Heme/Onc Flashcards
0
Q
- sarcoma in long bones (femur, humerus)
- more common in african americans
- teenager, usually going through a growth spurt that presents with pain
- main site of mets are lungs
- Tx: chemo, XRAD, surgery
A
Osteogenic sarcoma
1
Q
- sarcoma in the soft tissue component and long bones
- usually not seen in african americans
- Tx: chemo, XRAD, surgery
- can metastasize to lungs
A
Ewing Sarcoma
2
Q
- tibia or femur pain worse at night, relieved by Ibuprofen
- Xray: central radiolucent (white) area surrounded by thick sclerotic bone
A
Osteoid osteoma
3
Q
-most common type of solid tumors
A
Brain tumors
4
Q
- teenager with non-tender enlarged cervical LN or supraclavicular LN
- wt loss, fevers, night sweats
- Bx: Reed Sternberg cells
- CBC: incr WBC, decr lymphs
A
Hodgkins Lymphoma
5
Q
- MC pediatric emergency
- poor prognostic signs: 50, T cell
- good prognostic sign: B cell
- present with bone pain, joint pain, or swelling, fever, HSM
- pancytopenia, nL or incr WBC
- Dx: BM Bx
A
Acute Lymphocytic Leukemia
6
Q
- younger child with non-tender abd mass or head & neck region
- lymphoblastic (T cell region)
- non-lymphoblastic (B cell region)
- mediastinal masses & pleural effusions associated w/ lyphoblastic type
A
Non-Hodgkin Lymphoma
7
Q
- solitary focal bone lesion often in skull
- seborrheic rash, ear DC, DI, excessive urination
- Dx: skin Bx & electron microscopy
- Tx: surgery, steroids, chemo
A
Langerhans Cell Histiocytosis
8
Q
- 2nd MC solid tumor in childhood
- presents in the adrenal glands 50%
- 95% survival catecholamines
- Dx: biopsy, urine VMA, HAM, CT w/ contrast, pancytopenia, incr ferritin, incr LDH
- Bad prognosis: + n-myc amplification
A
Neuroblastoma
9
Q
-leucocoria
-5% will have a +FHx
-Tx: surgery, chemo, radiation
-Risk for osteosarcoma later in life
-parent 2 eyes: 50% chance to kid
parent with 1 eye: 5% chance to kid
A
Retinoblastoma
10
Q
- adolescent girl, neg HCG, and persistent abd pain
- benign: filled with fluid on US
- malignant: defined with echodensities
A
Ovarian masses
11
Q
- MC soft tissue sarcoma in childhood
- may present as a child with constipation
- visible or palpable rectal mass (grape-like mass protruding from the vagina)
- younger: head and neck
- older truncal or extremity (may be h/o trauma)
- Dx: Bx
- tx: chemo, surgery, XRAD
A
Rhabdomyosarcoma
12
Q
- -MC pediatric abdominal malignancy
- median age 3.5 years
- MC presentation is an asymptomatic abd mass with occasional hypertension and gross hematuria
- Dx: histology
- Tx: nephrectomy, chemo, XRAD
- associated with aniridia and hemihypertrophy
A
Wilms Tumor (Nephroblastoma)
13
Q
- rapid breakdown of a large number of tumor cells
- MC at the initiation of chemotherapy for large tumors or leukemia (Burkitts)
- high Phos, K, uric acid, low Ca
- Tx: hydration, alkalinization, allopurinol
A
Tumor Lysis Syndrome
14
Q
- tumor in or around the spine
- presents with neurologic symptoms or bowel.bladder dysfunction
- Tx: steroids or radiation
A
Cord Compression
15
Q
- respiratory distress when supine
- intubation is useless if mass is below vocal cords
- Thymoma, Teratoma, Thyroid, T-cell lymphoma*
- Tx: radiation in an emergency
A
Anterior mediastinal mass
16
Q
- extrinsic compression of the SVC by an anterior mediastinal tumor
- MC Hodgkins or T-cell lymphoma
- presents with red face (plethora), facial swelling, upper extremity edema, distended neck veins, and neurologic symptoms
- Tx: underlying disorder, steroids or radiation
A
SVC syndrome
17
Q
-causes hemorrhagic cystitis
A
Cyclophosphamide
18
Q
-causes pulmonary fibrosis
A
Bleomycin (Blow my icing)
19
Q
-causes cardiomyopathy
A
Anthracycline (Doxorubicin, Daunomycin)
20
Q
-causes neurotoxicity and SIADH
A
Vincristine/Vinblastine
21
Q
-causes pancreatitis
A
Aspariginase
22
Q
-causes CNS problems
A
Procarbazine
23
Q
-causes oral and GI ulcers
A
Methotrexate
24
- physiological drop in HCT by 2nd or 3rd month of life (8-10 weeks)
- no further evaluation is necessary
- etiology: low erythropoietin production
Anemia of the Newborn
25
- defect in one of the beta globin gene alleles
- asymptomatic
- mild microcytic anemia
Thalassemia Minor or Trait
26
- defects in both beta globin gene alleles
- -mild phenotype
- requires infrequent transfusions (after a viral infx)
Thalassemia Intermedia
27
- severe deficiency of beta globin due to a mutation in both beta globin gene alleles
- present in the first year of life with profound microcytic anemia
- require regular blood transfusions
- small for age Greek child with HSM
- skull Xray with hair on end appearance (extramedullary hematopoiesis)
- complications: cholelithiasis, hemosiderosis
Thalassemia major (Cooley's anemia)
28
- Dx: Hb electrophoresis
- very low HB A1, incr A2 (alpha--delta)
- incr Hb F (alpha-gamma)
Beta thalassemia
29
- microcytic anemia
| - deletion of one or more of the alpha globin gene alleles
Alpha Thalassemia
30
-high Hb F and Hb S
Sickle Cell Anemia
31
- higher risk in breast fed infants, infants given cows milk
- present with pallor and tachycardia
- low Hb and MCV
- high RDW
Iron Deficiency Anemia
32
- may be normocytic or microcytic
- low TIBC
- high ferritin
- Tx with Fe is not necessary
Anemia of chronic disease
33
- pale kid with vague symptoms (irritability, lethargy, sleplessness, headaches, belly pain, constipation)
- usually coexists with iron deficiency
- Dx: venous blood level
- Tx; chelation (penicillamine, dimercaprol, EDTA)
- elevated free erythrocyte protoporphyrin
- ringed sideroblast
Lead Poisoning
34
- instrinsic factor deficiency
- bacterial overgrowth
- s/p bowel resection
- infants whose mothers are vegetarians
- macrocytic anemia
- Schilling test for absorption
- AKA cobalamin
Vitamin B12 Deficiency
35
- infant fed goats milk
| - macrocytic anemia
Folate deficiency
36
- lack of intrinsic factor
- weakness, paresthesia, sore tongue
- Dx: erythrocyte folic acid concentration
- Tx: IM cobalamin
Pernicious anemia
37
- black or mediterranean boy with dark urine, jaundiced, and anemic
- exposure to mothballs, antimalarials, nitrofurantoin, bactrim, cipro or fava beans triggers hemolytic crisis
- sudden onset of pallor and anemia
- Heinz bodies: small purple granules in the red cell that form as a result of damage to the Hb molecule
- X linked
G6PD deficiency
38
-G6PD mainly found in African Americans
Type A
39
-G6PD mainly found in Mediterraneans
Type B
40
- defects in the surface of red cells resulting in loss of erythrocyte surface area
- MC autosomal dominant
- increased MCHC, low retic ct
- Dx: osmotic fragility testing
- etiology: spectrin deficiency or mutation
- presents with mod anemia, splenomegaly, and intermittent jaundice
- Tx: folic acid or splenectomy
- parvovirus causes aplastic crisis
Hereditary Spherocytosis
41
- -anemia, jaundice, and splenomegaly
- cells depleted of lactate and ATP--> buildup of DPG-->right shift of the oxygen binding curve
- increase in exercise tolerance
- Northern Europeans
- Autosomal recessive
Pyruvate Kinase Deficiency
42
- caused by a substitution at AA6 of the beta globin chain (valine for glutamic acid)
- newborn screening
- vasoocclusive crisis
- splenic sequestration
- aplastic crisis
- need PCN PPx
- Howell-Jolly Bodies
Sickle Cell Anemia
43
- chest pain, infiltrate, and hypoxia in a pt with sickle cell
- Dx: CXR, ABG
- Tx: transfusion
Acute chest syndrome
44
-when sickle cell children have trouble concentrating their urine
hyposthenuria
45
-MC cause of RUQ pain in a child with sickle cell
Gallstones
46
- pallor, mucosal ulcerations, and bruising
- anemia, low WBC, low plts
- increase in fetal Hb and macrocytosis
- normal liver and no LAD
- h/o chloramphenicol use
- Tx: bone marrow Tx
Aplastic anemia
47
- presents after age 3
- skin pigmentation, growth retardation, renal abnl, absent or hypoplastic thumb
- macrocytic anemia with elevated fetal Hb
- autosomal recessive
- at risk of AML or myelodysplastic syndrome
Fanconi Anemia
48
- arrest in the mutation of red cells
- anemia at birth, macrocytic
- dysmorphology (thumb abnl, UG abnl, craniofacial probs)
- need transfusions
- steroids sometimes helpful
Diamond Blackfan Anemia
49
- suppression of erythroid production
- toddlers
- spontaneous recovery
- normal MC
Transient Erythroblastopenia of Childhood
50
- WBC contain lysosomal granules
- abnl chemotaxis
- frequent lung and skin infx, easy bruisability, oculocutaneous albinism
- S. aureus, S. pyogenes, pneumococcus
- autosomal recessive
- Tx: BMT
Chediak-Higashi
51
- phagocytes cannot kill bugs they ingest
- recurrent bacterial and fungal infx in skin, lungs, GI, liver, spleen
- staph or gram negative infx
- Dx: NTB test measures the oxidative burst
- infx PPx, interferon, and BMT
Chronic Granulomatous Disease
52
- WBCs do not adhere well
- delayed separation of the umbilical cord, impaired wound healing, severe periodontal disease
- high WBC
- Tx: BMT
Leukocyte Adhesion Deficiency
53
- autosomal dominant
- low WBC x1 wk, reappears every month or so
- oral lesions in a child less than 10 yo
- Dx: CBC twice a week
- infx with Clostridium perfringens
- Tx: G-CSF if symptomatic
Cyclic Neutropenia
54
- autosomal recessive
- arrest in the development of neutrophils
- low ANC
- Tx: G-CSF
Kostmann Syndrome
55
- pancytopenia, EPI
- short statures, diarrhea, recurrent infx, skeletal abnl, clinodactyly/syndactyly
- monitor for leukemic transformation
Shwachman-Diamond Syndrome
56
- recent viral illness, low plts, ecchymoses, petichiae
- big plts
- Tx: IVIG, WinRho
- transfuse if plts <20K and bleeding
Immune Thrombocytopenic Purpura
57
- immunodeficiency only seen in boys (X-linked)-baby boy with really bad diaper rash, bruising, ear infx, and pneumonia
- low plts, low MPV, or small plts
- Tx: BMT
- at risk for other malignancies
Wiskott-Aldrich Syndrome
58
- hemangioma
- thrombocytopenia caused by consumptive coagulopahty
- at risk for DIC
- Tx: control hemangioma and transfuse
Kasabach-Merritt Syndrome
59
- thrombocytopenia, absent radius
- high WBC, nl Hb
- symptomatic in the first week of life
TAR Syndrome
60
- plts cannot aggregate
| - nl plts ct and coags
Glanzmann Thrombasthenia
61
-baby born at home who is breast fed and bleeding
Vit K Deficiency
62
- bruising, bleeding from circ, or site of venipuncture, deep joint bleeds
- prolonged pTT
- boys with a FHx in men on mom's side
- X-linked recessive
Hemophilia
63
- excessive bleeding after a dental procedure or T&A
- epistaxis
- menorrhagia in a female
- abnl pTT, normal PT
- prolonged bleeding time
- Tx: DDAVP, amicar, factor VIII
- autosomal dominant
Von Willebrand Disease
64
- h/o sepsis, malignancy, or burns
- low plts, fibrinogen, coags
- elevated D-dimer, thrombin
- Tx: plts, FFP, cryo, RBCs
DIC
