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General Peds Boards > Heme-Onc > Flashcards

Heme-Onc Flashcards

1
Q

What is leukodepletion used for for transfusions?

A
  • Removes any additional neutrophils that happened to get into pRBC product
  • Reduces risk of febrile transfusion reaction
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2
Q

What is irradiation used for in transfusions?

A
  • Small lymphocytes are not removed with leukodepletion but irradiation damages the DNA of donor lymphocytes which makes unable to undergo replication
  • Important for immunocompromised recipients
  • Eliminates risk of GVHD
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3
Q

Hereditary spherocytosis genetics

A
  • Autosomal dominant is most common (defect in gene encoding cell membrane protein ankyrin)
  • Common in European ancestry
  • Causes fragile red blood cell membranes and shortened life span
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4
Q

Hereditary spherocytosis complications

A
  • Parvovirus B19 infection: aplastic crisis leading to bone marrow suppression (low retics on CBC)
  • Gallstones
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5
Q

Barth syndrome

A
  • Congenital neutropenia
  • Cardiomyopathy
  • Proximal skeletal myopathy
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6
Q

Cartilage-hair hypoplasia

A
  • Congenital neutropenia
  • Impaired immunity
  • Short limbed dwarfism
  • Fine hair
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7
Q

Chediak-Higashi syndrome

A
  • Congenital neutropenia

- Albinism

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8
Q

Dyskeratosis congenita

A
  • Congenital neutropenia
  • Abnormal skin pigmentation
  • Nail dystrophy
  • Leukoplakia of oral mucosa
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9
Q

Schwachman Diamond syndrome

A

AUTOSOMAL RECESSIVE

  • Congenital neutropenia
  • Skeletal abnormalities (rib cage)
  • FTT due to exocrine pancreatic dysfunction
  • Recurrent bacterial infections
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10
Q

Langerhans cell histocytosis signs/symptoms

A
  • Osteolytic painful bony lesions
  • Pituitary lesions leading to DI
  • Skin lesions in diaper and scalp that don’t respond to normal therapies
  • Kid with rash and draining ears
  • Coin shaped lesions in scalp
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11
Q

Langerhans cell histiocytosis workup

A
  • Skeletal survey
  • MRI brain to evaluate pituitary
  • Bone marrow if any cytopenias
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12
Q

Von Willebrand disease labs

A
  • Abnormal PTT that corrects in a mixing study suggest abnormal intrinsic cascade (von Willebrand factor)
  • Need to check von willebrand factor and platelet function tests
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13
Q

Neonatal alloimmune thrombocytopenia cause

A
  • Maternal alloantibody to an antigen on the father’s platelets and the newborn’s platelets
  • Most commonly on chromosome 17
  • Transient, requires no treatment
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14
Q

Osteosarcoma symptoms/epidemiology

A
  • Most common in teenagers going through growth spurt
  • Present with weeks of unilateral limb pain, waking up at night with pain, limping
  • Mets to lungs (10-20% have mets at diagnosis)
  • Distal femur and proximal tibia (THE KNEE!) are most common, next is the shoulder
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15
Q

Osteosarcoma lab/imaging and treatment

A
  • Elevated LDH, ESR, and alkaline phosphatase
  • Xray shows sunbursting (calcified blood vessels)
  • Tx is surgery, chemo, radiation
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16
Q

Ewings sarcoma xray findings

A
  • Onionskinning (layers of periosteal reaction)

- Often has soft tissue component

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17
Q

Osteoid osteoma symptoms and xray findings

A
  • Tibia/femur pain, worse at night, relieved by ibuprofen

- Central radiolucent area surrounded by thick sclerotic bone

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18
Q

Conditions that increase risk for leukemia

A
  • Down syndrome
  • SCID
  • Ataxia telangiectasia
  • Fanconi anemia
  • Bloom syndrome
  • Klinefelter syndrome
  • Noonan syndrome
  • Neurofibromatosis
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19
Q

Symptoms of ALL

A
  • Pallor, fatigue, bruising, lymphadenopathy, BONE PAIN, fevers
  • PANCYTOPENIA
  • Most common in ages 2-5
  • More common in Caucasians and males
  • MCC death is infection/sepsis
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20
Q

Most common sites for relapse in ALL

A
  • CNS and testes

- Early relapse has a worse prognosis

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21
Q

Best test to get in a kid with unexplained lymphadenopathy

A

Chest xray to look for mediastinal mass –> lymphoma

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22
Q

Symptoms of Hodgkin’s lymphoma

A
  • Teenager with non-tender enlarged cervical/SUPRACLAVICULAR lymph nodes
  • Weight loss, fevers, night sweats
  • HAVE to get a chest xray
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23
Q

Reed sternberg cells on lymph node biopsy

A

Hodgkin’s lymphoma

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24
Q

Symptoms of non-hodgkin lymphoma

A
  • Younger child (not teen), rapid presentation and often have airway compression
  • Can also be foudn in the abdomen (non-tender mass)
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25
Q

Most common solid tumor of infancy

A

Neuroblastoma

  • Most common in kids less than 5
  • Highest rate of spontaneous regression of any malignancy
  • 95% survival if it occurs before 12 months of age –> most important prognostic factor
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26
Q

Presentation of neuroblastoma

A
  • Persistent bone/joint pain
  • Non-tender abdominal mass (2/3 of the time in the adrenal glands)
  • Weight loss, night sweats, fevers
  • UTI from obstructing abdominal mass
  • Raccoon eyes and proptosis (due to mets)
  • Horner syndrome (mediastinal tumor compressing the recurrent laryngeal nerve)
  • Irritability, hypertension, diarrhea (catecholamine production)
  • Opsoclonus-myoclonus paraneoplastic syndrome
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27
Q

Diagnostic tests for neuroblastoma

A
  • Biopsy of tumor
  • Elevated urine VMA and HMA along with neuroblasts in bone marrow
  • MIBG scan
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28
Q

Cause of raccoon eyes

A
  • Basal skull fractures (child abuse)

- But can also be associated with neuroblastoma

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29
Q

Symptoms of retinoblastoma

A
  • Absence of red reflex or strabismus

- Present under age 5

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30
Q

Genetics of retinoblastoma

A
  • If unilateral and unifocal –> sporadic
  • If bilateral –> Autosomal dominant with incomplete penetrence
  • RB1 gene on chromosome 13
  • If parent has it in 2 eyes there’s 50% chance of child getting it, if in only 1 eye then only 5% chance
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31
Q

Diagnosis/treatment of retinoblastoma

A
  • Diagnose with ultrasound or MRI (try to avoid CT)

- Tx with surgical excision, chemo, and radiation

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32
Q

Trilateral retinoblastoma

A
  • Pineal gland tumors
  • Bilateral retinoblastomas
  • At risk for osteosarcoma, other sarcomas, malignant melanomas
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33
Q

Rhabdomyosarcoma symptoms

A
  • MC soft tissue sarcoma
  • Constipation, rectal exam with a visible/palpated mass
  • Grape like mass protruding from the vagina
  • Head/neck rhabdo in young kids, truncal/extremity rhabdo in teens
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34
Q

Risk factors for tumor lysis syndrome

A
  • Initiation of chemo for large tumors, lymphomas, leukemias

- Can be triggered by systemic steroids

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35
Q

Labs in tumor lysis syndrome

A

Elevated:

  • Phosphate
  • Uric acid
  • Potassium
  • LDH
  • Creatinine (leading to low UOP)

Tx with hydration, alkalinization, allopurinol

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36
Q

Epidemiology of childhood cancers

A
  1. Leukemia (ALL more common than AML)
  2. CNS tumors
  3. Neuroblastoma
  4. Lymphoma
  5. Wilm’s Tumor
  6. Hodgkins
  7. Rhabdomyosarcoma
  8. Retinoblastoma
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37
Q

Symptoms of spinal cord compression

A
  • Bowel/bladder dysfunction
  • Neurologic symptoms
  • Tx with steroids and radiation
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38
Q

Complications of anterior mediastinal mass

A
  • Respiratory distress when supine

- DO NOT INTUBATE! –> airway compression is below the cords so can’t oxygenate/ventilate if you give them anesthesia

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39
Q

Causes of anterior mediastinal mass

A

Thymoma
Teratoma
Thyroid carcinoma
Terrible lymphoma

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40
Q

Red face (plethora), facial swelling, cyanosis, distended neck veins in cancer patient

A

Superior vena cava syndrome due to compression of SVC by an anterior mediastinal mass (most commonly lymphoma)

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41
Q

B-cell ALL prognostic factors

A
  • High risk:
    < 1 year of age or > 10 years of age
    WBC > 50K at presentation
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42
Q

B-cell ALL prognostic factors

A
  • High risk:
    < 1 year of age or > 10 years of age
    WBC > 50K at presentation
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43
Q

Treatment of leukemia

A
  • 95% achieve remission in induction phase
  • Consolidation
  • Interim maintenance
  • Delayed intensification
  • Maintenance
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44
Q

Auer rod in cells

A
  • AML
  • More common in congenital leukemias
  • Only 50% survival
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45
Q

Aklylating agents (cytoxan) chemotoxicity

A

Gonadal dysfunction, infertility issues

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46
Q

Cyclophosphamide chemotoxicity

A

Hemorrhagic cystitis

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47
Q

Cisplatin chemotoxicity

A

Hearing loss and peripheral neuropathy

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48
Q

Bleomycin chemotoxicicty

A

Pulmonary fibrosis

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49
Q

Anthracyclines (doxorubicin, daunomycin) chemototxicity

A

Cardiac toxicity

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50
Q

Vincristine/vinblastine chemotoxicity

A

Neurotoxicity and SIADH

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51
Q

Methotrexate chemotoxicity

A

Oral/GI ulcers, bone loss

52
Q

Chemo for hodgkins lymphoma

A

Cytoxan, doxorubicin, bleomycin, vincristine, etoposide, prednisone

53
Q

Relationship between staging and prognosis of neuroblastoma

A
  • Infant with stage III, favorable histology, unamplified N-MYC (3 year survival > 80%)
  • Infant with stage III, unfavorable histology, amplified N-MYC (3 year survival < 30%)
54
Q

Time for physiologic anemia of the newborn

A

Around 2-3 months of life (8-10 weeks) - Hgb 9 can be normal at this age

  • Cause is low erythropoietin level
  • Preemies can have this drop closer to 1-2 months
55
Q

Three main causes of microcytic anemia

A
  • Thalassemias
  • Too little iron
  • Too much lead
56
Q

Normal hemoglobin chains

A
  • Hgb F: 2 alpha and 2 gamma
  • Hgb A: 2 alpha and 2 beta
  • Hgb A2: 2 alpha and 2 delta

Alpha Always. Gamma Goes. Becomes Beta.

57
Q

Causes of hemolytic anemia with G6PD deficiency

A

Fava beans, aspirin, nitrofurantoin, chloramphenicol, antimalarial drugs, vitamin K analogs

58
Q

Bone with bone on xray disease correlation

A

Sickle cell disease

59
Q

Two defective alpha alleles causes what

A
  • Alpha thalassemia minor/trait
  • Symptomatic or have a mild hypochromic anemia
  • Dx via hemoglobin electrophoresis with a thalassemia panel (EP alone will be normal)
  • Newborn screen may show hemoglobin Bart
60
Q

Three defective alpha alleles causes what

A
  • Alpha thalassemia intermedia –> hemolysis and hepatomegaly
  • Hemoglobin Bart on newborn screen
  • Tx with transfusions or splenectomy
61
Q

Four defective alpha alleles causes what

A
  • Hydrops fetalis

- Intrauterine infusions can help survival but patients will need life long transfusions until bone marrow transplant

62
Q

Defect in one of the beta globin genes

A
  • Beta thalassemia minor/trait
  • Asymptomatic
  • Hemoglobin electrophoresis has elevated A2
63
Q

Defect in both beta globin gene alleles

A
  • Beta thalassemia major
  • Small for age Greek child with progressive microcytic hypochromic anemia, fatigue, enlarged liver/spleen
  • Skull xray with hair on end (due to extramedullary hematopoiesis
  • Newborn screen will have F only pattern
  • Tx with chronic transfusions or transplantation
  • Long term complications are cholelithiasis and hemochromatosis
64
Q

Sickle cell anemia on hemoglobin electrophoresis

A

Hemoglobin F and hemoglobin S

65
Q

Beta thalassemia on hemoglobin electrophoresis

A

Low/no hemoglobin A1, elevated hemoglobin A2, hemoglobin F

66
Q

Causes of increased RDW in microcytic anemia

A

Iron deficiency and lead toxicity

67
Q

Causes of normal RDW in microcytic anemia

A

Thalassemia

68
Q

Complication of low iron

A

Even without anemia it can cause mild delays in cognitive development

69
Q

Treatment for iron deficiency anemia

A

Ferrous sulfate until 2 months after hemoglobin normalizes (to replenish iron stores)

70
Q

Gold standard test for lead poisoning

A

WHOLE BLOOD lead level (not fingerstick)

- Would see ringed sideroblast (immature red blood cell with iron bloated mitochondria surrounding the nucleus)

71
Q

Causes of vitamin B12 deficiency

A
  • Crohn’s disease, following bowel resection
  • Vegetarian diets
  • Intrinsic factor deficiency (pernicious anemia)
  • Bacterial overgrowth
72
Q

Causes of folate deficiency

A
  • Goat’s milk
  • Make sure to treat with B12 and folate when correcting anemia (can lead to irreversible neurologic damage if you forget the B12)
73
Q

Signs of hemolysis in the urine

A

Hemosiderin

Bilirubin

74
Q

Hemolytic anemia with normal reticulocyte count

A

Parvovirus infection –> aplastic anemia

75
Q

Autoimmune hemolysis diagnostic test

A

Positive Coombs test

76
Q

Heinz bodies, helmet cells, blister cells

A

G6PD

- Small, purple granules in the red cell that form as a result of damage to teh hemoglobin molecule

77
Q

African American or Mediterranean boy (X-linked) with fatigue, back pain, jaundice, anemia, dark urine

A

G6PD deficiency

  • No HSM
  • If abdominal pain consider cholelithiasis
78
Q

Diagnosis for G6PD

A
  • Can’t test right after or during an episode because reticulocytes have a large amount of G6PD and can lead to a false negative
79
Q

Hereditary spherocytosis diagnostic clues

A
  • Mild/moderate anemia
  • Reticulocytosis
  • Nonimmune hemolysis so DAT will be negative
  • Osmotic fragility testing is classic
80
Q

Hereditary spherocytosis treatment

A
  • Splenectomy is curative (need to vaccinate against H. flue, S. pneumo, N. meningitidis), do after age 5
  • Monitoring if not severe, some may require transfusion
  • Need folic acid supplementation
81
Q

Sickle cell molecular cause

A
  • Amino acid substition at AA 6 of beta globin chain – > valine is substituted for glutamic acid
82
Q

CBC findings in sickle cell

A

Normocytic anemia with high reticulocyte count

- Can also have high indirect bili

83
Q

Sickle cell trait complications

A
  • 8% of African Americans have sickle cell trait on newborn scree
  • Need hemoglobin electrophoresis after 3 months of age
  • Dehydration with extreme exercise
  • Microscopic/gross hematuria
  • Increased risk of kidney disease ane renal medullary carcinoma
  • Rhabdomyolysis
  • Splenic infarction
84
Q

Sickle cell complications

A
  • Vasoocclussive: acute pain secondary to ischemia and infarction (dactylitis in infants) - rehydration and aggressive pain control
  • Sequestration crisis: signs of shock due to pooling of blood in liver/spleen in response to infection, need transfusion
  • Aplastic crisis: due to parvovirus infection, need blood transfusions
  • Hyperhemolytic crisis: infection as cause
  • Priapism
85
Q

Sickle cell infection prophylaxis

A
  • Give penicillin VK from 2 months of age through age 5

- Pneumococcal vaccine series (functional asplenia)

86
Q

Howell Jolly bodies

A

Sickle cell anemia once asplenic

87
Q

Management of acute chest syndrome

A
  • Chest pain, infiltrate on xray, hypoxia
  • Get an ABG
  • Tx with transfusion or exchange transfusion
88
Q

Complication of chloramphenicol

A

Aplastic crisis

89
Q

Physical characteristics associated with fanconi anemia

A
  • Presents usually after age 3
  • Macrocytic anemia with elevated fetal hemoglobin
  • Abnormal skin pigmentation
  • Short stature/microcephaly
  • Renal abnormalities
  • Abnormal thumbs/foreamrs
  • Developmental delays
  • Eye/ear anomalies
90
Q

Treatment of fanconi anemia

A
  • RBC/platelet transfusions

- Only cure is bone marrow transplant

91
Q

Cause of Diamond Blackfan Anemia vs. Transient Erythroblasopenia of Childhood

A
  • DBA caused by arrest in maturaiton of red cells
  • TEC caused by suppression of erythroid production
  • Both have low hemoglobin and reticulocyte count to start (DBA often presents at birth, TEC in toddlers)
92
Q

Thumb abnormalities, urogenital defects, craniofacial problems, severe anemia

A

Diamond Blackfan Anemia (presents at 2-3 months of age usually)

93
Q

Treatment of TEC vs DBA

A
  • Can use steroids in DBA and need transfusions

- Self resolving for TEC

94
Q

Cause of febrile nonhemolytic reactions

A
  • Fever and chills

- Reduce risk by leukocyte filtered blood

95
Q

Cause of hemolytic reactions

A

Happen if blood is not properly cross matched

96
Q

Definition of neutropenia

A

< 1000 in first year of life

< 1500 after that

97
Q

Common infections with neutropenia

A

Recurrent mucosal ulcerations (mouth/perirectal), gingivitis, cellulitis, abscess formation, pneumonia, septicemia
- S. aureus, S. epi, gram negatives, enterococci

98
Q

Classes of drugs that cause neutropenia

A

Antibiotics and anticonvulsants

99
Q

Isoimmune neonatal neutropenia

A

Maternal antibodies against infant’s neutrophils cross placenta
- Mild neutropenia in healthy infant, resolves without treatment

100
Q

Neutropenia for about a week that occurs once a month with oral ulcers, fever, enlarged lymph nodes, in a kid younger than 10 years old

A

Cyclic neutropenia - AUTOSOMAL DOMINANT

  • Less likely to have invasive infections
  • Need serial CBCs for 6 weeks
  • Tx with daily GCSF
  • Most deaths are from clostridia or gram negative organisms if untreated
101
Q

Chronic benign neutropenia

A
  • Often incidental finding in a 1-5 year old
  • Usually don’t have any symptoms but if they have oral ulcers or infections can use GCSF
  • Outgrowth this by age 5
102
Q

Severe congenital neutropenia

A

Kostmann syndrome

  • Autosomal recessive
  • Arrest in development of neutrophils
  • SEVERE Infections, need GCSF or bone marrow transplant
103
Q

Pancytopenia, pancreatic exocrine insufficiency (diarrhea), short stature, recurrent infections, skeletal abnormalities

A

Schwachman Diamond Syndrome

  • Monitor for leukemic transformation
  • Treat infections, give pancreatic supplements
104
Q

Common meds to cause thrombocytopenia

A

Sulfas, seizure meds, vancomyccin

  • ASA and ibuprofen affect platelet function but not the platelet count
105
Q

Causes of neonatal thrombocytopenia

A

Sepsis, allo/autoantibodies from mom, MOST COMMON is clumping from improper collection

106
Q

Neonatal alloimmune thrombocytopenia

A
  • Isolated, transient, severe thrombocytopenia in the first 48 hours of life due to maternal antibodies
  • If autoimmune then both mom and baby have low platelets
107
Q

Complications from ITP and treatment

A
  • Intracranial hemorrhage (headaches or neurological changes)
  • Chronic/recurrent ITP (more likely in kids > 10) –> may need splenectomy
  • IVIG for treatment if severely low or significantly bleeding
108
Q

Kasabach Merritt Syndrome

A

Hemangioma that traps the platelets (localized consumptive coagulopathy) so have thrombocytopenia, bone marrow is normal
- Tx is to control hemangioma

109
Q

TAR Syndrome

A
  • Thrombocytopenia
  • Absent Radius
  • Can also ahve renal agenesis
  • WBC is usually elevated
  • 50% are symptomatic in the first week of life, 90% have symptoms by 4 months of age
110
Q

Vitamin K dependent factors

A

2, 7, 9, 10 (extrinsic pathway)

- Deficiency in these creates an elevated PT

111
Q

Risk factors for early onset vitamin K deficiency

A

Exclusive breastfeeding and being born at home (no vitamin K shot)
- Tx is vitamin K and FFP if active bleeding

112
Q

Workup for child with persistent bleeding after a heelstick or circumcision

A

Workup for hemophilia - congenital factor deficiency

113
Q

Prolonged PT

A

VItamin K deficiency (2, 7, 9, 10)

114
Q

Prolonged PTT

A

Hemophilia (factors 8 and 9)

115
Q

Hemophilia A factor deficiency

A

8 –> X linked recessive (so mostly in boys)

116
Q

Hemophilia B factor deficiency

A

9 –> X linked recessive (so mostly in boys)

117
Q

Bleeding problems with hemophilia

A
  • Bleeding from circumcision/venipuncture
  • Deep joint bleeds
  • Intracranial bleed
118
Q

Treatment for hemophilia

A

Factor replacement

119
Q

Use of von Willebrand factor

A
  • Leads to normal factor VIII function and platelet aggregation
120
Q

Symptoms of Von Willebrand’s disease

A
  • Excessive bleeding after dental procedure or tonsillectomy
  • Epistaxis
  • Girl with menorrhagia
121
Q

Labs for von Willebrand’s disease

A
  • NORMAL PT
  • PTT can be slightly prolonged but is often normal
  • VWF activity will be low
122
Q

Treatment of von Willebrand’s disease

A
  • Often no treatment
  • If minor bleeding can do DDAVP (stimulates endogenous release of stored vWF and factor VIII)
  • replacement of factor VIII concentrate for major surgery or life threatening bleeds
123
Q

DIC labs

A
  • Platelets low
  • Low fibrinogen
  • Elevated D-dimers
  • PT/PTT unpredictable
  • Thrombin time will be prolonged
124
Q

Iron deficiency peripheral smear

A

Microcytosis, hypochromic

125
Q

Iron deficiency anemia iron studies

A
  • Low iron, MCV, ferritin, RBCs

- High TIBC, RDW

126
Q

Mentzer index

A

Used to distinguish thalassemia from iron deficiency anemia
= MCV / RBC
- If > 12 then iron deficiency
- If < 12 then beta thalassemia

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