hem onc Flashcards by Arya Stark

normal MCV

70-90

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range of alpha thal

missing one - silent
missing two - asymptomatic with mild microcytic hypochromic anemia
missing three - hgb H disease (hgb Barts as nbn) –> tx with splenectomy
missing four –> hydrops, stillbirth (or intrauterine transfusions –> BMT)

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small for age Mediterranean child with anemia, HSM

beta thal major
F only on NBS
thickened bone due to extramedullary hematopoeisis

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complications of beta thal major

cholelithiasis

hemochromatosis

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how long to tx iron def anemia

until 2 months after hgb normalizes (to replenish stores)

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anemias with high RDW

iron deficiency and lead toxicity

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anemia with normal/low RDW

thalassemias

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ringed sideroblast

lead poisoning vs sideroblastic anemia

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free erythrocyte protoporphyrin

elevated in lead poisoning and iron deficiency

normal in thalassemia

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why should you correct folate and B12 deficiency together?

if you only correct folate, could get reversible neuro damage

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G6PD inheritance pattern

X linked recessive

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smear findings in G6PD

heinz bodies, helmet cells, blister cells

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inheritance of hereditary spherocytosis

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dx of hereditary spherocytosis

osmotic fragility or

EMA flow cytometry

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amino acid substitution in SCD

glu –> val

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macrocytic anemia, renal/eye/ear anomalies, abnormal skin pigment, growth retardation, forearm anomalies

Fanconi anemia
AR
risk of transformation to AML/MDS

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infant with macrocytic anemia, dysmorphic facies

DBA

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how to reduce risk of febrile non hemolytic transfusion reaction

leukocyte reduced blood

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mucosal ulceration, gingivitis, cellulitis, abscesses, pneumonia

neutropenia

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infant with severe recurrent infections, neutropenia

Kostmann syndrome = severe congenital neutropenia
risk of transformation to MDS and AML
tx: gCSF, BMT

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cytopenias, pancreatic insufficiency, skeletal anomalies

Schwachmann Diamond

at risk for leukemic transformation

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mild neutropenia in healthy infant

isoimmune neonatal neutropenia

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monthly recurrent oral ulcers, fever, enlarged lymph nodes

cyclic neutropenia

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bugs responsible for death in cyclic neutropenia patients

clostridium perfringens and gram negative organisms

tx cyclic neutropenia

gCSF

incidental neutropenia in child

chronic benign neutropenia anti-neutrophil antibodies usually grow out of this by age 5

meds that cause thrombocytopenia

sulfas, seizure meds, vanc

meds that cause platelet dysfunction

aspirin, ibuprofen

anaphylactoid purpura

= HSP

age and prognosis in ITP

worse in older children - more likely to be chronic

other findings in TAR

renal agenesis | lymphocytosis

vit K dependent factors

2, 7, 9, 10

vit K deficiency and coag studies

prolonged PT and PTT

coag studies in hemophilia

prolonged PTT

dx hemophilia

measure factor levels

what to do for asymptomatic hemophilia pt with head trauma

give factors

coag studies in vWD

normal PT, normal or slightly prolonged PTT | vWF activity low

tx vWD

vasopressin for minor bleeding factor VIII for major bleeding/surgery amicar

bone tumor that metastasizes to lungs

osteosarcoma | sunbursting

bone tumor with onionskinning

Ewing

leg pain worse at night, relieved by NSAIDs, XR with radiolucent area surrounded by thick bone

osteoid osteoma | benign

sites with highest risk of relapse in ALL

CNS and testes - sanctuary sites

unexplained lymphadenopathy, cough, unexplained pruritus

lymphoma

most common solid tumor of infancy

neuroblastoma

persistent bone/joint pain, B symptoms, UTI, raccoon eyes, horner syndrome, opsoclonus-myoclonus, excess catechols

neuroblastoma

dx of neuroblastoma

biopsy | urine VMA and HMA (NOT urinary catecholamines)

age and neuroblastoma prognosis

< 1 yr: good | > 1 yr: bad

inheritance of RB

bilateral - AD with incomplete penetrance unilateral - sporadic RB1 gene on chr13

additional risk with RB

pineal gland tumors

most common soft tissue sarcoma

rhabdomyosarcoma

labs in tumor lysis

elevated uric acid, phosphate, potassium, LDH, renal insufficiency

why not to give steroids if can't rule out malignancy

risk of TLS

dz that can cause anterior mediastinal masses

thymoma teratoma thyroid carcinoma lymphoma (most likely to cause airway compromise)

cyclophosphamide SEs

hemorrhagic cystitis

cisplatin SEs

hearing loss and peripheral neuropathy

bleomycin SEs

pulmonary fibrosis

doxo/daunorubicin SEs

cardiac toxicity

vincristine/vinblastine SEs

neurotoxicity and SIADH

methotrexate SEs

oral ulcers, GI ulcers, bone loss