- no peak age - no sex predilection - leukoerythroblastic reaction - primitive WBC, nRBC, teardrop RBC - M1, M2, M3 --> Auer rods - hyperleukocytosis common --> sludging - extramedullary involvement (chloromas, leukemia cutis, gum hypertrophy, CNS disease) (nucleated RBC = 1. ineffective erythropoesis, 2. functional asplenia, 3. marrow replacement

Hematology-Oncology Flashcards by k k

What are some high risk indicators for ALL?

Age 10
WBC >50,000 (T-cell>B cell
prior steroid therapy
testicular disease
hypodiploid (<44 chromosomes)
Ph+
poor initial response

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Findings in Tumor Lysis Syndrome

Hyperuricemia
Hyperphosphatemia
Hypocalcemia
Hyperkalemia

(when Ca/PO4 is >60 precipitation will occur in brain, collecting tubules in kidney etc.)

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Hyperkalemia EKG changes?

6 –> peaked T waves
7 –> prolonged PR interval
8 –> absent P wave/widened QRS

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Features of AML

no peak age
no sex predilection
leukoerythroblastic reaction - primitive WBC, nRBC, teardrop RBC
M1, M2, M3 –> Auer rods
hyperleukocytosis common –> sludging
extramedullary involvement (chloromas, leukemia cutis, gum hypertrophy, CNS disease)

(nucleated RBC = 1. ineffective erythropoesis, 2. functional asplenia, 3. marrow replacement

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High risk prognostic factors in AML?

monosomy 7
monosomy 5/5q
FLT 3 internal tandem duplication
> 15% blasts after first cycle of induction

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Good prognostic factors in AML:

trisomy 8, t(8:21) higher remission rate
inv(16) or t(16;16) associated with M4 (eos) presentation
Trisomy 21 pt

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Genetic diseases associated with an increased risk of leukemia?

Klinefelter’s
Bloom’s
Fanconi’s Anemia
Neurofibromatosis
Kostman’s Neutropenia
Schwachman’s

*AML is most frequent secondary malignancy in survivors of Hodgkin’s Disease

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Mediastinal Mass

T-cell ALL

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DIC

APML (M3); t(15;17), ATRA/As2O3

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Extramedullary disease, infants

myelomonocytic/monocytic (M4/M5)

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Pancytopenia, Down Syndrome

Megakaryoblastic (M7)

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Name the cancer:

mean age mid-40’s
persistent neutrophilia without infection
absolute basophilia
thrombocytosis in chronic phase
low LAP score in chronic phase
increased serum B12

CML

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Tumor Markers - Hodgkin’s

ESR, Copper

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Tumor Markers - Neuroblastoma

Urine catecholamines, ferritin

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Tumor Markers - Hepatoblastoma

Alpha fetoprotein

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Tumor Markers - Teratocarcinoma, YST

Alpha fetoprotein

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Tumor Markers - Embryonal Carcinoma, Choriocarcinoma, Seminoma

Beta HCG

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Bad: hypodiploid, t(9:22)
Good: triple trisomy (4, 10, 17), TEL/AML = t(12:21)

ALL

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Bad: monosomy 7
Good: Down Syndrome

AML

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t(15;17)

APML

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t(9;22) = BCR-abl; Philadelphia chromosome

CML

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t(8;14) involves MYC oncogene

Burkitt’s Lymphoma

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Good: hyperdiploid (infants)
Bad: NMYC amplification, 1p-

Neuroblastoma

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11p- (sporadic cases)

Wilm’s Tumor

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13q- = Rb tumor suppressor

Retinoblastoma

t(11;22) = EWS-Fli

Ewing's/PNET

High risk factors for ALL

- prior treatment with steroids - 12 year old patient - testicular involvement - residual leukemia after induction

High risk factors for AML

- monosomy 7 | - residual leukemia after induction

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Abdominal Mass

Both

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Peripheral blasts

Neuroblastoma

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Hypertension

Both

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Elevated serum ferritin

Neuroblastoma

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Rapid, irregular eye movements

Neuroblastoma

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Commonly metastasizes to lungs

Wilm's

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Elevated alpha fetoprotein

Neither

Associated with Wilm's Tumor, Neuroblastoma, Both or Neither? Most common in toddler age group

Both

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? more common in second decade of life

Both

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? Li-Fraumeni Syndrome

Osteogenic sarcoma

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? Onion skin appearance on x-ray

Ewing's

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? Metaphyseal location

Osteogenic sarcoma

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? Down Syndrome

Neither

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? Fever

Ewing's

Associated with osteogenic sarcoma, ewing's sarcoma, both or neither? Pulmonary metastasis

Both

t(9;22)

Poor risk ALL

retinoic acid

APML (FAB M3)

translocation involving c-myc

Burkitt's lymphoma

rarely occurs in Blacks

Ewing's sarcoma

high risk ALL

hypodiploid chromosomes

LCH

Birbeck granules

Burkitt's lymphoma

Starry sky histology

AML

Auer rod

Hodgkin's lymphoma

Reed-Sternberg cell

Neuroblastoma

Elevated ferritin

Osteogenic sarcoma

Elevated alkaline phosphatase

High WBC, blasts, DIC

APML (Promyelocytic) M3

Gingival hypertrophy, CNS disease

Monoblastic (M5)

Mediastinal mass

T-cell ALL

Down syndrome

Megakaryoblastic (M7)

Chloroma

Monoblastic (M5)

Testicular relapse

T-cell ALL

1p-

poor prognosis neuroblastoma

Monosomy 7

poor prognosis AML

11 p-

Wilm's tumor

13q-

Retinoblastoma

Trisomy 21

good prognosis AML

5y/o M with DI and lytic skull lesions

Langerhans Cell Histiocytosis

12y/o M with pallor, new onset wheezing and cervical/supraclavicular adenopathy

T-cell ALL

14y/o M with massive splenomegaly on routine PE

CML

6y/o M with RLQ pain/mass

Burkitt's

16y/o boy with painless cervical and supraclavicular adenopathy

Hodgkin's Lymphoma

2y/o F with painless abdominal mass and hematuria

Wilm's Tumor

3y/o F with pallor, periorbital ecchymoses, hip pain

Neuroblastoma

14y/o M with painful swelling of right mid-femur

Ewing's

5y/o M with repeated bouts of vomiting

Cerebellar brain tumor

Hyperpigmentation, micro-cornea, thumb abnormalities

Fanconi's Anemia

Globin chains in HbF

a2g2

Globin chains of HbA

a2B2

Globin chains of HbA2

a2d2

Hb H

alpha-thal; unstable; non-functional

Barts (g4)

alpha-thal in BABIES

When do beta chain problems (SCD, beta thal) present?

4-6mos of age

Things that shift the O2 dissociation curve to the left?

HbF

Things that shift the O2 dissociation curve to the right? (Incr delivery to tissues)

Acidosis, hypoxia, 2,2DPG

Chocolate brown arterial blood

METHEMOGLOBINEMIA: - Fe2+ to Fe3+ (can't bind O2) - exposure to nitrites, NO, pyridium - AGE in infants --> stool bicarb losses --> acidosis --> bacterial overgrowth --> increased nitrites - Rx: methylene blue - Don't use in G6PD - insufficient NADPH for drug to work and may lead to hemolysis

What is lost first - folate stores or B12 stores?

Folate - small store; deficiency in 1month, anemia within 4 months of deprivation B12 - large stores; deficiency usually due to an absorptive problem - PA, ileal resection, fish tapeworm, or vegan diet Folate will fix anemia but won't stop neurologic deterioration from lack of B12 **Look for HYPERSEGMENTED POLYS**

Normocytic anemia, low serum Fe, but increased ferritin. Low epo levels relative to degree of anemia

Anemia of Chronic Inflammation

What decreases Fe absorption in the small intestine?

Oxalates | Vitamin C improves absorption

12mo boy with a Hct of 27%, MCV 69. Breast fed until 6mos of age, then given whole milk.

Iron Deficiency - determine the cause - ferritin --> serum Fe/FEP --> anemia - continue Fe therapy until ferritin normal - peak incidence between 6mos and 3 years (times of greatest growth)

The most important treatment for Acute Chest Syndrome?

Transfusion

What finding on a peripheral blood smear is most indicative of loss of splenic function in sickle cell disease?

nRBC

2y/o with 1 day h/o painful swelling over hands and feet with low grade fever.

SCD = dactylitis

Target cells, think...

Hb SC

A predominance of Hb Barts is associated with a defect in how many globin genes?

A 4y/o Vietnamese M with a microcytic, hypochromic anemia, Hb of 9, no response to therapeutic trial of iron

Alpha-thalassemia

3y/o Italian-American boy with mild microcytic, hypochromic anemia and hepatosplenomegaly. Hb 11.3, MCV 61, RBC 5 x 10^6. Smear shows target cells and basophilic stippling.

Beta-Thalassemia

Hair on end XR?

Beta thalassemia

Heredity of G6PD

X-linked

5m/o with recurrent skin infections and hypopigmentation of skin, hair and eyes. CBC shows moderate neutropenia with giant neutrophil granules

Chediak-Higashi

Giant neutrophil granules

Chediak-Higashi

Hb Bart's

Alpha-thalassemia

NBT test

CGD

Hydrops fetalis

Alpha-thal

Infections with catalase positive bacteria

CGD

Hb F

Higher O2 infinity

Which thal? In its MOST SEVERE FORM is characterized by both ineffective erythropoiesis AND hemolytic anemia

both

Which thal? The severity of clinical presentation is proportional to dose of defective genes

alpha thal

Which thal? Hb H is present

alpha

Which thal? Elevations of Hb A2 and Hb F

beta

Which thal? Diagnosis often made in newborn period

Alpha

Which thal? Iron responsive microcytic anemia

Neither

Which thal? Errors in transcription or translation of globin mRNA

beta

Folate or B12? Hypersegmented polys

both

Folate or B12? Poor diet, pregnancy, presence of chronic hemolysis

Folate

Folate or B12? Ileal resection

B12

Folate or B12? Normochromic/normocytic morphology, low retic

Neither

Folate or B12? Dyphyllobothrium latum infestation

B12

Folate or B12? Exclusively breast fed infants of strict vegan mothers

B12

Fe deficiency or lead poisoning? Hypochromic/microcytic anemia

both

Fe deficiency or lead poisoning? Acute encephalopathy

Lead poisoning

Fe deficiency or lead poisoning? Elevated Free Erythrocyte Protoporphyrin (FEP)

Both

Fe deficiency or lead poisoning? More common in children of low income families

both

Dimercaprol (BAL)

Lead poisoning

Fe decreased but ferritin increased

Chronic inflammation

B4 globin tetramer

Hb H

Parvovirus

Aplastic Crisis

Low EPO level

Chronic inflammation

rIFN-gamma

CGD

Malaria belt

G6PD

Non-megaloblastic macrocytosis

Liver disease

An 18m/o F is brought in for looking pale. PMH is unremarkable. There is a h/o URI 1 week prior. She eats a regular diet and is alert and active. Except for pallor PE is normal. CBC shows normal WBC and plt count but a Hb of 4.5 with MCV of 74. The most likely diagnosis is...

TEC

A 2m/o F is brought in for looking pale. CBC shows normal WBC and PLT count but a Hb of 5.5 with MCV of 105. The most likely diagnosis is...

Diamond Blackfan Anemia

A 3y/o F is well below 5th percentile for height and weight. Her PMx is unremarkable. Her PE shows micrognathia, hypoplastic thumbs and areas of hyperpigmentation on her chest and thighs. Her CBC is normal. The most likely hematologic diagnosis is...

Fanconi Anemia (DEB chromosome breakage study is test of choice)