Heme/Onc Flashcards by Cady Williams

Disorders at increased risk if ALL:

Downs, ataxia telengiectasia, Bloom syndrome, fanconi anemia

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What must a bone marrow biopsy show to diagnose ALL?

25%blasts

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Drug- name the alkylating agents and their SE:

Cyclophosphamide: inhibits DNA synthesis (hemorrhagic cystitis)

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Drugs- name the antimetabolites and their SE:

MTX: folic acid antagonist (myelosuppression, hepatotoxic, oral and GI ulcers)
6-MP: inhibits purine synthesis (myelosuppression, hepatotoxic)
Ara-C: inhibits DNA polymerase (myelosuppression)

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Drugs- name the chemo antibiotics and their SE:

Doxorubicin & daunorubicin: bind to DNA (cardiomyopathy)

Bleomycin: binds to DNA (pulmonary fibrosis)

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Drugs- name the chemo enzymes and their SE:

L-asparaginase: depletes L-asparagine (pancreatitis, increased glucose)

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Drugs- name the vinca alkaloids and their SE:

Vincristine: inhibits micro tubule formation (peripheral neuropathy, SIADH)
Vinblastine: inhibits micro tubule formation (leukopenia)

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What is M3 AML also called?

Acute promyelocytic leukemia- t(15;17), DIC at presentation. Treated with chemo + retinoic acid

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Drugs- SE of cisplatin & etoposide (VP-16):

Cisplatin: inhibits DNA synthesis (nephro toxic, ototoxic, neurotoxic)

VP-16: topoisomerase inhibitor (secondary leukemias)

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What findings in blasts on a bone marrow biopsy is associated with AML?

Auer rods

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What is the Philadelphia chromosome?

CML with t(9;22) translocation -> bcr-abl abnormal protein

Tmt: BMT, hydrourea, interferon alpha, tyrosine kinase inhibitors

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How does JMML present?

Juvenile myelomonocytic leukemia: <2 yrs of age, massive splenomegaly, leukocytosis/thrombocytopenia, ski findings (xanthoma, cafe au lait, eczema)

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What is the most common presentation for Hodgkin disease?

Asymptomatic cervical or supraclavicular LAN

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What are Pel-Ebstein fevers?

Classically associated with Hodgkin lymphoma, several days of fever followed by afebrile episodes (periodic type fevers)

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What are the chemotherapies commonly used in Hodgkins?

MOPP = nitrogen Mustard, vincristine(Oncovin), Procarbazine, Prednisone

ABVD = Adriamycin, Bleomycin, Vincristine, Dacarbazine

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What lab values are seen with tumor lysis? Prevention?

Hyperkalemia
Hyerphosphatemia
Renal insufficiency

Hydration, allopurinol (inhibits urate toxicity)

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What is the most common malignancy in infants?

Neuroblastoma

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Which ages of children have the best prognosis in Neuroblastoma?

< 1 year

4S staging has a good prognosis and is <1 with small tumor and dissemination in only liver and skin

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What two metabolites can be useful in Neuroblastoma diagnosis and screening?

HVA and VMA

What disorders are associated with Wilma tumor?

WAGR (Wilms, aniridia, GU abn, mental Retardation)

BWS

Denys Drash: Wilms, nephropathy, male pseudo hermaphroditism

Most common soft tissue tumor of childhood:

Rhabdomyosarcoma

What is the radio graphic appearance of osteosarcoma v Ewing’s sarcoma?

Osteosarcoma: metaphysis, sunburst

Ewing’s: diaphysis, lytic, onion skinning, more likely to have systemic symptoms

What chromosomal abnormality is associated with Ewing’s sarcoma?

T(11,22) mostly, some t(21,22)

What is an ostechondroma?

Benign bone tumor, occurs in metaphysis, stalk projection with cartilage cap

What is an osteoid osteoma?

Benign tumor with unremitted pain, worse at night, relieved with aspirin. Lucency with sclerotic bone

What tumor is associated with tuberous sclerosis?

Subependymal giant cell tumor

What is the most common malignant CNS tumor?

Primitive neurrctodermal tumors (medulloblastoma, pineoblastoma, central Neuroblastoma)

What is the most common CNS tumor?

Glioma (cerebellar astrocytoma & brainstem gliomas & high grade astrocytoma)

What are some germ cell CNS tumors? What so they often secrete?

Pineal tumor, germinomas, teratoma. Alpha feto protein and hcg

What is parinaud syndrome? What is it seen with?

Triad of (1) impaired upward gaze (2) dilated pupils with better reactivity to accommodation than light (3) conversion/retraction nystagmus. Seen with pineal germ cell tumors or pinealblastoma

What disease is meningioma associated with?

NF2

Where are most teratomas located?

Sacrococcyx, ovaries, testes, anterior mediastinum

What teratoma is most prone to malignant transformation?

Sacrococcygeal teratoma in infants over two months

Name the germ cell tumors and their associated tumor markers:

``` Teratoma- typically none Germinoma- marker negative Embryonal carcinoma- AFP & Bhcg Yolk sac (endodermal)- AFP + Choriocarcinoma- Bhcg + Gonadoblastoma- marker neg Sex cord tumors- leydig ->androgens, sertoli->estrogen ```

What tissue if found in a chorio carcinoma?

Syncytiotrophoblast tissue

What virus is associated with nasopharyngeal carcinoma?

EBV

What pancreatic tumor is associated with AD MEN-1?

Insulinoma and gastrinoma

What is the most common benign liver tumor?

Hemangioendothelioma, if under two, multiple lesions and AFP normal, think this.

Common findings with Hand-Schuller-Christian disease:

``` Mulitfocal LCH: Skull lytic lesions DI Exopthalmos Erosion of lamina dura or premature eruption of teeth Gingival hemorrhage Oral mucosa irritation ```

anterior mediastinal tumors

thymoma, teratoma, thyroid, t-cell lymphoma | *high risk of airway compromise, do not anesthetize

Mentzer index:

MCV/red blood cell count | <13 suggests beta thal trait

macrocytic anemia buzzwords

1. B12 def: vegan, pernicious anemia 2. Folate: goats milk, dx with erythrocyte folic acid level and r/o B12 def prior to tx 3. Diamond Blackfan: triphalyngeal thumbs 4. Fanconi: AR, short stature, café au laits, absent thumb/radial anomalies, HgF high

microcytic anemia buzzwords

1. iron deficiency: high RDW 2. beta thalassemia: high Hgb A2, Mentzer Index decreased, major (Cooleys)/intermedia/minor, target cells 3. alpha thal: carrier, trait/minor, HBH, hydrops fetalis 4. lead poisoning: ringed sideroblast, tx penicillamine/dimercaprol or EDTA 5. anemia of chronic disease: TIBC low, high ferritin, tx underlying illness

normocytic anemia buzzwords

1. SCD: AR, val instead of glutamic acid, howell jolly bodies when asplenic 2. HS: AD, spectrin def, tx with folate and splenectomy 3. G6PD: xlinked, Heinz bodies, test several weeks after an episode 4. PK def-AR and AD forms

definition of neutropenia

ANC<500