HEMATOLOGY/ONCOLOGY Flashcards by Sharmaine Lozano

Most common soft tissue tumor?

Rhabdomyosarcoma

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EVALUATION OF MICROCYTIC ANEMIA

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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

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What is the most common and most serious congenital coagulation factor deficiencies?

Hemophilia A

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renal tumor of embryonal origin; 2nd most common malignant abdominal tumor in childhood
2-5 years old

WILMS TUMOR

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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

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most important sign of IDA

Pallor (7 - 8 mg/dL)

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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

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Frontal bossing, prominence of malar and maxillary bones are seen in

Beta thalassemia major

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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.

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Hemoglobin H disease

3/4 alpha globin chains deleted or mutated

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MICROCYTIC ANEMIA MCV <80

CLITS

Chronic disease
Lead poisoning
IDA
Thalassemia
Sideroblastic Anemia

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Malignancy with highest mortality?

Brain (PNET)

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Most common solid tumor outside CNS?

Neuroblastoma

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Most common malignancy in children?

Leukemia (ALL)

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Where does ALL spread?

Liver, spleen, lymph nodes

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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

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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)

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Wilms Tumor vs. Neuroblastoma

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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)

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hydrops fetalis

Hemoglobin Bart (alpha thalassemia major)

4/4 alpha globin chains deleted or mutated

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Rhabdomyosarcomas are most commonly found in

the head and neck

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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

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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** ## Footnote Dx: decreased reticulocyte count (due to ineffective hematopoiesis), microcytic, hypochromic RBCs, normal RDW \*definitive diagnosis with Hb electrophoresis

IDA vs. THALASSEMIA

* autosomal recessive * a**plastic 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 n**ot 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 t**o 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:

1. Age of the patient at the time of diagnosis 2. Initial leukocyte count 3. 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