Heme/ONC Flashcards by Magda Kazmierczak

Sickle cell anemia Labs

Low hematocrit
Increased: reticulocytes, LDL, unconjugated bilirubin
Smear: howell-jolly bodies, polychromasia

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Maintenance of Sickle cell

Vaccincations
Penicillin (until age 5)
Folic acid supplementation
Hydroxyurea (for recurrent vasoocclusive events)

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Management of acute pain crisis in Sickle cell

Hydration
analgesia
+/- transfusion

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ALL C/F

typically Age 2-10

30-50% present with infection and about half with LAD/splenomegaly

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Dx ALL with smear

lymphoblast typically
>25% lymphoblasts in bone marrow
Lymphoblasts lack peroxidase positive granules but stain with PAS
TdT positive (pre B and pre T lymphoblast)

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Aplastic crisis in SC anemia due to

Parvo B19 virus
sudden arrest of erythropoiesis
reiculocytes need be less than 1%

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Polycythemia of Newborn

is what and causes (5)

Hct >65%
Causes: Inc Erythropoiesis from Intrauterine hypoxia:
Maternal DM
Maternal HTN
Smoker
IUGR
Delayed cord clamping

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CF/ of polycythemia in newborn (6)

Ruddy skin
hypoglycemia/hypocalcemia
Resp distress
cyanosis
apnea, irritability, jitterness
Abd distension

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Rx: Neonatal polycthemia

Partial exchange transfusion

remove blood, infuse normal Saline

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Dehydration in newborn typically occurs when? and why

unusual in first 2 days of life as they are born with excess extracellular water

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Anemia of prematurity labs (5)

smear: normocytic, normochromic anemia
low reticulocyte count, RBC precursores low in BM
Normal WBC/platelet count
Normal Total bilirubin
Hb: 7-10 g/dL

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Rx anemia of prematurity

Iron supplementation
periodic Hb check
blood transfucion if needed

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Causes of anemia of premi

Combo of:
diminished RBC production
Shortened RBC life spain
blood loss

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Acquired aplastic anemia labs

Pancytopenia: Increased MCV
Increased HbF
Bone marrow hypoplasia

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Fanconi Anemia C/F

BM: aplastic anemia and progressive BM failure
Apperance: short, microcephaly abnormal thumbs, hypogonadism
Skin: hypo/hyperpigmentation, cafe au lait spots, large freckles
Eye/ears: strabismus, low set ears, middle ear abnormalities: Headaches

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Fanconi anemia pathophys

spontaneous chromosomal breaks

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Fanconi anemia risk of developing

AML and cancers

BM failure

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Rx fanconi

Steroids, androgens

BM transplant definitive

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Diamond Blackfan syndrome

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Congenital hypoplastic anemia:
child w/ macrocytic anemia (no hypersegmented nuclei)
low reticulocyte count
congenital anomalies
usually sporadic although can be AD or AR 15% of times

Pathology in DBS?

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intrinsic defect of erythroid progenitor cells which results in creased apoptosis

Congenital anomlies associated with DBS

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Short stature
craniofacial deformities
defects of upper limbs: triphalangeal thumbs

Rx of DBS

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Corticosteroids
Transfusions and deferoxamine
stem cell transplant

in C1 inhibitor def. aka hereditary angioedema what is elevated that causes edema

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C2b

Bradykinin

Immune thrombocytopenia (ITP) C/F (3)

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mc around 2-5 yrs old
Antecedent viral infection
MC: asymptomatic petechiae and ecchymosis
Mucocutaneous bleeding

Labs in ITP

Isolated thrombocytopenia

Rx in kids for ITP

Skin manifestation only: observe | Bleeding: IVIG or Steroids

Rx in Adult for ITP

Plat: >30,000: observe Platelet below 30,000 or bleeding: IVIG or glucocorticoids

Sickle Cell disease: aplastic crisis

transient arrest of erythropoiesis that results in severe drop in Hb and virtual absence of reticulocyte on peripheral smear (

Rx aplastic crisis

blood transfusion

Sickle cell disease: hyperhemolytic crisis

sudden severe anemai w/ appropriate reiculocytosis

Sickle cell disease: acute chest syndrome

fever, chest pain, and infiltrate on chest xray Pulmonary infarction or infection Drop in Hb may be seen

Adolescent w/ nasal obstruction, visible nasal mass, frequent nosebleed consider:

Juvenile angiofibroma (JNA)

JNA is worrisome because

can invade bone highly vascularized: bleeding rx: surgicaly

Sickle cell disese sepsis organisms

mc: S. Pneumo | 2. HiB

Sickle cell disease kids should get routine wat

12 conjugate pneumococcus and HiB vaccines | plus 23 Polysaccharide pneumococus and meningococcal conjugate vaccines

Sickle cell kids shoudl get what pophylaxis

penicillin till age 5

Hereditary spherocytosis lab tests

Increased MCHC Negative coombs test Increased osmotic fragility on acidified glycerol lysis test Abnormal eosin 5 maleimide binding test

Hereditary spherocytosis genetic issue

AR mutation in ankyrin on RBC membran resulting in spectrin deficiency

Elevated RDW is what and what its useful for

RBC variability in size earliest sign in Iron def anemia Elevated in nutritional deficiences

MCHC is

Hb/HCT: measures Hb conc of each RBC Decreased in: Iron def, thallasemia Increased: spherocytosis

Hyperbilirubinemia in black infant is assoc w/

G6PD deficiency

Spherocytosis is seen in

Hereditary spherocytosis Hyperthermia G6PD def ABO incompatibility

in DIC there is a consumption of

factors II, V, VIII, and platelets

Toxicity of MTx

GI mucositis BMS skin erythema hepatic dysfunction

Toxicity of Vincristine

Peripheral neuropathy constipation jaw pain SIADH

Doxorubicin toxicity

``` alopecia N/V stomatitis tissue necrosis BMS Cardiotoxicity ```

G6PD presents in kids how/when

``` 3-4 months of age w/ failure to thrive hypoglycemia hepatomegaly acidosis ```

Heme/ONC Flashcards

(47 cards)