy

Labs
–anemia, thrombocytopenia

Bone Marrow Aspirate

  • -lymphoblasts
  • lymphoblasts are PAS, TdT positive

Complications

  • infxn: pneumocystis pneumonia
  • chemo sfx: tumor lysis syndrome –> hyperuricemia, hyperkalemia, hypophosphatemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Presentation

  • -painless, firm cervical or supraclavicular nodes, anterior mediastinal mass in 15-19 year old
  • -night sweats, fever, weight loss

Histo
–Reed-Sternberg cell (large cell with multiple, multilobular nuclei)

Dx?

A

Hodgkin Lymphoma

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Childhood Brain Tumors

Most common location?
–re: tentorium

Most common supratentorial tumors? (2)

Most common infratentorial tumor?

A

Childhood Brain Tumors

Supratentorial

  1. Cranipharyngioma
    - -panhypopituatarism, growth failure, visual loss
    - -calcification at sella turcica
  2. Optic nerve glioma
    - -unilateral visual loss, proptosis, eye deviation,
    - -assoc w neurofibromatosis

Infratentorial

  • -more common
  • -Juvenile Pilocytic Astrocytoma
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Wilms Tumor
–often an asymptomatic abdominal mass

Q: WAGR syndrome?

Rhabdomyosarcoma
–associated with neurofibromatosis

Q: Name of type that looks like grapes?

A

Wilms Tumor

WAGR: wilms tumor, aniridia, genitourinary anomalies, mental retardation

Rhabdomyosarcoma
–Botryoid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Presentation

  • -episodic severe HTN; palpitations, diaphoresis, h/a, abdominal pain, vomiting
  • -retinal papilledema, hemorrhages, exudate

Labs
–elevated catecholamines

Diagnosis
–use I-131 metaiodobenzylguanidine scan

Treament
–removal with pre-op alpha/beta blockade

Dx?
Mode of inheritance?
Association?

A

Pheocromocytoma

  • -AD
  • -associated with neurofibromatosis

*NB: also assoc w MEN-2A, MEN-2B, tuberous sclerosis, Sturge-Weber, ataxia-telangiectasia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Physiologic Anemia of Pregnancy

Why does EPO decrease at birth?

In a full-term infant, when does the physiologic nadir usually occur?

A

Physiologic Anemia of Infancy
(Physiologic Nadir)

Physiology
1. Intrauterine hypoxia –> increased EPO –> increased RBC production

  1. Birth –> increased FiO2 –> decreased EPO –> progressive drop in Hb, Hct over first 2-3m until tissue oxygen needs are greater than delivery
    - -normochromic, normocytic

*Occurs earlier in preterm infants

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Beta-Thalassemia

  1. Decreased synthesis of what chain?
  2. Why does it not present until 2-3m after birth?
  3. What is the uniting causal factor of expansion of face and skull; hepatosplenomegaly; “crewcut” on Xray; and “chipmunk facies”?
  4. What infection can cause aplastic crisis?
A

Beta-Thalassemia
1. decreased beta globin synthesis –> alpha tetramers; increased HbF at birth is temporarily protective

  1. after a few months of life –> anemia, hypersplenism, cardiac decompensation
  2. Extramedullary hematopoiesis –> expansion of face and skull; hepatosplenomegaly; “crewcut” on Xray; “chipmunk facies”
  3. Parvo B19 –> aplastic crisis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

DDx: Congenital Anemia

  1. triad: macrocytic anemia, low reticulocytes, congenital anomalies (eg, short stature, craniofacial deformities, triphalangeal thumbs)
  2. pancytopenia; absent or hypoplastic thumbs
A

DDx: Congenital Anemia

1. Blackfan-Diamond Syndrome
(Congenital Pure Red-Cell Anemia)
--increased RBC programmed cell death
--increased RBC adenosine deaminase
--Triad
*macrocytic anemia
*low reticulocytes
*congenital anomalies: short stature, craniofacial deformities, triphalangeal thumbs
  1. Fanconi Anemia
    - -AR; chromosomal breaks
    - -short stature; hyperpigmentation or cafe-au-lait spots; absent or hypoplastic thumbs; low set ears; middle ear abnormalities due to infection/hemorrhage
    - -increased risk of leukemia and cancer
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Sickle Cell Anemia

  1. First presentation? re: hands, feet
  2. Risk for what type of osteomyelitis?
  3. Altered splenic function leads to increased susceptibility to infection with encapsulated bacteria (3)
  4. Aplastic crisis after what infection?
  5. Sickled RBCs, target cells, and what other smear finding?
  6. What immunizations (2) and prophylactic antibiotic (1) are important?
A

Sickle Cell Anemia

  1. Hand-foot syndrome - acute distal dactylitis; symmetric painful swelling of hands and feet due to ischemic necrosis
  2. Salmonella osteomyelitis
  3. encapsulated bacteria: S. pneumococcus, H. influenzae, N. meningitidis
  4. Parvo B19 - aplastic crisis
  5. Sickled cells, target cells, Howell-Jolly bodies
  6. Immunization with pneumococcal, 23-valent meningococcal vaccines; daily penicillin prophylaxis age 2m-5y
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

G6PD Deficiency
–within 24-48 h after ingesting an oxidant (eg, acetasalicylic acid, sulfa drugs, anti-malarials, fava beans) or infection –> rapid drop in Hb, hemoglobinuria, and jaundice

  1. Mode of inheritance?
  2. Smear finding?
A

G6PD Deficiency

  • -within 24-48h after ingesting oxidant or infection –> rapid drop in Hb, hemoglobinuria, jaundice
  • -XLR
  • -Heinz bodies
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Iron Deficiency Anemia

  1. Histology? re: cell size, color
  2. Cow’s milk is a risk factor.
    - -Do not initiate before what age?
  3. To distinguish Fe Deficiency Anemia v thalassemia
    - -re: RDW; serum Fe and ferritin
A

Iron Deficiency Anemia

  1. microcytic, hypochromic
  2. No cow’s milk until age 1 yr
    * cow’s milk –> creates intestinal mucosa abnormalities –> occult blood loss
    * no more than 24oz per day
  3. Fe deficiency anemia
    - -increased RDW
    - -decreased serum Fe and ferritin
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Lead Poisoning
–microcytic, hypochromic anemia

Presentation

  • -behavioral changes
  • -cognitive/development dysfxn
  • -GI: constipation
  • -CNS: h/a, lethargy (due to cerebral edema)
  • -Oral: gingival lead lines

Screening at 12m, 24m
–5ug/dL is acceptable

  1. Chelation Therapy
    - -at 45-70 ug/dL? (1)
    - -at greater than 70: EDTA plus dimercaprol
A

Lead Poisoning

RFs: low SES; older housing

Presentation: hyperactivity; cognitive and developmental delay; constipation; gingival lead lines; cerebral edema and increased ICP

Screening: 12m, 24m
–5ug/dL is acceptable

Chelation Therapy

  • 45-70: DMSA (succimer, oral)
  • greater than 70: EDTA plus dimercaprol
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

DDx: Bone Tumors

  • osteogenic sarcoma
  • Ewing sarcoma
  • osteoid osteoma
  1. Which one on Xray appears lytic with laminar periosteal elevation (onion skin)?
  2. Which one on Xray shows sclerotic destruction (sunburst)?
  3. Which one on Xray shows a small round central lucency with a sclerotic margin?
  4. Which one is relieved with NSAIDs?
  5. Which one has risk factors of retinoblastoma and radiation?
A

DDx: Bone Tumors

Osteogenic Sarcoma

  • -sclerotic destruction (suburst)
  • -RFs: retinoblastoma, radiation
  • -elevated Alk Phos, LDH

Ewing Sarcoma

  • -central lytic lesion
  • -laminar periosteal elevation (onion skin)
  • -“onion skin” appearance is followed by mottled “moth-eaten” appearance with extension into soft tissue

Osteoid Osteoma

  • -small round central lucency with sclerotic margin
  • -relieved with NSAIDs
  • -pain worse at night, unrelated to physical activity

NB: systemic symptoms may be absent in OS, ES

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Sickle Cell Anemia

MoI?

sickling –> inflexible and fragile RBC membrane –> (extra-, intra-vascular) hemolysis

Lab Findings

  1. LDH?
  2. What type of hyperbilirubinemia?
  3. Reticulocytes?
  4. Haptoglobin?

What rx can be used as prophylaxis for vaso-occlusive pain crisis?

A

Sickle Cell Anemia

MoI: AR

sickling –> inflexible, fragile RBC membrane –> hemolysis

Labs

  • -increased LDH
  • -unconjugated hyperbilirubinemia
  • -reticulocytosis
  • -decreased haptoglobin

*Haptoglobin - binds free Hgb from destroyed RBCs; transports Hgb to liver for heme catabolism

Vaso-occlusive pain crisis prophylaxis

  • -Hydroxyurea
  • stimulates production of fetal Hgb
  • sfx: myelosuppression

NB: exchange transfusion can be used in acute crisis (eg, stroke)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Evaluation of Anemia

  1. decreased MCV (4 dx)
    - -Fe deficiency
    - -thalassemia
    - -what other 2?
  2. normal MCV, increased reticulocytes
    - -hemorrhage
    - -hemolysis
  3. normal MCV, decreased reticulocytes
    - -medication sfx
    - -leukemia
    - -what other 2?
  4. increased MCV
    - -B12 or folate deficiency
A

Evaluation of Anemia

  1. decreased MCV
    - -Fe deficiency
    - -thalassemia
    - -lead poisoning
    - -sideroblastic anemia
  2. normal MCV, increased reticulocytes
    - -hemorrhage
    - -hemolysis
  3. normal MCV, decreased reticulocytes
    - -leukemia
    - -medication sfx
    - -aplastic anemia
    - -infection
  4. increased MCV
    - -B12 or folate deficiency
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What protein helps absorb Fe from duodenum into blood?

What protein is responsible for carrying Fe in the blood?
–how is this protein affected by inflammation?

What protein is responsible for storing Fe in cells?
–how is this protein affected by inflammation?

A

Ferroportin

  • -helps absorb Fe from duodenum into blood
  • inhibited by Hepcidin, which is increased during inflammation

Transferrin (TIBC)

  • -transfers Fe in blood
  • -decreased during inflammation

Ferritin

  • -stores Fe in cells
  • -increased during inflammation
17
Q

Immune Thrombocytopenic Purpura

  • sudden onset of petechiae and purpura 1-4w post-viral infection
  • -decreased platelets; other cell lines normal

Pathogeneis: IgG autoAb against what platelet antigen?

Tx:

  1. NO transfusion!
  2. No tx if only cutaneous s/s; usually self-resolving
  3. IVIG or glucocorticoids if bleeding present
A

Immune Thrombocytopenic Purpura

  • -IgG autoAb against GP2b3a platelet Ag
  • -Ab-platelet complex destroyed in spleen
18
Q

Peripheral Blood Smear Findings

  1. RBCs with single round blue inclusions
    - -seen on Wright stain
    - -presence indicates lack of splenic function
    - -SCD, hereditary spherocytosis
  2. Due to hemoglobin precipitation to form insoluble precipitants
    - -visualized with dye such as Crystal Violet
    - -G6PD deficiency
A

Peripheral Blood Smear Findings

  1. Howell-Jolly Bodies
    - -RBCs with single round blue inclusions seen on Wright stain
    - -SCD, hereditary spherocytosis
  2. Heinz bodies
    - -Hgb precipitants visualized with crystal violet dye
    - -G6PD deficiency
19
Q

Peripheral Blood Smear Findings

Associated pathologies?

  1. Basophilic stippling (2)
  2. Bite cell
  3. Target cell (2)
  4. Heinz bodies
    * Hgb precipitants seen with crystal violet stain
  5. Howell-Jolly bodies
    * basophilic nuclear remnants
  6. Helmet cells
A

Peripheral Blood Smear Findings

  1. Basophilic stippling
    - -lead poisoning, thalassemia
  2. Bite cell
    - -G6PD deficiency
  3. Target cell
    - -thalassemia
    - -asplenia
  4. Heinz bodies
    - -G6PD deficiency
  5. Howell-Jolly bodies
    - -asplenia
  6. Helmet cells
    - -DIC, HUS, TTP
20
Q

Neonatal Polycythemia

Two uniting causal pathways?

A

Neonatal Polycythemia

  1. in-utero hypoxia –> increased erythropoiesis
    - -maternal HTN, diabetes, smoking, IUGR
  2. erythrocyte transfusion
    - -TTTS, delayed cord clamping

*viscous blood impairs organ perfusion

S/S

  • -plethoric skin
  • -respiratory distress, cyanosis
  • -hypoglycemia
  • -irritability, jitteriness
21
Q

Physiology of Coagulation

  1. Vitamin K is important for formation of which six proteins/factors?
  2. Which measure of bleeding is associated with the extrinsic pathway? Which factor is associated?
  3. Which measure of bleeding is associated with the intrinsic pathway? Which four factors are associated?
  4. Which factor is responsible for conversion of fibrinogen to fibrin? Which measure of bleeding is associated with this conversion?
A

Physiology of Coagulation

  1. Vit K –> formation of Factors II, VII, IX, X; proteins C and S
  2. Extrinsic pathway - PT
    - -factor VII
    - -prolonged by anticoagulants
  3. Intrinsic pathway - PTT
    - -factors VIII, IX, XI, XII
  4. Factor IIa (thrombin) converts fibrinogen to fibrin
    - -factor II (prothrombin)
    - -thrombin time
22
Q

Evaluation of Bleeding Disorders

Minor bleeds - von Willebrand Disease (vWD)
*petechiae, mucosal bleeding

Deep bleeds - clotting factor deficiency (hemophilia)

  • -Which measure is increased in hemophilia?
  • -Mode of inheritance for hemophilia?
  • -Hemophilia A - deficiency of what factor?
  • -Hemophilia B - deficiency of what factor?

Tx

  • -replace specific factor (prophylaxis)
  • -desmopressin/DDAVP
A

Evaluation of Bleeding Disorders

Hemophilia

  • easy bruising; hemarthroses
  • -increased PTT; all others normal
  • -XLR

Hemophilia A
–factor VIII deficiency

Hemophilia B
–factor IX deficiency

  • Complication: Hemophilic Arthropathy
  • -recurrent hemarthroses –> Fe and hemosiderin deposition in joints –> joint synovitis, fibrosis –> chronic worsening joint pain and swelling
23
Q

von Willebrand Disease

What are the two functions of vWF?

  • -re: platelets
  • -re: factors

Mode of inheritance for vWD?

S/S

  • -mucocutaneous bleeding
  • -what lab finding?

Name of diagnostic test?

Tx?

A

von Willebrand Diseae

vWF: two functions

  • -platelet adhesion
  • -carries factor VIII

vWF deficiency

  • -AD
  • -abnormal platelet adhesion –> mucocutaneous bleeding
  • -decreased factor VIII –> increased PTT

Dx: ristocetin test

Tx: desmopressin
–induces vWF release from W-P bodies

24
Q

DDx: pediatric malignant abdominal mass

  1. asymptomatic abdominal mass; age 2-5
  2. abdominal mass that cx midline; systemic s/s; 1st year of life
A

DDx: pediatric malignant abdominal mass

  1. Wilms Tumor
    - -asymptomatic abdominal mass; age 2-5
  2. Neuroblastoma
    - -abdominal mass that cx midline; 1st year of life; systemic s/s
25
Q

DDx: leukemia and lymphoma

  1. age 2-5y; fever, URI s/s; bone and joint pain; hepatosplenomegaly; pallor; epistaxis; petechiae
    * anemia, thrombocytopenia
  2. 15-19y; fever, weight loss, night sweats, anorexia; painless, firm cervical or supraclavicular LNs; anterior mediastinal mass
    * Reed-Sternberg cell (large cell with multiple nuclei)
  3. fever, cough; progressive respiratory s/s due to anterior mediastinal mass; abdominal pain and mass
A

DDx: leukemia and lymphoma

  1. Acute Lymphoblastic Leukemia
    * Dx: BM shows lymphoblasts
  2. Hodgkin Lymphoma
    * Dx: LN biopsy
  3. Non-Hodgkin Lymphoma
    * Dx: biopsy
26
Q

Hemolytic Uremic Syndrome

Context and Presentation

  • -recent diarrheal illness
  • -bloody diarrhea
  • -HTN
  • -pallor, petechiae
  • -oliguria

Etiologies

  • -E. coli O157:H7
  • -Shigella, Salmonella, Campylobacter

Two key findings on peripheral smear
–re: RBCs, platelets

A

Hemolytic Uremic Syndrome

Pathophysiology

  1. E coli verotoxin damages endothelial cells –> platelet microthrombi
  2. platelet microthrombi –> RBC shearing –> hemolytic anemia –> pallor
  3. platelet microthrombi –> thrombocytopenia –> petechiae
  4. platelet microthrombi –> intrarenal thrombi –> renal failure

Peripheral Smear

  • -schistocytes
  • -megakaryocytes (giant platelets)

Labs

  • -decreased Hgb
  • -elevated LDH
  • -elevated indirect bilirubin
  • -elevated reticulocytes
  • -elevated BUN, creatinine
  • -thrombocytopenia
27
Q

Triad: impaired upward gaze, obstructive hydrocephalus, precocious pubery

Dx?

A

Pinealoma

  1. Parinaud Syndrome
    - -impaired upward gaze
    - -upper eyelid retraction
    - -ptosis
    - -pupillary abnormalities
  2. Obstructive hydrocephalus
    - -h/a, ataxia, vomiting, papilledema
  3. Precocious puberty
    - -due to secretion of beta-hCG
28
Q

Hereditary Spherocytosis

MoI?

Pathophysiology
–defect of tethering proteins –> fragile spherocytes –> can’t pass thru spleen –> hemolytic anemia, splenomegaly

Name of two tethering proteins?

Features
–anemia, hyperbilirubinemia, biliary gallstones, susceptible to Parvo B19 aplastic crisis

Dx Tests

  • -increased osmotic fragility on acidified glycerol lysis test
  • -abnormal eosin-5-maleimide binding test
A

Hereditary Spherocytosis

MoI: AD

Pathophysiology

  • -defect of tethering proteins –> fragile spherocytes –> can’t pass thru spleen –> hemolytic anemia, splenomegaly
  • -tethering proteins: ankyrin, spectrin
  • -loss of membrane blebs –> increased MCHC

Features

  • -anemia
  • -hyperbilirubinemia
  • -biliary gallstones
  • -susceptible to Parvo B19 aplastic crisis

Dx Tests

  • -increased osmotic fragility on acidified glycerol lysis test
  • -abnormal eosin-5-maleimide binding test

Pediatrics (13 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions