Lymphoma, Leukemia, LAD, TTP - Newman, Vangarsse

Question 1

What are the central LNs that are enlarged in lymphoma?

Answer 1

Axillary, cervical, inguinal

Question 3

Generalized LAD w/respiratory symptoms requires what?

Answer 3

CXR

Question 4

What are the most common types of cancers in children under 15?

Answer 4

Leukemia

Brain tumors

Question 6

Neuroblastomas are tumors of what?

Derived from what?

Median age of dx?

Answer 6

Sympathetic ganglia and adrenal medulla

Neural crest cells

18 months

Question 7

Isolated thrombocytopenia in the absence of any obvious initiating or underlying cause is what?

How are the children normally described?

Answer 7

Acute ITP

otherwise well

Question 9

In a pt in which you suspect ITP, what does the presence of platelet morphology, schistocytes, +direct Coombs, or fibrin degradation products tell you?

Answer 9

The answer is NOT itp

Question 10

What labs should be ordered in a pt w/unexplained LAD?

Answer 10

CXR Before LN biopsy

Question 12

What is acute ITP classified as?

Chronic?

Answer 12

Less than 6 months

Greater than 6 months

Question 13

What are “B symptoms”?

Answer 13

Unexplained fever > 39C
Weight loss > 10% over 6mo
DRENCHING night sweats

Question 15

Peak incidence of Wilms tumor?

Answer 15

2-5 y/o

Question 16

Presentation of acute ITP?

Answer 16

Sudden onset of bruising/petechiae OR mucocutaneous bleeding
Otherwise healthy child
50% 1-2 was after viral illness

Question 17

What accounts for 60% of lymphomas in children and adolescents?

Answer 17

NHL

Question 18

What LN should never be enlarged and is cause for concern?

Answer 18

Supra-clavicular

Question 23

A young pt with chronic ITP needs what medications?

What treatment?

Platelets are usually what?

Answer 23

No Meds

Everything normal except cannot participate in contact sports

30-80K

Question 24

Burkitt lymphoma of the sporadic type manifests where?

Endemic?

Answer 24

Abdominal

Head and neck w/BM or CNS

Question 25

Blueberry muffin baby seen in what childhood disease?

Answer 25

Neuroblastoma

Question 26

What is a marker for hematologic malignancies?

Answer 26

LDH

Question 28

Treatment for acute ITP?

What is exceedingly rare but a feared complication?

Answer 28

None, maybe steroids

Intracranial hemorrhage

Question 30

What is indicated when a pt presents with a painless testicular mass w/no hx of trauma or injury?

Answer 30

CXR –> likely a malignancy

Question 31

Denys-Drash syndrome has what risk of developing Wilms tumor?

Genetic abnormality?

CF?

Answer 31

90%

mutation in Zn finger of WT1 protein (ch. 11)

Gonadal dysgenesis, early onset nephropathy

Question 33

What causes conjuctivitis, preauricular adenitis, and conjuctival granuloma?

Seen in what?

Answer 33

Parinaud’s syndrome

Cat scratch disease

Question 34

At what level of platelets is primary hemostasis impaired?

Spontaneous bleeding?

Clinically significant bleeding?

Life-threatening hemorrhage?

Answer 34

75K

50K

20K

10K

Question 35

What are the important lab values in neuroblastomas?

90% of them produce what?

Answer 35

Elevated urine VMA and HVA

Catecholamines

Question 36

Peak incidence of acute ITP?

Bw what ages usually?

Answer 36

2-5

2-10

Question 37

What kind of teratomas are the most common? What types are there?

Answer 37

Sacrococcygeal benign (mature), immature, mixed

Question 38

What is the only curative tx of chronic ITP?

Answer 38

Splenectomy

Question 39

NHL may be inherited or acquired, what common conditions?

Answer 39

SCID | Wiscott-Aldrich (X-linked Recessive)

Question 42

What is the most common cancer in adolescents and third most common cancer among US children under 14?

Answer 42

Lymphoma

Question 43

What results from malignant transformation and clonal expansion of hematopoietic cells at an early stage of differentiation that are then unable to undergo further maturation?

Answer 43

Leukemia

Question 45

WAGR syndrome has what risk of developing Wilms tumor? Chromosome deletion? CF?

Answer 45

33% 11 Aniridia, genital abnormalities, mental Retards

Question 46

Pain that worsens after ingestion of alcohol can occur in what?

Answer 46

HL

Question 49

When working up LAD, what is the most important component?

Answer 49

History and Physical

Question 50

Name a couple things a Wilms tumor may present with?

Answer 50

``` Large abdominal mass Hematuria Pain in abdomen after trauma Intestinal obstruction HTN Pulmonary metastases ```

Question 51

Large body organs, macroglossia, hemihypertrophy, omphalocele, abnormal large cells in adrenal cortex is what syndrome? Genetic abnormality?

Answer 51

Beckwith-Weismann syndrome Band 11p15.5 (WT2)

Question 52

Macular star or blurring of the optic disc seen in what?

Answer 52

Cat scratch disease

Question 59

ALL or AML of the following? Trisomy 21 White > blacks Increased in adolescence? EBV and L3 4 y/o peak age? Benzene exposure?

Answer 59

Both but under 3 y/o AML more likely ALL AML ALL ALL AML

Question 65

What is the age distribution of HL? What viruses may be indicated?

Answer 65

15-25 and older than 50 HHV6, CMV, EBV

Question 67

What size of a LN is considered enlarged?

Answer 67

Greater than or equal to 2cm

Question 68

What is described by recurrent sinopulmonary infections, eczema, and bleeding secondary to thrombocytopenia?

Answer 68

Wiscott-Aldrich syndrome Cole aldrich has bad skin and bleeds a lot bc he is a bitch

Question 71

What constitutes 97% of all childhood leukemias?

Answer 71

Acute leukemias | ALL or AML

Question 72

What are the CF of HL? What is rarely seen?

Answer 72

Painless, nontender, firm, rubbery cervical or supraclavicular LAD Mediastinal involvement Hepatosplenomegaly

Question 75

NHL in children present how?

Answer 75

De novo disease

Question 76

What gives a poor prognosis in HL?

Answer 76

Tumor bulk Stage B symptoms

Question 77

A large cell w/multiple or multilobulated nuclei (often bilobed) is what?

Answer 77

RS cell