Heme-onc Flashcards
Microcytic anemia with high rdw
Iron deficiency and lead toxicity
When do you see ringed sideroblast
Lead poisoning
Drinking goat milk lead to what deficiency
Folate
A hemolytic anemia without an elevated retic count would make you think of what
Parvovirus
Coombs test positive is what type of anemia
Autoimmune hemolytic
How does G6PD deficiency present
Hemolytic anemia in AA or Mediterranean boy (x linked)
Sudden onset of parlor and anemia, dark Uribe and jaundice
Agents that cause oxidative stress and exacerbate G6PD
Sulfa containing antibiotic, nitrofurantoin, moth balls, antimalarial meds (chloroquine and primaquine) fava beans, rasburicase
When should testing for G6PD be done
Several weeks after the episode
Heinz bodies are found when
G6PD deficiency
*bite and blister cells
Presentation of hereditary spherocytosis
Mild or moderate anemia, splenomegaly, intermittent jaundice, gallstones
Autosomal dominant
How do you diagnose hereditary sperocytosis
Osmotic fragility testing
Fragile red cells with increased MCHC are found in which condition
Hereditary spherocytosis
What is the etiology of hereditary spherocytosis
Spectrin deficiency or mutation
What is the etiology of sickle cell
Valine is substituted for a glutamic acid at amino acid 6 of the beta globin chain
Until what age is antibiotic prophylaxis for sickle cell disease indicated
Through age 5
What finding will you find in blood smear for a pt with sickle cell
Howell jolly bodies
What should you think of if you have a kid older than 5 with sickle cell in which you can palpate a spleen
By age 5 kids with sickle cell have functional asplenia and you can’t feel a spleen. Thus you want to think of a variation.
Hemoglobin SC or sickle trait and thalassemia together
Children with sickle cell and thalassemia together are resistant to what
Malaria
How is acute chest syndrome define and what would be the next study to obtain
Chest pain, infiltrate in chest X-ray, hypoxia
You want an ABG to confirm hypoxia
How does fanconi’s anemia present
After age 3
Macrocytic anemia with elevated fetal hemoglobin
Abnormal skin pigmentation
Growth retardation (short stature)
Renal abnormalities
Absent thumb
How is fanconi anemia inherited
Recessive
What are patients with fanconi anemia at risk for
Transformation to AML or myelodysplastic syndrome
How does diamond Blackfan anemia present (DBA)
DBA =
Dysmosphic (craniofacial problems, thumb abnormalities, urogenital defects)
Babies (infants 2-3 months)
Anemia (macrocytic, severe and chronic)
*require transfusions and steroids are helpful
How does transient erythroblastopenia of childhood present (TEC)
TEC =
Transient
Erythroid production suppression (anemia)
Children (18-26 months)
Lab findings for hemolytic anemias
Elevated retic count Low serum haptoglobin (bc it is used up transporting the released hemoglobin) Elevated LDH Elevated indirect bili Hemoglobinuria not hematuria
Drug that most frequently causes neutropenia
Macrolides
Presentation of cyclic neutropenia
Low WBC that lasts about 1 wk and reappears monthly
Oral lesions and enlarged lymph nodes
Child younger than 10 yrs
Pts with cyclic neutropenia that is untreated die due to which organisms
Clostridia perfringens and gram negative organisms
Tx for cyclic neutropenia
Daily rhG-CSF (recombinant human granulocyte colony stimulating factor)
Presentation of kostmann agranulocytosis
Like cyclic neutropenia except ANC remains low
Autosomal recessive vs dominant
Presentation of shwachman diamond syndrome
Pancreatic exocrine insufficiency (steatorrhea)
Pancytopenia - recurrent infections (especially upper respiratory and skin)
Skeletal abnormalities
Which medications can lower platelet count
Sulfa, seizure meds and vancomycin
What leads to worse prognosis in a pt with ITP
Kid older than 10 years
Presentation of TAR syndrome
Thrombocytopenia and absent radius!
Presents within the first month of life
Prolongued PT is associated with what
Vit K deficiency (factors 2,7,9,10)
Prolongued PTT is associated with what
Hemophilia
May be Von Willebrand if female
How is hemophilia inherited
X linked recessive
Lab findings in DIC
Low platelets, low fibrinogen, elevated d dimer, prolongued thrombin time
Presentation of osteoid osteoma
Tibia or femur pain, worse at night and relieved by ibuprofen
Central radiolucent surrounded by thick sclerotic bone
Presentation of osteogenic sarcoma
Teenager usually going through a growth spurt who presents with pain
Common sites are the distal long bones (femur and humerus)
How does Hodgkin’s lymphoma present
Typically in teens
Non tender enlarged cervical or supraclavicular lymph nodes
Weight loss, fever, night sweats
High WBC with a relatively low lymphocyte count
What would a lymph node biopsy of Hodgkin lymphoma show
Reed sternberg cells
How does non Hodgkin lymphoma present
Small child
Non tender mass in the abdomen
Airway compression
Rapid presentation
What is the survival rate when neuroblastoma presents before 1 year of age
95 percent
If a pt with neuroblastoma has hypertension what is the most likely reason
Renal artery compression
How do you diagnose neuroblastoma
Biopsy of tumor
Elevated urine VMA and HMA (not catecholamines)
What is the most important prognostic factor for neuroblastoma
Age
If <1 yr prognosis is excellent
How does sporadic retinoblastoma present
Usually unilateral
Only 5% will have a family Hx. If one parent had only 1 eye affected then there is only a 1/20 (5%) chance of a child having it
How does genetic retinoblastoma present
Autosomal dominant with incomplete penetrance. Gene is on the long arm of chromosome 13.
If a parent had both eyes affected there is 1/2(50%) chance of any child having it
What other malignancies in a child with retinoblastoma do you need to monitor for
Sarcomas and malignant melanomas
What is the most common solid tumor of infancy
Neuroblastoma
What is the most common pediatric abdominal malignancy
Wilms tumor
If an abdominal XRAY shows calcification on an abdominal mass what should you think of
Neuroblastoma
Wilms tumor does not calcify
What should you think of in a pt with hemihypertrophy
Wilms tumor
Beck with wiedemann syndrome
How does superior vena cava syndrome present
Red face, facial swelling, upper extremity edema, distended neck veins
Due to extrinsic compression of the SVC by an anterior mediastinal tumor
Side effect of cyclophosphamide
Hemorrhagic cystitis
Side effect of cisplatin
Hearing loss and peripheral neuropathy
Side effect of bleomycin
Pulmonary fibrosis
Side effect of doxorubicin and daunomycin
Cardiac toxicity
Side effect of vincristine/vinblastin
Neurotoxicity and SIADH
Side effect of methotrexate
Oral and GI ulcers
Bone loss
Would you intubate a pt with anterior mediastinal mass
No because the airway compression is below the vocal cords
What can cause symptoms associated with anterior mediastinal masses
Thymoma
Teratoma
Thyroid carcinoma
Terrible lymphoma
