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81 Cards in this Set

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Anemias of Inadequate Production-Macrocytic
megaloblastic changes (hypersegmented neutrophil): Folate, B12, inborn errors

No megaloblastic changes: Diamond-Blackfan, Pearson Marros-Pancreas, Congenital dyserythropoietic anemia
anemia with other hematologic abnormailities
aplastic anemia
leukemia
bone marrow replacement disorders
anmia with hemolysis (hyperbili, increased LDH)
Spherocyes: hereditary spherocytosis, autoimmune hemolytic anemia, Wilson disease
Sickle: SCC, Sickle beta thal
Target: Hb SC
Hypochromic: homozyg beta thal,
microangiopathy: HUS, thrombotic throbocytopenia
bite cells: G6PD
norbmocytic anemia with low retic
chronic diseaease
renal disease
transient erythroblastopenia of childhood
anemia with hypothyroidism
Diamond-Blackfan Anemia
Congenital Hypoplastic Anemia. 90% present in 1st yr. ave 3mo. macrocytic, retic low, no RBC precursors, nl bone marrow. Sporadic. defect in cell line causing too much apoptosis . High EPO in serum and urine. 1/2 have congen anomalies (triphalynx thumb, small, . Rx: steroids help 3/4
Pearson Marrow-Pancreas Syndrome
marrow failure anemia>neurtorpenia>throbocytopenia in neonatal period. Hb F elevated. FTT, pancreatic fibrosis, DM, muscle and neuro impariment, early death. mt DNA deletion
Transient erythroblastopenia of Childhood
most common acquired red cell aplasia in kids. severe, transient hypoplastic anemia 6mo-3yrs. . Transient immune suppression often post viral illness.low retic, neutropenia in 20%, platelets norml. Self-resolves.
anemia of chronic disease
common with cuinfection inflammation or tissue breakdown. Hb 6-9. normochromic and normocytic. retic nl to low. wbc hi. serum Fe low, TIBC nl is good indicator and diff from Fe def.
Congenital dyserythyropoietic anemia
Anemia despite ineffective active erythropoiesis. 3 types all singe gene.
Folic acid
mild megaloblastic anermia 4-7 mo before Fe deficiency. irritable, fail to gain weight, chronic diarrhea. goat's milk is deficient.
Pernicious anemia sx
polyendoronpathy s. adreanl, parathyroid, thryoid. megaloblastic anemia, GI, neuro
pernicious anemia describe
autoimmune destruction of parietal cells wthich make itrinsic factor. In kids, associated with polyendocrinopathy s.
when to give VZIG to immunocomprimised
within 96hrs of exposure to varicella.
hemophilia A factor
VIII
hemophilia B factor
IX
management of headtrauma in hemophilia
boost factor to 100% level.
Down syndrome leukemias
1/3 have transient leukemia, resolves spontaneously. 20% get ALL (worse than gen pub), or AML (better than gen pub)
hereditary spherocytosis, dx, sx
pallor, jaundice, splenomegaly, gallbladder d., often present with B19 virus, which suppresses RBC production. hemolytic anemia, hi MCHC, spherocytes (small and dense RBC with no central pallor). AD. dx by osmotic fragility test.
hereditary elliptocytosis, dx, sx
same as sperocytosis but elliptocytes are in smear. MCHC nl.
G6PD deficiency, inheritance and labs.
Xr, nl MCHC. hemolytic anemia
Blackfan-Diamond S.
anemia in 1st yerar. aplasia of RBC, no hemolysis. retic low, MCHC nl.
G6PD provocatures
bactrim, chloramphenicol, nitrofurantoin, antimalarials, DKA, aspirin,
autoimmune hemolysis, warm Ab (types)
most cases, idiopathic: primary
SLE, leukemia: secondary
acute: 3-6mo, ages 2-12
chronic: <2mo, >12ry mo-yrs,
autoimmune hemolysis, warm. Rx
prednisone 2mg/kg/day
Tx with least reactive blood may be necessary if Hb<5
pallor, fatigue, ictuerus during or after viral illness, think
autoimmune hemolytic anemia. direct coombs.
Osteoid osteoma
common benign bony tumor. sharpt boring pain. worse at night. naproxen helps. males, 5-24yrs. femur, tibia, spinke, tender radiolucent nidus, surrounding reactive bone,
Osteochondromas
most common benign tumor in kids. painless, hard, nontedner mass. metaphyses of long bones.
Osteogenic sarcoma, dx
pain, mass. bone destruction and production, periosteal elevation
Ewing Sarcoma, dx
pain, mass, XR: lytic, onion skin.
Lab dx of DIC
CBC, PT, PTT initially.
cyclic neutropenia sx
q21 days nadir. malaise, fever, gingivitis, oral ulcers, pharyngitis, lymphadenopathy.
cyclic neutropenia dx
recurrent fever, gingivitis, ulcers. ANC 2-3times week for 6-8wks.
tumor lysis risk malignancies
Burkitt > T-cell leukemia-lymphoma > ALL
treatment of problematic hemangiomas
around eye if setup for amblyopia. use prednisoneburst and slow taper.
von Willebrand lab dx
factor VIII clotting activity, mesaurements of von W proteins
von Willebrand disease sx
ecchymoses, epistaxis, menorrhagia, post op bleed. menorrhagia most common present in girls.
thalassemia majof
microcytic severe anemia. homozygous beta thal. Italian, Greek. almost no HbA as no beta globin. 6mo-2yr present.pallor, irritability, FTT, HSmegaly, jaundice. nucleated RBCs
bleeding time best corelates with:
platelet fxn.
transient erythroblastopenia of childhood
severe anemia and reticulocytopenia in a healthy child.toddlers. normochromic, normocytic severe. no retic response. self-limited. in recovery they retic hard, so may get jaundiced. No Rx unless CV unstable.
Diamond-Blackfan S.
2mo. 25% also have congenital malformations. hi fetal Hb. macrocytic. no retic. no precursors.
difference between transient erythroblastopenia of childhood and Diamond-Blackfan.
age. D-B presents when HbF weans off 2mo ish. Transient happens to toddlers.
Wiskott-Aldrich
recurrenbt infections, bleeding (thrombocytopenia), eczema. Xlinked
Fanconi S. sx
80% have congenital anomailes. hyperpigmentation, short stature, arm anomalies. aplastic anemia by 4yrs
differentiating aplastic anemia and ALL
very hard. aplastic anemia presents with palor, purpura, and all three cell lines down. ALL is 20 times more common and does not have HS megaly and bone pain. bone marrow differentiates.
ALL relapse sites
bone marrow moset. CNS, testes. painless testicular enlargement.
PTT
measures all but VII. Best measure of von Willebrand and hemophilia A. lupus anticoagulant more likely to affect PTT. more sensitive to heparin than PT. 20-35 is norm. prolonged in newborns
PT
measures VII, X, V, II, fibrinogen. onlyu PT gets VII. 10-13 nl. long PT with nl PTT is VII deficiency.
factor XIII deficiency
nl PTT, PT. delayed umbilical stump sep. elayed bruising following trauma. poor wound healing.
Hemophilia A genetics
X recessive. 1/3 are spontaneous. 1/2 have severe disease.
DVT causes
DVT in kids = hereditary thrombophilia.
factor V leiden def.
prothrombin G20210A
hyperhomocystinemia
neonatal abdominal mass
benign GU lesions. obstructive hydronephrosis, multicystic dyspoastic kidney.
Wilms sx
most common abdominal mass in kids. asx. but some have HTN, microscopic hematuria. flank mass, painless.
sickle cell d. hepatic and bil tract compications
cholelithiasis, liver infarction, gallstones in 40% of teens.
autoimmune hemolytic anemia sx
palor, fatigue, jaundice, abdominal pain, dark urine, following viral illness.
neuroblastoma prognosis
age and stage. <1yr great. >1yr depends on stage.
onset of sickle cell
hemolytic anemia starts 2-4mo/ first sx around 5-6 mo with hand-foot s. (symmetric swelling, painful of hands and feet)
Hand-foot syndrome in sickle cell. what is it
crowding of bone marrow chokes off blood supply. necrosis of small distal bones.
acute chest s
pulmonary infarction with pneumonitis or microscopic fat emboli (from bone marrow infarct).
CVA in sickle cell
embolic events10% of all sickle patients.
acute splenic sequestration
episodic event in infants and young children with SCC after acute febrile illness. Large amount of blood pools in spleen. Sudden massive enlargement, signs of circulatory collapse rapidly develop. Tx needed acutely.
Infections of SCC
pneumococcus, H flu infections. And Salmonella osteo. Spleen may be big, but it does not work
B19 virus and SCC
just like chronic hemolytic anemics. acute life-threatening decrease in Hb level with aplastic crisis.
CV disease in SCC
cardiomegaly by teen
GI disease in SCC
chronic high Fe hurts liver, gallstones in teens
HbA
mafor Hb of adult (a=adult)
2 alpha, 2 beta
Hb F
alpha 2, gamma 2. so beta thall not seen
Hb A2
minor adult Hb , alpha 2, delta 2
Hb Barts
tetramer of gamma, gamma 4
Hb H
tetramer of beta, beta 4. seen in alpha thal
Hb AS
sickle trait. one S, one A. can have splenic infarct but usually Asx
Hb C
alpha2, beta2with Lyx change.
in AC: (heterozygote) target cells but no anemia.
in CC: hemolytic anemia, , no sickling.
in SC: less severe than SS but similar. spindle-shaped RBCs.
Thalassemias
all cause microcytic anemia. all are from decreased transcription of alpha or beta. + indicates decreased transcription. 0 indicates no transcription. Only most severe alpha thal has abnormal hb (tetramers of beta or gamma.
B-Thal major
6m0-1yr presnts has severe progressive hemolytic anemia.transfusion dependent. untreated causes bones become thina nd fx occur. face and skull expand. serial tx to keep Hb>10. Hemosiderosis and splenectomy eventually occur in all.
alpha thal
a-/aa: silent carrier
a-/a-: trait. milkd microcytic anemia. Hb Barts in newborn up to 1 mo
a-/--: intermedia, Hb H disease (tetra-beta forms). Hb Barts in newborn and Hb H.micorytosis
--/--: hydrops
alpha thal in genes in different pools
Asians: -,-
Africans: -,a
so Asians can get Hb H and hydrops, usually not in Africans.
neonatal hemochromatosis
acquired s from severe liver disease, liver dysfunction, massive Fe stores
transfusion induced hemosiderosis, Rx
each 500cc of PRBC = 200mg Fe. deferoxamine chelates. SQ pump 5-6 times a wk
ITP sx
1-4yr old suddden petechiae and purpura with profound thrombocytopenia. viral infection 1-4weeks ago.
ITP DDx
severe isolated thrombocytopenia in healthy child has small diff. if anemia present do Coombs to rule out Evans S. (autoimmune hemolytic anemia with thrombocytopenia.
ITP course
up to 6 months of thrombocytopenia. 70-80% resolve by then. no problems as long as no significant bleed, including intracranial. No therapy demonstrated to improve short or long term complications
neonatal alloimmune thrombocytopenic purpura
Rh disease of platelets. healthy child who gest petechia in first few days. moms platelet count nl. Can occur in first born. worse in second. treat with maternal platelet tx.