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HEME ONC peds > HEME/ONC peds > Flashcards

HEME/ONC peds Flashcards

1
Q

What are part of the MYELOBLASTS?

A

Basophil, Neutrophil, Eosinophil

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2
Q

What is part of the lymphblasts?

A

Lymphocytes

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3
Q

What is part of the monblasts?

A

Monocytes

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4
Q

What is the most common childhood hematologic problem?

A

Anemia

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5
Q

This clinical presentation represents? Pallor, fatigue, dizzy, poor growth, exercise intolerance, tachycardia, murmur, HA, syncope,

Jaundice = hemolysis
Splenomegaly = hemolysis, splenic congestion
A

Anemia

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6
Q

What facts of history are looked at in anemia?

A 
Predisposing diet
Blood loss
Known congenital problems
Family hx
Abnormal newborn metabolic screen
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7
Q

What does this describe?
Tachycardia, FLOW MURMUR, poor exercise tolerance, HA, fatigue, irritable, poor feeding

More severe, hemolysis

A

Acute Anemia

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8
Q

This type is very well tolerated in children

Tachycardia, flow murmur

A

Chronic Anemia

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9
Q

Historical clues for anemia

A

nutrition, growth and development

  • sx of chronic dz = malabsorption, blood loss
  • hx of jaundice
  • ethnic background
  • Fam hx of splenomegaly, splenectomy, anemia, jaundice
  • Patient age
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10
Q

chronic disease or hypothyroid, Fe deficit

A

poor growth

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11
Q

aplastic anemia

A

congenital anomaly

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12
Q

leukemia, HUS

A

petechiae, purpura

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13
Q

hemolysis, HIV, JRA

A

jaundice

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14
Q

leukemia, sickle cell, hemoglobinopathy, spherocytosis

A

splenomegaly

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15
Q

what type is thalassemia and sickle cell?

A

hemoglobinopathy

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16
Q

impaired red cell production, increased red cell destruction, and blood loss causes what?

A

anemia in children and infants

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17
Q

What are the 2 best labs?

A

CBC and Retic count

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18
Q

What does it mean for retic count to be elevated?

A

Bone marrow response to anemia,

1-2% retics in circulation normally

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19
Q

size of rbc?

A

MCV

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20
Q

density of hemoglobin in RBC?

A

MCHC

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21
Q

range of size of mature RBC?

A

RDW

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22
Q

What is the best indicator in an iron study?

A

FERRITIN

Also in this : Serum FE, TIBC

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23
Q

What is low red blood cell production indicate?

A

low retic count

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24
Q

Parvovirus B19 and Diamond-Blackfan are what types of anemia?

A

low retic anemia

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25
Q

What does normal / elevated retic represent?

A

Bilirubin raised or normal

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26
Q

What if Bilirubin is raised?

A

hemolysis (beta thalassemia, sickle cell)

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27
Q

What if Bilirubin is normal?

A

Blood loss or ineffective erythropoiesis

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28
Q

red cell aplasia, respond to steroids, BMT curative, low retic

A

Diamond Blackfan anemia

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29
Q

EPO deficinecy

A

chronic renal failure, lot retic count

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30
Q

membrane defects - hereditary spherocytosis , enzyme 6gpd deficit, hemoglibinopathies, increased retic count

A

intrinsic hemolysis

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31
Q

auto-immune hemolytic anemia, DIC ,IV hemolysis, increased retic count

A

extrinsic hemolysis

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32
Q

Blood loss, increased retic count

A

Loss

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33
Q

Most common anemia in childhood
- term infants are born with enough iron for 4-5 months
- poor diet intake of iron (cows milk)
pallor , fatigued, irritable, poor growth
- micryoctytic, hypochromic, low MCV
- if neutropenic or thromboyctopenic –> check bone marrow
- oral __ is 6mg/kg/day

A

Fe deficit anemia

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34
Q

what is the treatment of iron deficit anemia?

A

oral supplements

  • increased retic 3-4 days
  • increased hemoglobin 4-30 days
  • increased iron stores 1-3 months
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35
Q

b12 deficit in what diet?

A

vegan diet

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36
Q

folate deficit in what diet?

A

goat’s milk, no veggies

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37
Q

chrons dz, and pernicious anemia

smooth tongue, beefy red, irritable, weak, paresthesia, unsteady gait

A

b12 deficit

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38
Q

celiac dz

A

folate deficit

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39
Q

Anemia of inflammation/chronic illness, arrested development, infiltration of bone marrow space

A

Nomoschromic, normocytic Anemia

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40
Q

congenital - fanconi anemia, Diamond- blackfan

acquired- parvovirus 19 infection

A

arrested development of cell

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41
Q

2-10 year olds at diagnosis, congenital anomalies 75% of time (skeletal, cafe au lait, renal microcephaly, thrombocytopenia, neutropenia –> pancytopenia
- rocker bottom feet, dangling thumbs, short

autosomal recessive

tx : transfusions ,bone marrow transplant

A

underproduction, congenital aplastic anemia, fanconi

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42
Q

birth - 1 year, respond to steroid, short, AD

ONLY RBC LINES AFFECTED

A

underproduction congenital hypoplastic anemia, Diamond Blackfan

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43
Q

underproduction - acquired

A

pancytopenia with hypocellular bone marrow

  • sulfonamides, nsaids, anticonvulsants rxns
  • infections (hep, hivm syphilis, mono)
  • 50% idiopathic
  • sx: anemia, thrombocytopenia, fevers. NO HSM, or jaundice
  • death from hemorrhage or infection

Tx: transfusions, immune modulators
TX: transfusions, immune modulators

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44
Q

Increased destruction

A

isoiz - hemolysis
–> rh/abo/ minor blood groups, hemolytic dz of the newborn

  • Congenital hemolysis
  • -> sphercytosis, G6PD deficit, pyruvate kinase deficit, hemoglobinopathy
  • Infections (bacterial, viral, protozoa)
  • Autoimmune hemolytic anemia
  • Thrombotic microangiopathy
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45
Q

sphercytosis, G6PD deficit, pyruvate kinase deficit, hemoglobinopathy, these are what?

A

Congenital Hemolysis

increased destruction

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46
Q

hemolytic disease of the newborn

A

mom rh- / baby rh +

  • mom makes anti-D antibodies that hemolyzes fetal cells
  • coombs test –> direct = antibodies on rbc, indirect = antibodies in sera

Jaundice within the FIRST 24 HOURS of life.

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47
Q

RBC membrane defects

A

hereditary spherocytosis

  • splenic sequestration of abn cells
  • anemia, jaundice, splenomegaly
  • gallstones 60-70% adults
  • increased retic
  • smear shows spherocytes, normoctyic, hyperchromic
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48
Q

most frequent inherited enzyme defect

  • x linked (males) recessive
  • anemia, HYPERBILIRUBINEMIA, jaundice
  • episodic hemolysis with oxidative stress
A

RBC ezyme defect : G6PD deficit

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49
Q

2nd most common RBC enzyme defect?

A

Pyruvate kinase deficit

50
Q

deficit in the production of globin chains (alpha and beta) , beta not present for first 4 months,

  • MICROCYTIC AND HYPOCHROMIC
  • severe = pallor, HSM
A

Hemoglobinopathy

Thalassemia

51
Q

high prevalence in central Africans, mediterranean, indians, middle east, carribean

  • Increased retic count ( normocytic, macroctyic)
  • smears have sickle cell and target cells

sx: 3-4 months of life, pallor fatigue, jaundice. increase risk of gallstones, splenomegaly d/t sequestration

recurrent vasocclusion and tissue ischemia

recurrent abdominal / msk pain

A

Sick Cell Disease

52
Q

Decreased production
Increased consumption
Impaired function

A

Disorders of platelets

53
Q

Hemophilias
Vitamin K deficit
DIC

A

Disorders of coagulation

54
Q

Petechiae, purpura, bruising, hemorrhage, GI bleed, frequent/prolonged epistaxis, heavy menses, hemarthrosis

A

SX of bleeding disorders

55
Q

Bleeding to the skin and mucous membranes is characteristic of what bleeding disorders?
There’s petechiae, and ecchymoses

A

Platelets and blood vessel bleeding disorder

56
Q

Bleeding into soft tissue muscle and joints is of what bleeding disorders

A

Hemophilia or other disorders of coagulation proteins

57
Q

Thrombocytopenia has what 3 things?

A

decreased production
increased consumption
impaired function

58
Q

infiltrative dz : leukemia, fanconi anemia

A

decreased production in thrombocytopenia

59
Q

immune thrombocytopenic purpura, DIC, kasaback - merritt syndrome

A

increased consumption (more common) in thrombocytopenia

60
Q

von willebrand dz

A

impaired fxn in thrombocytopenia

61
Q

low progenitor cells/ thrombopoietin

  • congenital viral syndrome (cmv, rubella, hiv, parvo)
  • marrow dz - congenital leukemia, neuroblastoma, fanconi anemia
A

decreased platelet production

62
Q

platelet destruction/ consumption

A

in peds- most often immune mediated –> ITP

  • can also be consumptive coagulopathy (dic, kasabach-merritt syndrome)
  • clots
  • infections ,shock, asphyxia
63
Q

What is the most common bleeding disorder of childhood?

A

ITP

64
Q

what are inherited bleeding disorders?

A

Hemophilia a,b

vWB

65
Q

what are acquired bleeding disorders?

A

DIC
Liver Dz
Henoch Schonlein Purpura (HSP)

66
Q

age 2-5
-often follows viral infection: MEASLES, rubella, varicella, EBV, influenza, parvo
- LOW PLATELETS, petechiae, ecchymosis, OTHERWISE HEALTHY KID
-

A

Immune Thrombocytopenia Purpura (ITP)

67
Q 
Factor 8
Factor  9 (christmas dz)
- PROLONGED PTT, normal PT
- Present in infancy with excessive bleeding after circumcision, or bruising after IM injection
- Joint bleeding
A

HEMOPHILIA (coagulation disorder)

68
Q

Factor 8 deficit (mild to severe)

  • mild d/t trauma
  • severe d/t spontaneous bruising, soft tissue bleeding
  • PROLONGED PTT, nl PT
  • x-linked males
  • TX: Desmopressin
A

Hemophilia A (Classic)

69
Q

x-linked
factor 9 deficit
prolonged ptt, nl pt

Tx: Factor 9

A

Hemophilia B (christmas dz)

70
Q

most common inherited bleeding disorder among caucasians - autosomal dominant

  • easy bruising and epistaxis from childhood
  • prolonged bleeding time, nl plt count

TX: desmopressin, IV or intranasal

A

Von Willebrand Disease

71
Q

platelet consumption, coagulation goes away

A

DIC

72
Q

major synthesis of prothrombin, fibrinogen, clotting factors, anticoags

A

LIVER DZ

73
Q

autoimmune mediated vasculitis –> severe abdominal pain

A

Henoch Schonlein Purpura

74
Q

nonthrombocytic : henoch-schonlein purpura, sepsis, trauma

A

child with petechiae or purpura

75
Q

thrombocytopenic: immune thrombocytopenia purpura, leukemia, DIC

A

child with petechiae or purpura

76
Q

acquired disorders: vitamin k deficit, liver disease, thrombocytopenia

A

child with abnormal bleeding to soft tissues, mucocutaneous, following surgery

77
Q

inherited disorders: hemophilias, von willebrand dz

A

child with abnormal bleeding into soft tissues, mucocutaneous, following surgery

78
Q

generally uncommon in children

A

clotting disorders

79
Q

most common hereditary cause of a predisposition to thrombus

A

factor V leiden mutation

80
Q

unconjugated (indirect)

  1. increased production
  2. impaired uptake
  3. decreased conjugation
  4. Increased enterohepatic recirculation (infancy)
A

these are all causes of jaundice

81
Q

conjugated (direct)

  1. impaired excretion into bile
  2. biliary obstruction
  3. hepatocyte destruction (hemolytic dz of newborn)
A

all causes of jaundice

82
Q

what catalyzes bilirubin conjugation in liver?

A

Glucuronyl transferase

83
Q

build up of unconugated bilirubin –> increased enterohepatic reuptake to blood

most healthy infants dont require intervention for jaundice

A

Physiologic Jaundice

84
Q

1Physiologic – breast milk jaundice
2Hemolytic – RH, ABO, G6PD, spherocytosis,
3Polycythemia – T21, too viscous, not flowing properly through blood vessels
4Hematoma / hemorrhage – scalp injury, excessive bruising
5Glucuronyl transferase defect – (unable to conjugate) - inherited

A

ddx of unconjugated hyperbilirubinemia

85
Q

decreased transcription of mRNA –> less enzyme

rx: no treatment needed except in neonate with excessive hyperbilirubinemia

A

gilbert’s syndrome (genetic cause - transferase gene)

86
Q

type 1 gene mutation –> no enzyme activity
Rx: liver transplant

type 2: gene mutation –> diminished activity
Rx: phenobarbital, phototherapy

A

Crigler-Najjar Syndrome (genetic cause - transferase gene)

87
Q

less common -bad sign
- always abnormal

signs of liver disease: biliary atresia, cholestasis

A

conjugated hyperbilirubinemia

88
Q

what is the most common cause of death by dz in children?

A

Cancer

89
Q

what is the most common CA in childhood?

A

ALL

90
Q

What is the second most common CA in childhood?

A

Brain tumor

91
Q

What is the most common solid tumor (CA) in childhood?

A

Brain tumor

92
Q

What cancerous tumor in the abdomen crosses the midline?

A

Neuroblastoma

93
Q

These are symptoms of what? Fatigue, bruising, anorexia, petechiae, malaise, bleeding, HA, pain, fever, vomit, abnormal lump/mass, vision changes, weight loss, pallor, night sweat

A

Cancer

94
Q

What is in a CA workup?

A

CBC w/ diff

  • Peripheral blood smear
  • CXR
  • Biopsy
  • CT of solid tumors
  • CSF : Leukemia, NH lymphoma, CNS tumors
95
Q

Uncontrolled proliferation of immature lymphcytes (lymphblasts)
- Peak age 4, 85% 2-10 yr
- Boys > girls
- White > Black
- Lymphoblasts seen on blood smear and BM bx
-

A

Acute Lymphoblastic Leukemia (ALL )

96
Q

What does the CXR for ALL look like?

A

mediastinal widening
anterior mediastinal mass
tracheal compression from lymphadenopathy

97
Q

What xrays are taken for ALL?

A

spine and long bones - demineralization, periosteal elevation

98
Q

induction - immune suppression

  • consolidation : chemotherapy
  • maintenance : methotrexate, vincristine, steroids

duration = 2-3 years with 80% cure rate

A

ALL therapy

99
Q

CBC: anemia, thrombocytopenia, neutropenia

  • malignant immature myeloblasts
  • poor prognosis than ALL
  • aggressive initial therapy with toxic substances
  • 80% achieve remission, high relapse rate
  • cure rate 50%
  • AUER RODS
A

Acute Myeloid Leukemia (AML)

100
Q

rare leukemia

  • peripheral smear has ___ cells in all stages of maturation with few blasts
  • philadelphia chromosome, very poor prognosis
  • translocation of chr 9 and 22
  • bone pain, fever, night sweats, fatigue can be asx
  • HSCT only cure (bone marrow transplant)
  • 50-80% survival rate
A

Chronic Myelogenous Leukemia CML

101
Q

malignant proliferation of lymphoid cells

  • arising from lymph nodes, thymus, spleen
  • 3rd most common malignancies in kids
  • hodgkin dz more common at 50%
  • non hodgkin is collective group
A

Lymphomas

102
Q

which hodgkin presents more in younger children?

A

Non hodgkin disease

103
Q

which hodgkin presents more in adolescence or >50?

A

hodgkin disease

104
Q

what clinical signs relate to both NH and H lymphoma?

A

lymphadenopathy

105
Q

painless cervical or supraclavicular adenopathy, mediastinal mass

  • fatigue, night sweats, cough, sob , anorexia, weight loss, fever
  • CBC ,CXR
  • BIOPSY : REED STERNBERG CELLS
  • TX: chemo
  • 5 year survival rate 90%
A

Hodgkin Essential

106
Q
  • arise in any site of lymphoid tissues (bone, marrow, CNS, skin, tests)
  • more malignant
  • cough, dyspnea, orthopnea, swelling of face, lymphadenopathy, mediastinal mass, pleural effusion
  • abdominal pain, distension, constipation, abd mass, ascites, hepatosplenomegaly
  • fever, neuro deficits, skin lesions
  • CBC, CXR, BIOPSY
  • TX: Chemo
A

Non Hodgkin Essentials

107
Q

morning headache, vomiting, papilledema

A

classic triad of brain tumors

108
Q

4th ventricular mass

A

medulloblastoma

109
Q

neural crest tissue of sympathetic ganglia or adrenal medulla

  • most common solid neoplasm outside CNS
  • 50% dx before 2y, 90% by 5y
  • most common: abdominal pain or mass that EXTENDS BEYOND MIDLINE
  • bone pain suggests mets
  • mets to : bone, marrow, lymph nodes
  • cbc, plain xrays : stipled calcifications of tumor, patho fractures
  • urine catecholamines (90%)
  • Bx is definitive and for staging

TX: chemo to shrink tumor and surgical resection

A

Neuroblastoma

110
Q

Where are mets of neuroblastoma?

A

Bone
Bone marrow
Lymph nodes

111
Q
  • nephroblastoma
  • from immature cells of kidney
  • encapsulated tumor from kidney
  • affect 2-5 year old, rarely older 6
  • Does NOT cross midline, abdomen increases, PAINLESS abd mass
  • CXR OR CT = PULMONARY METS

TX: surgical resection then chemo

A

Wilms Tumor

112
Q
  • from embryonic retinal cells

- 90% diagnosed

A

Retinoblastoma

113
Q
  • rhabdomyosarcoma
  • most common soft tissue ___ in children
  • peaks = 2-5 and adolescence
  • Younger pt : head, neck, GU
  • Older pt : extremities, trunk, male GU
  • Male > female
A

Sarcomas

114
Q

head, neck, GU are locations for sarcoma of what age?

A

younger patients

115
Q

extremities, trunk, male GU are locations for sarcoma of what age?

A

older patients

116
Q

symptoms result from disturbances in normal growth fxn due to tumor growth

  • orbital : proptosis, decreased extraocular motion
  • nasal mass: chronic otitis, ear discharge
  • bladder : hematuria, urinary obstriction, pelvic mass
  • xrays, ct, mri

TX: surgery if possible, chemo, radiation

A

Rhabodomyosarcoma

117
Q

most frequent in adolescents - peaks with growth spurts

  • pain, lump, swelling
  • cure rate 75%+ w/ no mets
  • tissue biopsy definitive
A

Sarcomas - Bone

118
Q
  • 60% cases
  • adolescents/ young adults
  • diagnosed during growth spurt
  • epiphysis, metaphysis most common
  • pain, palpable mass
  • xray : lytic lesion with calcifications around soft tissue
  • surgery chemo
A

Osteosarcoma

119
Q
  • toddlers to young adults
  • femur and pelvis most common
  • pain, swelling
  • systemic sx: fever, weight loss
  • chemo/surg, chemo/radiation
A

Ewing Sarcoma

120
Q

Short term side effects of chemotherapy

A

Bone marrow suppression = anemia, thrombocytopenia, bleeding, neutropenia

Immunosuppression = infection

Undernutrition from gut mucosal damage, nv, anorexia, alopecia

121
Q

Late effects of cancer therapy

A

60% will have some disability
Complications
- growth : pituitary gland
- endocrine/ growth = thyroid, pituitary
- cardiopulm = pulm fibrosis, cardiomyopathy
- renal = hemorrhagic cystitis, bladder fx
- neurospsych = non verbal tasks, math, short term memory
- second malignancies

HEME ONC peds (1 decks)

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