Heme/ONC Flashcards
Sickle cell anemia Labs
Low hematocrit
Increased: reticulocytes, LDL, unconjugated bilirubin
Smear: howell-jolly bodies, polychromasia
Maintenance of Sickle cell
Vaccincations
Penicillin (until age 5)
Folic acid supplementation
Hydroxyurea (for recurrent vasoocclusive events)
Management of acute pain crisis in Sickle cell
Hydration
analgesia
+/- transfusion
ALL C/F
typically Age 2-10
30-50% present with infection and about half with LAD/splenomegaly
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)
Aplastic crisis in SC anemia due to
Parvo B19 virus
sudden arrest of erythropoiesis
reiculocytes need be less than 1%
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
CF/ of polycythemia in newborn (6)
Ruddy skin hypoglycemia/hypocalcemia Resp distress cyanosis apnea, irritability, jitterness Abd distension
Rx: Neonatal polycthemia
Partial exchange transfusion
remove blood, infuse normal Saline
Dehydration in newborn typically occurs when? and why
unusual in first 2 days of life as they are born with excess extracellular water
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
Rx anemia of prematurity
Iron supplementation
periodic Hb check
blood transfucion if needed
Causes of anemia of premi
Combo of:
diminished RBC production
Shortened RBC life spain
blood loss
Acquired aplastic anemia labs
Pancytopenia: Increased MCV
Increased HbF
Bone marrow hypoplasia
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
Fanconi anemia pathophys
spontaneous chromosomal breaks
Fanconi anemia risk of developing
AML and cancers
BM failure
Rx fanconi
Steroids, androgens
BM transplant definitive
Diamond Blackfan syndrome
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?
intrinsic defect of erythroid progenitor cells which results in creased apoptosis
Congenital anomlies associated with DBS
Short stature
craniofacial deformities
defects of upper limbs: triphalangeal thumbs
Rx of DBS
Corticosteroids
Transfusions and deferoxamine
stem cell transplant
in C1 inhibitor def. aka hereditary angioedema what is elevated that causes edema
C2b
Bradykinin
Immune thrombocytopenia (ITP) C/F (3)
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
