Hema-Onco Flashcards
diagnostic test to distinguish ACD from IDA
Soluble transferrin receptor (sTfR)
- high in IDA, normal in ACD
progressive decline in Hb levels in normal full term infants persists until what age?
persists for 6-8 weeks
Hemoglobin concentration continues to decline until tissue oxygen needs become greater than oxygen delivery. This point is reached between what age?
8 and 12 weeks of age
blood smear in IDA
Hypochromic, microcytic RBCs
most accurate method of diagnosing IDA
Bone marrow iron staining
most practical means to establish the diagnosis of IDA
Increase in hemoglobin >1 g/dL after 1 month of iron therapy
therapeutic dose of Iron for IDA
3-6 mg/kg elemental Iron in 1 or 2 doses
reticulocytosis after initiation of iron therapy in IDA happens when?
48-72 hours
Subjective improvement, decreased irritability, increased appetite and serum iron after initiation of Iron therapy in IDA happens when?
12-24 hours
describe the primary pathologies in thalassemia and sickle cell disease
Thalassemia: quantity of globin produced
Sickle cell disease: quality of globin produced
major clinical complication of transfusion-dependent thalassemia
Hemosiderosis
major cause of death in thalassemia
Cardiac disease
Common presentation/manifestation of G6PD
acute episodic hemolytic anemia
neonatal jaundice
this constitutes the most important therapeutic measure in G6PD
prevention of hemolysis
Deficient factor in Hemophilia A
Factor VIII
Deficient factor in Hemophilia B
Factor IX
most common and serious congenital coagulation factor deficiencies
Hemophilia A and B
hallmark of hemophilic bleeding
hemarthrosis
Major long-term disability associated with hemophilia
chronic arthropathy
most common inherited bleeding disorder
Von Willebrand disease (VWD)
Patients with VWD typically present with this type of bleeding
Mucosal bleeding
most common type of VWD
Type 1
- mild to moderate quantitative defect
most severe type/form of VWD
Type 3
- VWF is completely absent
most common cause of acute onset of thrombocytopenia in an otherwise well child
Idiopathic thrombocytopenic purpura (ITP)
Most common viruses associated with ITP
EBV
HIV
In some patients, ITP appears to arise in children infected with this organism
Helicobacter pylori
classic presentation of ITP
sudden onset of generalized petechiae and purpura
What is Evans Syndrome?
Autoimmune hemolytic anemia + thrombocytopenia
first line of treatment for ITP presenting with mucocutaneous bleeding
IVIG (0.8-1 g/kg) single dose for 1-2 days or
short course of corticosteroids
most common malignant neoplasms in childhood
leukemias
accounts for 77% of cases of childhood leukemia
Acute Lymphoblastic Leukemia (ALL)
this infection is associated with B-cell ALL in certain developing countries
Epstein-Barr virus (EBV) infection
ITP is common in this age group
1-4 years old
most common immunophenotype of ALL
B-lymphoblastic leukemia
Diagnosis of ALL based on bone marrow evaluation
> 25% lymphoblasts
single most important prognostic factor in ALL
treatment
-without it, the disease is fatal
definition of standard risk of ALL
Age 1-10 yr
Leukocyte count <50,000/uL
definition of high risk for ALL
Children <1 yr or >10 yr
Leukocyte count >50,000/uL
definition of remission in ALL
<5% blasts in the marrow
return of neutrophil and platelet counts to near-normal levels
what are the phases of therapy in ALL and what are the drugs being given for each phase
Remission induction phase - weekly vincristine + steroids + aspariginase
Consolidation phase -with intrathecal chemo
Maintenance phase - daily mercaptopurine + weekly methotrexate
which among the types of ALL has worse outcome, B-ALL or T-ALL?
T-ALL
Characteristic feature of AML based on bone marrow evaluation
> 20% myeloid-monocyte-megakaryocyte blast cells
which is more common in Down syndrome during the 1st 3 yr of life, ALL or AML?
AML
Transient leukemia or myeloproliferative disorder in Down syndrome usually resolve within _____
1st 3 months of life
This type of leukemia is characterized by a specific translocation known as Philadelphia chromosome resulting in a BCR-ABL fusion protein
Chronic Myelogenous Leukemia (CML)
signs and symptoms only found in AML and uncommon in ALL
- subcutaneous nodules/“blueberry muffin” lesions
- infiltration of the gingiva
- signs and lab findings of DIC
- discrete masses: chloromas/granulocytic sarcomas
current standard treatment for pediatric CML
Tyrosine kinase inhibitor such as Imatinib
Patients with NF Type 1 and Noonan syndrome have a predilection for this type of leukemia
JMML/Juvenile Chronic Myelogenous Leukemia (JCML)
most common cancer in adolescents
Lymphoma
Infectious agents associated with Hodgkin Lymphoma
- Human herpesvirus 6
- Cytomegalovirus
- Epstein-Barr virus (EBV)
Pathognomic feature of Hodgkin Lymphoma
Reed-Sternberg (RS) cell
what are B symptoms?
- Unexplained fever >38 C
- Weight loss >10% of total body weight over 6 months
- Drenching night sweats
3 subtypes of Non-Hodgkin Lymphoma
Lymphoblastic Lymphoma (LBL)
Mature B-cell Lymphoma (2 pathologies: Burkitt Lymphoma (BL), diffuse large B-cell lymphoma (DLBCL)
Anaplastic Large Cell Lymphoma (ALCL)
electrolyte abnormalities in Tumor Lysis Syndrome
Hyperkalemia
Hyperuricemia
Hyperphosphatemia
Hypocalcemia
most common primary brain tumor in children with excellent prognosis
Pilocystic astrocytoma
classic sites of Pilocytic astrocytoma
Cerebellum
Optic pathway region
most common CNS tumors in children <1 yr old
Choroid Plexus Tumor
most common group of malignant CNS tumors of childhood, accounting 20% of pediatric CNS tumors
Embryonal tumors or Primitive Neuroectodermal tumors (PNET)
childhood brain tumor that most often metastasizes extraneurally
Medulloblastoma
most common extracranial solid tumor in children and the most commonly diagnosed malignancy in infants
Neuroblastoma
This associated syndrome in neuroblastoma is characterized by limping and irritability in young child with bone and bone marrow metastases
Hutchinson syndrome
Syndrome seen in neuroblastoma which presents as massive involvement of the liver with or without respiratory distress
Pepper Syndrome
Signs and symptoms of horner syndrome
Unilateral ptosis
Myosis
Anhidrosis
half of neuroblastoma tumors arise in what organ?
Adrenal glands
these tumor markers help to confirm the diagnosis of neuroblastoma
HVA and VMA
- elevated in the urine in 95% of patients
most common primary malignant renal tumor of childhood
Wilms tumor/Nephroblastoma
most common initial clinical presentation of Wilms Tumor
incidental discovery of an asymptomatic abdominal mass
The most common pediatric soft tissue sarcoma
Rhabdomyosarcomas
tumors that present as small round blue cell tumors in microscope
Rhabdomyosarcoma
Neuroblastoma
Non Hodgkin Lymphoma
Ewing sarcoma
favorable sites of rhabdomyosarcoma
Female genitalia
Paratesticular
Head and neck (nonparameningeal) regions
most common primary malignant bone tumor in children and adolescents
Osteosarcoma
More common malignant bone tumor among children <10 yr old
Ewing sarcoma
Patients with _______ have a significantly increased risk for development of Osteosarcoma
Hereditary retinoblastoma
classic radiographic appearance of this tumor is “sunburst appearance”
Osteosarcoma
common site of Osteosarcoma
Metaphysis
common site of Ewing sarcoma
Diaphysis
Standard chemotherapy regimen for osteosarcoma
Methotrexate (M), Doxorubicin (A), Cisplatin (P) (MDC)
Primary tumors arising in the chest wall in Ewing sarcoma are called?
Askin tumors
this type of bone tumor is often associated with systemic manifestations such as fever and weight loss
Ewing sarcoma
it has a radiographic appearance of a primary lytic bone lesion with periosteal reaction — “onion-skinning”
Ewing sarcoma
classic presentation of retinoblastoma
leukocoria/white pupillary reflex
what is trilateral retinoblastoma
primitive neuroectoderal tumor + bilateral retinoblastoma
most common benign tumors of infancy
Hemangiomas
more than 50% of hemangiomas are located in ___ and ___ region
Head, neck
primary site of visceral involvement in Hemangioma
Liver
hemangiomas involutes by this age
10-15 yr
after initiation of iron therapy in IDA, when is initial bone marrow response and erythroid hyperplasia are expected to happen?
36-48 hours
increase in hemoglobin level, mean corpuscular volume and ferritin after initiation of iron therapy in IDA happens when?
4-30 days
Iron therapy is indicated for all neonates with anemia of prematurity starting at what month of age, and it ends until what age
1 month until 1 year
most widespread and common nutritional disorder in the world
Iron deficiency anemia
how much iron is necessary to be absorbed to maintain positive iron balance in childhood?
1 mg daily
Features of Thalassemic facies
Maxillary hyperplasia
Flat nasal bridge
Frontal bossing
tests to differentiate IDA vs ACD
Serum Ferritin: Dec IDA, Inc ACD
TIBC: Inc IDA, Dec ACD
STRf: Dec IDA, Dec ACD
Organs that store Iron
Liver - Endocrine organs - Heart
Conditions that warrant Splenectomy in children with ITP
- older child >4 yo with severe ITP which lasted for >1 yr (chronic ITP) and whose symptoms are not easily controlled with therapy
- when life threatening hemorrhage complicates acute ITP, if platelet cannot be controlled rapidly with platelet transfusion and administration of IVIG and corticosteroids
favorable characteristics in ALL prognosis
Rapid response to therapy
Hyperdiploidy
Trisomy of specific chromosomes (4, 10, 17)
Rearrangements of the ETV6-RUNX1
definition of complete response in HL
complete resolution of disease on clinical examination and imaging studies or at least 70-80% reduction of disease and a change from initial positivity to negativity on PET scan
Poor prognostic features of Hodgkin Lymphoma
Tumor Bulk
Stage at diagnosis
Extralymphatic disease
Presence of b symptoms
significant morbidities associated with craniopharyngiomas
panhypopituitarism
growth failure
visual loss
syndrome associated with neuroblastoma which presents as intractable secretory diarrhea caused by tumor secretion of vasointestinal peptides
Kerner-Morrison Syndrome
