HEMATOLOGY/ONCOLOGY Flashcards
Most common soft tissue tumor?
Rhabdomyosarcoma
EVALUATION OF MICROCYTIC ANEMIA
SIGNS/SYMPTOMS OF ALL
- acute onset < 4 wks duration of Sx
- non-specific (anorexia, irritability, lethargy)
- signs of marrow failure (anemia, bleeding, purpuric/petechial lesions, low-grade fever)
- signs of infiltration
What is the most common and most serious congenital coagulation factor deficiencies?
Hemophilia A
- renal tumor of embryonal origin; 2nd most common malignant abdominal tumor in childhood
- 2-5 years old
WILMS TUMOR
- Painless, non-tender, firm, rubbery cervical or supraclavicular adenopathy
- symptoms important in staging (weight loss >10% of TBW over 3 months, unexplained high-grade fever, drenching night sweats)
Hodgkin / Non-hodgkin Lymphoma
most important sign of IDA
Pallor (7 - 8 mg/dL)
Hemophilia factor levels and severity of bleeding
o mild (5-25% normal activity): bleeding only after major trauma or surgery
o moderate (1-5%): bleeding with mild trauma
o severe (<1%): spontaneous bleeding
Frontal bossing, prominence of malar and maxillary bones are seen in
Beta thalassemia major
Why are the measurements of the total iron binding capacity important?
TIBC is high in iron deficiency anemia and low in anemia of chronic disease. Both illnesses have low serum iron levels.
Hemoglobin H disease
3/4 alpha globin chains deleted or mutated
MICROCYTIC ANEMIA MCV <80
CLITS
- Chronic disease
- Lead poisoning
- IDA
- Thalassemia
- Sideroblastic Anemia
Malignancy with highest mortality?
Brain (PNET)
Most common solid tumor outside CNS?
Neuroblastoma
Most common malignancy in children?
Leukemia (ALL)
Where does ALL spread?
Liver, spleen, lymph nodes
PHYSIOLOGIC ANEMIA OF INFANCY
- 1st week of life – progressive decline in Hgb that persists 6-8 weeks
- Usually lasts 8-12 weeks
- Hb levels around 11g/dL, rarely falls below 10g/dL
sites of relapse in ALL
bone marrow, CNS (increased ICP and isolated cranial nerve palsies), testes (painless swelling of one or both testes in 1-2% of males)
Wilms Tumor vs. Neuroblastoma
DIFFERENTIAL DIAGNOSIS OF SMALL, ROUND, BLUE-CELL TUMORS:
- Wilms tumor
- Acute leukemia
- Rhabdomyosarcoma
- Mesothelioma / Medulloblastoma
- Ewing sarcoma
- Retinoblastoma
- Primitive neuroectodermal tumor (PNET)
- Neuroblastoma
*** W-A-R-M-E-R-P-N (warmer in the Philippines)
hydrops fetalis
Hemoglobin Bart (alpha thalassemia major)
4/4 alpha globin chains deleted or mutated
Rhabdomyosarcomas are most commonly found in
the head and neck
TREATMENT OF VON WILLEBRAND DISEASE
- Mild bleeding in type 1: desmopressin (causes release of vWF from endothelial stores)
- Severe disease: Factor VIII concentrates which contain high vWF Ag
poor prognostic factors of ALL
o < 2 yrs or > 10 yrs
o male
o WBC > 100,000 u/L on presentation
o presence of CNS leukemia
o presence of a mediastinal mass
Cooley anemia
Beta-thalassemia major
- no beta-globin production
- anemia at 6 mos (decline in normal Hgb F & rise in Hgb A), splenomegaly, extramedullary hematopoiesis)
- ↓ or absent beta chain of Hgb → normal alpha Hgb chains build up → form insoluble aggregates that precipitate within the RBCs → hemolysis or damage to RBCs → susceptible to macrophage destruction & splenic sequestration → small & pale RBCs
t (9:22)
AML/CML
*Philadelphia chromosome
What is the most common hereditary hypercoagulable disorder?
Factor V Leiden
most common sites of metastasis of neuroblastoma
long bones & skull, BM, liver, lymph nodes, skin
invaginate and migrate along the neuroaxis where neural crest cells are present – sympathetic ganglia, adrenal glands
Neuroblasts (pluripotent stem cells)
What is the most common hereditary bleeding disorder?
von Willebrand Disease
o Disturbs both primary & secondary hemostasis
o Role in platelet adhesion to exposed subendothelium leads to increased bleeding time → mucous membrane bleeding, petechiae, purpura
o Often have a (+) family history
VON WILLEBRAND DISEASE
“crew cut” or “hair on end” appearance on skull radiographs
SICKLE CELL DISEASE
* Erythropoiesis must increase to compensate for hemolysis → marrow expansion → resorption of bone → new bone formation on the external aspect of the skull
* Dx: sickle cells and Howell-Jolly bodies on PBS; definitive diagnosis with Hb electrophoresis (HbS)
o Develops at around 6 mos old when HbS (result of a single missense mutation in the B-globin gene of Hgb) replaces HbF
o Episodes of painful crisis due to hypoxic tissue injury from microvascular occlusions
o Hgb becomes susceptible to polymerization in conditions of low oxygen or dehydrationàreduces flexibility of the RBC membrane
o Any organ can be affected by vascular congestion, thrombosis, and infarction caused by sickling cells
SICKLE CELL DISEASE
- S/Sx: painless abdominal pain with flank mass that does not cross the midline, hematuria (12-25%), hypertension
- Dx: abdominal UTZ, CT scan or MRI, abdominal X ray, liver & kidney function tests, chest x ray (pulmonary metastasis)
- Tx: transabdominal nephrectomy & post-surgical chemoTx
- Worse prognosis: large tumor (>500 gms), st III & IV, unfavorable histologic type (anaplastic)
- More than 60% of patients generally survive
- St I-III have a cure rate of >90%
WILMS TUMOR
a large protein made by endothelial cells and megakaryocytes; a carrier for factor VIII and is a cofactor for platelet adhesion
vWF
What is the earliest joint hemorrhages in children?
Ankles
S/Sx: reflect the tumor site & extent of disease; non-tender abdominal mass which may cross the midline, Horner syndrome, hypertension, cord compression (from a paraspinal tumor)
NEUROBLASTOMA
“birbeck granules”
Langerhans cell histiocytosis
Most children with leukemia present with:
Unexplained fever, pallor, and hemorrhage
What is the hallmark of hemophilia?
Prolonged bleeding
target cells and Heinz bodies on PBS
ALPHA THALASSEMIA
Dx: decreased reticulocyte count (due to ineffective hematopoiesis), microcytic, hypochromic RBCs, normal RDW
*definitive diagnosis with Hb electrophoresis
IDA vs. THALASSEMIA
- autosomal recessive
- aplastic anemia with microcephaly, microphthalmia, hearing loss, limb anomalies (absent radii & thumbs)
- Dx: clinical, cytogenetic analysis
- Tx: steroids, BMT, supportive care
FANCONI ANEMIA
measures vWF antigen levels and activity
Ristocetin cofactor assay
Auer rods deriving from the crystallisation of myeloperoxidase (MPO) granules are the hallmark of
Acute Myeloid Leukemia (AML)
Metastases: periorbital bruising (“raccoon eyes”), subcutaneous tumor nodules, opsoclonus /myoclonus (“dancing eyes, dancing feet”)
NEUROBLASTOMA
Most common solid tumor in children?
Brain Tumors
What are the typical peripheral blood smear findings in aplastic anemia?
Hypocellularity and pancytopenia
- embryonal tumor of neural crest cell origin
- 3 rd most common pediatric cancer
- 8% of childhood malignancies
NEUROBLASTOMA
International Neuroblastoma Staging System
- St 1: tumors confined to the organ or structure of origin
- St 2: tumors extend beyond the structure of origin but not across the midline with (2B) or without (2A) ipsilateral lymph node involvement
- St 3: extend beyond the midline w/ or w/o bilateral lymph node involvement
- St 4: disseminated to distant sites (bone, BM, liver, distant lymph nodes)
- St 4S: <1 yr old w/ dissemination to liver, skin, or BM w/o bone involvement & with a primary tumor
Diagnostic tests for neuroblastoma
Dx: abdominal CT scan (calcification & hemorrhage), 24-hr urine VMA & HVA (elevated in 95% of cases), BUN/Crea, CXR, bone scan, LFTs, CBC
associated with:
o Neurofibromatosis
o Beckwith-Wiedemann syndrome (hemihypertrophy, visceromegaly, macroglossia)
o WAGR syndrome (Wilms tumor, Aniridia, Genitourinary abnormalities, mental retardation)
WILMS TUMOR
Which type of leukemia in childhood will have the best response to chemotherapy?
ALL
LABORATORY FINDINGS IN BLEEDING DISORDERS
3 of the most important predictive factors for ALL:
- Age of the patient at the time of diagnosis
- Initial leukocyte count
- Speed of response to treatment
t (11:22)
Ewing Sarcoma
• associated with:
o N-myc oncogene – tuberous sclerosis
o Neurofibromatosis – Pheochromocytoma
o Hirschsprung disease
NEUROBLASTOMA
